WorldWideScience

Sample records for cell histiocytosis case

  1. Liver involvement in Langerhans' cell histiocytosis. Case report.

    Science.gov (United States)

    Dina, Ion; Copaescu, Catalin; Herlea, Vlad; Wrba, Fritz; Iacobescu, Claudia

    2006-03-01

    Langerhans'cell histiocytosis (Histiocytosis X) is a rare disease of unknown cause characterized by oligoclonal proliferation of Langerhans cells. It occurs mostly in children and young adults and involves one or more body systems such as bone, hypothalamus, posterior pituitary gland, lymph nodes, liver or various soft tissues. The diagnosis is always made by a histological approach. We report a case of Langerhans'cell histiocytosis in a young patient with clinical signs of diabetes insipidus and hepatic involvement in whom the immunohistochemical analysis of the liver tissue led to the definitive diagnosis.

  2. Unusual location of central nervous system langerhans cell histiocytosis: case report

    International Nuclear Information System (INIS)

    Kim, E. Yup; Lee, Jae Kyu; Kim, Chan Kyo; Lee, Chang Hyun; Kang, Chang Ho; Chung, Phil Wook

    2003-01-01

    Langerhans cell histiocytosis of the central nervous system (CNS) usually involves the hypothalamic-pituitary axis, and T1-weighted MR images normally demonstrate infundibular thickening and/or a mass lesion in the hypothalamus and the absence of a posterior pituitary 'bright spot'. We recently encountered a case of CNS langerhans cell histiocytosis with no posterior pituitary 'bright spot' and with lesions involving the cerebellum and basal ganglia but not the hypothalamic-pituitary axis

  3. Unusual location of central nervous system langerhans cell histiocytosis: case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, E. Yup; Lee, Jae Kyu; Kim, Chan Kyo; Lee, Chang Hyun; Kang, Chang Ho; Chung, Phil Wook [Armed Forces Capital Hospital, Seongnam (Korea, Republic of)

    2003-03-01

    Langerhans cell histiocytosis of the central nervous system (CNS) usually involves the hypothalamic-pituitary axis, and T1-weighted MR images normally demonstrate infundibular thickening and/or a mass lesion in the hypothalamus and the absence of a posterior pituitary 'bright spot'. We recently encountered a case of CNS langerhans cell histiocytosis with no posterior pituitary 'bright spot' and with lesions involving the cerebellum and basal ganglia but not the hypothalamic-pituitary axis.

  4. [Pulmonary Langerhans' cell histiocytosis (PLCH) revealed by pneumothorax: about a case].

    Science.gov (United States)

    Sajiai, Hafsa; Rachidi, Mariam; Serhane, Hind; Aitbatahar, Salma; Amro, Lamyae

    2016-01-01

    Langerhans cell histiocytosis is a rare disease of unknown etiology characterized by the infiltration of Langerhans cells in one or more organs. It has a polymorphic clinical presentation. We report the case of Mr R.Y, age 22, with 8 pack year history of smoking, admitted to hospital with complete spontaneous right-sided pneumothorax. Chest drainage was performed with good evolution. Control chest CT scan showed multiple diffuse cyst formations, predominant in the upper lobes. Lab and imaging tests were performed in order to detect systemic histiocytosis with negative results. Patient's evolution was marked by pneumothorax recurrence; pleurodesis and lung biopsy were performed which confirmed the diagnosis. The diagnosis of Langerhans cell histiocytosis should be evoked in front of pneumothorax associated with lung cystic. The diagnosis is easy in front of a suggestive clinical and radiological picture. Nevertheless, therapeutic options are limited and pneumothorax recurrence is common.

  5. Langerhans cell histiocytosis of bone in an adult: A case report

    Directory of Open Access Journals (Sweden)

    Zachary Christopher, MD

    2018-04-01

    Full Text Available Langerhans cell histiocytosis (LCH may clinically manifest in a variety of ways due to its ability to involve nearly every organ system. LCH may present as a single bone lesion, skin rash, or as invasive disseminated disease and occurs typically in the pediatric and adolescent population, affecting both males and females. Independent of its clinical presentation and severity, LCH lesions share the common histology of CD1a+/CD207+ dendritic cells along with an inflammatory infiltrate, and, based upon improved scientific understanding, is now classified as a myeloproliferative neoplasm. We present a case report of an adult diagnosed with LCH of the pelvis. Keywords: Langerhans cell histiocytosis, Adults, Pelvis

  6. Langerhans cell histiocytosis with involvement of the pons: case report

    International Nuclear Information System (INIS)

    Vourtsi, A.; Papadopoulos, A.; Moulopoulos, L.A.; Vlahos, L.; Xenellis, J.

    1998-01-01

    Central nervous system involvement is uncommon in Langerhans cell histiocytosis. The suprasellar region is more frequently affected. There have been few reports of involvement of the brain parenchyma shown on CT or MRI. We present a case of involvement of the pons, showing marked contrast enhancement on MRI. (orig.)

  7. Langerhans cell histiocytosis with involvement of the pons: case report

    Energy Technology Data Exchange (ETDEWEB)

    Vourtsi, A. [Xatzopoulou, Athens (Greece)]|[Department of Radiology, University of Athens Medical School, Athens (Greece); Papadopoulos, A.; Moulopoulos, L.A.; Vlahos, L. [Department of Radiology, University of Athens Medical School, Athens (Greece); Xenellis, J. [Department of Otorhinolaryngology, University of Athens Medical School, Athens (Greece)

    1998-03-01

    Central nervous system involvement is uncommon in Langerhans cell histiocytosis. The suprasellar region is more frequently affected. There have been few reports of involvement of the brain parenchyma shown on CT or MRI. We present a case of involvement of the pons, showing marked contrast enhancement on MRI. (orig.) With 2 figs., 17 refs.

  8. Langerhans Cell Histiocytosis Involving Maxilla and Mandible

    Directory of Open Access Journals (Sweden)

    M. Guna Shekhar

    2009-06-01

    Full Text Available Langerhans cell histiocytosis is a relatively rare unique disease process characterized by an abnormal proliferation of immature dendritic cells usually affecting children and young adults. Jaws are involved in less than 10% of children with the disease while mandibular involvement in young children is uncommon and bilateral affection is very rare. The purpose of this report is to describe a unique and very rare case of simultaneous and bilateral occurrence of Langerhans cell histiocytosis in both the jaws of a four-year-old boy.

  9. Persistent pulmonary interstitial emphysema in a case of Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Abbey, Pooja; Narula, Mahender K.; Anand, Rama; Chandra, Jagdish

    2014-01-01

    We present the case of a 10-month-old boy with multisystem Langerhans cell histiocytosis showing thin-walled lung cysts along with computed tomography (CT) evidence of persistent pulmonary interstitial emphysema (PPIE), in the absence of pneumothorax or pneumomediastinum. Follow-up CT performed after 6 months demonstrated complete resolution of interstitial emphysema

  10. Langerhans cell histiocytosis involving central nervous system: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Moon, Won Jin; Park, Dong Woo; Lee, Seung Ro; Hahm, Chang Kok; Ju, Kyung Bin [Hanyang University College of Medicine, Seoul (Korea, Republic of); Kim, Sung Tae [Ulsan University College of Medicine, Seoul (Korea, Republic of)

    1997-01-01

    Langerhans cell histiocytosis(LCH) is a systemic disorder characterized by idiopathic proliferation of histiocytes in the reticuloendothelial system; CNS involvement outside the hypothalamus or pituitary gland is uncommon. We present a case of LCH involving the brainstem, cerebellum, and temporal lobes, and also showing hypothalamic involvement. The lesions were isointense or hypointense on T1WI and hyperintense on T2WI, and showed multifocal enhancing nodules on post-contrast CT and Gd-enhanced MRI.

  11. Langerhans cell histiocytosis revisited: Case report with review

    Directory of Open Access Journals (Sweden)

    Y Pavan Kumar

    2015-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a group of idiopathic disorders characterized by proliferation of bone marrow derived Langerhans cells and mature eosinophils. Their clinical features simulate common oral findings such as gingival enlargement, oral ulcers, and mobility of teeth, along with nonspecific radiographic features; hence, diagnosing such lesions becomes difficult for the oral physicians. These lesions are commonly seen in childhood; however, we are reporting a case of LCH in 29-year-old adult male. A provisional diagnosis of giant cell granuloma was considered based on history and examination, although the lesion was histologically proven to be LCH and was confirmed with immunohistochemical staining of S100 protein and CD1a antigen. The purpose of this paper is to enhance the understanding of diverse, nonpathognomical oral presentation of LCH that is easily misdiagnosed and overlooked by dentist.

  12. Pituitary and pulmonary abnormalities in an adult patient with Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Perez, Adrian; Castro, Ricardo; Pantuso, Silvina

    2003-01-01

    Pulmonary histiocytosis is an uncommon interstitial lung disease, which is infrequent in adults and is part of a spectrum of disorders characterized by monoclonal proliferation and infiltration of organs by Langerhans cells. Histiocytosis X affects only the lungs in more than 85% of the cases. We report a case of a 21-years-old male patient with Diabetes Insipidus (DI) and respiratory symptoms due to a spontaneous pneumothorax with favorable clinical evolution. High-resolution CT demonstrated bilateral nodular and cystic pulmonary lesions. MRI revealed a nodular widening of the pituitary stalk. An endoscopic trans bronchial biopsy was negative for Langerhans cells. After thoracoscopy with surgical biopsy of the lung the diagnosis of Langerhans' cell histiocytosis was confirmed. (author)

  13. Langerhans' cell histiocytosis presenting with an intracranial epidural hematoma

    International Nuclear Information System (INIS)

    Lee, K.-W.; McLeary, M.S.; Zuppan, C.W.; Won, D.J.

    2000-01-01

    An 8-year-old boy developed vomiting and severe headache following minor head trauma. A CT scan of the head demonstrated a lytic lesion of the skull and adjacent epidural hematoma. Surgical evacuation and removal of the skull lesion and hematoma were carried out, and pathologic evaluation resulted in a diagnosis of Langerhans' cell histiocytosis (LCH). Epidural involvement of Langerhans' cell histiocytosis is very rare, and we report the first case of LCH presenting as an intracranial epidural hematoma. (orig.)

  14. Acute leukemia in association with Langerhans cell histiocytosis

    NARCIS (Netherlands)

    R.M. Egeler (Maarten); J.P. Neglia (J.); M. Aricò (Maurizio); B.E. Favara (B.); A. Heitger (A.); M.E. Nesbit (M.)

    1994-01-01

    textabstractLangerhans cell histiocytosis (LCH) and malignancy occurring in the same individual is unusual and has generally been the subject of isolated case reports. To better define the occurrence of these events a registry of cases with synchronous or asynchronous LCH and malignancy was

  15. Langerhans' cell histiocytosis presenting with an intracranial epidural hematoma

    Energy Technology Data Exchange (ETDEWEB)

    Lee, K.-W. [Department of Pediatrics, Loma Linda Children' s Hospital and University Medical Center, Loma Linda, CA (United States); McLeary, M.S. [Div. of Pediatric Radiology, Loma Linda Children' s Hospital and University Medical Center, Loma Linda, CA (United States); Zuppan, C.W. [Dept. of Pathology, Loma Linda Children' s Hospital and University Medical Center, Loma Linda, CA (United States); Won, D.J. [Div. of Pediatric Neurosurgery, Loma Linda University Children' s Hospital, Loma Linda, CA (United States)

    2000-05-01

    An 8-year-old boy developed vomiting and severe headache following minor head trauma. A CT scan of the head demonstrated a lytic lesion of the skull and adjacent epidural hematoma. Surgical evacuation and removal of the skull lesion and hematoma were carried out, and pathologic evaluation resulted in a diagnosis of Langerhans' cell histiocytosis (LCH). Epidural involvement of Langerhans' cell histiocytosis is very rare, and we report the first case of LCH presenting as an intracranial epidural hematoma. (orig.)

  16. Button sequestrum in a case of localized Langerhans' cell histiocytosis of the ilium: case report

    International Nuclear Information System (INIS)

    Tordeur, M.; Wybier, M.; Laporte, J.L.; Grenier, P.; Laredo, J.D.

    2000-01-01

    Langerhans' cell histiocytosis (LCH) is characterized by a proliferation of cells exhibiting the same immunohistochemical and ultra-structural characteristics as Langerhans' cells of the epidermis. Eosinophilic granuloma, chronic polyostotic disease (Hand-Schuller-Christian disease) and multisystemic disease (Letterer-Siwe disease) all belong to the spectrum LCH. Osseous lesions are the most common findings. the radiological appearance of skeletal LCH depends on the site and on disease activity. Button sequestrum has been described as uncommon in LCH of the skull and exceedingly rare in LCH at other sites. We report a case of localized LCH of the ilium with a button sequestrum. (author)

  17. Somatic activating ARAF mutations in Langerhans cell histiocytosis

    NARCIS (Netherlands)

    Nelson, David S.; Quispel, Willemijn; Badalian-Very, Gayane; van Halteren, Astrid G. S.; van den Bos, Cor; Bovée, Judith V. M. G.; Tian, Sara Y.; van Hummelen, Paul; Ducar, Matthew; MacConaill, Laura E.; Egeler, R. Maarten; Rollins, Barrett J.

    2014-01-01

    The extracellular signal-regulated kinase (ERK) signaling pathway is activated in Langerhans cell histiocytosis (LCH) histiocytes, but only 60% of cases carry somatic activating mutations of BRAF. To identify other genetic causes of ERK pathway activation, we performed whole exome sequencing on

  18. A Rare Disease in Adult: Langerhans Cell Histiocytosis

    Science.gov (United States)

    Berber, Ilhami; Erkurt, Mehmet Ali; Kuku, Irfan; Koroglu, Mustafa; Kaya, Emin; Unlu, Serkan

    2013-01-01

    Langerhans cell histiocytosis is a rare histiocytic disorder and has been diagnosed in all age groups, but is most common in children. This disease is very rare in adults. We presented a patient who was 62 years old man diagnosed langerhans cell histiocytosis. PMID:29147350

  19. Langerhans Cell Histiocytosis in Childhood: Review, Symptoms in the Oral Cavity, Differential Diagnosis and Report of One Case

    Directory of Open Access Journals (Sweden)

    Mohammad Shooriabi

    2016-08-01

    Full Text Available Background Langerhans cell histiocytosis (LCH is a rare disease in which monoclonal migration and proliferation of specific dendritic cells is seen. The disease primarily affects the bones and skin, but there is a possibility that involves other organs or appears as a multi-systemic disease. Case Report In oral examination of a nine-month girl, two deep wounds with a yellow membrane with approximate size of 1 × 1 cm on both sides of mandibular alveolar ridge were seen. The edges of the wounds were swollen and proliferated and redder than the surrounding mucosa. At the touch the edges of the wound were not indurated. The wound were created from the third-month and the size of wounds had become slightly larger within 6 months. According to the chronic wound and being non-responsive to various systemic and local treatments, incisional biopsy was taken from the wounds. Langerhans cell histiocytosis was confirmedhistologically and immune histochemically. Conclusion Mouth ulcers may be the only symptoms of Langerhans cell histiocytosis. Therefore, the role of dentist could be important in diagnosis of this disease.

  20. Two rare cases of laryngeal intralymphatic histiocytosis

    DEFF Research Database (Denmark)

    Reznitsky, Martin; Daugaard, Søren; Charabi, Birgitte Wittenborg

    2016-01-01

    We report two rare cases of intralymphatic histiocytosis causing, respectively, recurrent and persistent episodes of upper airway swelling and breathing difficulties. Case 1 was a 39-year-old man who was referred with recurrent upper airway swelling causing difficulty in breathing. A direct....... Extensive investigations were performed but discovered no abnormal findings. He received CO2 laser treatment twice and the swelling decreased. Intralymphatic histiocytosis is extremely rare in upper airway pathology. It is an important differential diagnosis in patients with recurrent and chronic laryngeal...

  1. Multisystem Langerhans cell histiocytosis coexisting with metastasizing adenocarcinoma of the lung: A case report

    Directory of Open Access Journals (Sweden)

    Lovrenski Aleksandra

    2013-01-01

    Full Text Available Introduction. Langerhans cell histiocytosis (LCH is an uncommon disease of unknown etiology characterized by uncontrolled proliferation and infiltration of various organs by Langerhans cells. Case report. We presented a 54-year-old man, heavy smoker, with dyspnea, cough, hemoptysis, headache and ataxia, who died shortly after admission to our hospital. On the autopsy, tumor was found in the posterior segment of the right upper pulmonary lobe as well as a right-sided occipitoparietal lesion which penetrated into the right ventricle resulting in internal and external hematocephalus. Histologically and immunohistohemically, the diagnosis of primary lung adenocarcinoma with brain metastasis was made (tumor cells showed positivity for CK7 and TTF-1 which confirmed the diagnosis. In the lung parenchyma around the tumor, as well as in brain tissue around the metastatic adenocarcinoma histiocytic lesions were found. Light microscopic examination of the other organs also showed histiocytic lesions involving the pituitary gland, hypothalamus, spleen and mediastinal lymph nodes. Immunohistochemical studies revealed CD68, S-100 and CD1a immunoreactivity within the histiocytes upon which the diagnosis of Langerhans' cells histiocytosis was made. Conclusion. The multisystem form of LCH with extensive organ involvement was an incidental finding, while metastatic lung adenocarcinoma to the brain that led to hematocephalus was the cause of death.

  2. Partial hypopituitarism and Langerhans cell histiocytosis

    Science.gov (United States)

    Balaguruswamy, S; Chattington, P D

    2011-01-01

    A case of multisystem Langerhans cell histiocytosis with pituitary involvement nearly 20 years after initial presentation. A 48-year-old man had histiocytosis X 22 years ago initially involving the groin; subsequently his external auditory meatus, scalp, gum, mandibular bone, perineum and axilla were involved and treated. The pituitary gland was involved 4 years ago. A thyrotropin-releasing hormone test showed delayed response suggestive of hypothalamic disease. Prolactin levels were normal. A gonadotropin-releasing hormone test showed impaired testosterone and gonadotrophin response in keeping with pituitary disease. A glucagon stimulation test showed an impaired growth hormone response but a normal cortisol increase. MRI pituitary showed an empty sella. There was no evidence of diabetes insipidus. Bone mineral densitometry was normal. He has partial hypopituitarism needing thyroxine and testosterone replacement. He also developed type 2 diabetes mellitus 9 years ago. He is closely monitored for any development of diabetes insipidus and the need for growth hormone supplementation. PMID:22715201

  3. Melanotic paraganglioma arising in the temporal horn following Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Yoo, Jeong Hyun; Rivera, Andreana; Naeini, Ramin M.; Yedururi, Sireesha; Megahead, Hatem; Bayindir, Petek; Fuller, Gregory N.; Suh, Jeong Soo; Adesina, Adekunle M.; Hunter, Jill V.

    2008-01-01

    Intracerebral paragangliomas are rare because of the lack of paraganglial cells in the cerebral tissue. We report a rare case of melanotic paraganglioma arising from the temporal horn of the lateral ventricle in a patient with prior Langerhans cell histiocytosis (LCH) treated with chemotherapy and radiation. (orig.)

  4. The rapid evolution of CT findings in pulmonary langerhans cell histiocytosis: a case report

    International Nuclear Information System (INIS)

    Kang, Tae Wook; Lee, Kyung Soo; Cho, Eun Yoon

    2007-01-01

    Imaging findings of pulmonary Langerhans cell histiocytosis (PLCH) demonstrate evolving changes over time, and the radiological transitions shown by imaging tools may allow a prediction of histopathological activity in PLCH. However, there are no reports describing how rapidly CT findings change with time. We describe a case of PLCH that showed a rapid evolutional change of the pulmonary lesions in a 48-year-old man, in which the nodular lesions showed cystic changes within two-month follow-up periods on chest CT scans

  5. Paediatric neuroradiological aspects of Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Demaerel, Philippe [University Hospital K.U.Leuven, Department of Radiology, Leuven (Belgium); Gool, Stefaan van [University Hospital K.U.Leuven, Paediatric Haemato-oncology/Neuro-oncology, Leuven (Belgium)

    2008-01-15

    Langerhans cell histiocytosis, previously known as histiocytosis X, is a complex disease consisting of three entities that are all characterized by a proliferation of the Langerhans cell. The clinical course is variable and ranges from a solitary lytic bone or skin lesion with complete remission to a multisystem disorder with possible lethal outcome. The clinical suspicion can be increased based on radiological findings that are important criteria in defining the extent of the disease involvement. A biopsy is often necessary for establishing the final diagnosis. The lytic craniofacial bone lesions are the most common craniospinal abnormality in Langerhans cell histiocytosis. Abnormalities in the hypothalamic-pituitary region are the most frequent manifestations, often accompanied with diabetes insipidus as the presenting symptom. A range of different central nervous system abnormalities can be recognized. It is important to be able to recognize the extensive spectrum of neuroradiological abnormalities in order to arrive at the diagnosis. Neuroimaging plays an even more important role in assessing the response to treatment or possible relapse. (orig.)

  6. Langerhans-cell histiocytosis of the cervical spine in an adult patient: Case report and review of the literature.

    Science.gov (United States)

    Vielgut, Ines; Liegl-Atzwanger, Bernadette; Bratschitsch, Gerhard; Leithner, Andreas; Radl, Roman

    2017-06-01

    Langerhans-cell histiocytosis (LCH) is a rare, benign bone tumor, usually occurring in children and younger adults under 20 years old. Only a few cases of solitary bone lesions of the adult spine are reported in literature, therapeutic guidelines or treatment regimens for lesions of the adult spine are not established yet to our knowledge.

  7. Thoracolumbar Langerhans cell histiocytosis in a toddler

    Directory of Open Access Journals (Sweden)

    Zhi Gang Lan

    2018-01-01

    Full Text Available Introduction: Langerhans cell histiocytosis (LCH is a rare uni or multisystem disorder associated with extreme production of immunoreactive Langerhans cells. Although this disorder has been reported in all age groups, spinal involvement especially thoracolumbar spine is seldom reported in toddlers. Case presentation: We present a one (1 year, four (4 months female child with a history of recurrent fever, irritability and severe anemia of one (1 month duration. CT-scan and MRI revealed a collapsed twelfth thoracic vertebra (T12 body height greater than 95% with a huge intradural soft tissue mass. T12 cortectomy via posterior thoracolumbar approach was used to decompress the soft tissue part followed by interbody fusion with titanium cage filled with autologous iliac crest bone graft, fixation using plates and screws. Conclusion: We are of the view that surgical decompression of spinal LHC lesions with interbody fusion with titanium cage filled with autologous iliac crest bone graft and fixation using plates and screws is very crucial in relieving neurological deficits. However, the patient will need repeated surgeries as she advances with age in a timely manner to avert any neurological deficit that may occur. Keywords: Langerhans cell histiocytosis (LCH, Letterer-Siwe disease, Hand-Schüller-Christian disease, Eosinophilic granuloma

  8. Radiotherapy for management of Langerhans' cell histiocytosis. Two case reports and a literature review

    International Nuclear Information System (INIS)

    Heyd, R.; Strassmann, G.; Martin, T.; Zamboglou, N.; Donnerstag, F.

    2000-01-01

    The use of radiotherapy in the treatment of Langerhans' cell histiocytosis was first reported in the literature in 1930 and has been proven as effective in numerous studies. We present the results of two female adults with eosinophilic granuloma of bone who underwent conventionally fractionated radiation therapy with total doses of 7x1.8 Gy and 7x2.0 Gy in four different sites. After observation periods raging from three months to six years local control of the disease was achieved in all treated locations. A review of 18 previously published studies include a total of 310 sites of eosinophilic granuloma of bone in 216 patients. It was demonstrated in 13 studies that the patients had complete relief of symptoms. An average of 94.3% had local control of the symptoms. Furthermore, in 12 studies for a total of 344 cases with involvement of other organs local control was reported in an average of 64.8% (range: 14.3-100%). Based on our own observations and on the literature review we conclude that low dose radiation therapy plays an important role in the management of localised Langerhans' cell histiocytosis. In order to minimise the risk of radiation induced neoplasms an accurate and precise radiation technique is required. (orig.) [de

  9. HISTIOCYTOSIS X: CLINICAL OBSERVATIONS

    Directory of Open Access Journals (Sweden)

    E. Y. Ponomareva

    2012-01-01

    Full Text Available Two clinical cases of pulmonary Langerhans cell histiocytosis X have been analyzed demonstrating lung and other inner organ pathology, common clinical and X-ray features but different life prognosis.

  10. The role of bone scintigraphy in Langerhans' cell histiocytosis; a case report; Interet de la scintigraphie osseuse dans l'histiocytose langerhansienne: a propos d'un cas

    Energy Technology Data Exchange (ETDEWEB)

    Razzouk, M.; Carrier, P.; Darcourt, J. [Centre TEP, Hopital de l' Archet-1, Service Universitaire de Medecine Nucleaire, 06 - Nice (France); Razzouk, M.; Carrier, P.; Darcourt, J. [Federation interhospitaliere, CHU, CAL universitaire de Nice, 06 - Nice (France); Deville, A. [Hopital de l' Archet, Service d' Hemato-Oncologie Pediatrique, 06 - Nice (France); Cadet, G. [Centre Hospitalier intercommunal de Grasse, Service de Pediatrie, 06 - Grasse (France)

    2008-09-15

    We present one case of bone-Langerhans cell histiocytosis in a three-year-old male child presenting osseous lesions in the skull and the femur, which are very frequent localizations in histiocytosis. Bone scintigraphy is useful for both initial staging and follow-up associated with other imaging modalities. (authors)

  11. Isolated thymic Langerhans cell histiocytosis discovered on F-18 fluorodeoxyglucose positron emission tomography/computed tomography (F-18 FDG PET/CT).

    Science.gov (United States)

    Turpin, Sophie; Carret, Anne-Sophie; Dubois, Josée; Buteau, Chantal; Patey, Natalie

    2015-11-01

    The thymic infiltration in young patients with multisystemic Langerhans cell histiocytosis and its radiologic features are well known. However, isolated thymic disease has seldom been reported in the literature. We report the case of a 10-month-old child admitted for fever of unknown origin. Whole-body F-18 fluorodeoxyglucose positron emission tomography/computed tomography (F-18 FDG PET/CT) was performed to identify a focus of infection. It demonstrated an unusual aspect of the thymus, which led to further investigation and revealed isolated infiltration of the thymus by Langerhans cell histiocytosis. The patient was treated accordingly and is now disease free. As evaluation of Langerhans cell histiocytosis patients with F-18 FDG PET/CT is becoming more frequent, it is important to be aware of the scintigraphical characteristics of thymic Langerhans cell histiocytosis.

  12. Isolated thymic Langerhans cell histiocytosis discovered on F-18 fluorodeoxyglucose positron emission tomography/computed tomography (F-18 FDG PET/CT)

    Energy Technology Data Exchange (ETDEWEB)

    Turpin, Sophie [CHU Sainte-Justine, Nuclear Medicine, Montreal (Canada); Carret, Anne-Sophie [CHU Sainte-Justine, Hemato-Oncology, Montreal (Canada); Dubois, Josee [CHU Sainte-Justine, Radiology, Montreal (Canada); Buteau, Chantal [CHU Sainte-Justine, Infectious Diseases, Montreal (Canada); Patey, Natalie [CHU Sainte-Justine, Pathology, Montreal (Canada)

    2015-11-15

    The thymic infiltration in young patients with multisystemic Langerhans cell histiocytosis and its radiologic features are well known. However, isolated thymic disease has seldom been reported in the literature. We report the case of a 10-month-old child admitted for fever of unknown origin. Whole-body F-18 fluorodeoxyglucose positron emission tomography/computed tomography (F-18 FDG PET/CT) was performed to identify a focus of infection. It demonstrated an unusual aspect of the thymus, which led to further investigation and revealed isolated infiltration of the thymus by Langerhans cell histiocytosis. The patient was treated accordingly and is now disease free. As evaluation of Langerhans cell histiocytosis patients with F-18 FDG PET/CT is becoming more frequent, it is important to be aware of the scintigraphical characteristics of thymic Langerhans cell histiocytosis. (orig.)

  13. Skull Base Langerhans Cell Histiocytosis with Diabetes Insipidus and Panhypopituitarism- A Rare Clinical Entity

    Directory of Open Access Journals (Sweden)

    Anirban Ghosh

    2017-12-01

    Case Report A 16 year old male presented with diminished vision, bilateral ptosis, left sided lateral rectus palsy, hypoesthesia of trigeminal nerve with nasal obstruction for last 5 months. There was polypoidal, bleeding mass in both nasal cavities. Contrast enhanced CT Scan showed a large homogenous mass arising from sphenoid extending into cavernous sinus and the suprasellar region. Endoscopic nasal biopsy revealed abundant Langerhans cell histiocytes, macrophages, neutrophils. Chemotherapy and radiotherapy were administered. But within 2 months the patient presented with Cushingoid features and further diminution of vision. Detailed work-up revealed Hypogonadotrophic hypogonadism and diabetes insipidus. Debulking of the tumour was done and left optic nerve decompression was done. PET scan was performed and showed large, well defined mass with increased FDG uptake in the skull base with suprasellar extension, reaching upto petrous temporal bone and causing bony erosion of ethmoid and sphenoid sinuses. Patient was then advised adjuvant chemotherapy.   Discussion Langerhans cell histiocytosis is a rare group of disorders characterised by abnormal clonal proliferation and accumulation of abnormal dendritic cells. Involvement of base of skull is even rarer. Though diabetes insipidus has been reported in Langerhans cell histiocytosis involving pituitary, panhypopituitarism is rare. These combinations of extensive Langerhans cell histiocytosis of base skull with clinical features of Diabetes insipidus and panhypopituitarism makes this case a rare clinical entity.

  14. Splenomegaly unresponsive to standard and salvage chemotherapy in Langerhans cell histiocytosis: a case of extramedullary hematopoiesis.

    Science.gov (United States)

    Christiansen, Ellen C; Ellwein, Marcine; Neglia, Joseph P

    2012-06-01

    Langerhans cell histiocytosis (LCH) is a proliferative disorder of dendritic cells which has evaded efforts to clearly define pathogenesis, diagnostic criteria, and therapeutic response markers. Strides have been made at classification with the recent development of a quantified score of disease severity. Splenic involvement is an indicator of poor prognosis, with spleen size its surrogate marker in evaluation and scoring. We describe a case of pediatric LCH with progressive splenomegaly despite treatment, which when examined at splenectomy revealed no LCH involvement but extramedullary hematopoiesis. These findings thus challenge our understanding of splenomegaly as a marker of disease. Copyright © 2011 Wiley Periodicals, Inc.

  15. High-resolution computed tomography findings in pulmonary Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Rodrigues, Rosana Souza [Universidade Federal do Rio de Janeiro (HUCFF/UFRJ), RJ (Brazil). Hospital Universitario Clementino Fraga Filho. Unit of Radiology; Capone, Domenico; Ferreira Neto, Armando Leao [Universidade do Estado do Rio de Janeiro (UERJ), Rio de Janeiro, RJ (Brazil)

    2011-07-15

    Objective: The present study was aimed at characterizing main lung changes observed in pulmonary Langerhans cell histiocytosis by means of high-resolution computed tomography. Materials and Methods: High-resolution computed tomography findings in eight patients with proven disease diagnosed by open lung biopsy, immunohistochemistry studies and/or extrapulmonary manifestations were retrospectively evaluated. Results: Small rounded, thin-walled cystic lesions were observed in the lung of all the patients. Nodules with predominantly peripheral distribution over the lung parenchyma were observed in 75% of the patients. The lesions were diffusely distributed, predominantly in the upper and middle lung fields in all of the cases, but involvement of costophrenic angles was observed in 25% of the patients. Conclusion: Comparative analysis of high-resolution computed tomography and chest radiography findings demonstrated that thinwalled cysts and small nodules cannot be satisfactorily evaluated by conventional radiography. Because of its capacity to detect and characterize lung cysts and nodules, high-resolution computed tomography increases the probability of diagnosing pulmonary Langerhans cell histiocytosis. (author)

  16. Primary Langerhans cell histiocytosis (LCH in the adult cervical spine: A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Sang-Deok Kim, M.D.

    2017-03-01

    Full Text Available Langerhans cell histiocytosis (LCH of the spine is a common benign disease in children and adolescents that rarely affects adults. Main management of single lesion (unifocal vertebral LCH is conservative method, unless there is neurological deficit due to mass effect, surgery must be considered. This is an interesting and rare case report of the patient with LCH at C5 vertebral body who underwent fusion surgery.

  17. MAP2K1 and MAP3K1 mutations in langerhans cell histiocytosis

    NARCIS (Netherlands)

    Nelson, David S.; van Halteren, Astrid; Quispel, Willemijn T.; van den Bos, Cor; Bovée, Judith V. M. G.; Patel, Bhumi; Badalian-Very, Gayane; van Hummelen, Paul; Ducar, Matthew; Lin, Ling; MacConaill, Laura E.; Egeler, R. Maarten; Rollins, Barrett J.

    2015-01-01

    Langerhans cell histiocytosis (LCH) is now understood to be a neoplastic disease in which over 50% of cases have somatic activating mutations of BRAF. However, the extracellular signal-related (ERK) pathway is activated in all cases including those with wild type BRAF alleles. Here, we applied a

  18. The presence of cytokines in Langerhans' cell histiocytosis

    NARCIS (Netherlands)

    deGraaf, JH; Tamminga, RYJ; DamMeiring, A; Kamps, WA; Timens, W

    1996-01-01

    Langerhans' cell histiocytosis (LCH) is characterized by an accumulation and/or proliferation of cells with a Langerhans' cell (LC) phenotype. The aetiology and pathogenesis of LCH are unknown; it is suggested that LCH is caused by an immunological dysregulation. Production of cytokines is a central

  19. A case report of orbital Langerhans cell histiocytosis presenting as a orbital cellulitis.

    Science.gov (United States)

    Albert-Fort, M; González-Candial, M

    2018-04-08

    A 10-year-old girl was seen with a 3-week history of right upper lid swelling and with no other symptoms or fever. There was no recent history of sinusitis, trauma, or previous infection involving the periorbital area, or response to oral antibiotic treatment. Orbital computed tomography showed a lesion involving the upper margin of the orbit, and bone destruction at the orbital roof. Biopsy performed revealed the presence of Langerhans cell Histiocytosis. The lesion was surgically debulked and corticosteroids were used intra-operatively. The lesion responded to treatment. The orbital involvement of Langerhans cell histiocytosis, despite its low incidence, should be considered in the examination of acute peri-orbital swelling. It usually presents as an osteolytic lesion, and it is confirmed with a histological examination and immunohistochemical techniques for CD1a and S100. An interdisciplinary approach is recommended to rule out multifocal or multisystemic diseases, as well as to develop an appropriate treatment strategy. Copyright © 2018 Sociedad Española de Oftalmología. Publicado por Elsevier España, S.L.U. All rights reserved.

  20. CT manifestations of pulmonary Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Qiang Jun; Yu Wei; Gao Wanqin; Song Haiqiao; Ma Yingjian

    2010-01-01

    Objective: To analyzes the CT manifestations of pulmonary Langerhans cell histiocytosis (PLCH). Methods: CT features of 11 patients with PLCH proved pathologically were analyzed retrospectively. Results: The main findings in 11 PLCHs were cysts and nodules. Two cases only had cysts, and 1 only had nodules, which most had cavitations. The other 8 cases showed cysts and nodules with 4 cases mainly manifested with cysts and nine mainly manifested with nodules. Two cases had pulmonary interstitial changes. One case only had cysts in the left upper lung field and 10 cases had lesions not only in the upper and middle but in the lower lung field, which 2 cases had more lesions in the lower and costo-phrenic angle field and 8 cases had less lesions in the costo-phrenic angle field. Three of these 8 cases had more lesions in the superior lobe and apical segment of lower lobe. Conclusion: CT manifestations of PLCH are helpful for the early diagnosis. (authors)

  1. Niemann-Pick disease, type B with TRAP-positive storage cells and secondary sea blue histiocytosis

    Directory of Open Access Journals (Sweden)

    R. Saxena

    2009-09-01

    Full Text Available We present 2 cases of Niemann Pick disease, type B with secondary sea-blue histiocytosis. Strikingly, in both cases the Pick cells were positive for tartrate resistant acid phosphatase, a finding hitherto described only in Gaucher cells. This report highlights the importance of this finding as a potential cytochemical diagnostic pitfall in the diagnosis of Niemann Pick disease.

  2. A rare case of langerhans cell histiocytosis of the gastrointestinal tract

    Institute of Scientific and Technical Information of China (English)

    Uday Shankar; Monika Prasad; Om P Chaurasia

    2012-01-01

    Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the proliferation of specialized,bone marrow-derived langerhans cells and mature eosinophils.The clinical spectrum ranges from an acute,fulminant,disseminated disease called LettererSiwe disease to solitary or few,indolent and chronic lesions of the bone or other organs called eosinophilic granuloma.Involvement of the gastrointestinal tract is very rare in LCH.We present the case of a 53-year-old woman referred by her primary care physician for a screening colonoscopy.A single sessile polyp,measuring 4 mm in size,was found in the rectum.Histopathological examination revealed that the lesion was relatively well circumscribed and comprised mainly a mixture of polygonal cells with moderate-to-abundant pink slightly granular cytoplasm.The nuclei within these cells had frequent grooves and were occasionally folded.Immunohistochemical staining was positive for CD-1a which confirmed the diagnosis of LCH.On further workup,there was no evidence of involvement of any other organ.On follow up colonoscopy one year later,there was no evidence of disease recurrence.Review of the published literature revealed that LCH presenting as solitary colonic polyp is rare.However,with the increasing rates of screening colonoscopy,more colonic polyps may be identified as LCH on histopathology.This underscores the importance of recognizing this rare condition and ensuring proper follow-up to rule out systemic disease.

  3. Cerebellar and basal ganglion involvement in Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Saatci, I.; Baskan, O.; Haliloglu, M.; Aydingoz, U. [Department of Radiology, Hacettepe University Hospital, Sihhiye 06100, Ankara (Turkey)

    1999-06-01

    Langerhans cell histiocytosis (LCH) is a disease of unknown cause characterised by proliferation of histiocytic granulomas in tissues; the primary cerebral manifestation is diabetes insipidus caused by hypothalamic infiltration. We present a patient in whom, except for the absence of high signal on T 1 weighting in the posterior pituitary, consistent with central diabetes insipidus, MRI showed no evidence of hypothalamic involvement by histiocytosis, despite the long duration of the disease. However, there was bilateral, symmetrical involvement of the cerebellum and globus pallidus in addition to a calvarial lesion. High signal in the cerebellar white matter on T 2-weighted images may represent demyelination, gliosis and cell loss, as previously reported on pathologic examination. (orig.) With 5 figs., 22 refs.

  4. Need for a cooperative study: Pulmonary Langerhans cell histiocytosis and its management in adults

    NARCIS (Netherlands)

    McClain, Kenneth L.; Gonzalez, Jorge Mario; Jonkers, Rene; de Juli, Emanuela; Egeler, Maarten

    2002-01-01

    BACKGROUND: Pulmonary involvement with Langerhans cell histiocytosis (LCH, formerly known as histiocytosis-X) presents as an interstitial process in children and adults either with or without symptoms. In contrast to other manifestations of LCH, most patients with pulmonary disease are adults.

  5. Epstein-Barr virus-containing T-cell lymphoma presents with hemophagocytic syndrome mimicking malignant histiocytosis.

    Science.gov (United States)

    Su, I J; Hsu, Y H; Lin, M T; Cheng, A L; Wang, C H; Weiss, L M

    1993-09-15

    The previously designated malignant histiocytosis (MH) may include lymphoid neoplasms of T-cell lineage as well as patients with benign virus-associated hemophagocytic syndrome (VAHS). In this study, the association of Epstein-Barr virus (EBV) with T cell lymphomas which present with clinicopathologic features indistinguishable from malignant histiocytosis (MH) was investigated further. Four adult patients, three women and one man, were admitted because of fever, cutaneous lesions, hepatosplenomegaly, and jaundice. Laboratory examinations revealed pancytopenia, abnormal liver functions and coagulopathy. All patients ran a fulminant course terminating in a hemophagocytic syndrome within 1 month. Immunophenotypic study, Southern blot analysis, and in situ hybridization were performed on the specimens obtained from the four patients. The biopsy-necropsy specimens from skin, liver, spleen, and bone marrow showed infiltration of atypical large cells with reactive histiocytosis and florid hemophagocytosis activity. Based on the clinical and histologic findings, these cases would have been designated as MH by previous criteria. Immunophenotypic, Southern blot, and in situ hybridization studies, however, showed clonotypic proliferation of EBV genomes in the nuclei of the large atypical cells that expressed T-cell antigens. Therefore, these patients should be diagnosed as a recently described EBV-associated peripheral T-cell lymphoma (EBV-PTCL). EBV-PTCL may present with a fulminant hemophagocytic syndrome indistinguishable from the previously designated MH. This finding represents a step forward in our changing concept regarding MH, some of which only recently has been suggested to be of T-cell lymphoma origin. Differentiation from benign VAHS is clinically important. Features useful in this distinction are tabulated and discussed.

  6. Pulmonary Langerhans Cell Histiocytosis with Lytic Bone Involvement in an Adult Smoker: Regression following Smoking Cessation

    Directory of Open Access Journals (Sweden)

    B. Routy

    2015-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare myeloid neoplasm characterized by the proliferation and dissemination of histiocytes. These in turn may cause symptoms ranging from isolated, infiltrative lesions to severe multisystem disease. Pulmonary Langerhans cell histiocytosis (PLCH presents as a localized polyclonal proliferation of Langerhans cells in the lungs causing bilateral cysts and fibrosis. In adults, this rare condition is considered a reactive process associated with cigarette smoking. Recently, clonal proliferation has been reported with the presence of BRAF V600E oncogenic mutation in a subset of PLCH patients. Spontaneous resolution was described; however, based on case series, smoking cessation remains the most effective way to achieve complete remission and prevent long term complications related to tobacco. Herein, we report the case of an adult woman with biopsy-proven PLCH presenting with thoracic (T8 vertebral bone destruction. Both the lung and the bone diseases regressed following smoking cessation, representing a rare case of synchronous disseminated PCLH with bone localization. This observation underscores the contribution of cigarette smoking as a systemic trigger of both pulmonary and extrapulmonary bone lesions. A review of similar cases in the literature is also presented.

  7. EXPRESSION OF CELLULAR ADHESION MOLECULES IN LANGERHANS CELL HISTIOCYTOSIS AND NORMAL LANGERHANS CELLS

    NARCIS (Netherlands)

    DEGRAAF, JH; TAMMINGA, RYJ; KAMPS, WA; TIMENS, W

    1995-01-01

    Langerhans cell histiocytosis (LCH) is characterized by lesions with an accumulation and/or proliferation of Langerhans cells (LCs). Little is known of the etiology and pathogenesis of LCH. Although the relation between the LCH cell and normal LCs is currently uncertain, the localizations of the LCH

  8. An unusual case of adult-onset multi-systemic Langerhans cell histiocytosis with perianal and incident thyroid involvement

    Directory of Open Access Journals (Sweden)

    Ozen Oz Gul

    2017-02-01

    Full Text Available Langerhans cell histiocytosis (LCH is a rare sporadic disease characterized by histiocytic neoplastic infiltration of various organ systems and a wide spectrum of clinical manifestations, ranging from benign and self-limiting to lethal. Herein, we report a rare case of adult-onset multi-systemic LCH in a 36-year-old male patient with an initial perianal presentation and incidental finding of subsequent thyroid gland involvement in the follow-up period. The patient with a history of perianal LCH treated with surgical excision and local radiotherapy was referred to our Endocrinology Department upon detection of hypermetabolic nodular lesions in the left lateral lobe of thyroid gland on positron emission tomography–computed tomography (PET/CT scan in the nineth month of follow-up. Current evaluation revealed euthyroid status, a hypoechoic solid lesion of 13 × 9 mm in size with irregular borders in the left thyroid lobe on thyroid USG and cytologic assessment of thyroid nodule. The patient was diagnosed with suspected, oncocytic lesion, Hashimoto thyroiditis or LCH. The patient underwent total thyroidectomy and pathological assessment confirmed the diagnosis of Langerhans cell histiocytosis. Assessments in the sixth month of postoperative follow-up revealed euthyroid status with no thyroid tissue remnants or pathological lymph node on thyroid USG. In view of the multifocal lesions indicating multi-system disease, a systemic chemotherapy protocol with combination of prednisone (PRED and vinblastine (VBL has been planned by the hematology department.

  9. Conventional radiology in the bony compromise of Langerhans cells Histiocytosis

    International Nuclear Information System (INIS)

    Morales, Nilson; Gonzalez, Claudia Patricia; Melendez, Patricia; Terselich, Gretty

    1999-01-01

    We present a descriptive study of 47 patients who attended the National Cancer Institute in Bogota, Colombia with pathological diagnosis of Langerhans cell histiocytosis. We reviewed the most frequent conventional x-ray findings

  10. High expression of markers of apoptosis in Langerhans cell histiocytosis

    DEFF Research Database (Denmark)

    Petersen, Bodil Laub; Lundegaard, Pia Rengtved; Bank, M I

    2003-01-01

    53 and the number of cells in apoptosis detected with TUNEL. Langerhans cell histiocytosis cells showed strong expression of p53 and in some cases co-expression of Fas and Fas-L. The expression of Fas-L was significantly higher in infiltrates from patients with single-system disease. The actual...... number of pathological Langerhans cells in apoptosis as estimated by TUNEL was low. CONCLUSIONS: The low number of TUNEL-reactive cells can be explained by the rapid turnover of apoptotic cells in the tissue, not leaving the apoptotic cells long enough in the tissue to be detected. The co......-expression of Fas and Fas-L in some Langerhans cells can lead to an autocrine apoptotic shortcut, mediating the death of the double-positive cells. Our findings suggest that apoptosis mediated through the Fas/Fas-L pathway may contribute to the spontaneous regression of lesions in single-system disease. A delicate...

  11. Magnetic resonance imaging of thickened pituitary stalk proceeding to langerhans cell histiocytosis in a child

    International Nuclear Information System (INIS)

    Halefoglu, A.M.

    2006-01-01

    Magnetic resonance imaging has shown isolated pituitary stalk thickening in certain cases of idiopathic or secondary central diabetes insipidus (Dl) due to infiltrative processes. We present a 4-year-old boy who was initially diagnosed as having central Dl. The MRI showed isolated pituitary stalk thickening with prominent homogeneous contrast enhancement. The remaining findings on MRI were within normal limits. The patient's personal and family history and laboratory and clinical findings were unremarkable; therefore, he was initially diagnosed as having idiopathic Dl. Since central Dl and isolated pituitary stalk thickening may be considered to be the first manifestations of Langerhans cell histiocytosis, we decided to follow up the patient. After 5 months, following the initial diagnosis, on skeletal X-ray survey, the patient did indeed develop multiple lytic skull lesions which, on biopsy, were histologically typical bone lesions of Langerhans cell histiocytosis. Copyright (2006) Blackwell Science Pty Ltd

  12. Intracranial non-Langerhans cell histiocytosis presenting as an isolated intraparenchymal lesion

    Energy Technology Data Exchange (ETDEWEB)

    Rajaram, Smitha; Shackley, Fiona; Raghavan, Ashok [Western Bank, Sheffield Children' s Hospital, Sheffield (United Kingdom); Wharton, Stephen B. [University of Sheffield, Department of Neurosciences, Sheffield (United Kingdom); Connolly, Daniel J.A. [Western Bank, Sheffield Children' s Hospital, Sheffield (United Kingdom); University of Sheffield, Academic Radiology, Sheffield (United Kingdom)

    2010-12-15

    Non-Langerhans cell histiocytosis in the absence of cutaneous or other organ involvement is very rare. A Caucasian boy age 3 years 11 months presented with episodes of recurrent right-side seizures over 2 weeks. Brain CT and MR imaging showed a single enhancing left frontal lobe lesion. Stereotactic biopsy was performed and histological examination showed diffuse infiltrate of macrophages with foamy cytoplasm. Four months later there was recurrence of seizure activity despite anti-epileptic medication and a repeat MR scan showed a persistent enhancing lesion in the left frontal lobe. Histological examination of the resection specimen resembled juvenile xanthogranuloma (JXG) involving the central nervous system. In the absence of skin lesions a diagnosis of non-Langerhans cell histiocytosis was made. The child made a full recovery following surgery with resolution of his symptoms. (orig.)

  13. BRAF V600E-Positive Multisite Langerhans Cell Histiocytosis in a Preterm Neonate

    Directory of Open Access Journals (Sweden)

    Sara V. Bates

    2013-10-01

    Full Text Available Hemorrhagic pustules with a “blueberry muffin” appearance accompanied by respiratory failure in a neonate present a challenging differential diagnosis that includes infections and neoplasms. We present a case of multiorgan, multisite Langerhans cell histiocytosis (LCH, positive for the oncogenic BRAF V600E mutation, in a preterm neonate. Infants with LCH pose a diagnostic challenge due to their heterogeneous presentations. This case is unusual in that the newborn presented with severe multiorgan involvement. Due to the rare incidence, wide spectrum of clinical manifestations, and high mortality rate, clinicians must maintain a high index of suspicion for LCH.

  14. Monostotic Langerhans' cell histiocytosis in a child with central diabetes insipidus.

    Science.gov (United States)

    Soares, Eduardo Costa Studart; Quidute, Ana Rosa Pinto; Costa, Fábio Wildson Gurgel; Gurgel, Maria Helane Costa; Alves, Ana Paula Negreiros Nunes; Fonteles, Cristiane Sá Roriz

    2012-01-01

    Langerhans'cell histiocytosis (LCH) comprises a rare group of reticuloendothelial system disorders that can produce focal or systemic manifestations. Diabetes insipidus is considered to be an important indicator of serious underlying diseases in children, including LCH. We report the case of a young patient with monostotic LCH confined to the mandibular ramus, who was diagnosed with the disease after presenting symptoms of central diabetes insipidus and was satisfactorily treated with multi-agent chemotherapy. Additionally, we discuss the clinical, radiographic, histological and immunohistochemical findings, as well as the multidisciplinary approach of this important disease, which should receive attention by dental practitioners, especially when it occurs in children.

  15. Liver involvement in Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Wong, Adelaine; Ortiz-Neira, Clara L.; Abou Reslan, Walid; Kaura, Deepak; Sharon, Raphael; Anderson, Ronald; Pinto-Rojas, Alfredo

    2006-01-01

    Liver involvement in Langerhans cell histiocytosis (LCH) typically presents with hepatomegaly and other signs of liver dysfunction. We present an 11-month-old child having only minimally elevated liver enzymes as an indication of liver involvement. Using sonography as the initial diagnostic tool followed by MRI, LCH of the liver was revealed. A review of sonographic, CT, MRI and MR cholangiopancreatography findings in liver LCH is presented. We recommend that physicians consider sonography and MRI screening for liver involvement in patients with newly diagnosed LCH, as periportal involvement may be present with little or no liver function abnormality present, as in this patient. (orig.)

  16. Histiocytosis versus (Hand-Schuller-Christian disease) - a case report

    International Nuclear Information System (INIS)

    Carneiro Filho, Jose Olimar; Leite, Marta Santos; Andrade Neto, Jose Moacyr

    2002-01-01

    The authors report a case of a 4-year-old girl with headache, irritability, othorrea and unilateral exophtalmus. Computed tomography and plain films showed large lytic areas in the vault and base of the skull. No other bones were involved. The diagnosis of histiocytosis X (Hand-Schuller-Christian disease) was confirmed by bone marrow biopsy. This paper presents the cytological and radiological findings of this patient as well as a review of the literature. (author)

  17. [Langerhans cell histiocytosis in adults].

    Science.gov (United States)

    Néel, A; Artifoni, M; Donadieu, J; Lorillon, G; Hamidou, M; Tazi, A

    2015-10-01

    Langerhans cell histiocytosis (LCH) is a rare disease characterized by the infiltration of one or more organs by Langerhans cell-like dendritic cells, most often organized in granulomas. The disease has been initially described in children. The clinical picture of LCH is highly variable. Bone, skin, pituitary gland, lung, central nervous system, lymphoid organs are the main organs involved whereas liver and intestinal tract localizations are less frequently encountered. LCH course ranges from a fulminant multisystem disease to spontaneous resolution. Several randomized controlled trials have enable pediatricians to refine the management of children with LCH. Adult LCH has some specific features and poses distinct therapeutic challenges, knowing that data on these patients are limited. Herein, we will provide an overview of current knowledge regarding adult LCH and its management. We will also discuss recent advances in the understanding of the disease, (i.e. the role of BRAF oncogene) that opens the way toward targeted therapies. Copyright © 2015 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

  18. Radiological signs of childhood Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Mas, F.; Menor, F.; Moreno, A.; Vallcanera, A.; Esteban, M.J.; Muro, D.; Cortina, H.

    1997-01-01

    To describe the most prominent radiological sings of Langerhans cell histiocytosis (LCH) in childhood and review the general aspects of greatest interest to the general radiologist. The clinical and radiological histories of 40 children diagnosed on the basis of biopsy and/or cytology as having LCH in our center over a 16-year period were reviewed. Bone involvement was observed in 95% of patients; flat bones were affected in 95% of the cases, most frequently the cranium. Radiological changes in temporal bone were viewed in 15% of cases. There were long-bone lesions in 21% of the patients, involving, metaphyseal-diaphyseal bones in every case; multiple epiphyseal dysplasia was found in one and a purely cortical lesion in another. Vertebral involvement was detected in 18% of patients. Clinical evidence of diabetes insipidus (DI) was present in 22.5% of cases, while there were clinical or radiological signs of lung involvement in 15%. Bone involvement is the most common association reported in childhood LCH and is usually the reason for medical consultation. The flat bones, especially the cranium, are those most often affected. Diagnosis and follow-up studies are mainly based on plain radiography. DI due to hypothalamic infiltration in usually associated with normal CT scan. Lung involvement, uncommon in pediatric patients, is the major clinical and radiological difference with respect to the adult form. (Author) 33 refs

  19. Intralymphatic Histiocytosis: A Report of 2 Cases.

    Science.gov (United States)

    Gómez-Sánchez, M E; Azaña-Defez, J M; Martínez-Martínez, M L; López-Villaescusa, M T

    Intralymphatic histiocytosis is a benign condition characterized by poorly defined erythematous plaques (sometimes forming a reticular pattern) as well as the presence of nodules and vesicles. Its etiology and pathogenesis appear to be related to chronic inflammation in the affected area, prior surgery, or systemic disease, particularly rheumatoid arthritis. We report on 2 new cases, both associated with joint surgery in the affected area and osteoarticular disease (primary synovial osteochondromatosis and rheumatoid arthritis). This is a chronic disease and there is no specific treatment. Different treatment options were chosen in the 2 cases described. A spectacular response to treatment with oral pentoxifylline and topical tacrolimus was observed in 1 of the patients. Copyright © 2017 AEDV. Publicado por Elsevier España, S.L.U. All rights reserved.

  20. LANGERHANS CELL HISTIOCYTOSIS - EXPRESSION OF LEUKOCYTE CELLULAR ADHESION MOLECULES SUGGESTS ABNORMAL HOMING AND DIFFERENTIATION

    NARCIS (Netherlands)

    DEGRAAF, JH; TAMMINGA, RYJ; KAMPS, WA; TIMENS, W

    Langerhans' cell histiocytosis (LCH) is characterized by an accumulation of cells with a Langerhans' cell (LC) phenotype. Most patients present with solitary skin or bone lesions, but multi-organ lesions may appear Twenty-two LCH-tissue sections from 13 children and adolescents, with lesions at

  1. Concomitant sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman Disease) and diffuse large B-cell lymphoma: A case report

    OpenAIRE

    Moore, JC; Zhao, X; Nelson, EL

    2008-01-01

    Abstract Introduction Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman Disease, is a rare and benign source of lymphadenopathy first described in 1969, which mimics neoplastic processes. This disease commonly presents in children and young adults with supra-diaphragmatic lymphadenopathy or extranodal lesions consisting of tissue infiltrates composed of a polyclonal population of histiocytes. Since its description greater than 400 cases have been described, sometim...

  2. Radiologic features in histiocytosis syndrome

    International Nuclear Information System (INIS)

    Hong, Sung Mo; Cho, Byung Jae; Yeon, Kyung Mo

    1980-01-01

    Histiocytosis syndrome is not rare disease of unknown etiology, characterized by development of granulomatous lesions with histiocytic proliferation. Authors analyzed 22 cases, which had been confirmed as histiocytosis syndrome from 1971 to Feb. 1980 with special attention to 15 cases showing positive findings on radiological examinations. The results are as follows. 1. Overall male to female ratio was about 2:1. The majority were between 1 and 7 years of age. 2. Skeletal system was involved in orders as follows: skull, pelvis, femur, rib, spine. 3. Four cases of pulmonary involvement were experienced. All cases had interstitial involvement with reticulonodular densities on roentgenograms. 4. We had experienced a pituitary tumor, presumably localized histiocytic mass, in a patient with diabetes insipidus. 5. In long bone involvement, diaphysis or metaphysis was usually involved, but in one patient, lesion were extended into epiphysis. 6. One case of platyspondyly was found, with symmetrical compression

  3. Radiologic features in histiocytosis syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Hong, Sung Mo; Cho, Byung Jae; Yeon, Kyung Mo [College of Medicine, Seoul National University, Seoul (Korea, Republic of)

    1980-12-15

    Histiocytosis syndrome is not rare disease of unknown etiology, characterized by development of granulomatous lesions with histiocytic proliferation. Authors analyzed 22 cases, which had been confirmed as histiocytosis syndrome from 1971 to Feb. 1980 with special attention to 15 cases showing positive findings on radiological examinations. The results are as follows. 1. Overall male to female ratio was about 2:1. The majority were between 1 and 7 years of age. 2. Skeletal system was involved in orders as follows: skull, pelvis, femur, rib, spine. 3. Four cases of pulmonary involvement were experienced. All cases had interstitial involvement with reticulonodular densities on roentgenograms. 4. We had experienced a pituitary tumor, presumably localized histiocytic mass, in a patient with diabetes insipidus. 5. In long bone involvement, diaphysis or metaphysis was usually involved, but in one patient, lesion were extended into epiphysis. 6. One case of platyspondyly was found, with symmetrical compression.

  4. Analysis of the BRAFV600E mutation in 19 cases of Langerhans cell histiocytosis in Japan.

    Science.gov (United States)

    Sasaki, Yuya; Guo, Ying; Arakawa, Fumiko; Miyoshi, Hiroaki; Yoshida, Noriaki; Koga, Yuhki; Nakashima, Kazutaka; Kurita, Daisuke; Niino, Daisuke; Seto, Masao; Ohshima, Koichi

    2017-09-01

    Langerhans cell histiocytosis (LCH) is a rare disease characterized by clonal proliferation of CD1a- and CD207 (langerin)-positive dendritic cells. Mutated BRAF (p.V600E) is observed in histiocyte-related diseases and dendritic cell-related diseases, including LCH. BRAFV600E is observed in some LCH cases and is thought to be involved in maintaining MAPK activation. We retrospectively analyzed BRAFV600E in 19 patients diagnosed with LCH. In our study, direct sequencing for exon 15, a mutation hotspot, demonstrated that 4 out of the 19 patients (21%) harbored a GTG > GAG (valine > glutamic acid) base substitution, which encodes BRAFV600E. The clinical impact of BRAFV600E in such diseases is unclear. The frequency of BRAFV600E in our LCH patients from Japan was lower than that reported in the United States and in Germany. However, reports from Asia tend to show a lower rate of the BRAFV600E mutation. These results imply the possibility of different genetic backgrounds in the pathogenesis of LCH across various ethnicities. We also performed an immunohistochemical analysis to detect BRAFV600E using the mutation-specific monoclonal antibody. However, immunohistochemical analysis failed to detect any mutated protein in any of the 4 BRAFV600E-positive cases. This implies that at present, BRAFV600E should be assessed by direct sequencing. Copyright © 2016 John Wiley & Sons, Ltd. Copyright © 2016 John Wiley & Sons, Ltd.

  5. Las histiocitosis Histiocytosis

    Directory of Open Access Journals (Sweden)

    Eva Svarch

    2001-12-01

    Full Text Available El término histiocitosis identifica un grupo de alteraciones que tienen en común la proliferación de células dendríticas (CD y los macrófagos, y se diagnostican más frecuentemente en niños. Dentro de las relacionadas con las CD las fundamentales son las histiocitosis a células de Langerhans (HCL. Las HCL tienen un comportamiento clínico muy variable, que puede ir desde una lesión que involucra un solo sitio o sistema hasta una enfermedad multisistémica. El tratamiento depende de la extensión del proceso. Una lesión única tiende a desaparecer espontáneamente. También la biopsia diagnóstica con o sin inyección de un esteroide puede iniciar la curación. Los pacientes con enfermedad multisistémica pueden beneficiarse con el tratamiento esteroideo y citostático o inclusive con el trasplante de células progenitoras hematopoyéticas. La histiocitosis sinusal con linfoadenopatías masivas o enfermedad de Rosai Dorfman se debe a la proliferación de los macrófagos, es de naturaleza benigna y usualmente autolimitada. Afecta sobre todo a niños y adultos jóvenes. La linfohistiocitosis hemofagocítica también se produce por la proliferación de los macrófagos y es una enfermedad rara con una alta mortalidad. Puede ser familiar (autosómica recesiva o secundaria a infecciones virales. Esta última forma se presenta más frecuentemente en el lactante pequeño. En la actualidad, sobre todo en la variedad familiar, el trasplante alogénico de células progenitoras hematopoyéticas puede ser la única medida curativaThe term histiocytosis identifies a group of disorders that have in common the proliferation of dentritic cells (DC and macrophages and is frequently diagnosed in children. Among the fundamental variants of histiocytosis related with DC, we find Langerhans cell histiocytosis (LCH. Langerhans cell histiocytosis has very variable clinical behavior that ranges from a lesion involving only one site or system to a multisystem

  6. Primary cerebral non-Langerhans cell histiocytosis: MRI and differential diagnosis

    Energy Technology Data Exchange (ETDEWEB)

    Ernemann, U.; Skalej, M.; Voigt, K. [Department of Neuroradiology, University Hospital Tuebingen, Hoppe-Seyler-Strasse 3, 72076 Tuebingen (Germany); Hermisson, M.; Platten, M. [Department of Neurology, University Hospital Tuebingen, Hoppe-Seyler-Strasse 3, 72076 Tuebingen (Germany); Jaffe, R. [Pathology Department, Children' s Hospital of Pittsburgh, 3705 Fifth Avenue, Pittsburgh, PA 15213 (United States)

    2002-09-01

    We report a young woman with primary cerebral non-Langerhans cell histiocytosis of the juvenile xanthogranuloma family. The clinical course was complicated by extensive infiltration of cranial nerves and meninges and epi- and intramedullary spinal dissemination. Whereas the cutaneous form of juvenile xanthogranuloma is usually benign and self-limited, central nervous system involvement is associated with high morbidity and mortality and might therefore be considered a separate clinical entity. (orig.)

  7. Langerhans Cell Histiocytosis: A Diagnostic Challenge in the Oral Cavity

    Directory of Open Access Journals (Sweden)

    Mehmet Ali Altay

    2017-01-01

    Full Text Available Background. Langerhans cell histiocytosis (LCH is a rare disorder of the reticuloendothelial system with unknown etiology. This report aims to present a case of LCH with diffuse involvement of the oral cavity and to raise awareness of the distinguishing features of this diagnostically challenging entity. Case Report. A 26-year-old male patient presented with complaints of teeth mobility, intense pain, and difficulty in chewing. Intraoral and radiological examinations revealed generalized gingival hyperplasia and severe teeth mobility with widespread alveolar bone loss. Periodontal therapy was performed with no significant improvement. An incisional biopsy revealed Langerhans cells and positive reaction to S-100 and CD1, and the patient was diagnosed with LCH. The patient underwent systemic chemotherapy with vinca alkaloids and corticosteroids. Regression of gingival lesions, as well as significant decrease in mobility of the remaining teeth and severity of pain, was achieved during 12 months of follow-up. Conclusion. The rarity and variable system involvement of LCH necessitate a multidisciplinary approach be carried out for accurate diagnosis, effective treatment, and an uneventful follow-up. Awareness of oral manifestations of LCH may aid clinicians greatly in reducing morbidity and mortality associated with this debilitating condition.

  8. New insights in lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis

    Directory of Open Access Journals (Sweden)

    Olga Torre

    2017-09-01

    Full Text Available Lymphangioleiomyomatosis (LAM and pulmonary Langerhans cell histiocytosis (PLCH are rare diseases that lead to progressive cystic destruction of the lungs. Despite their distinctive characteristics, these diseases share several features. Patients affected by LAM or PLCH have similar radiological cystic patterns, a similar age of onset, and the possibility of extrapulmonary involvement. In this review, the recent advances in the understanding of the molecular pathogenesis, as well as the current and most promising biomarkers and therapeutic approaches, are described.

  9. Pulmonary langerhans cell histiocytosis

    Directory of Open Access Journals (Sweden)

    Suri Harpreet S

    2012-03-01

    Full Text Available Abstract Pulmonary Langerhans Cell Histiocytosis (PLCH is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. The pathologic hallmark of PLCH is the accumulation of Langerhans and other inflammatory cells in small airways, resulting in the formation of nodular inflammatory lesions. While the overwhelming majority of patients are smokers, mechanisms by which smoking induces this disease are not known, but likely involve a combination of events resulting in enhanced recruitment and activation of Langerhans cells in small airways. Bronchiolar inflammation may be accompanied by variable lung interstitial and vascular involvement. While cellular inflammation is prominent in early disease, more advanced stages are characterized by cystic lung destruction, cicatricial scarring of airways, and pulmonary vascular remodeling. Pulmonary function is frequently abnormal at presentation. Imaging of the chest with high resolution chest CT scanning may show characteristic nodular and cystic abnormalities. Lung biopsy is necessary for a definitive diagnosis, although may not be required in instances were imaging findings are highly characteristic. There is no general consensus regarding the role of immunosuppressive therapy in smokers with PLCH. All smokers must be counseled on the importance of smoking cessation, which may result in regression of disease and obviate the need for systemic immunosuppressive therapy. The prognosis for most patients is relatively good, particularly if longitudinal lung function testing shows stability. Complications like pneumothoraces and secondary pulmonary hypertension may shorten life expectancy. Patients with progressive disease may require lung transplantation.

  10. 18-F-FDG PET/CT in Localizing Additional CNS Lesion in a Case of Langerhans Cell Histiocytosis: Determining Accurate Extent of the Disease.

    Science.gov (United States)

    Shamim, Shamim Ahmed; Tripathy, Sarthak; Mukherjee, Anirban; Bal, Chandrasekhar; Tripathi, Madhavi

    2017-01-01

    Central nervous system involvement is a rare manifestation of Langerhans cell histiocytosis (LCH), with bone and skin lesions being more frequent. MR remains the investigation of choice for localizing brain lesions. However, due to poor sensitivity of MRI in detecting osseous and pulmonary lesions, it is not used routinely in staging purposes until and unless indicated. We hereby discuss a case of 6-year-old boy of LCH who was referred for 18-F-FDG PET/CT for staging and knowing the extent of the disease, but a lesion in hypothalamus was picked up incidentally on FDG PET-CT study that was confirmed by MRI.

  11. Malignant histiocytosis in childhood: morphologic considerations.

    Science.gov (United States)

    Jurco, S; Starling, K; Hawkins, E P

    1983-12-01

    Eight cases diagnosed over a ten-year period as malignant histiocytosis (MH; histiocytic medullary reticulosis) were reviewed to clarify diagnostic criteria for the childhood disease and to identify sources of diagnostic confusion. Five of the eight cases met the authors' criteria for diagnosis; i.e., they were characterized by loose mixed infiltrates composed of three cell types--well-differentiated histiocytes, prohistiocytes, and malignant histiocytes--and they had no leukemic phase. Three cases did not share these features and were reclassified. The liver was found to be the organ most useful in premortem diagnosis, and immunoperoxidase staining for immunoglobulins and lysozyme was also helpful. The clinical and morphologic features of the five cases confirm the authors' view that diagnoses of MH should be limited to cases in which there is a loose pleomorphic population of all three types of histiocytes and that cases with monomorphous populations of aggregated malignant cells should be classified as lymphomas.

  12. Histiocitosis de células de Langerhans localizada en hueso malar: Presentación de un caso Localized langerhans cell histiocytosis of the zygomatic bone: A case report

    Directory of Open Access Journals (Sweden)

    María Loreto Castellón Zirpel

    2011-09-01

    Full Text Available La histiocitosis de células de Langerhans localizada (HCLL, conocida como granuloma eosinófilo, representa entre el 50 y el 60% de todos los casos de histiocitosis de células de Langerhans. El tratamiento clásico para la HCLL ha sido el curetaje o la resección de las lesiones óseas. Hay publicaciones de casos tratados con inyección intralesional de corticosteroides, combinado con curetaje. Se presenta un caso clínico de un paciente de tres años de edad con diagnóstico de HCLL que compromete en su extensión el hueso malar, tratado con infiltraciones de corticosteroides y posterior curetaje de la lesión. A un año de realizado el tratamiento, el paciente se encuentra asintomático y con una regeneración ósea del hueso malar, evidenciable en la tomografía axial computarizada.Localized Langerhans cell histiocytosis (LLCH, also known as eosinophilic granuloma, represents 50 to 60% of all cases of Langerhans cell histiocytosis. The standard treatment for LLCH has been lesion curettage or resection. Cases treated with intralesional corticosteroid injections combined with curettage have been described. We report the case of a three-year-old patient diagnosed of LLCH with extensive zygomatic bone involvement, who was treated with corticosteroid infiltrations and subsequent curettage of the lesion. One year after treatment, the patient is asymptomatic with zygomatic reossification evidenced on computed tomography.

  13. Hepatic involvement of Langerhans cell histiocytosis in children - imaging findings of computed tomography, magnetic resonance imaging and magnetic resonance cholangiopancreatography

    International Nuclear Information System (INIS)

    Shi, Yingyan; Qiao, Zhongwei; Gong, Ying; Yang, Haowei; Li, Guoping; Pa, Mier; Xia, Chunmei

    2014-01-01

    Langerhans cell histiocytosis is a rare disease that occurs mainly in children, and hepatic involvement is generally a poor prognostic factor. To describe CT and MRI findings of hepatic involvement of Langerhans cell histiocytosis in children, especially the abnormal bile duct manifestation on magnetic resonance cholangiopancreatography (MRCP). Thirteen children (seven boys, six girls; mean age 28.9 months) were diagnosed with disseminated Langerhans cell histiocytosis. They underwent CT (n = 5) or MRI (n = 4), or CT and MRI examinations (n = 4) to evaluate the liver involvement. Periportal abnormalities presented as band-like or nodular lesions on CT and MRI in all 13 children. The hepatic parenchymal lesions were found in the peripheral regions of the liver in seven children, including multiple nodules on MRI (n = 6), and cystic-like lesions on CT and MRI (n = 3). In 11 of the 13 children the dilatations of the bile ducts were observed on CT and MRI. Eight of the 13 children underwent MR cholangiopancreatography, which demonstrated stenoses or segmental stenoses with slight dilatation of the central bile ducts, including the common hepatic duct and its first-order branches. The peripheral bile ducts in these children showed segmental dilatations and stenoses. Stenosis of the central bile ducts revealed by MR cholangiopancreatography was the most significant finding of liver involvement in Langerhans cell histiocytosis in children. (orig.)

  14. Hepatic involvement of Langerhans cell histiocytosis in children - imaging findings of computed tomography, magnetic resonance imaging and magnetic resonance cholangiopancreatography

    Energy Technology Data Exchange (ETDEWEB)

    Shi, Yingyan; Qiao, Zhongwei; Gong, Ying; Yang, Haowei; Li, Guoping; Pa, Mier [Children' s Hospital of Fudan University, Department of Radiology, Shanghai (China); Xia, Chunmei [Shanghai Medical College of Fudan University, Physiology and Pathophysiology Department, Shanghai (China)

    2014-06-15

    Langerhans cell histiocytosis is a rare disease that occurs mainly in children, and hepatic involvement is generally a poor prognostic factor. To describe CT and MRI findings of hepatic involvement of Langerhans cell histiocytosis in children, especially the abnormal bile duct manifestation on magnetic resonance cholangiopancreatography (MRCP). Thirteen children (seven boys, six girls; mean age 28.9 months) were diagnosed with disseminated Langerhans cell histiocytosis. They underwent CT (n = 5) or MRI (n = 4), or CT and MRI examinations (n = 4) to evaluate the liver involvement. Periportal abnormalities presented as band-like or nodular lesions on CT and MRI in all 13 children. The hepatic parenchymal lesions were found in the peripheral regions of the liver in seven children, including multiple nodules on MRI (n = 6), and cystic-like lesions on CT and MRI (n = 3). In 11 of the 13 children the dilatations of the bile ducts were observed on CT and MRI. Eight of the 13 children underwent MR cholangiopancreatography, which demonstrated stenoses or segmental stenoses with slight dilatation of the central bile ducts, including the common hepatic duct and its first-order branches. The peripheral bile ducts in these children showed segmental dilatations and stenoses. Stenosis of the central bile ducts revealed by MR cholangiopancreatography was the most significant finding of liver involvement in Langerhans cell histiocytosis in children. (orig.)

  15. A Rare Case of Erdheim-Chester Disease and Langerhans Cell Histiocytosis Overlap Syndrome

    Directory of Open Access Journals (Sweden)

    Shahzaib Nabi

    2015-01-01

    Full Text Available A 48-year-old woman with a past medical history of seizures and end-stage renal disease secondary to obstructive uropathy from retroperitoneal fibrosis presented to the emergency department with seizures and altered mental status. A Glasgow Coma Scale of 4 prompted intubation, and she was subsequently admitted to the intensive care unit. Magnetic resonance imaging of the brain performed to elucidate the aetiology of her seizure showed a dural-based mass within the left temporoparietal lobe as well as mass lesions within the orbits. Further imaging showed extensive retroperitoneal fibrosis extending to the mediastinum with involvement of aorta and posterior pleural space. Imaging of the long bones showed bilateral sclerosis and cortical thickening of the diaphyses. Imaging of the maxillofacial structures showed osseous destructive lesions involving the mandible. These clinical and radiological features were consistent with a diagnosis of Erdheim-Chester disease; however, the patient’s skin biopsy was consistent with Langerhans cell histiocytosis.

  16. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): two case reports.

    Science.gov (United States)

    El Kohen, A; Planquart, X; Al Hamany, Z; Bienvenu, L; Kzadri, M; Herman, D

    2001-12-01

    Sinus histiocytosis with massive lymphadenopathy or Destombes-Rosai-Dorfman's syndrome is a rare benign disease of unknown etiology, usually seen in younger patients. The cases reported concerned a 15-month old Caucasian boy and an 8 year old black boy with unilateral cervical enlargement, occasional fever and without any extranodal involvement. Diagnosis was performed by superficial lymph node biopsy. No immunodeficiency was found. The patients received no therapy and a complete spontaneous resolution was seen after a few months in the two cases. The clinical presentation, histologic characteristics, pathogenesis and treatment of the Destombes-Rosai-Dorfman's syndrome are discussed.

  17. Histiocytosis X | Mohammed | Annals of African Medicine

    African Journals Online (AJOL)

    Histiocytosis X (Langerhan's cell granulomatosis) is a disease of unknown aetiology, but viral origin has been implicated.1 It is a clinicopathologic entity characterized by proliferation of Langerhan's cell (which are not primarily phagocytic) in an appropriate milieu.2 ...

  18. Cerebellar leukoencephalopathy: most likely histiocytosis-related

    NARCIS (Netherlands)

    van der Knaap, M.S.; Arts, W.F.M.; Garbern, J.Y.; Hedlund, G.; Winkler, F.; Barbosa, C.; King, M.D.; Bjornstad, A.; Hussain, N.; Beyer, M.K.; Gomez, C.; Patterson, M.C.; Grattan-Smith, P.; Timmons, M.; van der Valk, P.

    2008-01-01

    Background: Histiocytosis, both Langerhans and non-Langerhans cell type, can be associated with cerebellar white matter abnormalities, thought to be paraneoplastic. The associated clinical picture consists of ataxia, spasticity, and cognitive decline. Hormonal dysfunction is frequent. MRI shows

  19. Clinical features and treatment outcomes of Langerhans cell histiocytosis: a nationwide survey from Korea histiocytosis working party.

    Science.gov (United States)

    Kim, Bo Eun; Koh, Kyung-Nam; Suh, Jin Kyung; Im, Ho Joon; Song, Joon Sup; Lee, Ji Won; Kang, Hyoung Jin; Park, Kyung Duck; Shin, Hee Young; Choi, Hyoung Soo; Lee, Soo Hyun; Yoo, Keon Hee; Sung, Ki Woong; Koo, Hong Hoe; Jung, Hye Lim; Chung, Nak-Gyun; Cho, Bin; Kim, Hack Ki; Lyu, Chuhl Joo; Baek, Hee Jo; Kook, Hoon; Park, Jun Eun; Park, Hyeon Jin; Park, Byung-Kiu; Yoo, Eun Sun; Ryu, Kyung Ha; Lee, Kun Soo; Kim, Heung Sik; Lee, Jae Min; Park, Eun Sil; Yoon, Hoi Soo; Lee, Kwang Chul; Lee, Mee Jeong; Lim, Young Tak; Kim, Hwang Min; Park, Sang Kyu; Park, Jeong-A; Kim, Soon Ki; Park, Meerim; Lim, Yeon-Jung; Lee, Young Ho; Seo, Jong Jin

    2014-03-01

    A nationwide survey was conducted to clarify the clinical features and outcomes of Korean children with Langerhans cell histiocytosis (LCH). Korea Histiocytosis Working Party analyzed the data of 603 patients who were diagnosed with LCH between 1986 and 2010 from 28 institutions in Korea. Median age at diagnosis was 65 months (range, 0 to 276 mo). Bone was the most frequently affected organ (79.6%) followed by skin (19.2%). Initially, 419 patients (69.5%) had single-system involvement (SS), 85 (14.1%) with multisystem (MS) disease without risk organ involvement (MS-RO), and 99 (16.4%) multisystem disease with risk organ involvement (MS-RO). The 5-year overall survival (OS) rates in the SS, MS-RO, and MS-RO groups were 99.8%, 98.4%, and 77.0%, respectively (P<0.001), and the 5-year reactivation rates were 17.9%, 33.5%, and 34.3%, respectively (P<0.001). The OS rate was lower in patients with RO involvement (P=0.025) and lack of response to initial treatment (P=0.001). MS involvement (P=0.036) was an independent risk factor for reactivation. Permanent consequences were documented in 99 patients (16.4%). Reactivation of disease, MS involvement, and age at diagnosis ≤ 2 years were associated with higher incidence of permanent consequences. This study emphasized that further efforts are required to improve survival of MS-RO patients and reduce reactivation in younger patients with MS involvement.

  20. Perianal Ulcer and Diabetes Insipidus: A Rare Presentation of Langerhans Cell Histiocytosis in an Adult Male

    Directory of Open Access Journals (Sweden)

    Ng Chong Beng

    2017-07-01

    Full Text Available Langerhans cell histiocytosis (LCH, previously known as Histiocystosis X, is a rare idiopathic disease caused by a build-up of langerhan cells in the body. LCH has a heterogenous form of clinical presentation and presents with varying degree of severity. The severity often depends on the disease burden. LCH can manifest as a unifocal disease, multifocal-unisystem or multifocal-multisystem disease with the latter being more fatal than the rest. Risk organ involvement confers a grim prognosis. Perianal lesions are extremely rare skin manifestation of LCH with only few cases reported in the past. Vinca-alkaloids and steroid therapy have been shown to be effective in a large percentage of those presenting with LCH. This case report highlights the diagnosis and management of LCH presenting as a painful perianal ulcer in the background of newly diagnosed diabetes insipidus.

  1. Health-related quality of life, cognitive functioning and behaviour problems in children with Langerhans cell histiocytosis

    NARCIS (Netherlands)

    Vrijmoet-Wiersma, C. M. Jantien; Kooloos, Vicky M.; Koopman, Hendrik M.; Kolk, Annemarie M.; van der Laan, Ingrid; Grootenhuis, Martha A.; Egeler, R. Maarten

    2009-01-01

    BACKGROUND: This study was designed to evaluate generic and disease-specific health-related quality of life (HRQoL), cognitive functioning and behaviour problems of children with Langerhans Cell Histiocytosis (LCH). Furthermore, we investigated which medical determinants and social demographic

  2. Pulmonary Langerhans cell histiocytosis with cervical lymph node involvement, and coexistence with pulmonary tuberculosis and right pneumothorax: a case report and review of literature.

    Science.gov (United States)

    Gao, Limin; Li, Huifang; Li, Gandi; Liu, Weiping; Li, Jinnan; Zhang, Wenyan

    2015-01-01

    We report an uncommon 22-year-old male Pulmonary Langerhans Cell Histiocytosis (PLCH) case which co-existed with pulmonary tuberculosis (TB). Unlike the common PLCH cases, this PLCH case has cervical lymph node involvement and right pneumothorax. The diagnosis was established by the imaging of lung and the biopsies of the lung and left neck lymph node. Imaging of the chest showed characteristic small nodules and thin-walled cysts and right pneumothorax. The LCH cells in the lung and left neck lymph node were characterized by large convoluted nuclei with cerebriform indentations of the nuclear envelope and longitudinal grooves. The nuclei contained small eosinophilic nucleoli and moderate amount cytoplasm. Immunohistochemically, the histiocytoid cells were positive for Langerin, CD1a and S-100. Acid-fast bacilli were found in sputum and lung biopsy tissue. To the best of our knowledge, this is the first case of PLCH with cervical lymph node involvement, and coexisted with pulmonary tuberculosis, right pneumothorax. A contribution of this case and review three of the five cases of PLCH with extrapulmonary involvement to lymph nodes resolved spontaneously after smoking cessation constitute a novel addition that it is inappropriate to regard pulmonary/nodal LCH as multi-organ or disseminated disease, and the treatment methods are the same whether the PLCH patient with lymph node involvement or not.

  3. Langerhans′ cell histiocytosis involving posterior elements of the dorsal spine: An unusual cause of extradural spinal mass in an adult

    Directory of Open Access Journals (Sweden)

    Devendra K Tyagi

    2011-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is a clonal proliferation of Langerhans cells occurring as an isolated lesion or as part of a systemic proliferation. It is commoner in children younger than 10 years of age with sparing of the posterior elements in more than 95% of cases. We describe a case of LCH in an adult female presenting with paraplegia. MRI revealed a well-defined extradural contrast enhancing mass at D2-D4 vertebral level involving the posterior elements of spine. D2-5 laminectomy with excision of lesion was performed which lead to marked improvement of patients neurological status. Histopathology was suggestive of eosinophilic granuloma. We describe the case, discuss its uniqueness and review the literature on this rare tumor presentation.

  4. Pulmonary histiocytosis X - imaging aspects of pulmonary involvement

    International Nuclear Information System (INIS)

    Sabedotti, Ismail Fernando; Maeda, Lucimara; Ferreira, Daniel Miranda; Montandon, Cristiano; Marins, Jose Luiz C.

    1999-01-01

    Pulmonary histiocytosis X is an idiopathic disease which is and uncommon but important cause of pulmonary fibrosis in young adults. Chest radiographs and high resolution computed tomographic (HRCT) scans of the lungs of 7 patients diagnosed as pulmonary histiocytosis X were examined retrospectively. The authors reviewed the pathologic, clinical and radiographic features of pulmonary histiocytosis X, focusing on differential diagnosis and disease progression. Pulmonary histiocytosis X can be suspected on the basis of chest radiographic findings; predominantly upper lobe nodules and cysts present an increased sensitivity and are virtually pathognomonic of this disorder. Chest HRCT allows good assessment of the evolution of pulmonary histiocytosis X and is also valuable in distinguishing histiocytosis from other disorders that produces nodules or cysts. (author)

  5. BISPHOSPHONATES IN LANGERHANS CELL HISTIOCYTOSIS: AN INTERNATIONAL RETROSPECTIVE CASE SERIES

    Directory of Open Access Journals (Sweden)

    Deepak Chellapandian

    2016-07-01

    Full Text Available Background: Bone is the most common organ of involvement in patients with Langerhans cell histiocytosis (LCH, which is often painful and associated with significant morbidity from pathological fractures. Current first-line treatments include chemotherapy and steroids that are effective but often associated with adverse effects, whereas the disease may reactivate despite an initial response to first-line agents. Bisphosphonates are osteoclast inhibitors that have shown to be helpful in treating bone lesions of LCH. To date, there are no large international studies to describe their role in treating bone lesions of LCH. Method: We conducted a multicenter retrospective review of 13 patients with histologically proven LCH, who had received bisphosphonates either at diagnosis or at disease reactivation. Results: Ten patients (77% had a single system bone disease, and 3 (23% had bone lesions as part of multisystem disease. Median follow-up time post-bisphosphonate therapy was 4.6 years (range, 0.8 to 8.2 years. Treatment with bisphosphonates was associated with significant pain relief in almost all patients. Twelve  (92% achieved resolution of active bone lesions, and 10 out of them had no active disease for a median of 3.5 years (range, 0.8 to 5 years. One patient did not respond. No major adverse effects were reported in this series.  Conclusion: Bisphosphonates are well-tolerated drugs that can significantly improve bone pain and induce remission in active bone LCH. Future prospective studies evaluating the role of bisphosphonates in LCH are warranted.

  6. Polyclonal T-cells express CD1a in Langerhans cell histiocytosis (LCH lesions.

    Directory of Open Access Journals (Sweden)

    Jennifer A West

    Full Text Available Langerhans cell histiocytosis (LCH is a complex and poorly understood disorder that has characteristics of both inflammatory and neoplastic disease. By using eight-colour flow cytometry, we have identified a previously unreported population of CD1a(+/CD3(+ T-cells in LCH lesions. The expression of CD1a is regarded as a hallmark of this disease; however, it has always been presumed that it was only expressed by pathogenic Langerhans cells (LCs. We have now detected CD1a expression by a range of T-cell subsets within all of the LCH lesions that were examined, establishing that CD1a expression in these lesions is no longer restricted to pathogenic LCs. The presence of CD1a(+ T-cells in all of the LCH lesions that we have studied to date warrants further investigation into their biological function to determine whether these cells are important in the pathogenesis of LCH.

  7. Sinus Histiocytosis with Massive Lymphadenopathy | Sinclair-Smith ...

    African Journals Online (AJOL)

    Two cases of a recently described entity, 'sinus histiocytosis with massive lymphadenopathy,' occurring in Black males, are reported. Prominent cervical adenopathy was the main presenting feature in both. Histologically, these nodes were characterised by pronounced proliferation of sinus histiocytes which showed ...

  8. Pulmonary fibrosis caused by histiocytosis X

    International Nuclear Information System (INIS)

    Reinbold, W.D.; Seemann, W.R.; Ruehle, K.H.

    1984-01-01

    This paper reports on a 23-year-old man suffering from pulmonary fibrosis caused by localised histiocytosis X. Although chest film examination shows diffuse pulmonary involvement the patient is asymptomatic. No other organ systems are involved. The different forms and prognosis of histiocytosis X are discussed. (orig.)

  9. An Aggressive Form of Langerhan Cell Histiocytosis in an Adult: Therapeutic Challenges

    Directory of Open Access Journals (Sweden)

    Karan Seegobin

    2017-01-01

    Full Text Available Langerhans cell histiocytosis (LCH is rare in adults. Regular follow-up is mandatory due to reoccurrence. A 35-year-old male with an incidental left iliac bone lesion was diagnosed with LCH. He later became symptomatic with hip pain and spread of the disease. Despite excision of the symptomatic iliac lesion, he had progression while on cytarabine and nivolumab, evidenced by increased bone pain and involvement of other bones on imaging. He underwent excision of the jaw lesion followed by vinblastine; he was pain free and had stable disease on PET imaging after 3 months. LCH is an uncommon neoplasia. Treatment is reserved for symptomatic patients while asymptomatic patients are observed. Follow-up is imperative due to the risk of reoccurrence. Despite surgical treatment together with one of the front-line agents for refractory disease, in this case cytarabine, he still had progression of the disease. Furthermore, the trial of nivolumab was of no benefit. This case highlights good response to vinblastine which is previously reported to have good success. No trials are published, and the optimal strategy has yet to be defined. LCH with multiple bony involvement can be aggressive and therapeutically challenging.

  10. Langerhans Cell Histiocytosis of the Cranial Base: Is Low-Dose Radiotherapy Effective?

    Directory of Open Access Journals (Sweden)

    Andreas Meyer

    2012-01-01

    Full Text Available Introduction. Langerhans cell histiocytosis (LCH is a rare disease of unknown etiology with different clinical features. A standardised treatment has not been established so far. Case Report. We report a case of a 28-year-old patient who initially presented with hypesthesia of the fifth cranial nerve and pain of the left ear. Diagnosis showed a tumour localised in the cranial base with a maximum diameter of 4.1 cm. The diagnosis of LCH was confirmed histologically by biopsy. Diagnostic workup verified the cranial lesion as the sole manifestation of LCH. A total dose of 9 Gy (single dose 1.8 Gy was delivered. The symptoms dissolved completely within 6 months after radiation; repeated CT and MRI scans revealed a reduction in size of the lesion and a remineralisation of the bone. After a followup of 13 years the patient remains free of symptoms without relapse or any side effects from therapy. Discussion. Due to the indolent course of the disease with a high rate of spontaneous remissions the choice of treatment strongly depends on the individual clinical situation. In the presented case low-dose radiotherapy was sufficient to obtain long-term local control in a region with critical structures and tissues.

  11. Case report 457: Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) presenting as lesion in the sacrum

    Energy Technology Data Exchange (ETDEWEB)

    Unni, K K

    1988-03-01

    A 19-year-old women presented with a lytic lesion in the sacrum, associated with pain. Sinus histiocytosis (Rosai-Dorfman disease) was not diagnosed correctly until a biopsy specimen of an enlarged cervical lymph node showed the changes typical of sinus histiocytosis with massive lymphadenopathy. The disease was persistent in the patient and eventually involved the sternum. The patient improved with steroid therapy. The clinical, radiological and pathological aspects of this entity were discussed. It was stressed that radiologists, orthopedic surgeons and pathologists must be aware that sinus histiocytosis with massive lymphadenopathy may manifest initially as a bone 'tumor'.

  12. Concomitant occurrence of sinus histiocytosis with massive lymphadenopathy and nodal marginal zone lymphoma.

    Science.gov (United States)

    Pang, Changlee S; Grier, David D; Beaty, Michael W

    2011-03-01

    Sinus histiocytosis with massive lymphadenopathy (SHML), also known as Rosai-Dorfman disease, is a rare self-limiting disorder of histiocytes with unknown etiology. Sinus histiocytosis with massive lymphadenopathy is most common in children and young adults and is characterized by painless lymphadenopathy. Histologically there is a proliferation of sinus histiocytes with lymphophagocytosis or emperipolesis. On rare occasions, SHML has been associated with lymphoma, usually involving different anatomic sites and developing at different times. We report a case of concomitant SHML and nodal marginal zone lymphoma involving the same lymph node without involvement of other nodal or extranodal sites. The presence of concomitant SHML within the lymph node involved by nodal marginal zone lymphoma may represent the responsiveness of SHML histiocytes to B-cell-derived cytokines in lymphoproliferative disorders. To our knowledge, this is the first description of concomitant occurrence of SHML and nodal marginal zone lymphoma.

  13. CT findings of orbital langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Yi, Gina; Yoon, Hye Kyung; Han, Bokyung Kim; Kim, Kyeong Ah; Choo, In Wook

    2000-01-01

    To evaluate the CT findings in patients with Langerhans cell histiocytosis (LCH) involving the orbit. Orbital CT scans of six children with pathologically proven LCH were retrospectively analyzed. Follow-up CT (n=3D5) and MR (n=3D1) imaging findings were also reviewed. Initial CT scans revealed varying degree of bone destruction with soft-tissue masses, and on nonenhanced images the mean attenuation value was 44 Hounsfield units (HU). All masses showed mild to moderate enhancement with a mean attenuation value of 74 HU. The bony margins abutting onto soft tissue masses were irregular but clearly demarcated. No evidence of calcification or periosteal reaction was noted. Suprasellar mass and rib involvement was noted in one patient and hepatosplenomegaly in two. Follow-up CT and MR images showed that the soft tissue masses were almost completely resolved, with bone remodeling and reossification. A soft tissue mass with irregular but clearly demarcated bone destruction is thought to be a characteristic finding of LCH involving the orbit. Follow-up images after treatment showed bone remodeling and reossification. (author)

  14. Indeterminate cell histiocytosis in a pediatric patient: successful treatment with thalidomide.

    Science.gov (United States)

    Tóth, Béla; Katona, Mária; Hársing, Judit; Szepesi, Agota; Kárpáti, Sarolta

    2012-04-01

    The 15-year-old male patient presented several 2-6 mm large livid reddish-yellowish, shiny, compact papules on the head, trunk and extremities, which had developed within the last 4 months. Histology showed normal epidermis with dense dermal infiltrate of histiocytes accompanied by few eosinophils, Touton or foamy giant cells. The histiocytes were S100 positive, CD1a negative and did not contain Birbeck granules ultrastructurally. Chest X ray, EEG, skull MRI did not show pathology. Opthalmology, neurology, oto-rhino-laryngology did not reveal alterations. Based upon the clinical symptoms and the histopathology, the diagnosis of indeterminate cell histiocytosis was confirmed. Cryotherapy and cauterization did not stop the progression of the disease, however, under thalidomide treatment no new symptoms developed and the lesions healed with pigmentation.

  15. Whole-body MRI in children with Langerhans cell histiocytosis for the evaluation of the skeletal system

    International Nuclear Information System (INIS)

    Steinborn, M.; Woertler, K.; Rummeny, E.J.; Nathrath, M.; Schoeniger, M.; Hahn, H.

    2008-01-01

    Purpose: the usefulness of whole-body MRI (WB-MRI) for the detection of skeletal lesions in patients with Langerhans cell histiocytosis should be documented on the basis of case presentations. Materials and methods: in six patients with histologically proven Langerhans cell histiocytosis, 14 WB-MRI examinations were performed to evaluate the skeletal system within disease staging (6 primary, 8 follow-up examinations). The examinations were performed on a 1.5 Tesla, 32-channel whole-body scanner. The examination protocol consisted of T1-weighted and STIR sequences in coronal and sagittal orientation. For comparison, radiographs of the initial skeletal lesions and those that were additionally detected on WB-MRI were available. Results: in 4 patients no additional skeletal lesions were found on WB-MRI besides the initial lesion leading to the diagnosis of unifocal single system disease. In 2 patients WB-MRI was able to identify additional skeletal lesions. In a 5 1/2 year-old boy with the primary lesion located in the cervical spine, a second lesion was detected in the lumbar spine on the initial scan and in the skull and proximal femur during follow-up examination. In a 12 year-old girl with a primary lesion of the thoracic spine, WB-MRI diagnosed additional lesions in the pelvic bone and the tibia. In both patients the diagnosis of multifocal skeletal involvement led to chemotherapy. During follow-up examination, the healing response under therapy could be demonstrated. Comparison with conventional imaging showed that especially lesions located in the spine or the pelvis were not detectable on radiographs even when knowing the MR results. (orig.)

  16. Needle aspiration biopsy in the diagnosis of lytic bone lesions in histiocytosis X, Ewing's sarcoma and neuroblastoma

    International Nuclear Information System (INIS)

    Thommesen, P.; Frederiksen, P.; Loewhagen, T.; Willems, J.S.

    1978-01-01

    Cytologic smears obtained by needle aspiration biopsy of lytic bone lesions in 15 patients with histiocytosis X, Ewing's sarcoma and neuroblastoma were reviewed. After conventional staining, histiocytosis X could be diagnosed and differentiated from small cell tumours such as Ewing's sarcoma and neuroblastoma. The need for sampling material for cytochemical and ultrastructural analysis of these small cell tumours by needle aspiration is emphasized. (Auth.)

  17. The value of bone scintigraphy in diagnosing of langerhans cells histiocytosis

    International Nuclear Information System (INIS)

    Shao Hong; Shi Meihua; Wu Jinchuan

    2004-01-01

    Purpose: To investigate the clinical value of bone scintigraphy in the diagnosis of Langerhans cells histiocytosis (LCH). Methods: Whole-body bone scintigraphy was performed in 23 patients, 16 males and 7 females, average age was 4.5+1.4, with histologic diagnosis of LCH. The clinical presentation varied from local mass (10 cases), tenderness (12 cases), othemorrhea (1 case), limb fatigue (3 cases), restriction of spinal activity (2 cases) to irregular fever, cough and rash (4 cases). 99Tcm-MDP was injected intravenously , the dose modified as age, according to the formula of [ adult dosage x [(age+l)/(age+7)] ] , and sedation was given to the young children who could not cooperate well. After 3 hours of administration whole-body bone scan was performed. Images were acquired using GE Millennium SPR, with a high-resolution low-energy collimator, 8 min/m sweep speed, matrix 1024 x 256, zoom 1. Results: 19 of 23 patients (82.6%) showed positive in their bone scan. 8 cases with cranial abnormal uptake, some of them exhibited peripheral high and central low uptake; clavicle, rib and pelvis involvements were 3 respectively; 2 cases in upper limb, 4 in lower limb, most lesions of limb were located in the diaphysis; spinal abnormal uptakes were seen in 5 patients, and 1 child had photon deficient area in the lower of sternium. Among 19 positive scintigram, single bone lesion was shown in 9 patients (47.3%), the most common lesion located in cranium (5 cases). Conclusion: Some characters of bone scintigraphy in LCH are useful in the diagnosis of this disease, and accompanied with comprehensive sensation of clinical manifestation of LCH, the diagnostic accuracy will be promoted. (authors)

  18. [Neuroimaging of Langerhans cell histiocytosis in the central nervous system of children].

    Science.gov (United States)

    De La Hoz Polo, M; Rebollo Polo, M; Fons Estupiña, C; Muchart López, J; Cruz Martinez, O

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare disease characterized by the accumulation within tissues of anomalous dendritic cells similar to Langerhans cells. The clinical presentation varies, ranging from the appearance of a single bone lesion to multisystemic involvement. Central nervous system (CNS) involvement, manifesting as diabetes insipidus secondary to pituitary involvement, has been known since the original description of the disease. Two types of CNS lesions are currently differentiated. The first, pseudotumoral lesions with infiltration by Langerhans cells, most commonly manifests as pituitary infiltration. The second, described more recently, consists of neurodegenerative lesions of the CNS associated with neurologic deterioration. This second type of lesion constitutes a complication of the disease; however, there is no consensus about the cause of this complication. Our objective was to describe the radiologic manifestations of LCH in the CNS in pediatric patients. Copyright © 2013 SERAM. Published by Elsevier España, S.L.U. All rights reserved.

  19. Management and Outcome of Patients With Langerhans Cell Histiocytosis and Single-Bone CNS-Risk Lesions: A Multi-Institutional Retrospective Study

    NARCIS (Netherlands)

    Chellapandian, Deepak; Shaikh, Furqan; van den Bos, Cor; Somers, Gino R.; Astigarraga, Itziar; Jubran, Rima; Degar, Barbara; Carret, Anne-Sophie; Mandel, Karen; Belletrutti, Mark; Dix, David; Visser, Johannes; Abuhadra, Nour; Chang, Tiffany; Rollins, Barret; Whitlock, James; Weitzman, Sheila; Abla, Oussama

    2015-01-01

    Children with Langerhans cell histiocytosis (LCH) and single-bone CNS-risk lesions have been reported to be at increased risk of diabetes insipidus (DI), central nervous system neurodegeneration (CNS-ND), and recurrence of disease. However, it is unknown whether the addition of chemotherapy or

  20. Langerhans cell histiocytosis or tuberculosis on a medieval child (Oppidum de la Granède, Millau, France - 10th-11th centuries AD).

    Science.gov (United States)

    Colombo, Antony; Saint-Pierre, Christophe; Naji, Stephan; Panuel, Michel; Coqueugniot, Hélène; Dutour, Olivier

    2015-06-01

    In 2008, a skeleton of a 1 - 2.5-year-old child radiocarbon dated from the 10th - 11th century AD was discovered on the oppidum of La Granède (Millau, France). It presents multiple cranial osteolytic lesions having punched-out or geographical map-like aspects associated with sequestrum and costal osteitis. A multi 3D digital approach (CT, μCT and virtual reconstruction) enabled us to refine the description and identify the diploic origin of the lytic process. Furthermore, precise observation of the extent of the lesions and associated reorganization of the skeletal micro-structure were possible. From these convergent pieces of evidence, the differential diagnosis led to three possibilities: Langerhans cell histiocytosis, tuberculosis, or Langerhans cell histiocytosis and tuberculosis. Copyright © 2015 Elsevier Ltd. All rights reserved.

  1. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): a clinicoradiological profile of three cases including two with skeletal disease

    International Nuclear Information System (INIS)

    Gupta, Priya; Babyn, Paul

    2008-01-01

    Originally described as sinus histiocytosis with massive lymphadenopathy, Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder with a distinctive microscopic appearance. A rare entity, RDD is often under-diagnosed because of a low index of suspicion by both radiologist and pathologist. Through this article, we wish to apprise radiologists of the spectrum of disease that can be encountered in this disorder. RDD can mimic other common childhood skeletal diseases including benign Langerhans cell histiocytosis and lymphoma. The clinical and radiological manifestations of RDD vary depending upon organ involvement, and its imaging features are often confused with those of other disorders. RDD should be considered in the differential diagnosis of unifocal and multifocal skeletal involvement caused by granulomatous diseases, infections, pseudogranulomatous lesions and malignancy. As long-term outcome is usually good, a conservative approach is justified in most cases. Contrasted with its typical appearance, presenting with bilateral symmetrical cervical adenopathy (as shown in one patient), we also report extranodal involvement of bone in two patients. Extranodal disease occurs along with concomitant nodal disease in about 43% of patients. In 23% of patients, isolated extranodal RDD can be seen, most commonly in the head and neck. In two of our patients, we observed extranodal involvement with skeletal involvement away from the head and neck not associated with lymphadenopathy. Skeletal involvement in RDD without lymphadenopathy is rare, occurring only in 2% of all the patients reported to date. (orig.)

  2. Histiocitose das células de Langerhans na região anogenital Langerhans cell histiocytosis of the anogenital region

    Directory of Open Access Journals (Sweden)

    M. S. Neto

    1998-12-01

    Full Text Available A Histiocitose das células de Langerhans (HCL acometendo o trato genital tem uma incidência rara com apenas 48 casos relatados na literatura1-5 . Considerando somente as lesões da região anogenital, esse número cai para dois casos descritos1,2. Reportamos o 3º caso de HCL anogenital em uma paciente de 31 anos, branca, cuja comprovação diagnóstica foi feita através de microscopia eletrônica. O tratamento realizado foi quimioterapia sistêmica e excisão cirúrgica local.The Langerhans cell histiocytosis (LCH of the genital tract is rare, with only 48 cases related in the literature1-5. There were reported only 2 cases in the anogenital region1,2. We reported the third case of LCH in the anogenital region; patient was female, 31 years-old, caucasian and the diagnosis was confirmed by electron microscopic magnification. The treatment was local surgical excision and systemic chemotherapy.

  3. Central diabetes insipidus as a very late relapse limited to the pituitary stalk in Langerhans cell histiocytosis.

    Science.gov (United States)

    Nakagawa, Shunsuke; Shinkoda, Yuichi; Hazeki, Daisuke; Imamura, Mari; Okamoto, Yasuhiro; Kawakami, Kiyoshi; Kawano, Yoshifumi

    2016-07-01

    Central diabetes insipidus (CDI) and relapse are frequently seen in multifocal Langerhans cell histiocytosis (LCH). We present two females with multifocal LCH who developed CDI 9 and 5 years after the initial diagnosis, respectively, as a relapse limited to the pituitary stalk. Combination chemotherapy with cytarabine reduced the mass in the pituitary stalk. Although CDI did not improve, there has been no anterior pituitary hormone deficiency (APHD), neurodegenerative disease in the central nervous system (ND-CNS) or additional relapse for 2 years after therapy. It was difficult to predict the development of CDI in these cases. CDI might develop very late in patients with multifocal LCH, and therefore strict follow-up is necessary, especially with regard to symptoms of CDI such as polydipsia and polyuria. For new-onset CDI with LCH, chemotherapy with cytarabine might be useful for preventing APHD and ND-CNS.

  4. Solitary Langerhans cell histiocytosis of the occipital condyle: a case report and review of the literature.

    Science.gov (United States)

    Teranishi, Yu; Shin, Masahiro; Yoshino, Masanori; Saito, Nobuhito

    2016-02-01

    Despite the recent advent of various radiographic imaging techniques, it is still very difficult to correctly distinguish a pediatric osteolytic lesion in the occipital condyle, which makes it further complicated to decide on the necessity of and the adequate timing for radical resection and craniocervical fusions. To establish a legitimate therapeutic strategy for this deep-seated lesion, surgical biopsy is a reasonable choice for first-line intervention. The choice of surgical approach becomes very important because a sufficient amount of histological specimen must be obtained to confirm the diagnosis but, ideally, the residual bony structures and the muscular structures should be preserved so as not to increase craniocervical instability. In this report, we present our experience with a case of solitary Langerhans cell histiocytosis (LCH) involving the occipital condyle that was successfully treated with minimally invasive surgical biopsy with a far lateral condylar approach supported by preoperative 3D computer graphic simulation. An 8-year-old girl presented with neck pain. Magnetic resonance imaging and computed tomography (CT) revealed an osteolytic lesion of the left occipital condyle. At surgery, the patient was placed in the prone position. A 3-cm skin incision was made in the posterior auricular region, and the sternocleidomastoid and splenius capitis muscles were dissected in the middle of the muscle bundle along the direction of the muscle fiber. Under a navigation system, we approached the occipital condyle through the space between the longissimus capitis muscle and the posterior belly of the digastric muscle and lateral to the superior oblique muscle, verifying each muscle at each depth of the surgical field and, finally, obtained sufficient surgical specimen. After the biopsy, her craniocervical instability had not worsened, and chemotherapy was performed. Twelve weeks after chemotherapy, her neck pain had gradually disappeared along with her

  5. LANGERHANS CELL HISTIOCYTOSIS OF THE SCAPULA - DIAGNOSIS & TREATMENT OPTIONS

    Directory of Open Access Journals (Sweden)

    ROHIT PANDEY

    Full Text Available ABSTRACT Langerhans cell histiocytosis (LCH is characterised by an abnormal histiocytic accumulation in tissues such as the lung, spleen, bone marrow, skin, central nervous system, liver and lymph nodes, causing focal or systemic effects. No specific clinical & radiographic presentation of LCH is described in literature. This poses a diagnostic dilemma for surgeons. The scapula is the site of 3% of bone tumours, while for LCH it is the least common site. In a 10-year-old boy with isolated lesion of the scapula with no other systemic involvement, and no specific finding in MRI or CT scan of scapula, diagnosis was confirmed on biopsy. Division into single and multi-system disease is paramount in treatment, given that it is a single system disease. The patient improved clinically on follow-up of 2 years. The scapula is one of the rarest site of LCH, and because various lesions mimic each other, a biopsy is always required, with immunohistochemistry for CD68 & S-100. This was only a single system disease, so conservative management was performed, and the patent improved clinically.

  6. Histiocytosis in a 7 year old boy, a diagnostic dilemma ...

    African Journals Online (AJOL)

    by the infiltration/accumulation of histiocytic cells in affected tissues. Their mode of clinical presentation varies greatly and can represent a diagnostic challenge in our environment where there is a paucity of diagnostic facilities. This report is on a 7 year old boy with probable Histiocytosis who initially presented with signs ...

  7. [Sinus histiocytosis (Destombes-Rosai-Dorfman disease) revealed by extranodal spinal involvement].

    Science.gov (United States)

    Bernard, F; Sarran, N; Serre, I; Baldet, P; Callamand, P; Margueritte, G; Astruc, J

    1999-02-01

    Sinus histiocytosis with massive cervical lymphadenopathy (Rosai-Dorfman disease) is a non-neoplastic lymphoproliferative disorder. Extranodal involvement, especially of the nervous system, is unusual. We report a case revealed by neurological symptoms. A 10-year-old girl presented with paraparesis due to a dural extramedullary mass on magnetic resonance imaging. Massive cervical lymphadenopathy appeared secondarily. Radiological investigations showed mediastinal, paranasal sinus and lower eyelid involvement. The diagnosis of Rosai-Dorfman disease was established histologically and by immunohistochemical studies of nodal lesions by the demonstration of characteristic sinus histiocytosis with sheets of S-100 protein and CD-68 positive large histiocytes displaying lymphocyte phagocytosis. A dramatic response occurred with complete resolution of all clinical findings after treatment with corticosteroids and etoposide, although neurological lesions were unchanged on magnetic resonance imaging. Despite its rarity, this case underlines the unknown pathogenesis of this disease (immune dysfunction?) and the difficulties of treatment (choice of chemotherapeutic agents, duration).

  8. Intestinal lymphangiectasia associated with recurrence of histiocytosis X.

    Science.gov (United States)

    Hui, C K

    2011-09-01

    Intestinal lymphangiectasia may occur as a primary congenital disorder or a secondary disorder. Secondary lymphangiectasia could be associated with diseases such as abdominal carcinoma, retroperitoneal fibrosis or chronic pancreatitis. This is the first reported case of intestinal lymphangiectasia associated with recurrent histiocytosis X. This case report illustrates the need for more prospective, well-designed studies to determine the natural history and outcome of intestinal lymphangiectasia in the duodenum. Hopefully, these studies will also help clinicians identify which group of patients with intestinal lymphangiectasia in the duodenum is more likely to have a secondary cause.

  9. Histiocytosis versus (Hand-Schuller-Christian disease) - a case report; Histiocitose versus (sindrome de Hand-Schuller-Christian) - relato de caso

    Energy Technology Data Exchange (ETDEWEB)

    Carneiro Filho, Jose Olimar; Leite, Marta Santos [Hospital Geral de Fortaleza, CE (Brazil). Clinica Radiologica Beroaldo Jurema, Fortaleza, CE (Brazil)]. E-mail: olimar_carneiro@hotmail.com; Andrade Neto, Jose Moacyr [Hospital Universitario Lauro Wanderley, Joao Pessoa, PB (Brazil). Clinica Medica

    2002-04-01

    The authors report a case of a 4-year-old girl with headache, irritability, othorrea and unilateral exophtalmus. Computed tomography and plain films showed large lytic areas in the vault and base of the skull. No other bones were involved. The diagnosis of histiocytosis X (Hand-Schuller-Christian disease) was confirmed by bone marrow biopsy. This paper presents the cytological and radiological findings of this patient as well as a review of the literature. (author)

  10. Pulmonary histiocytosis X - imaging aspects of pulmonary involvement; Histiocitose X - aspectos radiologicos do acometimento pulmonar

    Energy Technology Data Exchange (ETDEWEB)

    Sabedotti, Ismail Fernando; Maeda, Lucimara; Ferreira, Daniel Miranda; Montandon, Cristiano; Marins, Jose Luiz C. [Universidade Estadual de Campinas, SP (Brazil). Faculdade de Ciencias Medicas. Dept. de Radiologia

    1999-06-01

    Pulmonary histiocytosis X is an idiopathic disease which is and uncommon but important cause of pulmonary fibrosis in young adults. Chest radiographs and high resolution computed tomographic (HRCT) scans of the lungs of 7 patients diagnosed as pulmonary histiocytosis X were examined retrospectively. The authors reviewed the pathologic, clinical and radiographic features of pulmonary histiocytosis X, focusing on differential diagnosis and disease progression. Pulmonary histiocytosis X can be suspected on the basis of chest radiographic findings; predominantly upper lobe nodules and cysts present an increased sensitivity and are virtually pathognomonic of this disorder. Chest HRCT allows good assessment of the evolution of pulmonary histiocytosis X and is also valuable in distinguishing histiocytosis from other disorders that produces nodules or cysts. (author)

  11. 18F-fluorodeoxyglucose positron emission tomography/computed tomography for primary thyroid langerhans histiocytosis: A case report and literature review

    International Nuclear Information System (INIS)

    Long, Qi; Shaoyan, Wang; Hui, Wang

    2015-01-01

    Langerhans cell histiocytosis (LCH) is a rare clonal proliferative disease, with an incidence rate of 4.0–5.4/1 million individuals. LCH encompasses a spectrum of disorders with diverse clinical presentations ranging from a single organ to multiple organ involvement. LCH rarely involves the thyroid gland. We presented a case with LCH of thyroid gland. The patient had painless progressive neck enlargement and then diabetes insipidus. Ultrasonic scan and magnetic resonance imaging scan revealed nodular goiter and pituitary stalk enlargement, respectively. Histopathological analysis revealed features of histiocytoid cells. 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography/computed tomography (PET/CT) was performed in order to rule out the presence of whole body infiltration. 18F-FDG PET/CT also demonstrated increased uptake in the thickening pituitary stalk and maxillofacial skin lesion, in addition to the bilateral thyroid nodules, CT showed the left lung nodule and the skull destruction without 18F-FDG uptake. This report emphasizes the role of 18F-FDG PET/CT in multiple organs involvement of patients with LCH

  12. From normal to abnormal MR findings within three weeks in a solitary pelvic Langerhans histiocytosis

    International Nuclear Information System (INIS)

    Hung, Po-Cheng; Wang, Huei-Shyong; Jaing, Tang-Her; Huang, Tsung-Jen; Pang, Leou-Chuan

    2003-01-01

    We present a histologically proven case of pelvic Langerhans histiocytosis (eosinophilic granuloma) which showed abnormality on MR imaging 3 weeks after a normal MR examination mimicking an infection. (orig.)

  13. Adult Multisystem Langerhans Cell Histiocytosis Presenting with Central Diabetes Insipidus Successfully Treated with Chemotherapy

    Directory of Open Access Journals (Sweden)

    Jung-Eun Choi

    2014-09-01

    Full Text Available We report the rare case of an adult who was diagnosed with recurrent multisystem Langerhans cell histiocytosis (LCH involving the pituitary stalk and lung who present with central diabetes insipidus and was successfully treated with systemic steroids and chemotherapy. A 49-year-old man visited our hospital due to symptoms of polydipsia and polyuria that started 1 month prior. Two years prior to presentation, he underwent excision of right 6th and 7th rib lesions for the osteolytic lesion and chest pain, which were later confirmed to be LCH on pathology. After admission, the water deprivation test was done and the result indicated that he had central diabetes insipidus. Sella magnetic resonance imaging showed a mass on the pituitary stalk with loss of normal bright spot at the posterior lobe of the pituitary. Multiple patchy infiltrations were detected in both lung fields by computed tomography (CT. He was diagnosed with recurrent LCH and was subsequently treated with inhaled desmopressin, systemic steroids, vinblastine, and mercaptopurine. The pituitary mass disappeared after two months and both lungs were clear on chest CT after 11 months. Although clinical remission in multisystem LCH in adults is reportedly rare, our case of adult-onset multisystem LCH was treated successfully with systemic chemotherapy using prednisolone, vinblastine, and 6-mercaptopurine, which was well tolerated.

  14. Aspects of pulmonary histiocytosis X on high resolution computed tomography

    International Nuclear Information System (INIS)

    Costa, N.S.S.; Castro Lessa Angela, M.T. de; Angelo Junior, J.R.L.; Silva, F.M.D.; Kavakama, J.; Carvalho, C.R.R. de; Cerri, G.G.

    1995-01-01

    Pulmonary histiocytosis X is a disease that occurs in young adults and presents with nodules and cysts, mainly in upper lobes, with consequent pulmonary fibrosis. These pulmonary changes are virtually pathognomonic findings on high resolution computed tomography, that allows estimate the area of the lung involved and distinguish histiocytosis X from other disorders that also produces nodules and cysts. (author). 10 refs, 2 tabs, 6 figs

  15. Successful treatment with cladribine of Erdheim-Chester disease with orbital and central nervous system involvement developing after treatment of langerhans cell histiocytosis

    Directory of Open Access Journals (Sweden)

    Perić Predrag

    2016-01-01

    Full Text Available Introduction. Erdheim-Chester disease (ECD is a rare, systemic form of non-Langerhans cell histiocytosis of the juvenile xantho-granuloma family with characteristic bilateral symmetrical long bone osteosclerosis, associated with xanthogranulomatous extras-keletal organ involvement. In ECD, central nervous system (CNS and orbital lesions are frequent, and more than half of ECD patients carry the V600E mutation of the proto-oncogene BRAF. The synchronous or metachronous development of ECD and Langerhans cell histiocytosis (LCH in the same patients is rare, and the possible connection between them is still obscure. Cladribine is a purine substrate analogue that is toxic to lymphocytes and monocytes with good hematoencephalic penetration. Case report. We presented a 23-year-old man successfully treated with cladribine due to BRAF V600E-mutation-negative ECD with bilateral orbital and CNS involvement. ECD developed metachronously, 6 years after chemotherapy for multisystem LCH with complete disease remission and remaining central diabetes insipidus. During ECD treatment, the patient received 5 single-agent chemotherapy courses of cladribine (5 mg/m2 for 5 consecutive days every 4 weeks, with a reduction in dose to 4 mg/m2 in a fifth course, delayed due to severe neutropenia and thoracic dermatomal herpes zoster infection following the fourth course. Radiologic signs of systemic and CNS disease started to resolve 3 months after the end of chemotherapy, and CNS lesions completely resolved within 2 years after the treatment. After 12-year follow-up, there was no recurrence or appearance of new systemic or CNS xanthogranu-lomatous lesions or second malignancies. Conclusion. In accordance with our findings and recommendations provided by other authors, cladribine can be considered an effective alternative treatment for ECD, especially with CNS involvement and BRAF V600E-mutation-negative status, when interferon-α as the first-line therapy fails.

  16. Contribution to the radiological study of the eosinophilic granuloma of the mandible (Unifocal granuloma due to Langherans' cell histiocytosis)

    International Nuclear Information System (INIS)

    Chigi, Gino; Pastremoli, Alessandro; Pisi, Paolo; Pastremoli, Alfredo

    2005-01-01

    Purpose: The radiological diagnosis of osteolytic lesions of the mandible still constitutes a challenge in some pathological conditions in which the clinical data and the case history are relatively uniform and the radiological picture is lacking in any characteristics. Materials and methods: We reviewed the conventional radiograms of six cases of Langherans' cell histiocytosis (LCH) of the mandible examined over the last ten years. The X-ray examinations were performed in the lateral-oblique projection to allow a view of the horizontal portion of the mandible almost completely free of overlapping images of other bone structures. Results: We identified a series of radiological patterns for these reticulo-endotheliopathies capable of causing granuloma formed by polinuclear eosinophils, plasma cells, lymphocytes, and large mononuclear macrophages with granulopexic and phagocytic activity that proliferate in the bone tissue and can be identified as the Langherans' cells of skin, mucosa, periodontal cavities and bone marrow. The granulomatous tissue penetrates the affected organ elements and, in its spread, it compresses, atrophies, and destroyed the damaged tissue, replacing it. The alterations produced by Langherans' cell histiocytic granuloma are most common in the skeletal system affecting, in order of frequency, the skull, the long bones of the limbs, the foot, the ribs and the spine. The oral mucosa is rarely involved. Conclusions: The radiological investigation of unifocal Langherans' cell histiocytic granuloma of the mandible is essential in the study of perimandibular swelling, although diagnosis is based on biopsy alone. In addition to digital or conventional radiography, other useful examinations are bone scintigraphy, Colour Doppler US, MR and CT, which enables a correct localisation necessary for planning the biopsy and treatment [it

  17. Langerhans' cell histiocytosis: pathology, imaging and treatment of skeletal involvement

    Energy Technology Data Exchange (ETDEWEB)

    Azouz, E. Michel [University of Miami, Pediatric Radiology Section, Department of Radiology, Miami, FL (United States); Saigal, Gaurav [McGill University, Department of Medical Imaging, Quebec (Canada); Rodriguez, Maria M. [University of Miami, Department of Pathology, Miami, FL (United States); Podda, Antonello [University of Miami, Division of Pediatric Hematology/Oncology, Miami, FL (United States)

    2005-02-01

    Langerhans' cell histiocytosis (LCH) is manifested in a variety of ways, the most common being the eosinophilic granuloma, a localized, often solitary bone lesion that occurs predominantly in the pediatric age group. The hallmark of LCH is the proliferation and accumulation of a specific histiocyte: the Langerhans' cell. In bone this may cause pain and adjacent soft-tissue swelling, but some lesions are asymptomatic. LCH can involve any bone, but most lesions occur in the skull (especially the calvarium and temporal bones), the pelvis, spine, mandible, ribs, and tubular bones. Imaging diagnosis of the disease in bone is first based on the plain radiographic appearance, which is usually a central destructive, aggressive-looking lesion. In the skull, the lesions develop in the diploic space, are lytic, and their edges may be beveled, scalloped or confluent (geographic), or show a ''button sequestrum.'' Vertebral body involvement usually causes collapse, resulting in vertebra plana. With significant recent improvements in the quality of gamma cameras, imaging techniques, and in studying children, bone scintigraphy at diagnosis and on follow-up usually reveals the sites of active disease, especially when the involvement is polyostotic. CT and MR imaging are very useful in providing detailed cross-sectional anatomic detail of the involved bone, including the bone marrow and the adjacent soft tissues. CT is better suited for demonstrating bone detail and MR imaging for bone marrow and soft-tissue involvement. (orig.)

  18. Localized pulmonary crystal-storing histiocytosis complicating pulmonary mucosa-associated lymphoid tissue lymphoma presenting with multiple mass lesions.

    Science.gov (United States)

    Kokuho, Nariaki; Terasaki, Yasuhiro; Kunugi, Shinobu; Onda, Naomi; Urushiyama, Hirokazu; Terasaki, Mika; Hino, Mitsunori; Gemma, Akihiko; Hatori, Tsutomu; Shimizu, Akira

    2017-07-01

    Crystal-storing histiocytosis (CSH) is an uncommon finding in lymphoplasmacytic disorders that presents histiocytes with abnormal intralysosomal accumulations of immunoglobulin light chains as crystals of unknown etiology. A 38-year-old woman with antiphospholipid syndrome had a surgical lung biopsy because of multiple lung mass lesions. In a right middle lobe lesion, lymphoplasmacytic cells had a monocytoid appearance, destructive lymphoepithelial lesions, and positive immunoglobulin heavy chain (IGH) gene rearrangements. A right upper lobe lesion manifested proliferating rounded histiocytes with abundant, deeply eosinophilic cytoplasm and negative IGH gene rearrangements. Electron microscopy and mass spectrometry revealed a case of pulmonary CSH: abnormal proliferation of the immunoglobulin κ chain of a variable region that may be crystallized within plasma cells and histiocytes. We report a rare case of localized pulmonary CSH complicating pulmonary mucosa-associated lymphoid tissue lymphoma with multiple mass lesions. We demonstrate advances in the understanding of the pathogenesis of CSH by various analyses of these lesions. Copyright © 2017 Elsevier Inc. All rights reserved.

  19. Pulmonary langerhans cell histiocytosis in adults: high-resolution CT - pathology comparisons and evolutional changes at CT

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Hyo Jin; Lee, Ho Yun; Kim, Tae Sung [Sungkyunkwan University School of Medicine, Department of Radiology and Center for Imaging Science, Samsung Medical Center, Seoul (Korea, Republic of); Lee, Kyung Soo [Sungkyunkwan University School of Medicine, Department of Radiology and Center for Imaging Science, Samsung Medical Center, Seoul (Korea, Republic of); Samsung Medical Center, Department of Radiology, Seoul (Korea, Republic of); Johkoh, Takeshi [Kinki Central Hospital of Mutual Aid Association of Public School Teachers, Department of Radiology, Hyoko (Japan); Tomiyama, Noriyuki [Osaka University Graduate School of Medicine, Department of Radiology, Osaka (Japan); Han, Joungho [Sungkyunkwan University School of Medicine, Department of Pathology, Samsung Medical Center, Seoul (Korea, Republic of)

    2011-07-15

    To compare high-resolution (HR) CT and histopathological findings and to evaluate serial CT findings in pulmonary Langerhans cell histiocytosis (PLCH). We reviewed CT of lung lesions in 27 adults (M:F = 20:7, mean age, 41 {+-} 12.3 years) with PLCH. After evaluating lung abnormalities including nodules, micronodules, thick-walled, thin-walled, and bizarre-shaped cysts and reticulation, observers compared CT findings obtained at lung biopsy sites with histopathological findings. The final CT was compared with the initial CT to determine disease extent changes. The most frequently observed patterns of lung abnormalities were micronodules (n = 24, 89%), thick-walled (n = 22, 82%), and thin-walled (n = 22, 82%) cysts. Even thin-walled and bizarre cysts harboured active inflammatory Langerhans cell sheets and eosinophils in their walls. In thin-walled cysts, we noted pericystic inflammatory cell infiltrations along the alveolar walls, as well as pericystic emphysema. Thin-walled or bizarre cysts demonstrated a tendency to coalesce with surrounding cysts via their cystic wall destruction. Fourteen (52%) patients showed improvement and nine (33%) showed progressing disease. More than half of patients with pulmonary PLCH show improvement at follow-up CT. Even thin-walled cysts harbour active inflammatory cells on histopathology and exhibit improvement at follow-up CT. (orig.)

  20. The natural history of skin-limited Langerhans cell histiocytosis: a single-institution experience.

    Science.gov (United States)

    Ehrhardt, Matthew J; Humphrey, Stephen R; Kelly, Michael E; Chiu, Yvonne E; Galbraith, Sheila S

    2014-11-01

    Prior reports of Langerhans cell histiocytosis (LCH) suggest that isolated skin involvement is rare and often progresses to systemic disease. More rapid access to pediatric subspecialty care has likely led to more frequent representation of this condition. The purpose of this study is to characterize the natural history of skin-limited LCH in an era of increased access to pediatric subspecialty care. A retrospective chart review was performed on all patients newly diagnosed with LCH between 2001 and 2012 at the Children's Hospital of Wisconsin. Extensive review of laboratory, physical examination, and imaging reports was performed and data collected for patients with biopsy-proven skin LCH. Sixteen individuals with skin-limited LCH were identified. The median age at onset of skin eruption was birth (range, birth to 6 mo), and median duration of follow-up was 19.5 months (range, 2 wk to 10 y) from diagnosis. One patient (6%) developed pituitary disease and 1 patient (6%) had refractory skin involvement. All others experienced complete resolution. For patients without progressive or refractory disease, resolution of skin findings occurred within 7 months from onset. Progression of skin-limited to multisystem LCH likely may be less frequent than previously described.

  1. Langerhans Cell Histiocytosis—Patient Version

    Science.gov (United States)

    Langerhans cell histiocytosis is a rare blood cancer that forms when a type of white blood cell called Langerhans cells becomes abnormal and grows in different parts of the body. LCH is most common in young children but can occur at any age. Start here to find information on Langerhans cell histiocytosis treatment.

  2. Early Diagnosis and Monitoring of Neurodegenerative Langerhans Cell Histiocytosis.

    Directory of Open Access Journals (Sweden)

    Elena Sieni

    Full Text Available Neurodegenerative Langerhans Cell Histiocytosis (ND-LCH is a rare, unpredictable consequence that may devastate the quality of life of patients cured from LCH. We prospectively applied a multidisciplinary diagnostic work-up to early identify and follow-up patients with ND-LCH, with the ultimate goal of better determining the appropriate time for starting therapy.We studied 27 children and young adults with either ND-LCH verified by structural magnetic resonance imaging (MRI (group 1 or specific risk factors for (diabetes insipidus, craniofacial bone lesions, but no evidence of, neurodegenerative MRI changes (group 2. All patients underwent clinical, neurophysiological and MRI studies.Seventeen patients had MRI alterations typical for ND-LCH. Nine showed neurological impairment but only three were symptomatic; 11 had abnormal somatosensory evoked potentials (SEPs, and five had abnormal brainstem auditory evoked potentials (BAEPs. MR spectroscopy (MRS showed reduced cerebellar NAA/Cr ratio in nine patients. SEPs showed sensitivity, specificity, positive predictive value (PPV and negative predictive value (NPV for predicting ND-LCH of 70.6% (95%CI, 44.0%-89.7%, 100% (69.2%-100%, 100% (73.5%-100%, and 66.7% (38.4%-88.2%, respectively. Repeated investigations in group 1 revealed increasingly abnormal EP parameters, or neurological examination, or both, in nine of fifteen patients while MRI remained unchanged in all but one patient.A targeted MRI study should be performed in all patients with risk factors for ND-LCH for early identification of demyelination. The combined use of SEPs and careful neurological evaluation may represent a valuable, low-cost, well-tolerated and easily available methodology to monitor patients from pre-symptomatic to symptomatic stages. We suggest a multidisciplinary protocol including clinical, MRS, and neurophysiological investigations to identify a population target for future therapeutic trials.

  3. CT and MR findings of langerhans cell histiocytois involving the spleen: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Pyun, Hae Wook; Kim, Mee Eun; Kim, Jang Ho [Fatima Hospital, Taegu (Korea, Republic of)

    2002-02-01

    Langerhans cell histiocytosis (LCH) is systemic disease resulting from the proliferation and dissemination of abnormal histiocytic cells of the Langerhans cell system. Common sites of involvement include the skin, bone, bone marrow, lung, lymph nodes and central nervous system, and the condition manifests in variety of ways. We present the CT and MR findings of a case of LCH involving the spleen, an organ invloved relatively rarely. Post-contrast CT revealed multiple hypodense nodules. T1-weighted MR images of the spleen depicted no definitive lesion, but T2-weighted images showed abnormal low signals scattered throughout this organ. In addition, post-contrast, fat-saturated T1-weighted MR images lesions showed multiple, low-signal-intensity lesions.

  4. {sup 18}F-fluorodeoxyglucose positron emission tomography-computed tomography in the management of adult multisystem Langerhans cell histiocytosis

    Energy Technology Data Exchange (ETDEWEB)

    Obert, Julie [Universite Paris Diderot, Paris (France); Assistance Publique-Hopitaux de Paris, Centre National de Reference de l' Histiocytose Langerhansienne, Service de Pneumologie, Hopital Saint-Louis, Paris (France); Vercellino, Laetitia [Assistance Publique-Hopitaux de Paris, Service de Medecine Nucleaire, Hopital Saint-Louis, Paris (France); Van der Gucht, Axel [Universite Paris Diderot, Paris (France); Assistance Publique-Hopitaux de Paris, Service de Medecine Nucleaire, Hopital Saint-Louis, Paris (France); De Margerie-Mellon, Constance [Universite Paris Diderot, Paris (France); Assistance Publique-Hopitaux de Paris, Service de Radiologie, Hopital Saint-Louis, Paris (France); Bugnet, Emmanuelle; Lorillon, Gwenael [Assistance Publique-Hopitaux de Paris, Centre National de Reference de l' Histiocytose Langerhansienne, Service de Pneumologie, Hopital Saint-Louis, Paris (France); Chevret, Sylvie [Universite Paris Diderot, Paris (France); Assistance Publique-Hopitaux de Paris, Service de Biostatistique et Information Medicale, Hopital Saint-Louis, Paris (France); Biostatistics and Clinical Epidemiology Research Team, U1153 CRESS, Paris (France); Tazi, Abdellatif [Universite Paris Diderot, Paris (France); Assistance Publique-Hopitaux de Paris, Centre National de Reference de l' Histiocytose Langerhansienne, Service de Pneumologie, Hopital Saint-Louis, Paris (France); Biostatistics and Clinical Epidemiology Research Team, U1153 CRESS, Paris (France)

    2017-04-15

    The standard evaluation of multisystem Langerhans cell histiocytosis (LCH) includes a clinical evaluation, laboratory tests and a skeleton/skull X-ray survey, with chest high-resolution computed tomography (HRCT) in the case of pulmonary involvement. Preliminary reports suggest that {sup 18}F-fluorodeoxyglucose positron emission tomography-computed tomography ({sup 18}F-FDG PET-CT) may be useful for evaluating patients with LCH. Fourteen consecutive adult patients with multisystem LCH were included in this retrospective study, and were evaluated using standard procedures and {sup 18}F-FDG PET-CT. The two sets of findings were compared both at baseline and during follow-up. Serial HRCT and pulmonary function tests were used to evaluate outcome in patients with lung involvement. At the baseline evaluation, PET-CT identified every LCH localization found with the standard evaluation (except a mild cecum infiltration). PET-CT showed additional lesions in seven patients, mostly involving bones, and differentiated inactive from active lesions. Thyroid {sup 18}F-FDG uptake was identified in three cases. No pituitary stalk {sup 18}F-FDG uptake was observed in patients with pituitary LCH. Only 3/12 (25 %) patients with pulmonary LCH displayed moderate pulmonary {sup 18}F-FDG uptake. During follow-up, variations (≥50 % of maximum standardized uptake) in bone {sup 18}F-FDG uptake intensity were correlated with disease state and response to treatment. The absence of lung {sup 18}F-FDG uptake did not preclude lung function improvement after treatment. Except for cases with pulmonary and pituitary involvement, {sup 18}F-FDG PET-CT could replace the standard evaluation for staging of adult patients with multisystem LCH. Serial PET-CT scans are useful for evaluating treatment responses, particularly in cases with bone LCH involvement. (orig.)

  5. Histiocitose das células de Langerhans: experiência de 16 anos Langerhans cell histiocytosis: a 16-year experience

    Directory of Open Access Journals (Sweden)

    Márcia Kanadani Campos

    2007-02-01

    Langerhans cell histiocytosis followed at Hospital das Clínicas, Universidade Federal de Minas Gerais, Brazil, between 1988 and 2004. RESULTS: Age at diagnosis ranged from 2 months to 16 years (median: 2.5 years. Seventeen children were male. The follow-up period varied from 21 days to 16.2 years (median: 3.4 years. The most common clinical manifestations at diagnosis were osteolytic lesions, enlarged lymph nodes and skin lesions. The overall survival rate for the whole group was 86.1% at 16 years (95%CI 66.6-94.6%. Deaths occurred in patients with multisystem disease and organ dysfunction at diagnosis. Those patients who had a "better" response to treatment in the sixth week were likely to have a significantly higher overall survival rate than those who showed disease progression. Overall survival rate was significantly higher for patients with single-system disease. The disease-free survival rate for the whole group was 30.9% at 16 years (95%CI 15.6-47.5%, and was significantly higher for those with single-system disease. Age groups were not associated with different disease-free survival rates. Diabetes insipidus was the most common sequela. No cases of secondary neoplasms were observed. CONCLUSION: The clinical manifestations of Langerhans cell histiocytosis vary widely, with a high relapse rate and low mortality rate.

  6. Radiographic skeletal survey and radionuclide bone scan in Langerhans cell histiocytosis of bone

    International Nuclear Information System (INIS)

    Nieuwenhuyse, J.P. van; Clapuyt, P.; Malghem, J.; Everarts, P.; Melin, J.; Pauwels, S.; Brichard, B.; Ninane, J.; Vermylen, C.; Cornu, G.

    1996-01-01

    Background. The lack of a consensus in the literature on the imaging strategy in Langerhans cell histiocytosis (LCH) bone lesions in childhood. Objective. To evaluate the relative value of radionuclide bone scan (RBS) and radiographic skeletal survey (RSS) in the detection of LCH bone lesions, both in the initial work-up of the disease and during the follow-up period. Materials and methods. Ten children with bone lesions evaluated by means of RSS and RBS in a retrospective study (1984-1993). Results. Fifty radiologically and/or scintigraphically abnormal foci were detected: 27 anomalies in the initial work-up (12 by both RSS and RBS, 8 by RSS only and 7 by RBS only) and 23 additional anomalies during follow-up (10 by both RSS and RBS, 10 by RSS only and 3 by RBS only). RSS+/RBS- lesions (n = 18) are more frequently encountered in the skull (P = 0.038), and more frequently lack radiologic signs of osteoblastic activity (P = 0.020), than RSS+/RBS+ lesions (n = 22). RSS-/RBS+ abnormalities (n = 10) were most frequently insignificant. Conclusion. In the initial work-up both RSS and RBS should be carried out, while in the follow-up only RSS should be performed. (orig.). With 2 figs., 4 tabs

  7. Langerhans cell histiocytosis of the urinary bladder in a patient with bladder cancer previously treated with intravesical Bacillus Calmette-Guérin therapy.

    Science.gov (United States)

    Numakura, Satoe; Morikawa, Teppei; Ushiku, Tetsuo; Toyoshima, Toyoaki; Fukayama, Masashi

    2014-02-01

    We report an extremely rare case of Langerhans cell histiocytosis (LCH) of the urinary bladder. A 68-year-old man presented with gross hematuria. Cystoscopy showed multiple papillary tumors in the urinary bladder, and transurethral resection was performed. Pathological diagnosis was high-grade papillary urothelial carcinoma with lamina propria invasion. The patient received six treatments with intravesical Bacillus Calmette-Guérin (BCG) therapy. Seven months after surgery, follow-up cystoscopy showed three elevated lesions in the urinary bladder, two of which were identified histologically as recurrent urothelial carcinoma. Microscopic examination of the lesion at the anterior wall revealed diffuse infiltration of medium to large histiocytoid cells in the lamina propria, many of which had distorted nuclei and nuclear grooves. Dense eosinophilic infiltration was also observed. Immunohistochemically, the histiocytoid cells were diffusely positive for S-100 and CD1a, but negative for cytokeratin AE1/AE3 and melanosome-associated antigen recognized by HMB-45. Based on the histological and immunohistochemical features, we diagnosed the lesion as LCH of the urinary bladder. There was no evidence of recurrence of either bladder cancer or LCH after an 18-month follow-up. To avoid misdiagnosis, urologists and pathologists should be aware that LCH may develop in the urinary bladder after intravesical BCG therapy for bladder cancer. Copyright © 2013 Elsevier GmbH. All rights reserved.

  8. MRI and clinical features of Langerhans cell histiocytosis (LCH) in the pelvis and extremities: can LCH really look like anything?

    International Nuclear Information System (INIS)

    Samet, Jonathan; Weinstein, Joanna; Fayad, Laura M.

    2016-01-01

    To assess clinical and MRI features of Langerhans cell histiocytosis in the pelvis and extremities. The MRI and clinical features of 21 pathologically proven cases of LCH involving the pelvis and extremities were studied. Multiple characteristics of the lesions were evaluated (location, size, T1/ T2/post-contrast features, perilesional bone and soft tissue signal, endosteal scalloping, periosteal reaction, soft tissue mass, pathologic fracture). Pre-biopsy radiologic diagnoses were collected from the original clinical reports. Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), temperature, and white blood cell count (WBC) were collected at the time of diagnosis when available. The locations of the LCH lesions included five humerus, four femur, five ilium, one tibia, one clavicle, and three scapula. Lesional size ranged from 1.8 to 7.1 cm, with a mean of 3.6 cm. All lesions demonstrated perilesional bone marrow edema, periosteal reaction, endosteal scalloping, and post-contrast enhancement. An associated soft tissue mass was present in 15/21 (71.4 %). Clinically, the WBC, ESR, and CRP were elevated in 2/14 (14 %), 8/12 (67 %), and 4/10 (40 %) of cases, respectively. Fever was documented in 1/15 (7 %) patients and pain was reported in 15/15 (100 %). The clinical and radiologic features of LCH in the pelvis and extremities overlap with infection and malignancy, but LCH must be considered in the differential diagnosis, as it routinely presents with aggressive MRI features, including endosteal scalloping, periosteal reaction, perilesional edema, and a soft tissue mass. Furthermore, an unknown skeletal lesion at presentation without aggressive MRI features is unlikely to represent LCH. (orig.)

  9. MRI and clinical features of Langerhans cell histiocytosis (LCH) in the pelvis and extremities: can LCH really look like anything?

    Energy Technology Data Exchange (ETDEWEB)

    Samet, Jonathan [Northwestern University Feinberg School of Medicine, Department of Medical Imaging, Ann and Robert H. Lurie Children' s Hospital of Chicago, Chicago, IL (United States); Weinstein, Joanna [Northwestern University Feinberg School of Medicine, Departments of Pediatrics, Division of Hematology/Oncology/Stem Cell Transplant, Ann and Robert H. Lurie Children' s Hospital of Chicago, Chicago, IL (United States); Fayad, Laura M. [The Johns Hopkins University School of Medicine, The Russell H. Morgan Department of Radiology and Radiological Science, Baltimore, MD (United States)

    2016-05-15

    To assess clinical and MRI features of Langerhans cell histiocytosis in the pelvis and extremities. The MRI and clinical features of 21 pathologically proven cases of LCH involving the pelvis and extremities were studied. Multiple characteristics of the lesions were evaluated (location, size, T1/ T2/post-contrast features, perilesional bone and soft tissue signal, endosteal scalloping, periosteal reaction, soft tissue mass, pathologic fracture). Pre-biopsy radiologic diagnoses were collected from the original clinical reports. Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), temperature, and white blood cell count (WBC) were collected at the time of diagnosis when available. The locations of the LCH lesions included five humerus, four femur, five ilium, one tibia, one clavicle, and three scapula. Lesional size ranged from 1.8 to 7.1 cm, with a mean of 3.6 cm. All lesions demonstrated perilesional bone marrow edema, periosteal reaction, endosteal scalloping, and post-contrast enhancement. An associated soft tissue mass was present in 15/21 (71.4 %). Clinically, the WBC, ESR, and CRP were elevated in 2/14 (14 %), 8/12 (67 %), and 4/10 (40 %) of cases, respectively. Fever was documented in 1/15 (7 %) patients and pain was reported in 15/15 (100 %). The clinical and radiologic features of LCH in the pelvis and extremities overlap with infection and malignancy, but LCH must be considered in the differential diagnosis, as it routinely presents with aggressive MRI features, including endosteal scalloping, periosteal reaction, perilesional edema, and a soft tissue mass. Furthermore, an unknown skeletal lesion at presentation without aggressive MRI features is unlikely to represent LCH. (orig.)

  10. Disseminated sinus histiocytosis with massive lymphadenopathy: its pathologic aspects.

    Science.gov (United States)

    Buchino, J J; Byrd, R P; Kmetz, D R

    1982-01-01

    Sinus histiocytosis with massive lymphadenopathy (SHML) is generally regarded as a benign, self-limited, pseudolymphomatous process requiring little or no therapy. We studied a 13-year-old black boy with a ten-year clinical course of SHML that had varying, intermittent sites of extranodal involvement, including bone, submandibular gland, trachea, eye, and spinal cord. At the time of death, which was attributed to SHML, additional extranodal sites of involvement included thymus, kidney, heart, liver, and base of brain. Microscopic examination of the SHML lesions at the time of autopsy revealed varying stages of development, from proliferation to involution. This case illustrates that SHML may involve multiple organ systems, can kill, and that histologic evaluation of disease activity at one site cannot be used as an indicator of activity at another.

  11. Total pleurectomy as the surgical treatment for recurrent secondary spontaneous pneumothorax in a child with severe pulmonary Langerhans cells histiocytosis.

    Science.gov (United States)

    Abdul Aziz, Dayang Anita; Abdul Rahman, Nur Afdzillah; Tang, Swee Fong; Abdul Latif, Hasniah; Zaki, Faizah Mohd; Annuar, Zulfiqar Mohd; Alias, Hamidah; Abdul Latiff, Zarina

    2011-12-01

    Pulmonary Langerhans cell histiocytosis (LCH) in children is more extensive and is a rare cause of spontaneous secondary pneumothorax (SSP) which tends to be recurrent and refractory to conventional treatment. Its occurrence in paediatric patients posed great challenge to the choice of surgical management. Surgery in the form of pleurodesis is only considered if SSP does not improve after chemotherapy and after considering all relevant risk and benefits of surgery to patients. Chemical pleurodesis will not give the expected effect to eradicate SSP in this patient. Therefore mechanical pleurodesis is the treatment of choice. There are various techniques to perform mechanical pleurodesis; from pleural abrasion to pleurectomy. In the authors' experience, bilateral total pleurectomy provided the best outcome for this 9-year-old patient with persistent respiratory distress from SSP due to extensive pulmonary LCH.

  12. Erdheim-Chester disease: a two-case report

    International Nuclear Information System (INIS)

    Hexsel, Fernando Fernandez; Suwa, Eiji; Aguiar, Paula Musa; Maciel, Antonio Carlos; Hospital de Clinicas de Porto Alegre, RS

    2009-01-01

    Erdheim-Chester disease is a rare non-Langerhans cell histiocytosis of unknown etiology, affecting multiple organ system, involving bones, central nervous system, eyes, lungs, mediastinum, kidneys and retroperitoneum. The authors report two cases that progressed with the typical presentation of the disease. Radiological findings were in agreement with literature and guided the diagnosis, confirmed by immunohistochemistry. (author)

  13. Histiocytosis X Involving the Skeletal System in a Black Girl

    African Journals Online (AJOL)

    1974-10-19

    Oct 19, 1974 ... Lieberman et a/.' reported no deaths in a group of 74 patents with unifocal or muitifocal eosinophilic granuloma, but noted consider- able morbidity associated with the multifocal lesions. Our patient has none of the features which severely limit the prognosis in histiocytosis X, and appears to have responded ...

  14. 111In-Octreotide and 99mTc-MDP scintigraphy in the detection of bone lesions in Langerhans cell histiocytosis

    International Nuclear Information System (INIS)

    Hervas, I.; Bello, P.; Gonzalez-Cabezas, P.; Flores, D.; Perez-Velasco, R.; Torres, I.; Castel, V.; Mateo, A.; Canete, A.

    2002-01-01

    Aim: Langerhans cell histiocytosis (LCH) is a granulomatous disease which can involve multiples sites of the body. Diagnostic imaging is of utmost importance in the management of these patients. Since now radiographic skeletal survey and bone scintigraphy (BS) have been used to assess bone involvement (both with low specificity). Magnetic resonance and CT have been used to assess visceral involvement but with the limitation that can not give information about the functional status. Recently two groups of investigators (Lastoria et al. and Calming et al.) have proposed somatostatin receptor scintigraphy (SSRS) to detect active lesions and for monitoring the response to treatment, due to the somatostatin analogue octreotide binds to the cell membrane of activated lymphocytes expressing somatostatine receptors. The aim of this study is to assess bone and somatostatine receptor scintigraphy in the detection of bone involvement in LCH in children. Visceral involvement has not been assessed due to none of the patients presented it at the moment of the SRS. Methods: 17 scintigraphies (11 SSRS and 6 BS) were performed in seven patients (3 girls and 4 boys) aged at diagnosis: 18 month- 12 years (mean age 6,2 years). The findings obtained in the scintigraphies were compared with clinical evolution and other imaging techniques. Results: All the BS detected the diagnosed lesions, that decreased the uptake after the treatment. In three cases BS detected one unknown bone lesion. Two SSRS could not detect a lesion on right rib in two patients, both at the moment of diagnosis. SSRS was true negative in one patient (clinical and other imaging remission) and true positive in the other four cases. SSRS detected three new unknown bone lesions. SRSS showed decreased uptake after treatment and increased uptake in the relapses. Conclusions: Somatostatin receptor and Bone scintigraphy can be used to detect active LCH bone lesions in children and can help in monitoring the response to

  15. Radiologic findings of adult pelvis and appendicular skeletal Langerhans cell histiocytosis in nine patients

    International Nuclear Information System (INIS)

    Song, You Seon; Lee, In Sook; Kim, Do Kyung; Yi, Jae Hyuck; Cho, Kil Ho; Song, Jong Woon

    2011-01-01

    The purpose of this article was to evaluate the radiologic findings of adult pelvis and appendicular skeletal Langerhans cell histiocytosis (LCH), emphasizing the CT and MR findings. The images of nine patients with pathologically proven LCH (five men and four women; mean age, 37.11 years) were retrospectively reviewed. Imaging analysis was confined to the long and flat bones. CT scans were performed in five patients and MR imaging was performed in eight. Images were assessed for the following features on CT and MRI: the location and number of lesions; the presence of cortical destruction, endosteal scalloping, and a periosteal reaction on CT or MRI; the margin of soft tissue masses, the presence of bone marrow edema, and a ''budding'' appearance on MRI; and the presence of sclerotic margins or septations on CT. The involved skeletal sites were the pelvis (seven), femurs (five), humeri (two), tibias (two), fibula (one), clavicle (one), scapula (one), and sternum (one). Endosteal scalloping, a periosteal reaction, and a budding appearance were common on MRI or CT images. Although cortical destruction and the soft tissue lesion formation were rare, soft tissue masses had well-defined margins. Endosteal scalloping and a budding appearance with a periosteal reaction on CT and MRI may be helpful signs for differentiation of LCH from malignant tumors in adults. (orig.)

  16. Hand-Schüller-Christian disease

    Directory of Open Access Journals (Sweden)

    Deepak Bhargava

    2012-01-01

    Full Text Available Langerhan cell histiocytosis, formerly known as histiocytosis X, traditionally denotes a group of diseases that stem from proliferative reticuloendothelial disturbances.The etiology and pathogenesis of the disease remain debatable. In this paper we report a case of Langerhan cell histiocytosis in a 3 1 / 2 -year-old child who reported to the department of oral pathology with gingival enlargements. The radiological features and histopathological features are also discussed.

  17. Evolving radiological features of hypothalamo-pituitary lesions in adult patients with Langerhans cell histiocytosis (LCH)

    International Nuclear Information System (INIS)

    Makras, P.; Samara, C.; Antoniou, M.; Nikolakopoulou, Z.; Zetos, A.; Papadogias, D.; Piaditis, G.; Kaltsas, G.A.; Toloumis, G.; Andreakos, E.; Kontogeorgos, G.

    2006-01-01

    Langerhans cell histiocytosis (LCH) is a rare, systemic disease caused by monoclonal expansion of dendritic cells that shows a particular predilection for the hypothalamic-pituitary system (HPS). We studied the function (anterior and posterior pituitary hormonal secretion) and morphology using magnetic resonance imaging (MRI) of the HPS in 17 adult patients (seven males, median age 35 years, range 18-59 years) with multisystem LCH. We also evaluated the evolution of structural HPS abnormalities in relation to pituitary function and response to treatment in 12 of these patients during a median follow-up period of 3.75 years (range 1.5-10 years). Of the 17 patients, 14 (82%) had abnormal HPS imaging, and 12 (70%) had more than one area involved. Lack of the bright spot of the posterior pituitary lobe was typically found in all patients with the diagnosis of diabetes insipidus (DI). Eight patients (47%) had infundibular enlargement, six (35%) pituitary infiltration, four (24%) partially or completely empty sella, three (18%) hypothalamic involvement, and two (12%) infundibular atrophy. DI was found in 16 patients (94%) and anterior pituitary hormonal deficiency (APHD) in 10 patients (59%); two patients had single (12%) and 8 (47%) multiple APHD. During the follow-up period there was improvement of the initially demonstrated HPS pathology in seven (47%) patients, and five (33%) of them had received at least one form of treatment. APHD and DI persisted in all patients except in one in whom established gonadotrophin deficiency recovered. In summary, DI and APHD are very common in patients with multisystem LCH and are almost always associated with abnormal HPS imaging. (orig.)

  18. Aspects of pulmonary histiocytosis X on high resolution computed tomography; Aspectos da histiocitose X pulmonar na tomografia computadorizada de alta resolucao (TCAR)

    Energy Technology Data Exchange (ETDEWEB)

    Costa, N.S.S.; Castro Lessa Angela, M.T. de; Angelo Junior, J.R.L.; Silva, F.M.D.; Kavakama, J.; Carvalho, C.R.R. de; Cerri, G.G. [Sao Paulo Univ., SP (Brazil). Faculdade de Medicina. Hospital das Clinicas. Inst. do Coracao

    1995-01-01

    Pulmonary histiocytosis X is a disease that occurs in young adults and presents with nodules and cysts, mainly in upper lobes, with consequent pulmonary fibrosis. These pulmonary changes are virtually pathognomonic findings on high resolution computed tomography, that allows estimate the area of the lung involved and distinguish histiocytosis X from other disorders that also produces nodules and cysts. (author). 10 refs, 2 tabs, 6 figs.

  19. Diagnósticos diferenciales de la histiocitosis a células de Langerhans The differential diagnostics of Langerhans cell histiocytosis

    Directory of Open Access Journals (Sweden)

    C. N. Chirino

    2007-06-01

    Full Text Available La histiocitosis a células de Langerhans (HCL debe diferenciarse de las siguientes entidades: eritema tóxico neonatorum (ETN, dermatitis seborreica (DS, foliculitis pustulosa eosinofílica (FPE, incontinencia pigmenti (IP, mastocitosis/urticaria pigmentosa (M/UP, acrodermatitis enteropática (ADE, síndrome de Wiskott-Aldrich (WAS, acropustulosis infantil (API. Además se deben considerar la enfermedad de Rosai- Dorfman (ERD, xantomas diseminados, melanosis pustulosa neonatal (MPN, candidiasis congénita, listeriosis neonatal, herpes simple perinatal y la varicela neonatal. Debido a que los métodos auxiliares de laboratorio no siempre están disponibles o los resultados laboratoriales algunas veces son extemporáneos, y puesto que el médico práctico a menudo necesita tomar decisiones precozmente, es que la epidemiología resulta útil, pues brinda el marco adecuado para ordenar y jerarquizar las sospechas diagnósticas frente a un caso concreto, con un paciente determinado, en un momento específico.The differential diagnostics of Langerhans cell histiocytosis should include the following disorders: erythema toxicum neonatorum, seborrheic dermatitis, eosinophilic pustular folliculitis, incontinentia pigmenti, mastocytosis / urticaria pigmentosa, acrodermatitis enteropathica, Wiskott-Aldrich syndrome, infantile acropustulosis, Rosai- Dorfman disease, xanthoma disseminatum, neonatal pustular melanosis, congenital candidiasis, perinatal listeriosis, perinatal herpes simplex, neonatal varicella. Since the auxiliary methods of lab are not always available, or lab results are sometimes extemporaneous, the physicians often needs to make quick decisions. The epidemiology is useful because it offers the appropriate mark to prioritize the diagnostic in specific cases

  20. Central nervous system imaging in childhood Langerhans cell histiocytosis – a reference center analysis

    International Nuclear Information System (INIS)

    Porto, Luciana; Schöning, Stefan; Hattingen, Elke; Sörensen, Jan; Jurcoane, Alina; Lehrnbecher, Thomas

    2015-01-01

    The aim of our study was (1) to describe central nervous system (CNS) manifestations in children with Langerhans cell histiocytosis (LCH) based on images sent to a reference center and meeting minimum requirements and (2) to assess the inter-rater agreement of CNS-MRI results, which represents the overall reproducibility of this investigation. We retrospectively reviewed brain MRI examinations in children with LCH, for which MRI minimum requirements were met. Abnormalities were rated by two experienced neuroradiologists, and the inter-rater agreement was assessed. Out of a total of 94 imaging studies, only 31 MRIs met the minimum criteria, which included T2w, FLAIR, T1w images before/after contrast in at least two different section planes, and thin post contrast sagittal slices T1w through the sella. The most common changes were osseous abnormalities, followed by solid enlargement of the pineal gland, thickened enhancing stalk and signal changes of the dentate nucleus. Whereas inter-rater agreement in assessing most of the CNS lesions was relatively high (κ > 0.61), the application of minimum criteria often did not allow to evaluate the posterior pituitary. The diversity of radiological protocols from different institutions leads to difficulties in the diagnosis of CNS abnormalities in children with LCH. Although the inter-rater agreement between neuroradiologists was high, not all the LCH manifestations could be completely ruled out when using the minimum criteria. Brain MRIs should therefore follow LCH guideline protocols and include T1 pre-gadolinium sagittal images, and be centrally reviewed in order to improve the comparison of clinical trials

  1. Histiocytosis X: Scintigraphic and roentgenological findings

    International Nuclear Information System (INIS)

    Fezoulidis, I.; Wickenhauser, J.; Schurawitzki, H.; Gritzmann, N.

    1987-01-01

    The aim of this study was to compare the roentgenological and scintigraphic osseous changes in 25 resp. 18 patients with histiocytosis X and to analyse them. In particular, it was also interesting to discuss the divergent reports in the literature in respect of scintigraphic storage behaviour. In all patients examined before initiation of therapy (39 osseous foci) high activities were found scintigraphically. The extension of the scintigraphically active zone was much greater than the corresponding translucencies on the X-ray film. On the other hand, patients after therapy still showed foci on the X-ray film whereas the scan had already become inactive. This striking discrepancy between scan findings before and after therapy could be one of the reasons for divergent literature reports on this problem. (orig.) [de

  2. Sinus histiocytosis with massive lymphadenopathy Rosai-Dorfman's disease as cause of isolated hilar lymphadenopathy and complete remission after high dose steroid

    International Nuclear Information System (INIS)

    Al-Jahdali, Hamdan H.; Al-Shirawi, Nehad N.; Bamefleh, Hana S.; Yamani, Nizar M.

    2008-01-01

    Rosai-Dorfman's Disease, also known as sinus histiocytosis with massive lymphadenopathy SHML, is a rare histiocytic proliferative disorder and a distinct clinic-pathological feature of unknown origin. Painless cervical lymphadenopathy is the most common clinical presentation. Different treatment modalities have been tried with variable responses, however, there is no consensus on the best modality of treatment. Here, we present a case report of SHML causing isolated hilar lymphadenopathy with complete remission for more than 6 years, after a short course of high dose steroid dexamethasone 20 mg daily for 3 days. (author)

  3. CD34+ (Non-Malignant) Stem Cell Selection for Patients Receiving Allogeneic Stem Cell Transplantation

    Science.gov (United States)

    2017-07-13

    Bone Marrow Failure Syndrome; Severe Aplastic Anemia; Severe Congenital Neutropenia; Amegakaryocytic Thrombocytopenia; Diamond-Blackfan Anemia; Schwachman Diamond Syndrome; Primary Immunodeficiency Syndromes; Acquired Immunodeficiency Syndromes; Histiocytic Syndrome; Familial Hemophagocytic Lymphocytosis; Lymphohistiocytosis; Macrophage Activation Syndrome; Langerhans Cell Histiocytosis (LCH); Hemoglobinopathies; Sickle Cell Disease; Sickle Cell-beta-thalassemia

  4. Irradiation of the hypothalamic-hypophyseal area induces complete regression of mucocutaneous lesions in disseminated histiocytosis X

    International Nuclear Information System (INIS)

    Palmieri, G.; Stefani, S.; Gridelli, C.; Conte, A.; Airoma, G.; Contegiacomo, A.; Bianco, A.R.

    1989-01-01

    We report on a 54-year-old woman with disseminated histiocytosis X who had a complete regression of all mucocutaneous lesions within 1 month from the completion of radiation therapy (4500 cGy) to the hypothalamic-hypophyseal (H-H) area. This response lasted 12 months, after which new cutaneous and bone lesions appeared

  5. [Generalized eruptive histiocytoma. Apropos of a case. Review of the literature].

    Science.gov (United States)

    Bobin, P; Carsuzaa, F; Seurat, P; Lucas, D

    1983-01-01

    About one case of generalized eruptive histiocytoma (G.E.H.) (the second French case) and about thirteen others cases mentioned in literature, the authors remind the clinical, histological, immuno-histochemical and evolutive aspects of this misunderstanding disease, described by Winkelmann in 1963. After having classed it, in the group of nodular, non X, non lipidique, benign histiocytosis, they expose the imprecision of this nosological limits of the G.E.H., in comparison to the bordering diseases. At last, they insist upon the fact that the few number of cases published up to this time, the lack of stand back and the casual transformation into a multicentric reticulo-histiocytosis must induce to watchfulness by requiring for each patient affected by G. E. H. and for a long time, a regular, clinical, radiological and hematological supervision.

  6. [Juvenile xanthogranuloma: 3 cases report and literature review].

    Science.gov (United States)

    Liu, Zi-qin; Liu, Rong; Shi, Xiao-dong; Li, Jing-xian; Zou, Ji-zhen

    2011-09-01

    To report the clinical characteristics and treatment of 3 patients with juvenile xanthogranuloma (JXG). A retrospective review of the medical records of 3 patients with JXG. JXG was characterized by solitary or multiple yellowish cutaneous nodules, or eye involvement . It could also affect pituitary. JXG was easily misdiagnosed as Langerhans cell histiocytosis (LCH). Treatment for JXG was surgical excision of a solitary skin lesion and some cases might be, spontaneous regression. In cases with multisystem involvement, chemotherapy regimens used to treat LCH may be effective. JXG is one of the more common non-Langerhans histiocytic proliferations and is frequently seen in infants and children. LCH-like chemotherapy is effective for patients with symptomatic multisystem JXG.

  7. Preliminary study on the evaluation of Langerhans cell histiocytosis using F-18-fluoro-deoxy-glucose PET/CT

    Institute of Scientific and Technical Information of China (English)

    Zhou Wenlan; Wu Hubing; Han Yanjiang; Wang Shaobo; Dong Ye; Wang Quanshi

    2014-01-01

    Background Limited number of studies have been reported regarding the utilization of F-18-fluoro-deoxy-glucose (F-18-FDG) positron emission tomography/computed tomography (F-18-FDG PET/CT) in Langerhans cell histiocytosis (LCH).The aim of this study was to assess the role of F-18-FDG PET/CT in the diagnosis and treatment of LCH.Methods Eight newly diagnosed and seven recurrent patients with LCH received F-18-FDG PET/CT scans.The diagnosis of LCH was established by pathology,multi-modality imaging,and clinical follow-up.Results F-18-FDG PET/CT was positive in 14 patients with 13 true positives and one false positive.All 45 LCH lesions were F-18-FDG avid including six small bone lesions <1.0 cm in diameter.The mean maximal standardized uptake value (SUVmax) was 7.13±4.91.F-18-FDG uptake showed no significant difference between newly diagnosed lesions vs recurrent lesions (SUVmax:6.50±2.97 vs.7.93±6.60,t=-0.901,P=0.376).Among 45 LCH lesions,68.9% (31/45) were found in bones and 31.1% (14/45) in soft tissue.The most commonly involved bones were the pelvis and vertebrae.There was no significant difference in F-18-FDG uptake between bone lesions vs.non-bone lesions (SUVmax:6.30±2.87 vs.8.97±7.58,t=1.277,P=0.221).In two patients,changes in F-18-FDG uptake on serial PET/CT scans reflected response of lesions to treatment.Conclusions The present study suggests that F-18-FDG PET/CT may be useful for diagnosis and assessing the treatment response of LCH.Because of the small sample size,further research is warranted to confirm our findings.

  8. Pulmonary Langerhans Cell Histiocytosis Case with Diabetes ...

    African Journals Online (AJOL)

    2016-07-12

    Jul 12, 2016 ... thin-walled cystic lesions in different sizes more dominantly in the upper lobes and consolidated areas ... zones inside, which started from the hilar region on ... increase of infiltrations in the control PA radiography. Right lung ...

  9. Quantitative PCR and immunohistochemical analyses of HMGB1 and RAGE expression in canine disseminated histiocytic sarcoma (malignant histiocytosis).

    Science.gov (United States)

    Sterenczak, Katharina A; Kleinschmidt, Sven; Wefstaedt, Patrick; Eberle, Nina; Hewicker-Trautwein, Marion; Bullerdiek, Jörn; Nolte, Ingo; Murua Escobar, Hugo

    2011-05-01

    Disorders of histiocytic origin affecting humans and dogs share various similarities. Canine disseminated histiocytic sarcoma (DHS) (formerly known as malignant histiocytosis) is an aggressive neoplasm of interstitial dendritic cells (DCs). The receptor for glycation end products (RAGE) and the high mobility group box1 protein (HMGB1) have been shown to be required for the maturation and migration of DCs. Thus, deregulation of the expression of these genes could have a major effect on the progression of histiocytic disorders. Neoplastic canine DHS samples and non-neoplastic control samples were analysed immunohistochemically and via real-time PCR. Significant down-regulation of RAGE in the lung tumour samples and down-regulation of HMGB1 in the lung, lymph node and spleen tumour samples were detected compared to their non-neoplastic counterparts. RAGE and HMGB1 expression down-regulation in canine DHS points to a role in the progression of histiocytic disorders.

  10. The pituitary gland in patients with Langerhans cell histiocytosis: a clinical and radiological evaluation.

    Science.gov (United States)

    Kurtulmus, Neslihan; Mert, Meral; Tanakol, Refik; Yarman, Sema

    2015-04-01

    Langerhans cell histiocytosis (LCH) is a rare disease in which the most common endocrine manifestation is diabetes insipidus (DI). Data on anterior pituitary function in patients with LCH are limited. Thus, the present study investigated anterior pituitary function in LCH patients with DI via the evaluation of clinical and radiological findings at disease onset and during follow-up. The present study retrospectively evaluated nine patients with LCH (five males and four females). All diagnoses of LCH were made following histological and/or immunophenotypic analyses of tissue biopsies, bronchoalveolar lavage, or cerebrospinal fluid (CSF). Basal and, if necessary, dynamic pituitary function tests were used to assess anterior pituitary function, and magnetic resonance imaging (MRI) scans were used to image the pituitary. The LCH treatment modality was based on organ involvement. The mean age at onset of DI was 27.6 years (range 15-60 years). One patient (11%) exhibited single organ involvement, while eight patients (89%) displayed multisystem organ involvement. On admittance, one patient had hypogonadotropic hypogonadism, one patient exhibited panhypopituitarism [hypogonadotropic hypogonadism, central hypothyroidism, hypocortisolism, and growth hormone (GH) deficiency], and four patients (44%) displayed hyperprolactinemia. The MRI data revealed infundibular enlargement in seven patients (78%), a thalamic mass in one patient (11%), and the absence of the bright spot in all patients. A single patient (11%) showed a mass in the pons that had a partially empty sella. The patients were treated with radiation therapy (RT), chemotherapy (CT), or a combination of both (RT+CT) and were followed up for a median of 91.8 months (range 2-318 months). Seven patients were assessed during the follow-up period, of whom four patients (57.1%) developed anterior pituitary hormone deficiency, three (43%) were diagnosed with GH deficiency, and one (14%) exhibited gonadotropin deficiency

  11. Browse Title Index

    African Journals Online (AJOL)

    Vol 20, No 4 (2017), Pulmonary langerhans cell histiocytosis case with ... Management Of Impacted Coin In The Oesophagus – A Case Report .... Vol 20, No 5 (2017), Relationship between alexithymia and chronic periodontitis, Abstract PDF.

  12. Palbociclib in Treating Patients With Relapsed or Refractory Rb Positive Advanced Solid Tumors, Non-Hodgkin Lymphoma, or Histiocytic Disorders With Activating Alterations in Cell Cycle Genes (A Pediatric MATCH Treatment Trial)

    Science.gov (United States)

    2018-05-15

    Advanced Malignant Solid Neoplasm; RB1 Positive; Recurrent Childhood Ependymoma; Recurrent Ewing Sarcoma; Recurrent Glioma; Recurrent Hepatoblastoma; Recurrent Kidney Wilms Tumor; Recurrent Langerhans Cell Histiocytosis; Recurrent Malignant Germ Cell Tumor; Recurrent Malignant Glioma; Recurrent Medulloblastoma; Recurrent Neuroblastoma; Recurrent Non-Hodgkin Lymphoma; Recurrent Osteosarcoma; Recurrent Peripheral Primitive Neuroectodermal Tumor; Recurrent Rhabdoid Tumor; Recurrent Rhabdomyosarcoma; Recurrent Soft Tissue Sarcoma; Refractory Ependymoma; Refractory Ewing Sarcoma; Refractory Glioma; Refractory Hepatoblastoma; Refractory Langerhans Cell Histiocytosis; Refractory Malignant Germ Cell Tumor; Refractory Malignant Glioma; Refractory Medulloblastoma; Refractory Neuroblastoma; Refractory Non-Hodgkin Lymphoma; Refractory Osteosarcoma; Refractory Peripheral Primitive Neuroectodermal Tumor; Refractory Rhabdoid Tumor; Refractory Rhabdomyosarcoma; Refractory Soft Tissue Sarcoma

  13. Langerhans cell histiocytosis: A case presentation and literature ...

    African Journals Online (AJOL)

    evaluation imaging (skeletal survey, skeletal isotopes and CT ... recent imaging confirmed that he still has .... include small stature, growth hormone deficiency, hypothyroidism, ... lesions or multisystem non-risk organ involvement, even a short.

  14. Genetics Home Reference: Langerhans cell histiocytosis

    Science.gov (United States)

    ... as viral infections and environmental toxins, may also influence the development of this complex disorder. Learn more about the ... occur? How can gene mutations affect health and development? More about Mutations and ... in people with no history of the disorder in their family. A few families with multiple cases of Langerhans ...

  15. Eosinophilic granuloma in jaw bone: a pare pediatric case report ...

    African Journals Online (AJOL)

    Background: Eosinophilic granuloma (EG), one of the three clinical forms of Langerhans cell histiocytosis (LCH), is a benign inflammatory reaction to an unknown etiologic agent. It most commonly occurs in children and young adults. The most frequently involved bones are the skull, the ribs and the femurs. Alongside the ...

  16. Erdheim-Chester disease: a comprehensive review.

    Science.gov (United States)

    Abdelfattah, Ahmed Maher; Arnaout, Karim; Tabbara, Imad A

    2014-07-01

    Erdheim-Chester disease is a rare form of non-Langerhans' cell histiocytosis characterized by multi-system infiltration by xanthogranulomas composed of foamy histiocytes surrounded by fibrosis. Approximately 400 cases have been reported in the literature, and the recent increase in the number of cases is likely due to the increased awareness of its associated morbidity and mortality. The etiology of this disease remains unknown, the clinical course is variable and treatment is still not well-established. The objective of this review is to describe the pathogenesis, clinical manifestations, and diagnosis of this rare disorder, and to review its prognosis and treatment. Erdheim-Chester disease (ECD) is a rare form of non-Langerhans' cell histiocytosis. It was first described in 1930. Approximately 400 cases have been reported in the literature. Copyright© 2014 International Institute of Anticancer Research (Dr. John G. Delinassios), All rights reserved.

  17. Critical Pitfalls in the use of BRAF Mutation as a Diagnostic Tool in Thyroid Nodules: a Case Report.

    Science.gov (United States)

    Kuhn, Elisabetta; Ragazzi, Moira; Zini, Michele; Giordano, Davide; Nicoli, Davide; Piana, Simonetta

    2016-09-01

    Thyroid fine-needle aspiration (FNA) cytology is the primary tool for the diagnostic evaluation of thyroid nodules. BRAF mutation analysis is employed as an ancillary tool in indeterminate cases, as recommended by the American Thyroid Association management guidelines. Hereby, we report the case of a 73-year-old woman who presented an 8-mm-size, ill-defined, left thyroid nodule. FNA resulted "suspicious for papillary thyroid carcinoma". BRAF mutation status was analyzed, and somatic BRAF (V600E) mutation identified. The patient underwent a total thyroidectomy. At histological examination, the nodule was composed of Langerhans cells, admixed with many eosinophils. A final diagnosis of Langerhans cell histiocytosis of the thyroid was made. Our case emphasizes the critical diagnostic pitfalls due to the use of BRAF (V600E) mutation analysis in thyroid FNA. Notably, BRAF (V600E) mutation is common in melanoma, colorectal carcinoma, lung carcinoma, ovarian carcinoma, brain tumors, hairy cell leukemia, multiple myeloma, and histiocytoses. Therefore, in cases of indeterminate FNA with unclassifiable atypical cells BRAF (V600E) mutated, the possibility of a localization of hystiocytosis or a secondary thyroid malignancy should be taken into account.

  18. Baby with neonatal systemic juvenile xanthogranuloma born within a cross-cousin marriage

    Directory of Open Access Journals (Sweden)

    Hikmet Tekin Nacaroglu

    2015-12-01

    Full Text Available Juvenile xanthogranuloma is a non-Langerhans cell histiocytosis seen most commonly in childhood and adolescence. Extracutaneous involvement is rare. We report an interesting and extremely rare case of systemic (skin, lung, spleen, and colon “juvenile xanthogranuloma” in the neonatal period. Our case was the first ever reported case born to a cross-cousin marriage.

  19. Dendritic cell neoplasms: an overview.

    Science.gov (United States)

    Kairouz, Sebastien; Hashash, Jana; Kabbara, Wadih; McHayleh, Wassim; Tabbara, Imad A

    2007-10-01

    Dendritic cell neoplasms are rare tumors that are being recognized with increasing frequency. They were previously classified as lymphomas, sarcomas, or histiocytic neoplasms. The World Health Organization (WHO) classifies dendritic cell neoplasms into five groups: Langerhans' cell histiocytosis, Langerhans' cell sarcoma, Interdigitating dendritic cell sarcoma/tumor, Follicular dendritic cell sarcoma/tumor, and Dendritic cell sarcoma, not specified otherwise (Jaffe, World Health Organization classification of tumors 2001; 273-289). Recently, Pileri et al. provided a comprehensive immunohistochemical classification of histiocytic and dendritic cell tumors (Pileri et al., Histopathology 2002;59:161-167). In this article, a concise overview regarding the pathological, clinical, and therapeutic aspects of follicular dendritic, interdigitating dendritic, and Langerhans' cell tumors is presented.

  20. The Rosai Dorfman Syndrome-The Report of A Rare Case

    OpenAIRE

    R, Snehalatha; P, Senthilnathan; Ramani, Prathiba; Herald, Sherlin J

    2013-01-01

    Rosai Dorfman syndrome known as Sinus Histiocytosis with Massive Lymphadenopathy (SHML) is an uncommon benign systemic histio-proliferative disease which affects lymph nodes , most often those of the neck (cervical lymphadenopathy). The characteristic histological feature is lymphophagocytosis which is specific for this syndrome. Evidence based survey reveals that many cases that have been reported in india are from medical fraternity other than dental colleagues. As dental professionals we d...

  1. Sinus Histiocytosis with Massive Lymphadenopathy (Rosai-Dorfman Disease: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Pradhananga, Rabindra Bhakta

    2014-08-01

    Full Text Available Introduction Rosai-Dorfman disease (RDD is a rare histiocytic proliferative disorder of unknown etiology. Usually it presents with massive painless cervical lymph node enlargement. Histologically, it shows proliferation of distinctive histiocytic cells that demonstrate emperipolesis in the background of a mixed inflammatory infiltrates. Immunohistochemically, the cells are positive for markers such as CD68 and S100. Objective To report a case of a 12-year-old patient with multiple sites of cervical lymphadenitis, which was diagnosed as RDD histopathologically as well as immunohistologically. Resumed Report A 12-year-old girl presented with multiple painless sites of cervical lymphadenitis without any systemic and other ear, nose, and throat manifestations. The biopsy report of the lymph node showed dilatation of the sinuses, filled with histiocytes having foamy cytoplasm. Many of the histiocytes were engulfing mature lymphocytes. The sinus histiocytes were strongly positive for S-100 protein. Conclusion RDD must be considered in the differential diagnosis of massive or multiple lymphadenopathies.

  2. Trials with TALL-1O4 Cells for Treatment of Metastatic Breast Cancer

    Science.gov (United States)

    1999-10-01

    Cesano, A.*, Jeglum, K. A., and Santoli, D. Adjuvant treatment of canine osteosarcoma with the human cytotoxic T cell line TALL-104. Clin. Cancer... canine malignant histiocytosis with the human MHC non-restricted cytotoxic T cell line TALL-104. Clin. Cancer Res., 3: 1789-1797, 1997. 14. Visonneau, S...Visonneau, S., Cesano, A., Jeglum, K. A., and Santoli, D. Adoptive therapy of canine metastatic mammary carcinoma with the human MHC non-restricted

  3. ROSAI-DORFMAN DISEASE WITH CERVICAL LYMPHADENOPATHY AND ORBITAL INVOLVEMENT: A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Sameer Saleem, Sundas Younas, Kamran Qayyum

    2015-07-01

    Full Text Available Rosai-Dorfman disease (RDD, which is also called as sinus histiocytosis with massive lymphadenopathy (SHML, is a rare histiocytic disorder which occurs due to the over-production of non Langerhans sinus histiocytes. It is a nonmalignant disorder that most frequently affects children and young adults and typically presents with fever, night sweats, nonpainful cervical lymphadenopathy, leukocytosis and an elevated ESR. Extranodal involvement may also occur, thus a variety of organs in the body can be affected. Although some viral etiology has been implicated, the disease generally is considered to have an unknown cause. RDD can often be misdiagnosed as lymphoma, leukemia or tuberculosis, so it is imperative to distinguish it from these conditions as well as other forms of histiocytosis because of difference in the modes of treatment. Diagnosis of Rosai-Dorfman disease is based on biopsy of affected tissue. Biopsy showing the presence of emperipolesis, or the engulfment of lymphocytes and other immune cells by histiocytes that express S-100 antigen is diagnostic of Rosai-Dorfman disease. Once diagnosed, further workup including imaging studies are undertaken in order to determine the extent of the disease. In majority of cases, the disease resolves on its own however, treatments including corticosteroids, chemotherapy, surgical treatment or radiotherapy are carried out in severe or persistent disease or when organ function is at stake (e.g. breathing obstruction, kidney failure, visual problems. The case we report is that of a 16 year old girl who presented with a 6 month history of gradual onset drooping of left upper eyelid with mild proptosis of the left eye alongwith mild drooping of right upper eyelid, low grade fever, night sweats and cervical lymphadenopathy. Blood workup showed increased ESR, CT scan of orbits showed superior orbital masses and diagnostic biopsy revealed Rosai-Dorfman disease.

  4. 18F-FDG PET/CT in follow-up evaluation in pediatric patients with Langerhans histiocytosis.

    Science.gov (United States)

    Garcia, J R; Riera, E; Bassa, P; Mourelo, S; Soler, M

    We evaluated the impact of 18 F-FDG PET/CT in identifying sites of active disease and to assess therapeutic follow up in a group of pediatric patients with Langerhans cell histiocytosis (LCH). During 2007-2013, 13 18 F-FDG PET/CT studies were performed for follow-up in 7 patients with a diagnosis of LCH (4 female, 3 male; 1-12 years-old). PET findings were analyzed and correlated with the CT and MRI. Findings were also follow-up by these techniques. PET was negative in 4 patients (all diagnosed with bone lesions and one with pituitary involvement also). CT findings showed residual morphological bone lesions in all patients, and hypophysis MRI study showed no abnormal signal. PET remained negative at 10, 14, 25 and 28 months, and no new lesions on CT and MRI were detected. PET was positive in 3 patients (one with cervical lymphadenopathy and 2 with bone lesions, one also with pituitary involvement not identified by PET). CT findings showed pathological cervical lymphadenopathy (n=1), bone lesions (n=2) and also a pituitary MRI lesion (n=1). In a patient with cervical lymphadenopathy histology demonstrated LCH involvement. In the other 2 patients, PET remained positive with an increase of 18 F-FDG bone uptake at 17 and 19 months. In our preliminar study, 18 F-FDG PET is a useful imaging procedure, along with other diagnostic tools, for identification of active lesions. Copyright © 2017 Elsevier España, S.L.U. y SEMNIM. All rights reserved.

  5. Congenital self-healing reticulohistiocytosis - an important diagnostic challenge

    DEFF Research Database (Denmark)

    Jensen, Marie Louise Slott; Bygum, Anette; Clemmensen, Ole

    2011-01-01

    Aim:  To present current and new knowledge on congenital self-healing reticulohistiocytosis, a benign variant of cutaneous Langerhans cell histiocytosis presenting with skin lesions in the neonatal period. Methods:  We describe and photo document two cases of this rare disease and review the lite......Aim:  To present current and new knowledge on congenital self-healing reticulohistiocytosis, a benign variant of cutaneous Langerhans cell histiocytosis presenting with skin lesions in the neonatal period. Methods:  We describe and photo document two cases of this rare disease and review...... the literature. Results:  Only few newborns have acute access to a neonatal dermatologist and we demonstrate how the spontaneous cutaneous involution may happen even prior to the first dermatological assessment. As no sole criterion can reliably distinguish the self-healing form from disseminated disease......, multidisciplinary assessment and follow up are essential. Conclusion:  Our data document how easily the diagnosis congenital self-healing reticulocytosis may be missed and emphasize the importance and value of instant clinical photographing at the neonatal unit and the use of teledermatology whenever congenital...

  6. Erdheim Chester disease with appendicular skeletal, renal and pleural involvement responding to Zelboraf (BRAF inhibitor) treatment: case report

    International Nuclear Information System (INIS)

    Borys, Dariusz; Nystrom, Lucas; Song, Albert; Lomasney, Laurie M.

    2016-01-01

    Erdheim Chester disease is a rare non-Langerhans cell histiocytosis which may involve multiple organs including bone, soft tissue, lungs, cardiovascular system, kidneys (retroperitoneum), skin, and central nervous system. Bone involvement is most common followed by other organs. This case report describes a 58-year-old man who presented with progressive renal dysfunction presumed due to obstruction. The patient failed multiple urinary tract interventions, and clinical course was complicated by recurrent low-grade fevers, and bilateral knee pain. Advanced imaging and histopathological features on bone biopsy were consistent with Erdheim Chester disease. Molecular studies of tissue showed BRAF V600 mutation. This patient was treated with Zelboraf (vemurafenib) BRAF inhibitor with subsequent improvement in renal and pleural dysfunction as well as decreased histiocytic soft tissue masses on CT. (orig.)

  7. Erdheim Chester disease with appendicular skeletal, renal and pleural involvement responding to Zelboraf (BRAF inhibitor) treatment: case report

    Energy Technology Data Exchange (ETDEWEB)

    Borys, Dariusz [Loyola University Medical Center Chicago, Department of Pathology, Maywood, IL (United States); Loyola University Medical Center Chicago, Departmet of Orthopaedic Surgery, Maywood, IL (United States); Loyola University Medical Center, Maywood, IL (United States); Nystrom, Lucas [Loyola University Medical Center Chicago, Departmet of Orthopaedic Surgery, Maywood, IL (United States); Song, Albert [Loyola University Medical Center Chicago, Department of Radiology, Maywood, IL (United States); Lomasney, Laurie M. [Loyola University Medical Center Chicago, Departmet of Orthopaedic Surgery, Maywood, IL (United States); Loyola University Medical Center Chicago, Department of Radiology, Maywood, IL (United States)

    2016-10-15

    Erdheim Chester disease is a rare non-Langerhans cell histiocytosis which may involve multiple organs including bone, soft tissue, lungs, cardiovascular system, kidneys (retroperitoneum), skin, and central nervous system. Bone involvement is most common followed by other organs. This case report describes a 58-year-old man who presented with progressive renal dysfunction presumed due to obstruction. The patient failed multiple urinary tract interventions, and clinical course was complicated by recurrent low-grade fevers, and bilateral knee pain. Advanced imaging and histopathological features on bone biopsy were consistent with Erdheim Chester disease. Molecular studies of tissue showed BRAF V600 mutation. This patient was treated with Zelboraf (vemurafenib) BRAF inhibitor with subsequent improvement in renal and pleural dysfunction as well as decreased histiocytic soft tissue masses on CT. (orig.)

  8. Cystic lung disease: a comparison of cystic size, as seen on expiratory and inspiratory HRCT scans

    International Nuclear Information System (INIS)

    Lee, Ki Nam; Yoon, Seong Kuk; Nam, Kyung Jin; Choi, Seok Jin; Goo, Jin Mo

    2000-01-01

    To determine the effects of respiration on the size of lung cysts by comparing inspiratory and expiratory high-resolution CT (HRCT) scans. The authors evaluated the size of cystic lesions, as seen on paired inspiratory and expiratory HRCT scans, in 54 patients with Langerhans cell histiocytosis (n = 3), pulmonary lymphangiomyomatosis (n = 4), confluent centrilobular emphysema (n = 9), paraseptal emphysema and bullae (n = 16), cystic bronchiectasis (n = 13), and honeycombing (n = 9). Using paired inspiratory and expiratory HRCT scans obtained at the corresponding anatomic level, a total of 270 cystic lesions were selected simultaneously on the basis of five lesions per lung disease. Changes in lung cyst size observed during respiration were assessed by two radiologists. In a limited number of cases (n = 11), pathologic specimens were obtained by open lung biopsy or lobectomy. All cystic lesions in patients with Langerhans cell histiocytosis, lymphangiomyomatosis, cystic bronchiectasis, honeycombing, and confluent centrilobular emphysema became smaller on expiration, but in two cases of paraseptal emphysema and bullae there was no change. In cases in which expiratory CT scans indicate that cysts have become smaller, cystic lesions may communicate with the airways. To determine whether, for cysts and cystic lesions, this connection does in fact exist, paired inspiratory and expiratory HRCT scans are necessary

  9. Cystic lung disease: a comparison of cystic size, as seen on expiratory and inspiratory HRCT scans

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Ki Nam; Yoon, Seong Kuk; Nam, Kyung Jin [Donga University College of Medicine, Pusan (Korea, Republic of); Choi, Seok Jin [Inje University College of Medicine, Gimhae (Korea, Republic of); Goo, Jin Mo [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2000-06-01

    To determine the effects of respiration on the size of lung cysts by comparing inspiratory and expiratory high-resolution CT (HRCT) scans. The authors evaluated the size of cystic lesions, as seen on paired inspiratory and expiratory HRCT scans, in 54 patients with Langerhans cell histiocytosis (n = 3), pulmonary lymphangiomyomatosis (n = 4), confluent centrilobular emphysema (n = 9), paraseptal emphysema and bullae (n = 16), cystic bronchiectasis (n = 13), and honeycombing (n = 9). Using paired inspiratory and expiratory HRCT scans obtained at the corresponding anatomic level, a total of 270 cystic lesions were selected simultaneously on the basis of five lesions per lung disease. Changes in lung cyst size observed during respiration were assessed by two radiologists. In a limited number of cases (n = 11), pathologic specimens were obtained by open lung biopsy or lobectomy. All cystic lesions in patients with Langerhans cell histiocytosis, lymphangiomyomatosis, cystic bronchiectasis, honeycombing, and confluent centrilobular emphysema became smaller on expiration, but in two cases of paraseptal emphysema and bullae there was no change. In cases in which expiratory CT scans indicate that cysts have become smaller, cystic lesions may communicate with the airways. To determine whether, for cysts and cystic lesions, this connection does in fact exist, paired inspiratory and expiratory HRCT scans are necessary.

  10. Bone scintigraphy in Erdheim-chester disease: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Siqueira, V.L.; Soares, L.M.M.; Ribeiro, V.P.B.; Coura Filho, G.B.; Sapienza, M.T.; Ono, C.R.; Watanabe, T.; Costa, P.L.A.; Hironaka, F.; Buchpiguel, C.A. [Universidade de Sao Paulo (FM/USP), SP (Brazil). Fac. de Medicina. Hospital das Clinicas

    2008-07-01

    Full text: Introduction: Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis, of unknown etiology, characterized by infiltration of foamy histiocytes. Clinically, patients usually present with bone pain, and various extraskeletal manifestations. ECD differs from Langerhans cell histiocytosis (LCH) by radiologic and immunohistochemistry features. Case report: A 57-year-old woman presented with a history of intense pain on her left hand, besides eyelid xanthelasmas and xanthoms on frontal area ten years ago. Four years late she presented with pain on hips, legs and feet. Xanthoms spread to perioral area, mento and neck. Radiographs of the hands showed osteolysis of carpal bones bilaterally, osteolysis of fifth left metacarpal bone, osteosclerosis of all metacarpal bones bilaterally, except the fifth, and osteosclerosis of the second and third proximal falanges bilaterally. The legs showed bilateral diaphyseal and metaphyseal osteosclerosis. Bone scintigraphy demonstrated increased uptake on face bone (maxilla), and symmetric intense uptake on elbows, distal radii and ulnae, hands, distal area of femurs, tibias particularly on proximal and distal area, and feet. A tibia biopsy and a biopsy of neck lesion were made. The analysis of histology and immunohistochemistry were consistent with ECD. She has been treated with a-interferon for 1,5 year, and she reports delay in xanthoms progression and bone pain remission. Discussion: ECD is an adult multisystemic xanthogranulomatous infiltrative disease of unknown etiology. It may be confused with LCH, however ECD have distinctive immunohistochemistry and radiologic findings. LCH shows typically lytic bone lesions on axial skeleton, whereas symmetrical long-bone osteosclerosis is the radiologic sign for ECD. LCH stain positive for CD1a and S-100 protein, and the electron microscopy of cytoplasm discloses Biberck granules. ECD stain positive for CD68, negative for CD1a and S-100 protein, shows absent of

  11. Peculiar Distribution of Tumorous Xanthomas in an Adult Case of Erdheim-Chester Disease Complicated by Atopic Dermatitis

    Directory of Open Access Journals (Sweden)

    Yukako Murakami

    2011-05-01

    Full Text Available Erdheim-Chester disease is a rare non-Langerhans form of histiocytosis with multiple organ involvement. Approximately 20% of patients have xanthoma-like lesions, usually on the eyelids. We report a case of Erdheim-Chester disease in a 32-year-old male who showed peculiar xanthomatous skin lesions and also had atopic dermatitis. His skin manifestations included ring-like yellowish tumors on his periorbital regions, rope necklace-like tumors on his neck, and spindle-shaped tumors on his right preauricular region and cubital fossas. He also had exophthalmos and diabetes insipidus. Chronic eczematous lesions were present on the flexor aspect of his extremities, and his serum eosinophil numbers and immunoglobulin E levels were elevated. A histological examination of his right neck tumor showed foamy macrophages and touton-type giant cells, which were positive for CD68 and CD163 and negative for S-100 and CD1a. We suggest that the complication of atopic dermatitis may have contributed to the uncommon clinical features in this case.

  12. Breast. cancer. Prognosis factors - preliminar study

    International Nuclear Information System (INIS)

    Rotstein, S.; Fonseca, N.M.

    1984-01-01

    A preliminar study of prognosis factors in 8 cases of breast cancer is made. Are used as parameters the dimension, the localization and the nuclear differentiation degree (gN) of the primary tumor, the vascular invasion and the axillary histologic status (pN) and the sinus histiocytosis phenomenon. Among the studied factors, have special importance the presence of vascular invasion and the negative sinus histiocytosis (minimal or absent sinus histiocytosis). Both phenomena are considered as an expression of potential systemic disease, independent of the clinical stage. Consequently the use of chemotherapy in the surgery complementation is preconized, to a best control of the disease. (author)

  13. Contribution to the radiological study of the eosinophilic granuloma of the mandible (Unifocal granuloma due to Langherans' cell histiocytosis); Contributo allo studio radiologico del granuloma eosinofilo della mandibola (granuloma unifocale da istiocitosi delle cellule di Lagherhans)

    Energy Technology Data Exchange (ETDEWEB)

    Chigi, Gino; Pastremoli, Alessandro; Pisi, Paolo; Pastremoli, Alfredo [Bologna Univ., Bologna (Italy). Dipartimento di scienze odontomastologiche; Bianchi, Giuseppe [Istituti Ortopedici Rizzoli, Bologna (Italy)

    2005-04-01

    Purpose: The radiological diagnosis of osteolytic lesions of the mandible still constitutes a challenge in some pathological conditions in which the clinical data and the case history are relatively uniform and the radiological picture is lacking in any characteristics. Materials and methods: We reviewed the conventional radiograms of six cases of Langherans' cell histiocytosis (LCH) of the mandible examined over the last ten years. The X-ray examinations were performed in the lateral-oblique projection to allow a view of the horizontal portion of the mandible almost completely free of overlapping images of other bone structures. Results: We identified a series of radiological patterns for these reticulo-endotheliopathies capable of causing granuloma formed by polinuclear eosinophils, plasma cells, lymphocytes, and large mononuclear macrophages with granulopexic and phagocytic activity that proliferate in the bone tissue and can be identified as the Langherans' cells of skin, mucosa, periodontal cavities and bone marrow. The granulomatous tissue penetrates the affected organ elements and, in its spread, it compresses, atrophies, and destroyed the damaged tissue, replacing it. The alterations produced by Langherans' cell histiocytic granuloma are most common in the skeletal system affecting, in order of frequency, the skull, the long bones of the limbs, the foot, the ribs and the spine. The oral mucosa is rarely involved. Conclusions: The radiological investigation of unifocal Langherans' cell histiocytic granuloma of the mandible is essential in the study of perimandibular swelling, although diagnosis is based on biopsy alone. In addition to digital or conventional radiography, other useful examinations are bone scintigraphy, Colour Doppler US, MR and CT, which enables a correct localisation necessary for planning the biopsy and treatment. [Italian] Scopo: La diagnosi radiologica delle lesioni osteolitiche della mandibola costituisce un problema

  14. A review of histiocytic diseases of dogs and cats.

    Science.gov (United States)

    Moore, P F

    2014-01-01

    Histiocytic proliferative disorders are commonly observed in dogs and less often cats. Histiocytic disorders occur in most of the dendritic cell (DC) lineages. Canine cutaneous histiocytoma originates from Langerhans cells (LCs) indicated by expression of CD1a, CD11c/CD18, and E-cadherin. When histiocytomas occur as multiple lesions in skin with optional metastasis to lymph nodes and internal organs, the disease resembles cutaneous Langerhans cell histiocytosis of humans. Langerhans cell disorders do not occur in feline skin. Feline pulmonary LCH has been recognized as a cause of respiratory failure due to diffuse pulmonary infiltration by histiocytes, which express CD18 and E-cadherin and contain Birbeck's granules. In dogs and cats, histiocytic sarcomas (HS) arise from interstitial DCs that occur in most tissues of the body. Histiocytic sarcomas begin as localized lesions, which rapidly disseminate to many organs. Primary sites include spleen, lung, skin, brain (meninges), lymph node, bone marrow, and synovial tissues of limbs. An indolent form of localized HS, progressive histiocytosis, originates in the skin of cats. Hemophagocytic HS originates in splenic red pulp and bone marrow macrophages in dogs and cats. In dogs, histiocytes in hemophagocytic HS express CD11d/CD18, which is a leuko-integrin highly expressed by macrophages in splenic red pulp and bone marrow. Canine reactive histiocytic diseases, systemic histiocytosis (SH) and cutaneous histiocytosis, are complex inflammatory diseases with underlying immune dysregulation. The lesions are dominated by activated interstitial DCs and lymphocytes, which invade vessel walls and extend as vasocentric infiltrates in skin, lymph nodes, and internal organs (SH).

  15. Osseous eosinophilic granuloma in children

    International Nuclear Information System (INIS)

    Leblan, I.; Gaucher, H.; Marinard, L.; Galloy, M.A.; Phi, I.N.; Hoeffel, J.C.

    2001-01-01

    Eosinophilic granuloma of bone or Langerhans cell histiocytosis is mostly uni-focal. It appears on plain X Ray as a solitary destructive lesion of long bones or flat bones. CT is useful to define the extension to the cortical bone and also to precisely localize the lesion when the anatomy is complex (hip, spine, base of the skull). MR is very useful in case of more aggressive lesions when there is extension to soft tissues. Differential diagnosis includes circumscribed osteitis and tumors prognosis is more serious in case of multiple lesions. (authors)

  16. Rheumatoid arthritis and pulmonary nodules: An unexpected final diagnosis.

    Science.gov (United States)

    Zurita Prada, Pablo Antonio; Urrego Laurín, Claudia Lía; Assyaaton Bobo, Sow; Faré García, Regina; Estrada Trigueros, Graciliano; Gallardo Romero, José Manuel; Borrego Pintado, Maria Henar

    We report the case of a 50-year-old female smoker with an 11-year history of seropositive rheumatoid arthritis (rheumatoid factor and anti-cyclic citrullinated peptide antibodies) receiving triple therapy. She developed pulmonary nodules diagnosed as Langerhans cell histiocytosis by lung biopsy. We found no reported cases of the coexistence of these two diseases. Smoking abstinence led to radiologic resolution without modifying the immunosuppressive therapy. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.

  17. Osseous eosinophilic granuloma in children; Le granulome eosinophile des os chez l'enfant

    Energy Technology Data Exchange (ETDEWEB)

    Leblan, I.; Gaucher, H.; Marinard, L.; Galloy, M.A.; Phi, I.N.; Hoeffel, J.C. [Hopital de Brabois-Vandoeuvre, 54 - Nancy (France). Service de radiologie

    2001-02-01

    Eosinophilic granuloma of bone or Langerhans cell histiocytosis is mostly uni-focal. It appears on plain X Ray as a solitary destructive lesion of long bones or flat bones. CT is useful to define the extension to the cortical bone and also to precisely localize the lesion when the anatomy is complex (hip, spine, base of the skull). MR is very useful in case of more aggressive lesions when there is extension to soft tissues. Differential diagnosis includes circumscribed osteitis and tumors prognosis is more serious in case of multiple lesions. (authors)

  18. [Xanthoma disseminatum with asymptomatic multisystem involvement].

    Science.gov (United States)

    Zinoun, M; Hali, F; Marnissi, F; Lazaar, S; Benchikhi, H

    2015-04-01

    Xanthogranulomas belong to non-Langerhans histiocytosis of the second group in the Histiocyte Society classification. They comprise a heterogeneous group of rare entities frequently involving cutaneous tropism. Xanthoma disseminatum belongs to this group of non-Langerhans histiocytosis. We report a case of xanthoma disseminatum (XD) in which localized skin and mucous impairment revealed multisystem involvement. A 28-year-old man presented with a two-year history of progressive yellow-orange and infiltrated xanthomatous papulonodular lesions of the face. Lesions of the oral mucosa and genital region were seen, with no functional repercussions. No ophthalmic or other complications were found. Histopathology showed a dense histiocytic infiltrate within the dermis with Touton giant cells, foamy multinucleated giant cells and inflammatory cells, without necrobiosis. Histiocytes were positive for CD68 but negative for CD1a. Gastric and lung involvement was seen and was confirmed at histology. Bone scintigraphy showed suspicious left ulnar hyperfixation suggesting bone involvement. No monoclonal gammopathy or diabetes insipidus was seen. Our patient was treated with corticosteroids 1mg/kg/day and thalidomide 100 mg/day. The outcome was marked by regression and exfiltration of the cutaneous lesions from the second week of treatment, with subsidence continuing at 3 months. This case involves a very rare form of xanthoma disseminatum. The localized facial skin lesions revealed multifocal non-Langerhans histiocytosis that was in fact asymptomatic. The diagnosis of XD was based on clinical, histological and immunohistochemical criteria. Xanthoma disseminatum is a non-Langerhans histiocytic proliferation first described by Montgomery in 1938. This rare entity is characterized by skin and mucous membrane xanthomatosis in which the facial involvement is common, together with diabetes insipidus and normal lipid metabolism. The prognosis is determined by the presence of mucosal

  19. Solitary eosinophilic granuloma of humerus in a 2-month-old infant: A case report with 3 years follow-up

    Directory of Open Access Journals (Sweden)

    Prateek S Joshi

    2015-01-01

    Full Text Available Eosinophilic granulomais (EG a benign self-limiting disease which belongs to the spectrum of Langerhans cell histiocytosis. It is characterized by single or multiple skeletal lesions involving skull, mandible, ribs, spine and long bones predominately in children <12 years. We report a relatively rare case of left proximal humerus solitary EG in a month old infant who was brought to us with reduced movements of left upper limb and swelling of left shoulder. X-ray revealed osteolytic lesion in left upper humerus. No associated lesions were revealed by other imaging modalities. Open biopsy and curettage of lesion revealed proliferation of histiocytes with an infiltration of eosinophils. Immunohistochemistry was positive for S-100 and CD1a. Hence, diagnosis of solitary EG was made. Baby was followed up every 6 monthly for 3 years. There was no evidence of recurrence or detection of new lesion elsewhere at last follow-up.

  20. A Case Study on Idiopathic Orbital Pseudotumor: Surgery and ...

    African Journals Online (AJOL)

    one year revealed a gradual improvement in the vision of all the patients. The VA of the right eye for all the patients ... Hunt syndrome, pituitary histiocytosis, idiopathic meningitis ... computer tomography (CT) and magnetic resonance imaging ...

  1. Can p63 serve as a biomarker for giant cell tumor of bone? A Moroccan experience

    Directory of Open Access Journals (Sweden)

    Hammas Nawal

    2012-09-01

    Full Text Available Abstract Background Multinucleated giant cell-containing tumors and pseudotumors of bone represent a heterogeneous group of benign and malignant lesions. Differential diagnosis can be challenging, particularly in instances of limited sampling. The purpose of this study was to evaluate the contribution of the P63 in the positive and differential diagnosis of giant cell tumor of bone. Methods This study includes 48 giant cell-containing tumors and pseudotumors of bone. P63 expression was evaluated by immunohistochemistry. Data analysis was performed using Epi-info software and SPSS software package (version 17. Results Immunohistochemical analysis showed a P63 nuclear expression in all giant cell tumors of bone, in 50% of osteoid osteomas, 40% of aneurysmal bone cysts, 37.5% of osteoblastomas, 33.3% of chondromyxoide fibromas, 25% of non ossifiant fibromas and 8.3% of osteosarcomas. Only one case of chondroblastoma was included in this series and expressed p63. No P63 immunoreactivity was detected in any of the cases of central giant cell granulomas or langerhans cells histiocytosis. The sensitivity and negative predictive value (NPV of P63 immunohistochemistry for the diagnosis of giant cell tumor of bone were 100%. The specificity and positive predictive value (PPV were 74.42% and 59.26% respectively. Conclusions This study found not only that GCTOB expresses the P63 but it also shows that this protein may serve as a biomarker for the differential diagnosis between two morphologically similar lesions particularly in instances of limited sampling. Indeed, P63 expression seems to differentiate between giant cell tumor of bone and central giant cell granuloma since the latter does not express P63. Other benign and malignant giant cell-containing lesions express P63, decreasing its specificity as a diagnostic marker, but a strong staining was seen, except a case of chondroblastoma, only in giant cell tumor of bone. Clinical and radiological

  2. Chronic myelomonocytic leukemia masquerading as cutaneous indeterminate dendritic cell tumor: Expanding the spectrum of skin lesions in chronic myelomonocytic leukemia.

    Science.gov (United States)

    Loghavi, Sanam; Curry, Jonathan L; Garcia-Manero, Guillermo; Patel, Keyur P; Xu, Jie; Khoury, Joseph D; Torres-Cabala, Carlos A; Nagarajan, Priyadharsini; Aung, Phyu P; Gibson, Bernard R; Goodwin, Brandon P; Kelly, Brent C; Korivi, Brinda R; Medeiros, L Jeffrey; Prieto, Victor G; Kantarjian, Hagop M; Bueso-Ramos, Carlos E; Tetzlaff, Michael T

    2017-12-01

    Chronic myelomonocytic leukemia (CMML) is a hematopoietic stem cell neoplasm exhibiting both myelodysplastic and myeloproliferative features. Cutaneous involvement by CMML is critical to recognize as it typically is a harbinger of disease progression and an increased incidence of transformation to acute myeloid leukemia. Cutaneous lesions of CMML exhibit heterogeneous histopathologic features that can be challenging to recognize as CMML. We describe a 67-year-old man with a 3-year history of CMML who had been managed on single-agent azacitidine with stable disease before developing splenomegaly and acute onset skin lesions. Examination of these skin lesions revealed a dense infiltrate of histiocytic cells morphologically resembling Langerhans type cells (lacking frank histopathologic atypia), and with the immunophenotype of an indeterminate cell histiocytosis (S100+ CD1a+ and langerin-). Given the history of CMML, next-generation sequencing studies were performed on the skin biopsy. These revealed a KRAS (p.G12R) mutation identical to that seen in the CMML 3 years prior, establishing a clonal relationship between the 2 processes. This case expands the spectrum for and underscores the protean nature of cutaneous involvement by CMML and underscores the importance of heightened vigilance when evaluating skin lesions of CMML patients. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  3. MR evaluation of diabetes insipidus

    International Nuclear Information System (INIS)

    Tien, R.D.; Newton, T.H.; Kucharczyk, J.

    1989-01-01

    The authors have used MR imaging to evaluate the hypothalamic/pituitary region in 21 patients with central diabetes insipidus. Diagnoses included histiocytosis-X, germ cell tumors trauma, metastases, tuberculosis, pituitary abscess, and Erdheim-Chester disease. Gd-DTPA was administered in eight patients. Seventeen of 21 patients had a pituitary infundibular stalk that was thicker than that of normal subjects, but the signal intensity of the abnormal stalk was normal. The pituitary stalk was uniformly thickened in the histiocytosis-X and tuberculosis patients. Asymmetric thickening was seen in the patients with germ cell tumors, metastases, and Erdheim-Chester disease. Pituitary stalk was disrupted in the posterior pituitary lobe was absent in all patients

  4. [Spinocerebellar ataxia type 8: the case of a Spanish family].

    Science.gov (United States)

    Mayo-Cabrero, D; Sánchez-Migallón, M; Cantarero, S; García-Ruiz Espiga, P J; Giménez-Pardo, A; Trujillo-Tiebas, M; Ayuso-García, C

    Dominant autosomic ataxias include a group of neurodegenerative diseases characterized by the abnormal expansion of triplets. Male aged 33, with expansion of the SCA 8 gene (100 repetitions), who presented a clinical picture compatible with a pancerebellar syndrome. The patient had been diagnosed 11 years earlier as suffering from previously of histiocytosis X. A clinico genetic study was conducted on the patient and several members of his family (parents and two sisters). Both sisters and the father were found to be carriers of the expansion (110 and 150 repetitions, respectively), and are currently asymptomatic. There is no relation between the number of repetitions and the age of onset of the disease. The normal interval in our population oscillates between 16 37 repetitions, and the pathological interval has not been well determined. There may be a relation between the SCA 8 form and histiocytosis X.

  5. Eosinophilic granuloma of bone in children

    International Nuclear Information System (INIS)

    Leblan, I.; Gaucher, H.; Hoeffel, J.C.; Arnould, V.; Galloy, M.A.; Mainard, L.

    1995-01-01

    Eosinophilic granuloma of bone or Langerhans cell histiocytosis is mostly unifocal. It appears on plain X Ray as a solitary destructive lesion of long bones or flat bones. Computerized tomography (CT) is useful to define the extension to the cortical bone and also to precisely localize the lesion when the anatomy is complex (hip, spine, base of the skull). Magnetic resonance (MR) is very useful in case of more aggressive lesions when there is extension to soft tissues. Differential diagnosis includes circumscribed osteitis and tumors in the case of extensive destruction. The natural course of solitary lesions is favorable, spontaneously or with therapy. The prognosis is more serious in the case of multiple lesions. (authors)

  6. p53 expression in biopsies from children with Langerhans cell histiocytosis

    DEFF Research Database (Denmark)

    Bank, Micha I; Lundegaard, Pia Rengtved; Carstensen, Henrik

    2002-01-01

    based on CD1a positivity. The slides were stained with p53 antibody and semiquantitatively evaluated using a grading system from 1 to 5 as an estimate for 0% to 20%, 20% to 40%, 40% to 60%, 60% to 80%, and 80% to 100% p53-positive for pathologic Langerhans cells (pLC), respectively. RESULTS: The p53...... protein was expressed in various degrees in pLC in all lesions. The degree of p53 expression could not be correlated to either clinical manifestation or outcome. CONCLUSIONS: An increased expression of p53 in pLC indicates an altered DNA repair control with or without abnormal control of apoptosis....

  7. August 2017 pulmonary case of the month

    Directory of Open Access Journals (Sweden)

    Wesselius LJ

    2017-08-01

    Full Text Available No abstract available. Article truncated after the first page. History of Present Illness: The patient is a 60-year-old woman with dyspnea on exertion when she had a pulmonary embolism following knee surgery 3 years earlier. She smoked 1 pack per day for the past 40 years. She was seen at another hospital and had pulmonary function testing which showed only a DLco which was 66% of predicted. Serologic studies were negative for a rheumatologic disorder. A CT scan was also performed (Figure 1. The CT scan was interpreted as showing a few small nodules and possible very early interstitial lung disease. Which of the following are true? 1. A pulmonary embolism can reduce the DLco; 2. Her CT scan is characteristic of Langerhans cell histiocytosis; 3. Smoking can reduce the DLco; 4. 1 and 3; 5.\tAll of the above …

  8. Mantle cell lymphoma of the larynx: Primary case report

    Directory of Open Access Journals (Sweden)

    Naciri Sarah

    2012-07-01

    Full Text Available Abstract Introduction Primary laryngeal lymphomas are exceedingly rare. Only about a hundred cases have been reported. They consist mainly of non-Hodgkin lymphoma, especially of diffuse large B-cell lymphoma and mucosa-associated lymphoid tissue. We report the first case of a primary laryngeal mantle cell lymphoma. Case presentation We report a case of a primary mantle cell lymphoma of the larynx in a 70-year-old North African non-smoker male. We present a detailed report of his clinical and paraclinical data as well as treatment options. Conclusions Mantle cell lymphoma is a very aggressive lymphoma subset associated with poor prognosis. Laryngeal mantle cell lymphoma is exceedingly rare. To the best of our knowledge, this is the first case to ever be reported.

  9. First experiences from Copenhagen with paediatric single photon emission computed tomography/computed tomography

    DEFF Research Database (Denmark)

    Andersen, Julie Bjerglund; Mortensen, Jann; Bech, Birthe Højlund

    2011-01-01

    bone scintigraphy combined with In-labelled leukocyte study (osteomyelitis), three I-meta-iodobenzylguanidine scintigraphies (neuroblastoma), three In-octreotide scintigraphies (two carcinoid tumours, one Langerhans cell histiocytosis) and one Tc-dimercaptosuccinic acid scintigraphy (suspected renal...

  10. [Pseudo-tumoral and ischemic encephalic Erdheim-Chester disease].

    Science.gov (United States)

    Amezyane, T; Abouzahir, A; Bassou, D; Zoubeir, Y; Hammi, S; Mahassin, F; Ohayon, V; Archane, M-I

    2009-01-01

    Erdheim-Chester disease (ECD) is a rare non-langerhans cell histiocytosis of unknown etiology. It is a multi-systematic xanthogranulomatous infiltration with almost constant bone involvement; the neurological manifestations are not specific and occur in 15-20% of cases. We report the case of a 59-year-old woman hospitalized for a frontal syndrome and right hemiparesis. Imaging revealed a left caudate nucleus process with recent infarct. Cardiovascular involvement and bilateral osteosclerosis of long bones strongly suggested ECD, confirmed after biopsies of the pericardium and bone. Pseudo-tumor encephalic ECD is very rare; the caudate nuclei is an unusual localization; ischemic stroke has been exceptionally described. Prognosis depends largely on the involvement of the central nervous and cardiovascular systems.

  11. A Classic Case of Basal Cell Nevus Syndrome

    Directory of Open Access Journals (Sweden)

    Dattaprasad Dadhe

    2015-01-01

    Full Text Available The basal cell nevus syndrome is an autosomal dominant inherited condition characterized mainly by basal cell carcinomas, multiple keratinizing odontogenic tumors, and other systemic anomalies. As these manifestations do not alter the patient′s lifestyle, most of the cases are diagnosed through oral abnormalities. A classic case of basal cell nevus syndrome fulfilling almost all the major and minor criteria has been reported here.

  12. Squamous cell carcinoma of temporal bone: four case reports

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Jun Ha; Sung, Ki Joon; Sim, Young; Shim, Sue Yoen; Yoon, Byoung Moon [Wonju College of Medicine, Yonsei University, Wonju (Korea, Republic of)

    2000-04-01

    We report the CT findings of four cases of squamous cell carcinoma, paying special attention to the epicenter of the lesion and the pattern of bony destruction. All four patients had a past history of chronic otitis media. Squamous cell carcinoma affected mainly the hypotympanum and inferior wall of the external auditory canal. and in all cases revealed an irregular pattern of bony destruction. Irregular destruction of the tegmen tympani occurred in two cases. In cases of squamous cell carcinoma, CT findings suggesting involvement of the promontory are usually noted. (author)

  13. Squamous cell carcinoma of temporal bone: four case reports

    International Nuclear Information System (INIS)

    Lee, Jun Ha; Sung, Ki Joon; Sim, Young; Shim, Sue Yoen; Yoon, Byoung Moon

    2000-01-01

    We report the CT findings of four cases of squamous cell carcinoma, paying special attention to the epicenter of the lesion and the pattern of bony destruction. All four patients had a past history of chronic otitis media. Squamous cell carcinoma affected mainly the hypotympanum and inferior wall of the external auditory canal. and in all cases revealed an irregular pattern of bony destruction. Irregular destruction of the tegmen tympani occurred in two cases. In cases of squamous cell carcinoma, CT findings suggesting involvement of the promontory are usually noted. (author)

  14. Metastatic giant basal cell carcinoma: a case report.

    Science.gov (United States)

    Bellahammou, Khadija; Lakhdissi, Asmaa; Akkar, Othman; Rais, Fadoua; Naoual, Benhmidou; Elghissassi, Ibrahim; M'rabti, Hind; Errihani, Hassan

    2016-01-01

    Basal cell carcinoma is the most common skin cancer, characterised by a slow growing behavior, metastasis are extremely rare, and it occurs in less than 0, 1% of all cases. Giant basal cell carcinoma is a rare form of basal cell carcinoma, more aggressive and defined as a tumor measuring more than 5 cm at its largest diameter. Only 1% of all basal cell carcinoma develops to a giant basal cell carcinoma, resulting of patient's negligence. Giant basal cell carcinoma is associated with higher potential of metastasis and even death, compared to ordinary basal cell carcinoma. We report a case of giant basal cell carcinoma metastaticin lung occurring in a 79 years old male patient, with a fatal evolution after one course of systemic chemotherapy. Giant basal cell carcinoma is a very rare entity, early detection of these tumors could prevent metastasis occurrence and improve the prognosis of this malignancy.

  15. Laryngeal Rosai-Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy: A Retrospective Study of 5 Cases

    Directory of Open Access Journals (Sweden)

    Yanyan Niu

    2017-01-01

    Full Text Available This study was performed to investigate the clinical manifestations, treatment methods, and prognosis of Rosai-Dorfman disease (RDD with laryngeal involvement. Five clinical cases of RDD with laryngeal involvement diagnosed between 1986 and 2015 were retrospectively analyzed. The laryngeal lesions of these 5 patients mostly involved the glottis and subglottis, with the main symptoms being a hoarse voice and airway obstruction. In addition, the patients mostly exhibited a unilateral or asymmetric onset that was manifested by a laryngeal submucosal nodular mass. The patients were subjected to a regimen of hormone treatment combined with surgical resection. The median follow-up duration was 101 months (8–384 months. One case was lost, and the remaining 4 subjects are alive with disease. The follow-up examinations revealed that 4 subjects had stable laryngeal conditions, whereas one showed minor progression. RDD with laryngeal involvement is clinically rare and differs considerably from classical RDD in age of onset, gender composition, and extranodal involvement. The regimen of hormone treatment combined with surgical resection can stabilize the patient’s general condition and laryngeal lesion. Tracheotomies are recommended for patients with dyspnea. After their conditions stabilize, decannulation can be successfully performed in most cases. This therapeutic regimen generally delivers a good prognosis.

  16. Laryngeal Rosai-Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy): A Retrospective Study of 5 Cases.

    Science.gov (United States)

    Niu, Yanyan; Li, Yongjin; Wang, Jian; Jin, Xiaofeng; Yang, Dahai; Huo, Hong; Li, Wuyi

    2017-01-01

    This study was performed to investigate the clinical manifestations, treatment methods, and prognosis of Rosai-Dorfman disease (RDD) with laryngeal involvement. Five clinical cases of RDD with laryngeal involvement diagnosed between 1986 and 2015 were retrospectively analyzed. The laryngeal lesions of these 5 patients mostly involved the glottis and subglottis, with the main symptoms being a hoarse voice and airway obstruction. In addition, the patients mostly exhibited a unilateral or asymmetric onset that was manifested by a laryngeal submucosal nodular mass. The patients were subjected to a regimen of hormone treatment combined with surgical resection. The median follow-up duration was 101 months (8-384 months). One case was lost, and the remaining 4 subjects are alive with disease. The follow-up examinations revealed that 4 subjects had stable laryngeal conditions, whereas one showed minor progression. RDD with laryngeal involvement is clinically rare and differs considerably from classical RDD in age of onset, gender composition, and extranodal involvement. The regimen of hormone treatment combined with surgical resection can stabilize the patient's general condition and laryngeal lesion. Tracheotomies are recommended for patients with dyspnea. After their conditions stabilize, decannulation can be successfully performed in most cases. This therapeutic regimen generally delivers a good prognosis.

  17. Health related quality of life, cognitive functioning and behaviour problems in children

    NARCIS (Netherlands)

    Vrijmoet-Wiersma, C.M.J.; Kooloos, V.M.; Koopman, H.M.; Kolk, A.M.; van der Laan, I.; Grootenhuis, M.A.; Egeler, R.M.

    2009-01-01

    Background: This study was designed to evaluate generic and disease-specific health-related quality of life (HRQoL), cognitive functioning and behaviour problems of children with Langerhans Cell Histiocytosis (LCH). Furthermore, we investigated which medical determinants and social demographic

  18. Basal cell nevus syndrome: 2 case reports

    International Nuclear Information System (INIS)

    Kim, Jae Duk; Seo, Yo Seob; Kim, Jin Soo

    2008-01-01

    The basal cell nevus syndrome (BCNS) is an autosomal dominant disorder, characterized by basal cell carcinomas, odontogenic keratocysts and skeletal abnormalities. We experienced two cases that represented several characteristics of BCNS. Case 1: a thirty three year-old man visited CSU hospital. His radiographs showed four cystic lesions at both maxillary sinus and both mandibular angle, with bifid rib and ectopic calcification of falx cerebri. After marsupialization and enucleation, recurrent and newly developing tendency were found on his follow-up radiographs. Case 2: a seventeen year-old man had four large cystic lesions which were diagnosed as odontogenic keratocysts. He had craniofacial anomalies which included ectopic calcification and frontal bossing.

  19. Isolated lacrimal gland involvement in Rosai-Dorfman-Destombes disease

    Directory of Open Access Journals (Sweden)

    Gulwani Hanni

    2008-01-01

    Full Text Available Rosai-Dorfman-Destombes (sinus histiocytosis with massive lymphadenopathy disease is an uncommon disease characterized by benign proliferation of histiocytes, with painless lymph node enlargement and frequent extranodal disease. Orbital involvement occurs in 9-11% of cases. However, isolated Rosai-Dorfman-Destombes disease of the lacrimal gland without any systemic involvement is very rare with only three case reports. We describe here one such young male patient with unilateral lacrimal gland swelling. Excision biopsy revealed almost complete replacement of the lacrimal gland by lymphocytes, plasma cells and large pale histiocytes. The latter exhibited emperipolesis and stained positive for S-100 and CD68 on immunohistochemistry. Patient is well and has no other manifestation or recurrence of the disease during a follow-up of 24 months.

  20. General Information about Langerhans Cell Histiocytosis (LCH)

    Science.gov (United States)

    ... speaking. Trouble seeing. Headaches. Changes in behavior or personality. Memory problems. These signs and symptoms may be ... National Institutes of Health FOLLOW US Facebook Twitter Instagram YouTube Google+ LinkedIn GovDelivery RSS CONTACT INFORMATION Contact ...

  1. A young man with multiple pulmonary cysts | Ibrahim | Libyan ...

    African Journals Online (AJOL)

    Several diseases cause cystic or cyst-like parenchymal lung abnormalities including adult pulmonary Langerhan\\'s cell histiocytosis (PLCH), lymphangioleiomyomatosis (LAM), emphysema, end-stage interstitial lung disease, and cystic bronchiectasis. Many of these diseases can now be diagnosed with high accuracy by ...

  2. Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis: response to HLH-04 treatment protocol.

    Science.gov (United States)

    Jiménez-Hernández, Elva; Martínez-Villegas, Octavio; Sánchez-Jara, Berenice; Martínez-Martell, María Angélica; Hernández-Sánchez, Beatriz; Loza-Santiaguillo, Paloma Del Rocío; Pedro-Matías, Eduardo; Arellano-Galindo, José

    Hemophagocytic syndrome, macrophage activation syndrome, reactive histiocytosis or hemophagocytic lymphohistiocytosis (HLH) represent a group of diseases whose common thread is reactive or neoplastic mononuclear phagocytic system cells and dendritic cell proliferation. We present a case of an HLH probably associated with Epstein-Barr virus (EBV) in a 4-year-old male patient treated with HLH-04 protocol. Viral etiology in HLH is well accepted. In this case, clinical picture of HLH was assumed secondary to EBV infection because IgM serology at the time of clinical presentation was the only positive factor in the viral panel. Diagnosis of HLH is the critical first step to successful treatment. The earlier it is identified, the less the tissue damage and reduced risk of multiple organ failure, which favors treatment response. Copyright © 2016 Hospital Infantil de México Federico Gómez. Publicado por Masson Doyma México S.A. All rights reserved.

  3. Giant Cell Angiofibroma in Unusual Localization: A Case Report

    Directory of Open Access Journals (Sweden)

    Emel Ebru Pala

    2012-01-01

    Full Text Available Giant cell angiofibroma (GCA was initially described as a potentially recurrent tumor in the orbit of adults. However, it is now recognized that it can also present in other locations. The morphological hallmark is a richly vascularized patternless spindle cell proliferation containing pseudovascular spaces and floret like multinucleate giant cells. Our case was a 32-years-old female complaining of painless solitary nodule arising on the occipital region of the scalp, which was diagnosed as giant cell angiofibroma. We report the case because of its extremely rare localization.

  4. Case-positive versus case-negative designs for low-rate lithium thionyl chloride cells

    Science.gov (United States)

    Mahy, T. X.

    1982-03-01

    Case polarity design choices are discussed. Two examples of case-negative designs are presented. One battery is thionyl chloride limited and the other is lithium limited. The case-positive design is thionyl chloride limited. It is found that the case-positive/case-negative design consideration does not seem to have much bearing on storage. However, during low rate discharge, the case-negative cells show a steadily decreasing capacity as you go to lower and lower rates.

  5. Interstitial lung diseases with fibrosis - the pattern at high resolution

    International Nuclear Information System (INIS)

    Jarzemska, A.; Lasek, W.; Nawrocka, E.; Meder, G.; Zapala, M.

    2003-01-01

    Surgical lung biopsy, either open thoracotomy or video-assisted thoracoscopy is recommended in the diagnosis of interstitial lung diseases (ILD). In some cases, however, the repetitive pattern of radiological features in high-resolution computed tomography is often sufficient to confirm the diagnosis in a non-invasive manner. The purpose of the study was to determine whether patients with ILD can be selected on the basis of the HRCT pattern. Thin-section CT scans were performed in 40 patients with histologically proven idiopathic interstitial pneumonia (26 patients with usual interstitial pneumonia UIP, 2 patients with desquamative interstitial pneumonia DIP, 2 patients with bronchiolitis obliterans organizing pneumonia BOOP, 2 patients with non-specific interstitial pneumonia NSIP, 11 patients with hypersensitivity pneumonitis, and 3 patients with pulmonary histiocytosis X). The location and the intensity of lesions were taken into consideration. Clinical and histopathological findings were compared. HRCT features of interstitial lung diseases such as nodules and cystic spaces in hypersensitivity pneumonitis and pulmonary histiocytosis, and ground-glass opacities in idiopathic interstitial pneumonias (IIP) were statistically significant for differential diagnosis in ILD cases. Combination of honeycombing and ground-glass opacities found in UIP and nodules found in DIP were also statistically significant features in IIP subtypes diagnosis. In some cases, HRCT patterns of hypersensitivity pneumonitis, pulmonary histiocytosis X and IPF combined with clinical findings allowed for the accurate diagnosis without resorting to lung biopsy. Within a group of idiopathic interstitial pneumonia only in usual interstitial pneumonia characteristic pattern in thin-section CT can be defined. In other subgroups some typical features can imply a diagnosis. (author)

  6. Periurethral granular cell tumor: a case report

    International Nuclear Information System (INIS)

    Kim, Jeong Kon; Choi, Hyo Gyeong; Cho, Kyoung Sik

    1998-01-01

    Granular cell tumors are uncommon soft tissue tumors which arise as solitary or multiple masses. Lesions commonly arise in the head, neck, and chest wall, but can occur in any part of the body. To our knowledge, periurethral granular cell tumor has not been previously reported. We report one such case

  7. A rare case of vulvar squamous cell carcinoma; case presentation

    Directory of Open Access Journals (Sweden)

    Cristina N. Cozma

    2018-05-01

    Full Text Available Objectives. Vulvar cancer is a rare gynecological malignancy, with an incidence of 1.5 per 100 000 women/year. The most common vulvar cancer is developed in squamous cells, the most encountered type of skin cells. Case report. We report a case of a 72-year-old female admitted in the Department of Plastic Surgery of Emergency Clinical Hospital “Prof. Dr. Agrippa Ionescu” with a 5/4.2 cm painful ulcerated tumoral mass located in the vulvar area. The lesion slowly increased in size over the past 12 months. The tumour was surgically removed with oncological safety margins and sent for histopathological evaluation. The histopathological examination revealed an ulcerated squamous carcinoma with lymphovascular and perineural invasion, but with negative margins. Postoperative results were favorable, and no local or general complications were observed. Conclusion. We highlight this case due to its unusual presentation in the clitoral area. Moreover, considering the potential for recurrence we point out the importance of the radical vulvectomy with regional lymphadenectomy and histopathological examination, in order to put a precise diagnosis and ensure the best possible treatment for the patient.

  8. Breast spindle cell tumours: about eight cases

    Directory of Open Access Journals (Sweden)

    Abd El All Howayda S

    2006-07-01

    Full Text Available Abstract Background Breast spindle cell tumours (BSCTs, although rare, represent a heterogeneous group with different treatment modalities. This work was undertaken to evaluate the utility of fine needle aspiration cytology (FNAC, histopathology and immunohistochemistry (IHC in differentiating BSCTs. Methods FNAC of eight breast masses diagnosed cytologically as BSCTs was followed by wide excision biopsy. IHC using a panel of antibodies against vimentin, pan-cytokeratin, s100, desmin, smooth muscle actin, CD34, and CD10 was evaluated to define their nature. Results FNAC defined the tumors as benign (n = 4, suspicious (n = 2 and malignant (n = 3, based on the cytopathological criteria of malignancy. Following wide excision biopsy, the tumors were reclassified into benign (n = 5 and malignant (n = 3. In the benign group, the diagnosis was raised histologically and confirmed by IHC for 3 cases (one spindle cell lipoma, one myofibroblastoma and one leiomyoma. For the remaining two cases, the diagnosis was set up after IHC (one fibromatosis and one spindle cell variant of adenomyoepithelioma. In the malignant group, a leiomyosarcoma was diagnosed histologically, while IHC was crucial to set up the diagnosis of one case of spindle cell carcinoma and one malignant myoepithelioma. Conclusion FNAC in BSCTs is an insufficient tool and should be followed by wide excision biopsy. The latter technique differentiate benign from malignant BSCTs and is able in 50% of the cases to set up the definite diagnosis. IHC is of value to define the nature of different benign lesions and is mandatory in the malignant ones for optimal treatment. Awareness of the different types of BSCTs prevents unnecessary extensive therapeutic regimes.

  9. Height and bone age development in children with a malignant disease

    NARCIS (Netherlands)

    Tamminga, RYJ; Zweens, M; Drayer, NM; Kamps, WA

    2000-01-01

    Because controversy exists about the growth inhibiting effect of chemotherapy, we studied up to 4 years after diagnosis, height, growth and bone age de development in 28 children treated with cytostatic drugs for a solid tumor or Langerhans cell histiocytosis. The results were compared with those

  10. Smoking-related interstitial lung diseases: histopathological and imaging perspectives

    Energy Technology Data Exchange (ETDEWEB)

    Desai, S.R.; Ryan, S.M.; Colby, T.V

    2003-04-01

    The present review focuses on the interstitial lung diseases related to smoking. Thus, the pathology and radiology of Langerhans cell histiocytosis, desquamative interstitial pneumonia, respiratory bronchiolitis and respiratory bronchiolitis-associated-interstitial lung disease are considered. The more tenuous association between pulmonary fibrosis and smoking is also discussed.

  11. Smoking-related interstitial lung diseases: histopathological and imaging perspectives

    International Nuclear Information System (INIS)

    Desai, S.R.; Ryan, S.M.; Colby, T.V.

    2003-01-01

    The present review focuses on the interstitial lung diseases related to smoking. Thus, the pathology and radiology of Langerhans cell histiocytosis, desquamative interstitial pneumonia, respiratory bronchiolitis and respiratory bronchiolitis-associated-interstitial lung disease are considered. The more tenuous association between pulmonary fibrosis and smoking is also discussed

  12. Laryngeal Rosai-Dorfman Disease (Sinus Histiocytosis with Massive Lymphadenopathy): A Retrospective Study of 5 Cases

    OpenAIRE

    Yanyan Niu; Yongjin Li; Jian Wang; Xiaofeng Jin; Dahai Yang; Hong Huo; Wuyi Li

    2017-01-01

    This study was performed to investigate the clinical manifestations, treatment methods, and prognosis of Rosai-Dorfman disease (RDD) with laryngeal involvement. Five clinical cases of RDD with laryngeal involvement diagnosed between 1986 and 2015 were retrospectively analyzed. The laryngeal lesions of these 5 patients mostly involved the glottis and subglottis, with the main symptoms being a hoarse voice and airway obstruction. In addition, the patients mostly exhibited a unilateral or asymme...

  13. Leiomyosarcoma of the skin with osteoclast-like giant cells: a case report

    Directory of Open Access Journals (Sweden)

    Sarma Deba P

    2007-12-01

    Full Text Available Abstract Introduction Osteoclast-like giant cells have been noted in various malignant tumors, such as, carcinomas of pancreas and liver and leiomyosarcomas of non-cutaneous locations, such as, uterus and rectum. We were unable to find any reported case of a leiomyosarcoma of the skin where osteoclast-like giant cells were present in the tumor. Case presentation We report a case of a 59-year-old woman with a cutaneous leiomyosarcoma associated with osteoclast-like giant cells arising from the subcutaneous artery of the leg. The nature of the giant cells is discussed in light of the findings from the immunostaining as well as survey of the literature. Conclusion A rare case of cutaneous leiomyosarcoma with osteoclast-like giant cells is reported. The giant cells in the tumor appear to be reactive histiocytic cells.

  14. Basal Cell Carcinoma in Cases with or without Xeroderma Pigmentosum.

    Science.gov (United States)

    Ghartimagar, Dilasma; Ghosh, Arnab; Shrestha, Sushil Ram; Shrestha, Sachet; Thapa, Sushma; Narasimhan, Raghavan; Talwar, O P

    2017-01-01

    Basal cell carcinoma is the most common form of cancer in humans and comprises the vast majority of skin cancers. It predominantly affects fair-skinned individuals, and its incidence is rapidly increasing. The objective of the study is to identify the epidemiology, its topography and different histological subtypes of basal cell carcinoma in patients with or without Xeroderma Pigmentosum. A cross-sectional descriptive study was conducted at Manipal Teaching Hospital, Pokhara from Jan 2009 to Dec 2016. Ethical approval was taken from MEMG/IRC/GA. The study included patients with a confirmed diagnosis of basal cell carcinoma irrespective of their age and sex. This study showed 77 individuals with 91 biopsies of BCC including 5 cases of Xeroderma Pigmentosum. The predominant histological subtype was nodular with 41 (53.94%) cases, followed by the 14 (18.42%) cases of pigmented and 10 (13.15%) cases baso-squamous subtype. The most frequent sites of involvement were the head and neck, with predominance in the nasal and orbital region. The mean age was 57.68 years but the basal cell carcinoma in cases of Xeroderma Pigmentosum was seen more in younger age groups. There were 43 (55.84 %) male patients and 34 (44.16 %) female patients with a male to female ratio of 1.26:1. Nodular and pigmented varieties were the most frequent subtypes with nose being the commonest site of involvement. Basal cell carcinomas in cases of Xeroderma Pigmentosum were noted in younger age group with multiple lesions.

  15. Myonecrosis in Sickle Cell Anemia: Case Study.

    Science.gov (United States)

    Turaga, Lalita Prabha; Boddu, Prajwal; Kipferl, Steve; Basu, Anupam; Yorath, Martin

    2017-01-30

    BACKGROUND Myonecrosis is one of the more poorly studied, painful manifestations of sickle cell crisis. Medical literature is sparse detailing the manifestations and management of such symptoms. In myonecrosis, red cells containing sickle hemoglobin become rigid, resulting in reduced blood flow and myonecrosis. CASE REPORT We present a case study of a patient in sickle cell crisis with an episode of acute pain and swelling to the intrinsic muscles of the foot as a prominent feature of the crises. Although muscle biopsy is considered the gold standard for the diagnosis of myositis or myonecrosis, a low intensity signal on T1 and high intensity signal on T2 at the affected muscle belly can be as conclusive as imaging studies. In an actively sickling patient any invasive intervention should be avoided as it can result in ischemic necrosis of the tissues, due to interruption of capillary flow in end-arteries. CONCLUSIONS Early recognition is critical in sickle cell disease management, allowing for prompt and aggressive fluid resuscitation which remains a cornerstone in the management of most sickle cell vaso-occlusive crises. In this instance, off loading the extremity and early fluid resuscitation resolved the pain and swelling and prevented myonecrosis.

  16. Primary Small Cell Neuroendocrine Carcinoma of Vagina: A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Jignasa N. Bhalodia

    2011-01-01

    Full Text Available Primary small cell neuroendocrine carcinoma of vagina is an extremely rare disease. There have been only 26 previously reported cases in literature. Here, we report a case of primary small cell neuroendocrine carcinoma of vagina. Immunohistochemistry (IHC showed tumor cells positive for synaptophysin, chromogranin, and neuron-specific enolase (NSE.

  17. Merkel Cell Carcinoma Metastatic to Pleural Fluid: A Case Report

    Directory of Open Access Journals (Sweden)

    Ye-Young Rhee

    2018-05-01

    Full Text Available Merkel cell carcinoma (MCC is a rare aggressive neuroendocrine carcinoma of the skin that shows locoregional or distant metastasis. Metastasis of MCC to body cavity effusion is extremely rare; only three cases have been reported so far. Metastatic MCC in effusion cytology shows small blue round cells with fine stippled chromatin like other small blue round cell tumors such as small cell lung carcinoma or lymphoma. The diagnosis of metastatic MCC can grant patients good chances at recently advanced therapeutic options. Here, we present a case of metastatic MCC to pleural effusion with characteristic single file-like pattern.

  18. Merkel Cell Carcinoma Metastatic to Pleural Fluid: A Case Report.

    Science.gov (United States)

    Rhee, Ye-Young; Kim, Soo Hee; Kim, Eun Kyung; Kim, Se Hoon

    2018-05-01

    Merkel cell carcinoma (MCC) is a rare aggressive neuroendocrine carcinoma of the skin that shows locoregional or distant metastasis. Metastasis of MCC to body cavity effusion is extremely rare; only three cases have been reported so far. Metastatic MCC in effusion cytology shows small blue round cells with fine stippled chromatin like other small blue round cell tumors such as small cell lung carcinoma or lymphoma. The diagnosis of metastatic MCC can grant patients good chances at recently advanced therapeutic options. Here, we present a case of metastatic MCC to pleural effusion with characteristic single file-like pattern.

  19. Rare Case of Duodenal Metastasis From Pulmonary Squamous Cell Carcinoma

    Directory of Open Access Journals (Sweden)

    Zain Memon DO

    2017-10-01

    Full Text Available Pulmonary squamous cell carcinoma is the second most common non–small cell malignancy of the lung. It commonly metastasizes to the adrenal glands, bone, liver, brain, and kidneys. Most occurrences of metastatic squamous cell carcinoma involving the gastrointestinal tract originate from primary lung tumors. Metastasis to the duodenum, however, is exceedingly rare, with very few cases of stomach or duodenal involvement described in the literature. We report the case of a patient with stage IV pulmonary squamous cell carcinoma metastasizing to the duodenum with an uncommon presentation to add to the paucity of literature available regarding this rare finding.

  20. Computed tomography of cystic lung lesions

    International Nuclear Information System (INIS)

    Grgic, A.; Heinrich, M.; Girmann, M.; Kramann, B.; Wilkens, H.; Uder, M.

    2004-01-01

    A cystic lesion in the lung is defined as a well-demarcated epithel-lined cavity, that can be mostly filled with air, water, as well as solid material content. This definition includes a wide variety of diseases such as bronchogenic cyst, abscess formation, lymphangioleiomyomatosis, Langerhans cell histiocytosis, emphysema, bronchiectasis, and pneumatoceles. Despite the difficulties in differential diagnosis, there are some diagnostic criteria for CT-scanning helping the radiologist to differentiate between these cystic entities. Moreover, clinical informations are extremely important. The most important clinical parameters include age, sex, clinical history and symptoms. Thus, a better understanding of classic CT appearance of cystic lung disease will allow more definitive diagnosis and could, in some cases, avoid biopsy. (orig.)

  1. Computed tomography of cystic lung lesions; Computertomographie bei zystischen Lungenerkrankungen

    Energy Technology Data Exchange (ETDEWEB)

    Grgic, A.; Heinrich, M.; Girmann, M.; Kramann, B. [Inst. fuer Radiodiagnostik, Universitaetskliniken des Saarlandes, Homburg/Saar (Germany); Wilkens, H. [Innere Medizin V-Pneumonologie, Universitaetskliniken des Saarlandes, Homburg/Saar (Germany); Uder, M. [Inst. fuer Diagnostische Radiologie, Friedrich-Alexander-Univ. Erlangen Nuernberg (Germany)

    2004-07-01

    A cystic lesion in the lung is defined as a well-demarcated epithel-lined cavity, that can be mostly filled with air, water, as well as solid material content. This definition includes a wide variety of diseases such as bronchogenic cyst, abscess formation, lymphangioleiomyomatosis, Langerhans cell histiocytosis, emphysema, bronchiectasis, and pneumatoceles. Despite the difficulties in differential diagnosis, there are some diagnostic criteria for CT-scanning helping the radiologist to differentiate between these cystic entities. Moreover, clinical informations are extremely important. The most important clinical parameters include age, sex, clinical history and symptoms. Thus, a better understanding of classic CT appearance of cystic lung disease will allow more definitive diagnosis and could, in some cases, avoid biopsy. (orig.)

  2. Case report 429: Adult T-cell leukemia (ATL)

    International Nuclear Information System (INIS)

    Aoki, Jun; Yamamoto, Itsuo; Hino, Megumu; Torizuka, Kanji; Kyoto Univ.; Uchiyama, Takahashi; Uchino, Haruto

    1987-01-01

    The radiological and pathological skeletal manifestations in a case of adult T-cell leukemia are presented. The authors have emphasized the presence of multiple areas of localized subperiosteal resorption as a helpful finding in the differential diagnosis between adult T-cell leukemia and multiple myeloma and hyperparathyroidism. A possible mechanism for these radiological features and its similarity to those of other T-cell malignancies are discussed briefly. (orig./SHA)

  3. Five cases of squamous cell carcinoma induced by irradiation

    International Nuclear Information System (INIS)

    Omoto, Kayo; Tani, Tasaburo; Nagata, Hiroyuki; Kohda, Mamoru; Ueki, Hiroaki

    1985-01-01

    Five cases of squamous cell carcinoma (skin) induced by irradiation are reported. Three cases had been given radiotherapy for benign skin disorders, tinea pedis, lichen Vidal, and dermatitis papillaris capillitis. The other two cases were medical doctors who had developed carcinoma as the result of advanced radiodermatitis. (author)

  4. Imaging findings of juvenile xanthogranuloma of the penis

    Energy Technology Data Exchange (ETDEWEB)

    Bradford, Ray K. [Penn State College of Medicine, Hershey, PA (United States); Hershey Medical Center, Hershey, PA (United States); Choudhary, Arabinda K. [Hershey Medical Center, Department of Radiology, Hershey, PA (United States)

    2009-02-15

    Juvenile xanthogranuloma (JXG) is a rare benign self-limiting lesion presenting in early childhood. It is the commonest variant of non-Langerhans cell histiocytosis and usually presents as a cutaneous mass. It might have a systemic component and also might be associated with other conditions, notably neurofibromatosis and juvenile chronic myelogenous leukemia. Penile masses are unusual in childhood and we describe a case of JXG involving the penis. Although four cases of JXG of the penis have been reported in the literature, this is the first with imaging of the penile lesion. We discuss the clinical and radiological findings, differential diagnosis and management of these cases. High awareness of these lesions in the differential diagnosis of penile masses presenting in early childhood is important to avoid potentially unnecessary ablative genital surgery. Careful assessment should also be made for any systemic involvement and for associated pathologies. (orig.)

  5. Imaging findings of juvenile xanthogranuloma of the penis

    International Nuclear Information System (INIS)

    Bradford, Ray K.; Choudhary, Arabinda K.

    2009-01-01

    Juvenile xanthogranuloma (JXG) is a rare benign self-limiting lesion presenting in early childhood. It is the commonest variant of non-Langerhans cell histiocytosis and usually presents as a cutaneous mass. It might have a systemic component and also might be associated with other conditions, notably neurofibromatosis and juvenile chronic myelogenous leukemia. Penile masses are unusual in childhood and we describe a case of JXG involving the penis. Although four cases of JXG of the penis have been reported in the literature, this is the first with imaging of the penile lesion. We discuss the clinical and radiological findings, differential diagnosis and management of these cases. High awareness of these lesions in the differential diagnosis of penile masses presenting in early childhood is important to avoid potentially unnecessary ablative genital surgery. Careful assessment should also be made for any systemic involvement and for associated pathologies. (orig.)

  6. Nevoid Basal Cell Carcinoma Syndrome : A Case Report

    Directory of Open Access Journals (Sweden)

    K Rajanikanth

    2004-01-01

    Full Text Available The nevoid basal cell carcinoma syndrome (NBCCS or Gorlin - Goltz syndrome is an autosomal disorder principally characterized by cutaneous basal cell carcinomas, multiple keratocysts, and skeletal anomalies. The major organ systems involved are skin, bones, central nervous system, eyes, gonads and endocrine. This particular syndrome is extensively described in the literature under different names. However, there are only few cases reported in the Indian literature. An unusual case of a 33-year old male with large odontogenic keratocyst involving impacted canine in the mandible, along with multiple cysts and impacted teeth in the maxilla; bifid rib and vertebral anomalies has been described.

  7. Peripheral giant cell granuloma: A review of 123 cases

    Directory of Open Access Journals (Sweden)

    Niloofar Shadman

    2009-01-01

    Full Text Available Background: Peripheral giant cell granuloma is one of the reactive hyperplastic lesions of the oral cavity, which originates from the periosteum or periodontal membrane following local irritation or chronic trauma. The purpose of this study was to present the clinical characteristics of peripheral gi-ant cell granuloma in a group of Iranian population. Methods: A series of 123 consecutive confirmed cases of peripheral giant cell granuloma after biopsy were evaluated. Age, sex, anatomic location, consistency, etiologic factor, pain and bleeding history, color, surface texture, and pedicle situation were recorded and were analyzed by chi-square test and values were considered to be significant if P < 0.05. Results: Age ranged from 6 to 75 years (mean 33 years. Women affected more than men (M/F 1:1.1. Peripheral giant cell granuloma was seen in the mandible more than in the maxilla and in the anterior region more than in the posterior region. In most cases, lesions were pink, pedunculated and had non-ulcerated surface. In less than half of the cases, there was no history of bleeding and also pain was rarely reported. Calculus was the most common etiologic factor. Conclusion: The results confirmed that the clinical features of peripheral giant cell granuloma in a group of Iranian population are almost similar to those reported by other investigators.

  8. Basal cell carcinoma of the nipple. Report of two cases.

    Science.gov (United States)

    Cain, R J; Sau, P; Benson, P M

    1990-02-01

    Two cases of basal cell carcinoma of the nipple are presented, bringing the total number of reported cases to 15. The majority, including our two patients, are elderly men. This finding suggests a causal role of exposure to ultraviolet radiation. In our cases excision was curative.

  9. Linear Basal Cell Carcinoma: A Case Report

    Directory of Open Access Journals (Sweden)

    Yuko Ichinokawa

    2011-07-01

    Full Text Available Basal cell carcinoma (BCC presents with diverse clinical features, and several morphologic and histologic variants of BCC have been reported [Sexton et al.: J Am Acad Dermatol 1990;23:1118–1126]. Linear BCC was first described as a new clinical subtype in 1985 by Lewis [Int J Dematol 1985;24:124–125]. Here, we present a case of linear BCC that we recently encountered in an elderly Japanese patient, and review other cases reported in Japan.

  10. Squamous cell carcinoma of the breast : a case report

    NARCIS (Netherlands)

    Flikweert, Elvira R.; Hofstee, Mans; Liem, Mike S. L.

    2008-01-01

    Background: Squamous cells are normally not found inside the breast, so a primary squamous cell carcinoma of the breast is an exceptional phenomenon. There is a possible explanation for these findings. Case presentation: A 72-year-old woman presented with a breast abnormality suspected for breast

  11. Spindle Cell Metaplastic Breast Cancer: Case Report

    Directory of Open Access Journals (Sweden)

    Dursun Ozgur Karakas

    2013-08-01

    Conclusion: Spindle cell metaplastic breast cancer must be considered in differential diagnosis of breast cancers, and preoperative immunohistochemical examination, including cytokeratin and vimentin, must be added to pathological examination in intervening cases. [Arch Clin Exp Surg 2013; 2(4.000: 259-262

  12. Granulosa cell tumor of scrotal tunics: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Ji, Eun Kyung; Cho, Kyoung Sik [Pochon CHA University, Seoul (Korea, Republic of)

    2001-06-01

    We report a case of adult granulosa cell tumor arising in the scrotal tunics. The patient was a 34-year-old man who presented with right scrotal swelling, first noticed four months previously. Under the initial clinical impression of epididymoorchitis, antibiotic treatment was instituted but there was no response. The paratesticular nodules revealed by ultrasound and magnetic resonance imaging mimicked intratesticular lesion, and radical orchiectomy was performed. Although several cases of adult testicular granulosa cell tumor, have been reported, the occurrence of this entity in the paratesticular area has not, as far as we are aware, been previously described.

  13. Langerhans Cells Histiocytosis: Features of Clinical and Laboratory Manifestations and Course of the Disease

    Directory of Open Access Journals (Sweden)

    O.I. Dorosh

    2014-08-01

    Results of the Study. An analysis of 25 cases of LCH in children was presented. Monosystem LCH most often affects the skeletal system. Multisystem LCH is characterized by diversity of clinical manifestations, more severe course and high risk of death. One third of patients with multisystem LCH are infants. In children with monosystem LCH we observed complete clinical response to first-line therapy. At the same time, complete response to polychemotherapy is observed only in 30 % of children with multisystem LCH. Prognosis of the disease depends on the initial affection of risk organs (bone marrow, liver, lungs, spleen, their dysfunction and the child’s age at the time of diagnosis. Process reactivation in children with multisystem LCH occurs in the first 12 months from the onset of the disease.

  14. Genetics Home Reference: histiocytosis-lymphadenopathy plus syndrome

    Science.gov (United States)

    ... body. ENT3 is found in cellular structures called lysosomes , which break down large molecules into smaller ones ... the breakdown of DNA and RNA out of lysosomes into the cell so they can be reused. ...

  15. Squamous cell carcinoma of the lacrimal caruncle : case reports

    NARCIS (Netherlands)

    van de Put, Mathijs A. J.; Haeseker, Barbara I.; De Wolff-Rouendaal, Did; De Keizer, Robert J. W.

    2014-01-01

    Purpose: To report 2 cases of squamous cell carcinoma of the lacrimal caruncle. Methods: Two patients, a 38-year-old man and a 72-year-old woman, presented with a painful mass in the medial angle of the eyelid aperture, with signs of inflammation. Biopsy was performed in both cases. Results:

  16. A rare case of avascular necrosis in sickle cell trait: a case report.

    Science.gov (United States)

    Sanders, William J

    2018-01-01

    Sickle cell trait is usually an asymptomatic presentation of a patient with slightly different hemoglobin molecule makeup than normal. It is similar to a more serious disease, sickle cell disease, in which a person's hemoglobin is mutated in such a way that causes their red blood cells to easily change shape in certain environmental and internal states; this causes red blood cells to adhere to the walls and occlude the lumen of the arteries in which they travel, leading to downstream effects secondary to ischemia. Sickle cell trait does not have these ischemic effects, usually. In this case, a young African American female patient presents to the clinic with severe right hip pain. Her past medical history includes sickle cell trait and asthma. She has not been symptomatic of her asthma for years and is not on therapy for it. The pain has lasted for several months and has not improved with anti-inflammatory medication. There is severe pain with internal and external rotation of the hip. The neurovascularity of the lower extremities is intact bilaterally. MRI of the femur shows stage 2 or 3 avascular necrosis of the femoral head, while X-rays of the femur are unremarkable. Non weight-bearing for several weeks was unsuccessful; shortly thereafter, the patient underwent core decompression of the right femoral head as well as starting bisphosphonates. The patient improved temporarily but regressed shortly thereafter. Her avascular necrosis worsened radiographically over the next several months. At this point, the only other option would be to do a total hip arthroplasty, but the patient may need several more throughout her lifetime due to the lifespan of the artificial replacement. There have only been scarce reports of avascular necrosis in patients with sickle cell trait. This manuscript presents such a case and includes the trials and tribulations associated with its management.

  17. Immunohistochemical detection of the apoptosis-related proteins FADD, FLICE, and FLIP in Langerhans cell histiocytosis

    DEFF Research Database (Denmark)

    Bank, Micha I; Gudbrand, Charlotte; Lundegaard, Pia Rengtved

    2005-01-01

    -apoptotic)-in lesions from LCH patients. Immunohistochemistry was performed on paraffin-embedded tissue specimens from 43 children with LCH. The infiltrates were scored according to the amount of positive pathologic Langerhans cells (pLCs). In all investigated specimens, the majority of the pLCs expressed FADD, active...... FLICE, and FLIP. The clinical outcome of the disease could not be correlated to the expression of the investigated proteins. This study shows a high expression of the apoptosis-related proteins FADD, active FLICE, and FLIP in pLCs. The authors previously showed that pLCs express Fas and Fas ligand...

  18. Annular elastolytic giant cell granuloma of conjunctiva: A case report

    Directory of Open Access Journals (Sweden)

    Karabi Konar

    2014-01-01

    Full Text Available Annular elastolytic giant cell granuloma is a condition characterized histologically by damaged elastic fibers associated with preponderance of giant cells along with absence of necrobiosis, lipid, mucin, and pallisading granuloma. It usually occurs on sun-damaged skin and hence the previous name actinic granuloma. A similar process occurs on the conjunctiva. Over the past three decades only four cases of conjunctival actinic granuloma have been documented. All the previous patients were females with lesions in nasal or temporal bulbar conjunctiva varying 2-3 mm in size. We report a male patient aged 70 years presenting with a 14 mm × 7 mm fleshy mass on right lower bulbar conjunctiva. Clinical differential diagnoses were lymphoma, squamous cell carcinoma in situ and amyloidosis. Surgical excision followed by histopathology confirmed it to be a case of actinic granuloma. This is the first case of isolated conjunctival actinic granuloma of such a large size reported from India.

  19. Urethral metastasis from non-seminomatous germ cell tumor: a case report

    Directory of Open Access Journals (Sweden)

    Joffe Johnathan

    2011-01-01

    Full Text Available Abstract Introduction We present a case of nonseminomatous germ cell tumor of the testes with acute urinary retention secondary to urethral metastasis. This presentation, and similar cases of urethral metastasis from this tumor, have not been reported previously. Case presentation A 35-year-old Caucasian man presented to hospital with a history of acute urinary retention. On examination he was found to have right testicular enlargement with raised β-human chorionic gonadotrophin, serum α-fetoprotein and lactate dehydrogenase levels. He underwent radical left inguinal orchidectomy and histology confirmed a nonseminomatous germ cell tumor of the testes. Cystoscopy carried out due to urinary retention showed penile metastasis and the biopsy confirmed metastatic malignant undifferentiated teratoma. Staging computed tomography scan and magnetic resonance imaging of the pelvis showed pulmonary, pelvic nodal, ischial and penile metastasis. The diagnosis of the International Germ Cell Cancer Collaborative Group of poor prognosis metastatic nonseminomatous germ cell tumor was made, following which he received four cycles of bleomycin, etoposide and cisplatin chemotherapy with curative intent. He had a complete marker and an excellent radiological response. He is currently under follow up. Conclusion The unusual presentation of lymphovascular spread in this case of nonseminomatous germ cell tumor highlights the need to include routine pelvic imaging in the assessment and follow up of testicular cancer.

  20. Spindle Cell Carcinoma of Nasal Cavity- A Case Report

    Science.gov (United States)

    Mittal, Abhishek; Nagpal, Tapan

    2016-01-01

    Spindle Cell Carcinoma (SpCC), also known as Sarcomatoid Carcinoma, is a rare and peculiar biphasic malignant neoplasm that occurs mainly in the upper aero-digestive tract, mostly in larynx. SCC accounts for 3% of all squamous cell carcinomas (SCCs) in the head and neck region. It is a rare variant of SCC which shows spindled or pleomorphic tumour cells simulating a true sarcoma. We present a case report of SpCC nasal cavity in a 50-year-old female patient, presented with intermittent epistaxis from left nasal cavity. On physical examination, the patient had an ulcero-exophytic type of mass in the left nasal cavity and a smooth bulge on the left side of anterior hard palate. Patient underwent excision of nasal mass along with partial palatectomy by facial degloving approach and reconstruction of palate with naso-labial flap. The postoperative histopathological report showed SCC. Surgery forms the mainstay of treatment. Radiotherapy and Chemotherapy is warranted in order to improve treatment results. As only few cases have been reported, we report a case of this rare entity to contribute for better understanding and awareness of this rare malignancy. PMID:27190843

  1. Occurrence of mesothelial/monocytic incidental cardiac excrescences in material from open-heart procedures: case reports and literature review.

    Science.gov (United States)

    Grossi, Vitor Gabriel Ribeiro; Lee, Karen; Demarchi, Léa Maria; Castelli, Jussara Bianchi; Aiello, Vera Demarchi

    2018-01-01

    Mesothelial/monocytic incidental cardiac excrescences (MICE) are unusual findings during the histological analysis of material from the pericardium, mediastinum, or other tissues collected in open-heart surgery. Despite their somewhat worrisome histological appearance, they show a benign clinical course, and further treatment is virtually never necessary. Hence, the importance of recognizing the entity relays in its differential diagnosis, as an unaware medical pathologist may misinterpret it for a malignant neoplasm. Other mesothelial and histiocytic proliferative lesions, sharing very close histological morphology and immunohistochemistry features with MICE, have been described in sites other than the heart or the mediastinum. This similarity has led to the proposal of the common denomination "histiocytosis with raisinoid nuclei." We report three cases from the pathology archives of the Heart Institute of São Paulo University (Incor/HC-FMUSP), diagnosed as "mesothelial/monocytic incidental cardiac excrescence," with immunohistochemical documentation, and provide a literature review of this entity.

  2. Breast implant capsule-associated squamous cell carcinoma: a report of 2 cases.

    Science.gov (United States)

    Olsen, Daniel L; Keeney, Gary L; Chen, Beiyun; Visscher, Daniel W; Carter, Jodi M

    2017-09-01

    The use of prosthetic implants for breast augmentation has become commonplace. Although implants do not increase the risk of conventional mammary carcinoma, they are rarely associated with anaplastic large cell lymphoma. We report 2 cases of breast implant capsule-associated squamous cell carcinoma with poor clinical outcomes. Both patients (56-year-old woman and 81-year-old woman) had long-standing implants (>25 years) and presented with acute unilateral breast enlargement. In both cases, squamous cell carcinoma arose in (focally dysplastic) squamous epithelium-lined breast implant capsules and widely invaded surrounding breast parenchyma or chest wall. Neither patient had evidence of a primary mammary carcinoma or squamous cell carcinoma at any other anatomic site. Within 1 year, one patient developed extensive, treatment-refractory, locoregional soft tissue metastasis, and the second patient developed hepatic and soft tissue metastases and died of disease. There are 2 prior reported cases of implant-associated squamous cell carcinoma in the plastic surgery literature; one provides no pathologic staging or outcome information, and the second case was a capsule-confined squamous cell carcinoma. Together, all 4 cases share notable commonalities: the patients had long-standing breast implants and presented with acute unilateral breast pain and enlargement secondary to tumors arising on the posterior aspect of squamous epithelialized implant capsules. Because of both its rarity and its unusual clinical presentation, implant capsule-associated squamous cell carcinoma may be underrecognized. The aggressive behavior of the tumors in this series underscores the importance of excluding malignancy in patients with long-standing breast implants who present with acute unilateral breast pain and enlargement. Copyright © 2017 Elsevier Inc. All rights reserved.

  3. Squamous cell carcinoma of the breast: a case report

    Directory of Open Access Journals (Sweden)

    Hofstee Mans

    2008-12-01

    Full Text Available Abstract Background Squamous cells are normally not found inside the breast, so a primary squamous cell carcinoma of the breast is an exceptional phenomenon. There is a possible explanation for these findings. Case presentation A 72-year-old woman presented with a breast abnormality suspected for breast carcinoma. After the operation the pathological examination revealed a primary squamous cell carcinoma of the breast. Conclusion The presentation of squamous cell carcinoma could be similar to that of an adenocarcinoma. However, a squamous cell carcinoma of the breast could also develop from a complicated breast cyst or abscess. Therefore, pathological examination of these apparent benign abnormalities is mandatory.

  4. Usefulness of [18F]FDG-PET in diagnosis of bone and soft tissue tumors. Study with multi-center survey by questionnaire

    International Nuclear Information System (INIS)

    Kato, Katsuhiko; Hosono, Makoto; Okada, Masahiro; Komeya, Yoshihiro; Im, Sung-Woon; Tsuchiya, Norio; Ito, Kengo; Torizuka, Kanji

    2008-01-01

    In the aspect of future additional approval of [ 18 F]fluorodeoxyglucose positron emission tomography (FDG-PET) diagnosis of the title tumors in the health insurance, its usefulness was studied by questionnaire to 18 facilities, where PET had been conducted for those tumors in the period July, 2005-February, 2006. Major questions concerned the purpose and finding of PET, findings by other imaging and by tumor markers, and judgment of PET effectiveness compared with other imaging (more useful, equally or less, and its reason). Subjects were 75 cases (42 males, 33 females; 3-82 years old) in 20 diseases, which involved 21 cases of osteosarcoma, 7 of leiomyosarcoma, 8 of Ewing sarcoma, 6 of liposarcoma, 5 of hemangiosarcoma, 4 of synovial sarcoma, each 3 of rhabdomyosarcoma, giant cell tumor, Schwannoma, malignant fibrous histiocytoma, each 2 of chondrosarcoma, alveolar soft part sarcoma, each one of epithelioid sarcoma, endometrial storomal sarcoma, hibernoma, fibrosarcoma, multiple osteochondroma, sacral chondroma, Langerhans cell histiocytosis and neurofibromatosis. Obtained were the judgments of highly useful in 5 diseases, fairly useful in 4, useful in 3, and useful/inconclusive due to the only one case in 8. FDG-PET was thus found useful in all diseases examined. (R.T.)

  5. Squamous Cell Carcinoma in African Children with Xeroderma Pigmentosum: Three Case Reports.

    Science.gov (United States)

    Kaloga, Mamadou; Dioussé, Pauline; Diatta, Boubacar Ahy; Bammo, Mariama; Kourouma, Sarah; Diabate, Almamy; Gueye, Ndiaga; Dione, Haby; Diallo, Moussa; Diop, Bernard Marcel

    2016-01-01

    Xeroderma pigmentosum is a rare autosomal recessive genetic disease. This disease predisposes patients to early-onset skin cancers, particularly squamous cell carcinoma. Here, we report 3 pediatric cases, including 2 deaths. The subjects included 2 boys and 1 girl with skin type VI. All subjects were from consanguineous marriages, and the average age was 7.6 years. The patients all had ulcerative budding tumor lesions in the cephalic region, and the mean disease duration was 18 months. In all 3 cases, the diagnosis of xeroderma pigmentosum was made before the poikilodermal appearance of sun-exposed areas and photophobia. Neurological-type mental retardation was noted in 1 case. Histology confirmed squamous cell carcinoma in all 3 cases. The evolutions were marked by the death of 2 children (cases 1 and 3). In one case, the outcome was favorable following cancer excision and subsequent chemotherapy with adjuvant radiotherapy. Squamous cell carcinoma is a serious complication related to xeroderma pigmentosum in Sub-Saharan Africa. Prevention is based on the early diagnosis of xeroderma pigmentosum, black skin photoprotection, screening and early treatment of lesions, and genetic counseling.

  6. A case report of the clear cell variant of gallbladder carcinoma.

    Science.gov (United States)

    Maharaj, Ravi; Cave, Christo; Sarran, Kevin; Bascombe, Nigel; Dan, Dilip; Greaves, Wesley; Warner, Wayne A

    2017-01-01

    Clear cell gallbladder carcinoma accounts for less than 1% of all gallbladder malignancies and demonstrates its unique histopathological characteristics in patients with no prior medical illness or familial predisposition. Here we present a case of a 56-year-old female, with no prior medical conditions presented with a 2-month history of upper abdominal pain. Routine hematological and biochemical tests were unremarkable. An abdominal ultrasound revealed the presence of a gallbladder calculi, and a fundic mass while magnetic resonance cholangiopancreatography revealed a 8.0cm×3.5cm gallbladder mass. Computed tomography imaging excluded any distant haematogenous metastases. An open cholecystectomy with lymphadenectomy was proceeded by staging laparoscopy. Upon pathologic investigation, the morphologic and immunophenotypic features supported a diagnosis of clear cell variant of gallbladder carcinoma. Pathological prognostications for primary clear cell gall bladder carcinomas are not well defined due to the rarity of cases and possible misidentification as secondary metastases. Foci of adenocarcinoma within the tumor along with immunohistochemical staining probes can be informative in consideration of differential diagnosis. In these cases, clinical case management should be personalized for increased survival with the possible incorporation of next generation sequencing approaches to guide therapeutic algorithms. We discuss this exceedingly rare case of the clear cell variant of gallbladder carcinoma in detail, highlighting some of the diagnostic, and clinical challenges. Copyright © 2017 The Authors. Published by Elsevier Ltd.. All rights reserved.

  7. The Evolution of Biochemical Indices After Basal Cell Epithelioma Removal - Case Report

    Directory of Open Access Journals (Sweden)

    Gurgas L.

    2017-05-01

    Full Text Available The paper proposes new exposure data on etiopathogenesis basal cell epithelioma and present a clinical case investigated dermatoscopic, biochemically, treated surgically and guided to avoid relapses. The case presented is part of typical cases of pigmented basal cell carcinoma. Biochemical and haematological investigations performed one day before the excisional intervention (results 1 and 30 days (results 2 after the intervention: It is recommended to monitor biochemical investigations in which alterations were found, and ways for raising the immunological status.

  8. Squamous cell carcinoma of the mastoid: a report of two cases

    International Nuclear Information System (INIS)

    Lasisi, O. A.; Ogunleye, A.O.; Akang, E.E.U.

    2005-01-01

    Malignant tumours of the mastoid are rare, the majority being squamous cell carcinomas. We report two cases whose clinical presentation mimicked mastoid abscess with intracranial complications. The first case is a twenty year Nigerian lady who presented to the Emergency Room of the Otorhinolaryngology Department with a one month history of headache, low grade fever, left facial palsy, neck stiffness and left post-auricular swelling on a background of left chronic suppurative otitis media since childhood. An initial diagnosis of meningitis and mastoid abscess secondary to chronic suppurative otitis media was made but histology of the mastoid specimen revealed keratinizing squamous cell carcinoma, which was treated with palliative primary radiotherapy. The second case is a 45-year old man with a chronic mastoiditis and mastoid abscess that was later found to be squamous cell carcinoma of the mastoid and was managed with combination of surgery and radiotherapy. The report highlights advanced stage of the disease at presentation, and discusses the etiology and management. To the best of our knowledge, these are the first cases of this entity to be documented in Africans. (au)

  9. A case report of giant cell reparative granuloma

    Energy Technology Data Exchange (ETDEWEB)

    Park, Chang Sik; Lee, Yoo Dong [College of Dentistry, Seoul National University, Seoul (Korea, Republic of)

    1974-11-15

    The authors observed in the routine roentgenographic examination, a rare case of Giant cell Reparative Granuloma found in the mandible of woman 23 years of age who had visited Infirmary of Dental College, Seoul National University be cause of the traffic accident. In the serial roentgenograms, authors had obtained the results as follows; 1. Giant cell Reparative Granuloma occurred below the 20 years of age, and occurred in the mandible of female. 2. In roentgenograms, it figures the radiolucent lesion with multilocular appearance. 3. The growing process of Giant Cell Reparative Granuloma is not by the neoplastic reaction but by the local reparative reaction.

  10. Langerhan’s cell sarcoma: two case reports

    Directory of Open Access Journals (Sweden)

    Tasneem A. Kaleem

    2016-04-01

    Full Text Available Langerhan’s cell sarcoma (LCS is a rare neoplasm with a poor prognosis. To our knowledge, only sixty-six cases have been published. We discuss two patients who presented very differently with LCS, as well as a recently published review of all sixty-six cases. Our first case had a complicated history of metastatic, high-grade myxofibrosarcomas and presented with a single skin lesion of LCS which was treated with resection to a positive margin and adjuvant radiotherapy. The LCS recurred locoregionally and was again resected. The patient is alive two years after initial diagnosis. The second case presented with bone marrow and splenic involvement, leukocytosis, and thrombocytopenia. This patient had an excellent response to etoposide, prednisone, oncovorin, cyclophosphamide, and adriamycin, with normalization of the complete blood count, negative bone marrow biopsy at follow up, and splenectomy without viable neoplasm. This patient is alive without signs of disease at 16 months after initial diagnosis.

  11. Intratubular large cell hyalinizing Sertoli cell tumor of the testis presenting with prepubertal gynecomastia: a case report.

    Science.gov (United States)

    Tuhan, Hale; Abaci, Ayhan; Sarsık, Banu; Öztürk, Tülay; Olguner, Mustafa; Catli, Gonul; Anik, Ahmet; Olgun, Nur; Bober, Ece

    2017-08-01

    Intratubular large cell hyalinizing Sertoli cell neoplasia (ITLCHSCN) resulting from Sertoli cells of the testis are mainly reported in young adults and these are rarely seen in childhood. The most common presenting symptoms of the patients diagnosed with ITLCHSCN are gynecomastia, enlargement in the testicles, increase in growth velocity, and advanced bone age. Symptoms are basically resulting from increased aromatase enzyme activity in Sertoli cells. In this case report, an eight-and-a-half-year-old case presenting with complaint of bilateral gynecomastia since two years, showing no endocrine abnormality in laboratory during two years of follow-up, determined to have progression in bilateral gynecomastia, increase in testicular volumes, advanced bone age, increase in growth velocity in the clinical follow-up, and diagnosed with ITLCHSCN after testis biopsy was presented.

  12. Spindle-cell carcinoma of esophagus: a case report

    International Nuclear Information System (INIS)

    Kim, Ji Chang; Lee, Jae Mun; Jung, Seung Eun; Lee, Kyo Young; Hahn, Seong Tai; Kim, Man Deuk

    2001-01-01

    Spindle-cell carcinoma of the esophagus is a rare malignant tumor composed of both carcinomatous and sarcomatous elements, and has generated many terminology problems. It is characterized by a bulky polypoid intraluminal mass with a lobulated surface located in the middle third of the esophagus. Local expansion of this organ is observed. The lesion may be pedunculated but despite its bulk, causes little obstruction. We report the imaging findings of a case of spindle-cell carcinoma arising in the upper esophagus

  13. A case report of the clear cell variant of gallbladder carcinoma

    Directory of Open Access Journals (Sweden)

    Ravi Maharaj

    2017-01-01

    Conclusion: In these cases, clinical case management should be personalized for increased survival with the possible incorporation of next generation sequencing approaches to guide therapeutic algorithms. We discuss this exceedingly rare case of the clear cell variant of gallbladder carcinoma in detail, highlighting some of the diagnostic, and clinical challenges.

  14. Breast Metastasis from Renal Cell Carcinoma: A Case Report

    International Nuclear Information System (INIS)

    Kim, Seon Jeong; Kim, Ji Young; Jeong, Myeong Ja; Kim, Jae Hyung; Kim, Soung Hee; Kim, Soo Hyun; Jun, Woo Sun; Kim, Hyun Jung; Han, Se Hwan

    2010-01-01

    Metastatic breast cancer from renal cell carcinoma is extremely rare and has non-specific findings that include a well circumscribed lesion without calcification on mammography and a well circumscribed hypoechoic lesion without posterior acoustic shadowing on sonography. We report a case of metastatic breast cancer from renal cell carcinoma and describe the radiologic findings in a 63-year-old woman who has no history of primary neoplasm

  15. Breast Metastasis from Renal Cell Carcinoma: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Seon Jeong; Kim, Ji Young; Jeong, Myeong Ja; Kim, Jae Hyung; Kim, Soung Hee; Kim, Soo Hyun; Jun, Woo Sun; Kim, Hyun Jung; Han, Se Hwan [Sanggye Paik Hospital, Seoul (Korea, Republic of)

    2010-01-15

    Metastatic breast cancer from renal cell carcinoma is extremely rare and has non-specific findings that include a well circumscribed lesion without calcification on mammography and a well circumscribed hypoechoic lesion without posterior acoustic shadowing on sonography. We report a case of metastatic breast cancer from renal cell carcinoma and describe the radiologic findings in a 63-year-old woman who has no history of primary neoplasm.

  16. MR imaging for evaluation of lesions of the cranial vault: a pictorial essay

    Energy Technology Data Exchange (ETDEWEB)

    Amaral, Lazaro; Chiurciu, Miriam; Almeida, Joao Ricardo; Ferreira, Nelson Fortes; Mendonca, Renato; Lima, Sergio Santos [Hospital da Beneficiencia Portuguesa, Sao Paulo, SP (Brazil). MEDIMAGEM]. E-mail: lazden@terra.com.br

    2003-09-01

    A variety of diseases affect the calvaria. They may be identified clinically as palpable masses or incidentally in radiologic examinations. There are many diagnostic possibilities, including congenital, neoplastic, inflammatory and traumatic lesions. The purpose of this study is to illustrate the main calvarial lesions through MR imaging, their signal intensity and extension to neighboring sites. A retrospective analysis of 81 cases, from November 1996 to July 2001, was conducted. The examinations were performed on a 1.5 T equipment and each one of the cases was pathologically proven. The results were: dermoid cysts [4 cases (5%)], epidermoid cysts [2 cases (2.5%)], cephalocele [14 cases (17.5%)], sinus pericranii [3 cases (3.7%)], leptomeningeal cysts [4 cases (5%)], Langerhans cell histiocytosis [10 cases (12.5%)], lipoma [4 cases (5%)], fibrous dysplasia [13 cases (16.2%)], osteoma [8 cases (10%)], hemangioma [1 case (1.2%)], meningioma [3 cases (3.7%)], chondrosarcoma [5 cases (6.2%)], hemangiosarcoma [1 case (1.2%)], multiple myeloma [3 cases (3.7%)], sarcomatous transformation of Paget disease [1 case (1.3%)], and metastasis [5 cases (6.2%)]. MRI identifies bone marrow abnormalities and invasion of adjacent tissues at an early stage. Therefore, it is an essential method when it comes to properly evaluating calvarial lesions. (author)

  17. MR imaging for evaluation of lesions of the cranial vault: a pictorial essay

    International Nuclear Information System (INIS)

    Amaral, Lazaro; Chiurciu, Miriam; Almeida, Joao Ricardo; Ferreira, Nelson Fortes; Mendonca, Renato; Lima, Sergio Santos

    2003-01-01

    A variety of diseases affect the calvaria. They may be identified clinically as palpable masses or incidentally in radiologic examinations. There are many diagnostic possibilities, including congenital, neoplastic, inflammatory and traumatic lesions. The purpose of this study is to illustrate the main calvarial lesions through MR imaging, their signal intensity and extension to neighboring sites. A retrospective analysis of 81 cases, from November 1996 to July 2001, was conducted. The examinations were performed on a 1.5 T equipment and each one of the cases was pathologically proven. The results were: dermoid cysts [4 cases (5%)], epidermoid cysts [2 cases (2.5%)], cephalocele [14 cases (17.5%)], sinus pericranii [3 cases (3.7%)], leptomeningeal cysts [4 cases (5%)], Langerhans cell histiocytosis [10 cases (12.5%)], lipoma [4 cases (5%)], fibrous dysplasia [13 cases (16.2%)], osteoma [8 cases (10%)], hemangioma [1 case (1.2%)], meningioma [3 cases (3.7%)], chondrosarcoma [5 cases (6.2%)], hemangiosarcoma [1 case (1.2%)], multiple myeloma [3 cases (3.7%)], sarcomatous transformation of Paget disease [1 case (1.3%)], and metastasis [5 cases (6.2%)]. MRI identifies bone marrow abnormalities and invasion of adjacent tissues at an early stage. Therefore, it is an essential method when it comes to properly evaluating calvarial lesions. (author)

  18. Giant cell tumor of the frontal sinus: case report

    Energy Technology Data Exchange (ETDEWEB)

    Matushita, Joao Paulo, E-mail: jpauloejulieta@gmail.com [Universidade Federal de Minas Gerais (UFMG), Belo Horizonte, MG (Brazil). Hospital das Clinicas; Matushita, Julieta S.; Matushita Junior, Joao Paulo Kawaoka [Centro de Diagnostico por Imagem Dr. Matsushita, Belo Horizonte, MG (Brazil); Matushita, Cristina S. [Universidade Federal do Rio de Janeiro (UFRJ), RJ (Brazil). Hospital Universitario Clementino Fraga Filho; Simoes, Luiz Antonio Monteiro; Carvalho Neto, Lizando Franco de

    2013-06-15

    The authors report the case of a giant cell tumor of the frontal sinus in a 54-year-old male patient. This tumor location is rare, and this is the third case reported in the literature with radiographic documentation and histopathological confirmation. The patient underwent surgery, with curettage of frontal sinus and placement of a prosthesis. He died because a voluntary abrupt discontinuation of corticosteroids. (author)

  19. Splenic littoral cell angioma. Radio pathological correlation in two cases

    International Nuclear Information System (INIS)

    Asensio, J.; Montero, N.; Perez-Cidoncha, P.

    2000-01-01

    We present two cases of Littoral Cell Angiomas (LCA), a recently described variant of splenic angioma which originates in the cells that line the sinusoids from the red pulp (littoral cell). The histopathological and immunohistochemical characteristics of this neoplasm verifies its origin in the littoral cell with an intermediate origin between the endothelial and histiocyte cell and makes it possible to consider it as a pathological entity which is differentiated from the hemangiomas. The imaging findings are indistinguishable from the other splenic vascular neoplasms and the role of the Magnetic Resonance (MRI) stands out. (Author) 21 refs

  20. Imaging Findings of Embryonal Cell Carcinoma in Ovary:A Case Report

    International Nuclear Information System (INIS)

    Kim, Hee Kyung; Park, Cheol Min; Choi, Jae Woong; Seol, Hae Young; Kim, Kyeong Ah

    2004-01-01

    Embryonal cell carcinoma is one of the malignant germ cell tumors. This tumor is commonly encountered in the testis, however, it rarely occurs in the ovary. To the best of our knowledge, no imaging findings of ovarian embryonal cell carcinoma have previously been reported. We describe the US and MRI findings of such a case

  1. Percutaneous vertebroplasty in osteoporosis, myeloma and Langerhans' cell histiocytosis.

    LENUS (Irish Health Repository)

    Kevane, B

    2012-01-09

    This review aims to assess the effectiveness of percutaneous vertebroplasty as a treatment for the severe refractory pain associated with vertebral fracture, in a group of patients with fractures secondary to either osteoporotic or neoplastic disease. A retrospective review of 20 patients treated with percutaneous vertebroplasty in Cork University Hospital up until March 2007 was carried out and a questionnaire was prepared and distributed. Prior to vertebroplasty, patients had been symptomatic with severe pain for a mean of 20.9 weeks. Of those thirteen whom replied to a postal questionnaire, 12 (92.3%) reported pain relief and this improvement occurred within 7 days in 9 (81.8%). This was associated with decreased analgesic requirements, as determined on chart review. Prior to the procedure only 5 (38.4%) were independently mobile and this figure rose to 10 (76.9%) afterwards, occurring within one week in the majority. Subjective outcomes were better in the group of patients with neoplasm-induced fractures.

  2. Feasibility of combination allogeneic stem cell therapy for spinal cord injury: a case report

    Directory of Open Access Journals (Sweden)

    Ichim Thomas E

    2010-11-01

    Full Text Available Abstract Cellular therapy for spinal cord injury (SCI is overviewed focusing on bone marrow mononuclear cells, olfactory ensheathing cells, and mesenchymal stem cells. A case is made for the possibility of combining cell types, as well as for allogeneic use. We report the case of 29 year old male who suffered a crush fracture of the L1 vertebral body, lacking lower sensorimotor function, being a score A on the ASIA scale. Stem cell therapy comprised of intrathecal administration of allogeneic umbilical cord blood ex-vivo expanded CD34 and umbilical cord matrix MSC was performed 5 months, 8 months, and 14 months after injury. Cell administration was well tolerated with no adverse effects observed. Neuropathic pain subsided from intermittent 10/10 to once a week 3/10 VAS. Recovery of muscle, bowel and sexual function was noted, along with a decrease in ASIA score to "D". This case supports further investigation into allogeneic-based stem cell therapies for SCI.

  3. Bronchoalveolar lavage: role in the pathogenesis, diagnosis, and management of interstitial lung disease

    International Nuclear Information System (INIS)

    Daniele, R.P.; Elias, J.A.; Epstein, P.E.; Rossman, M.D.

    1985-01-01

    Bronchoalveolar lavage has emerged as a useful technique for the study of pulmonary interstitial disorders. Several types of information are provided by the evaluation of lavage fluid. First, the identification of cellular constituents helps to separate inflammatory processes in which lymphocytes predominate (for example, sarcoidosis, hypersensitivity pneumonitis, and berylliosis) from those in which neutrophils or macrophages predominate (for example, idiopathic pulmonary fibrosis and histiocytosis X). Second, the cells removed during lavage can be studied for their immune properties and function; tested with specific antigens, in diseases such as berylliosis and hypersensitivity pneumonitis; and examined for the presence of unique surface antigens with monoclonal antibodies (for example, histiocytosis X). Third, in conjunction with scanning electron microscopy and electron probe analysis, lavage makes possible the identification of inorganic particles in alveolar macrophages of patients with pneumoconiotic lung disease. Finally, although lavage is still an investigative procedure for most pulmonary disorders, it has an established role in the diagnosis of opportunistic infections in the immunocompromised patient

  4. Conjunctival mass as an initial presentation of mantle cell lymphoma: a case report

    Directory of Open Access Journals (Sweden)

    Khanlari Mahsa

    2012-12-01

    Full Text Available Abstract Background To describe a rare manifestation of mantle cell lymphoma (MCL in conjunctiva, with clinical, hisologic, immunohistologic and genetic findings together with review of the Literature. Case presentation Most ocular adnexal lymphomas are extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue (MALT. A few cases of ocular adnexal mantle cell lymphomas have been reported in the literature. We present a case of mantle cell lymphoma presenting as right conjunctival mass of at least three months duration in a 64-year-old man. Histopathologic examination showed a proliferation of monomorphous small-to-medium-sized lymphoid cells with cleaved nuclei in the subconjunctiva. By immunohistochemistry, the infiltrate was positive for CD20, CD5, BCL-2, cyclin D1, and the transcription factor SOX11. Fluorescent in situ hybridization demonstrated the presence of IGH-CCND1 fusion indicating t(11;14. Conclusion A rigorous approach to initial diagnosis and staging of small cell lymphomas of the ocular adnexa is needed. The recognition of ocular MCL requires appropriate immunohistochemical staining and/or genetic confirmation to differentiate this rare form of presentation of MCL from other more frequent small cell lymphomas.

  5. Ischial apophyseal fracture in an abused infant

    International Nuclear Information System (INIS)

    Bixby, Sarah D.; Kleinman, Paul K.; Wilson, Celeste R.; Barber, Ignasi

    2014-01-01

    We report a previously healthy 4-month-old who presented to the hospital with leg pain and swelling and no history of trauma. Radiographs demonstrated a comminuted left femur fracture. Given the concern for child abuse, skeletal survey was performed and revealed four vertebral compression deformities. Although abuse was suspected, the possibility of a lytic lesion associated with the femur fracture and multiple spinal abnormalities raised the possibility of an underlying process such as Langerhans cell histiocytosis. Subsequently 18F-NaF positron emission tomographic (PET) scintigraphy revealed increased tracer activity in the ischium, and MRI confirmed an ischial apophyseal fracture. Pelvic fractures, particularly ischial fractures, are extremely rare in the setting of child abuse. This case report describes the multimodality imaging findings of an ischial fracture in an abused infant. (orig.)

  6. Ischial apophyseal fracture in an abused infant

    Energy Technology Data Exchange (ETDEWEB)

    Bixby, Sarah D.; Kleinman, Paul K. [Boston Children' s Hospital, Department of Radiology, Boston, MA (United States); Wilson, Celeste R. [Boston Children' s Hospital, Child Protection Program, Department of Pediatrics, Boston, MA (United States); Barber, Ignasi [Hospital Vall d' Hebron, Department of Pediatric Radiology, Barcelona (Spain)

    2014-09-15

    We report a previously healthy 4-month-old who presented to the hospital with leg pain and swelling and no history of trauma. Radiographs demonstrated a comminuted left femur fracture. Given the concern for child abuse, skeletal survey was performed and revealed four vertebral compression deformities. Although abuse was suspected, the possibility of a lytic lesion associated with the femur fracture and multiple spinal abnormalities raised the possibility of an underlying process such as Langerhans cell histiocytosis. Subsequently 18F-NaF positron emission tomographic (PET) scintigraphy revealed increased tracer activity in the ischium, and MRI confirmed an ischial apophyseal fracture. Pelvic fractures, particularly ischial fractures, are extremely rare in the setting of child abuse. This case report describes the multimodality imaging findings of an ischial fracture in an abused infant. (orig.)

  7. Giant basal cell carcinoma of the eyelid: a case history | Fetohi | Pan ...

    African Journals Online (AJOL)

    Giant basal cell carcinoma of the eyelid: a case history. ... Abstract. Basal cell carcinoma is a type of skin cancer and rare, aggressive forms of basal cell ... She died 09 months after the end of irradiation in Intensive care unit due to septic shock.

  8. Ovarian Small Cell Carcinoma Hypercalcemic Type: A Case Report

    LENUS (Irish Health Repository)

    Rahma, M B.

    2016-09-01

    A 31-year-old female was diagnosed with small cell carcinoma of the ovary hypercalcaemic type (OSCCHT) post left oophorectomy. This is a rare aggressive ovarian tumour of which less than 300 cases were reported.

  9. Small Cell Carcinoma of the Ovary of Hypercalcemic Type: A Case Report

    Directory of Open Access Journals (Sweden)

    S. Zaied

    2012-01-01

    Full Text Available Introduction. The small cell carcinoma of hypercalcemic type of ovary is a very aggressive tumor. It is associated with two-thirds of cases with hypercalcemia most often asymptomatic. It occurs mostly for young women. The treatment combines surgery, chemotherapy, and radiotherapy. Case Presentation. We report a case of small cell carcinoma of the ovary hypercalcemic type in a young Tunisian woman aged 25 years after a severe abdominal pain syndrome and a large ovarian mass discovered in scanner; a laparotomy was performed by radical surgery. The pathological examination of the specimen confirmed the diagnosis. The radiological assessment performed after surgery showed a continuing evolution. Palliative chemotherapy was established, and the patient had died two months after diagnosis. Conclusion. The hypercalcemic small cell carcinoma of the ovary is a rare disease of poor prognosis.

  10. [Diabetes insipidus and pregnancy].

    Science.gov (United States)

    Gutiérrez Cruz, Oswaldo; Careaga Benítez, Ricardo

    2007-04-01

    Diabetes insipidus is an uncommon pathology; its incidence varies from two to six cases in 100,000 pregnancies. It has multiple etiologies and it is classified in central and neurogenic. Patients with diabetes insipidus generally show intense thirst, polyuria, neurologic symptoms and hypernatremia. It does not seem to alter the patient's fertility. Diabetes insipidus is usually associated with pre-eclampsia, HELLP syndrome, and fatty liver disease of pregnancy. This is a report of a case seen at the Hospital General de Cholula, in Puebla, Mexico. A 19 year-old female, with 37.2 weeks of pregnancy, had a history of Langerhans cell histiocytosis since she was four years. Patient was treated with intranasal desmopressin until 2005. She went to an obstetric evaluation; laboratory and cabinet studies were obtained. A healthy 1900 g female was obtained through vaginal delivery, with a 7/9 Apgar score. We should be familiarized with this uncommon pathology because of its association with several obstetric emergencies.

  11. Basal Cell Epithelioma (A Clinico-pathological Study of 172 Cases

    Directory of Open Access Journals (Sweden)

    R L Solanki

    1989-01-01

    Full Text Available The present paper deals with a clinico-pathological analysis of 172 cases of basal cell epithelioma representing 28% of the total skin cancer case. 1.44% of the total malignant tumours, 1% of the total tumours and 0.24% of the hospital admissions. M:F ratio was 1.26:1. The mean age was 54.06 years with the maximum number of cases in the fifth decade., Most (94% of the lesions were seen on the head and neck region. Histopathologically it showed solid (60.5%, adenoid cystic (15,7%, keratotic (9.3%, pigmented (6.4%, basi-squamous (3.5% and morphoea patterns (2.3%. Perineural lymphatic invasion was seen in 52% of the adenoid cystic basal call epithelioma. Amyloid (12.8% and amyloid like deposits (29.65% were seen in 42% of the case. Amyloid deposits were seen as lichen amyloidosis in 10.46% cases and in or around the tumour cell masses in 16.8% cases. It appeared as structureless homogeneous eosinophilic material and showed congophilia with apple green birefringence on polarising microscopy. It was found to be KMN04 sensitive (AA type protein- secondary amyloidosis. Amyloid like deposits appeared as less homogeneous,, basophilic, fractured material which showed non-specific congophilia with apple- green birefringence and stained with acid orcein stain indicating degenerated collagen (elastoid-degeneration.

  12. [Extensive scabies in a baby (author's transl)].

    Science.gov (United States)

    Maleville, J; Derrien, A; Boineau, D; Mollard, S; Marc-Antoine, H; Guillet, G

    The authors are reporting a new case of widespread scabies in a baby. They take this opportunity to emphasize on the atypical erythematous and excoriated papular rash which sometimes may be vesicular and hyper-keratotic. This widespread eruption may mimic generalised dermatitis, pustular psoriasis and even histiocytosis X. They also underline importancy of longlasting ointment with fluorinated steroid being responsible for this widespread eruption.

  13. Plasma cell gingivitis - A rare case related to Colocasia (arbi) leaves.

    Science.gov (United States)

    Bali, Deepika; Gill, Sanjeet; Bali, Amit

    2012-09-01

    Plasma cell gingivitis is an uncommon inflammatory condition of uncertain etiology often flavoured chewing gum, spices, foods, candies, or dentifrices. The diagnosis of plasma cell gingivitis is based on comprehensive history taking, clinical examination, and appropriate diagnostic tests. Here we are presenting a rare case of plasma cell gingivitis caused by consumption of colocasia (arbi) leaves. Colocasia is a kind of vegetable, very commonly consumed in the regions of North India.

  14. Nasosinusal Lymphoma of T Natural Killer Cells: Case Report

    Directory of Open Access Journals (Sweden)

    Castro, Victor Labres da Silva

    2011-01-01

    Full Text Available Introduction: The primary nasal lymphoma is an uncommon extranodal tumor and represents 0.44% of all Extranodal lymphomas in this region. The primary nasal lymphoma derives from the T-lineage in nearly 75% of the cases. Objective: To describe a case of nasosinusal lymphoma of T Natural Killer cells, attended in the Clinical Hospital of the Federal University of Goiás. Case Report: 48-year-old female patient with diffuse tumefaction in the left hemiface of firm-elastic consistency and painful upon digital compression. Face sinuses tomography identified a total maxillary veiling to the left and some posterior ethmoidal cells. With the diagnostic hypothesis of a tumor affection, we opted for the surgical removal via a transmaxillary approach and the material was sent for biopsy. The histopathological exam diagnosed a highly necrotic tumor of angiocentric pattern, polymorphic and atypical lymphoid population (T /NK Lymphoma; with the prognosis, the patient was submitted to chemical therapy with total regression of the facial edema. Final Comments: The otorhinolaryngologist must be attentive as regards the existence of lymphomas among the nasosinusal diseases, because the early diagnosis improves the survival as it prevents metastases, growth and local destruction.

  15. Two cases of seborrheic keratosis with basal clear cells.

    Science.gov (United States)

    Anan, Takashi; Fukumoto, Takaya; Kimura, Tetsunori

    2017-03-01

    Seborrheic keratosis with basal clear cells (SKBCC) is an extremely rare histopathological variant of seborrheic keratosis that has histological similarities to melanoma in situ. We herein report two cases of SKBCC and provide the first description of the dermoscopic features of this condition, in addition to the histopathological findings. Both of the two lesions showed typical histological architectures of seborrheic keratosis with rows or focal clusters of monomorphic clear cells with abundant pale cytoplasm and small round nucleus in the basal layer. Immunohistochemical examination revealed that most clear cells were positive for high molecular weight cytokeratin (34βE12) in a peripheral pattern but were negative tor Melan-A. Dermoscopy revealed typical features of ordinary seborrheic keratosis, while unfortunately did not reflect the presence of basal clear cells. © 2016 Japanese Dermatological Association.

  16. Smoking-related interstitial lung diseases

    International Nuclear Information System (INIS)

    Marten, K.

    2007-01-01

    The most important smoking-related interstitial lung diseases (ILD) are respiratory bronchiolitis, respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and Langerhans' cell histiocytosis. Although traditionally considered to be discrete entities, smoking-related ILDs often coexist, thus accounting for the sometimes complex patterns encountered on high-resolution computed tomography (HRCT). Further studies are needed to elucidate the causative role of smoking in the development of pulmonary fibrosis

  17. Smoking-related interstitial lung diseases; Interstitielle Lungenerkrankungen bei Rauchern

    Energy Technology Data Exchange (ETDEWEB)

    Marten, K. [Technische Univ. Muenchen (Germany). Klinikum rechts der Isar, Inst. fuer Roentgendiagnostik

    2007-03-15

    The most important smoking-related interstitial lung diseases (ILD) are respiratory bronchiolitis, respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and Langerhans' cell histiocytosis. Although traditionally considered to be discrete entities, smoking-related ILDs often coexist, thus accounting for the sometimes complex patterns encountered on high-resolution computed tomography (HRCT). Further studies are needed to elucidate the causative role of smoking in the development of pulmonary fibrosis.

  18. Combined basal cell carcinoma and Langerhans cell histiocytosis of the scrotum in a patient with occupational exposure to coal tar and dust

    Energy Technology Data Exchange (ETDEWEB)

    Izikson, L.; Vanderpool, J.; Brodsky, G.; Mihm, M.C.; Zembowicz, A. [Harvard University, Boston, MA (US). Massachusetts General Hospital

    2004-09-01

    The patient was a 77-year-old male former smoker, with history of several basal cell carcinomas (BCCs) in sun-protected areas around the waistline, who presented with another small ulceration on the anterior right upper scrotum near the base of the penis. BCC was suspected clinically and the lesion was treated with cryosurgery. The tumor recurred, became raised, and began to bleed. An excisional biopsy was performed. It showed nodular BCC surrounded by a cellular proliferation of round histiocytic cells with convoluted, lobulated and reniform nuclei and abundant cytoplasm . The patient had no history of exposure to ionizing radiation, chemotherapy, immunosuppressive medications, prior lymphoma or other malignancy. However, he spent 4 years on a ship loading coal into the furnace of a steam engine, during which he slept in adjacent quarters that were covered with coal dust. Additionally, he had a several-year history of occupational skin exposure to machine oil, oil refinery waste, sulfur waste, hydraulic fluid, and asbestos. He also reported a history of nude sunbathing. The scrotal lesion was re-excised and the patient remains disease-free more than 1 year after the diagnosis.

  19. Plasma cell gingivitis - A rare case related to Colocasia (arbi leaves

    Directory of Open Access Journals (Sweden)

    Deepika Bali

    2012-01-01

    Full Text Available Plasma cell gingivitis is an uncommon inflammatory condition of uncertain etiology often flavoured chewing gum, spices, foods, candies, or dentifrices. The diagnosis of plasma cell gingivitis is based on comprehensive history taking, clinical examination, and appropriate diagnostic tests. Here we are presenting a rare case of plasma cell gingivitis caused by consumption of colocasia (arbi leaves. Colocasia is a kind of vegetable, very commonly consumed in the regions of North India.

  20. Nevoid Basal Cell Carcinoma Syndrome: A Case Report

    Directory of Open Access Journals (Sweden)

    Razavi

    2016-09-01

    Full Text Available Nevoid basal cell carcinoma syndrome (BCNS is an autosomal dominant inherited disorder. Multiple organ systems may be affected in this syndrome including abnormalities of the skin, skeletal system, genitourinary system and central nevus system. In this report, we present a case of Nevoid basal cell carcinoma syndrome in a 26-year-old male patient. The patient had multiple odontogenic keratocyst in the posterior of mandible, syndactyly in both hand and bifid rib. After enucleation and curettage, he was followed for two years. A number of both clinical and radiological criteria are used to diagnose this syndrome. Basal cell carcinoma syndrome is diagnosed with two major criteria or one major and two minor criteria. We must suspect this disorder in young patients with multiple odontogenic keratocyst and dental abnormalities whether related or not with other clinical manifestations or familial history.

  1. Targeting BCL2 Family in Human Myeloid Dendritic Cells: A Challenge to Cure Diseases with Chronic Inflammations Associated with Bone Loss

    Directory of Open Access Journals (Sweden)

    Selma Olsson Åkefeldt

    2013-01-01

    Full Text Available Rheumatoid arthritis (RA and Langerhans cell histiocytosis (LCH are common and rare diseases, respectively. They associate myeloid cell recruitment and survival in inflammatory conditions with tissue destruction and bone resorption. Manipulating dendritic cell (DC, and, especially, regulating their half-life and fusion, is a challenge. Indeed, these myeloid cells display pathogenic roles in both diseases and may be an important source of precursors for differentiation of osteoclasts, the bone-resorbing multinucleated giant cells. We have recently documented that the proinflammatory cytokine IL-17A regulates long-term survival of DC by inducing BCL2A1 expression, in addition to the constitutive MCL1 expression. We summarize bibliography of the BCL2 family members and their therapeutic targeting, with a special emphasis on MCL1 and BCL2A1, discussing their potential impact on RA and LCH. Our recent knowledge in the survival pathway, which is activated to perform DC fusion in the presence of IL-17A, suggests that targeting MCL1 and BCL2A1 in infiltrating DC may affect the clinical outcomes in RA and LCH. The development of new therapies, interfering with MCL1 and BCL2A1 expression, to target long-term surviving inflammatory DC should be translated into preclinical studies with the aim to increase the well-being of patients with RA and LCH.

  2. Paraneoplastic scleroderma-like tissue reactions in the setting of an underlying plasma cell dyscrasia: a report of 10 cases.

    Science.gov (United States)

    Magro, Cynthia M; Iwenofu, Hans; Nuovo, Gerard J

    2013-07-01

    Systemic plasma cell dyscrasias have diverse manifestations in the skin and include an inflammatory paraneoplastic process. We encountered cases of scleroderma and eosinophilic fasciitis in the setting of an underlying plasma cell dyscrasia. Ten cases of scleroderma-like tissue reactions in the setting of an underlying plasma cell dyscrasia were encountered. The biopsies were stained for Transforming growth factor (Transforming growth factor) beta, IgG4, kappa, and lambda. Patients presented with a sclerodermoid reaction represented by eosinophilic fasciitis (5 cases), morphea (3 cases), and systemic scleroderma (2 cases). The mean age of presentation was 70 years with a striking female predominance (4:1). Acral accentuation was noted in 8 cases. In 6 of the cases, the cutaneous sclerosis antedated (4 cases) by weeks to 2 years or occurred concurrently (2 cases) with the initial diagnosis of the plasma cell. The biopsies showed changes typical of eosinophilic fasciitis and/or scleroderma. In 5 cases, light chain-restricted plasma cells were present on the biopsy. There was staining of the plasma cells for Transforming growth factor beta in 3 out of 5 cases tested. In any older patient presenting with a sudden onset of eosinophilic fasciitis or scleroderma especially with acral accentuation, investigations should be conducted in regards to an underlying plasma cell dyscrasia.

  3. Granulosa cell tumor of ovary: A clinicopathological study of four cases with brief review of literature

    Directory of Open Access Journals (Sweden)

    B R Vani

    2014-01-01

    Full Text Available Introduction: Adult granulosa cell tumor (GCT is a rare ovarian malignancy having good prognosis in comparison with other epithelial tumors. The study aims to collect data of all granulosa cell tumors diagnosed in ESIC Medical College & PGIMSR, Rajajinagar, Bangalore over the last 3 years and to describe the patient profile, ultrasonographic and various histopathological features.Materials and Methods: A total of 4 granulosa cell tumors were diagnosed in ESIC Medical College & PGIMSR, Rajajinagar, Bangalore during the period from June 2010 to June 2013. The patient′s age, clinical manifestations, radiological and histopathological findings were evaluated.Results: All 4 patients were diagnosed as adult granulosa cell tumor, three of four cases were in premenopausal age group and one case was in perimenopausal age. The clinical manifestations were menorrhagia and abdominal pain. Ultrasonographically, 2 cases of granulosa cell tumors were both solid and cystic and one case each was either solid or cystic. Histologically, variety of patterns like diffuse, trabecular, cords, spindle and clear cells were noted. Both Call-Exner bodies and nuclear grooves were observed in all cases. All four cases showed simple hyperplasia without atypia endometrial findings. Follow up on all patients revealed no evidence of recurrence.Conclusion: Granulosa cell tumor of the ovary is a rare ovarian entity. The important prognostic factor is staging of the tumor. Staging and histopathology helps in prediction of survival. Also diligent endometrial pathology has to be sorted to rule out endometrial carcinoma.

  4. A case of clear cell sarcoma

    DEFF Research Database (Denmark)

    Juel, Jacob; Ibrahim, Rami Mossad

    2017-01-01

    INTRODUCTION: Clear cell sarcoma (CCS) is a rare tumour of the soft tissue often misdiagnosed, as it shares characteristics with malignant melanoma (MM). Previously, CCS has been characterised, as malignant melanoma of the soft tissue, contemporary immunohistochemical techniques, however, have made...... this designation obsolete. The true incidence remains unknown, but CCS is believed to represent less than one percent of all sarcomas. PRESENTATION OF CASE: A 22-year-old patient presented with a mass sized 2.6×2.7×2.7cm of the left gluteal region, pain, and malaise. Initially, the symptoms were interpreted...

  5. Choroid plexus metastasis of renal-cell carcinoma. A case report

    Energy Technology Data Exchange (ETDEWEB)

    Shigemori, Minoru; Shimamoto, Houtetsu; Noguchi, Shinji; Yoshitake, Yasuhiro; Sugita, Yasuo; Kuramoto, Shinken

    1987-10-01

    A rare case of the choroid plexus metastasis of renal-cell carcinoma is reported. A 58-year-old man was admitted on March 3, 1982, with complaints of mild headache and a transient attack of muscle weakness of the left upper extremity. He had undergone a left nephrectomy because of renal-cell carcinoma 2 years before this admission. A CT scan revealed a small mass in the right lateral ventricle that was markedly enhanced by the contrast medium. A carotid angiogram was normal, but a left vertebral angiogram showed a round tumor stain in the distal portion of the right posterior choroidal artery. To determine the nature of the tumor, it was successfully removed via the right frontal transventricular approach. The immediate recovery from the operation was uneventful, but the patient became semicomatose 6 hours later because of a large subdural hematoma over the left hemisphere. An emergency operation for clot removal and external decompression failed to improve the patient's status, and he died on the 3rd postoperative day. An histological examination of the tumor determined the diagnosis of clear-cell-type renal-cell carcinoma. The CT demonstration of choroid plexus metastasis is quite rare. To our knowledge, only two cases have been described.

  6. [Anesthetic considerations in sickle cell anemia: a case report].

    Science.gov (United States)

    Fernández-Meré, L A; Sopena-Zubiría, L A; Alvarez-Blanco, M

    2009-01-01

    Sickle cell anemia is the most common hemoglobinopathy. Advances in therapeutic techniques and anesthetic procedures have led to a considerable increase in the success of surgical procedures in these patients. We report the case of a 16-year-old black boy diagnosed with sickle cell anemia and beta-thalassemia who presented with chronic osteomyelitis of the tibia. He was scheduled for debridement of the lesion and musculocutaneous flap repair. We emphasize the importance of communication between anesthesiologists, surgeons, and hematologists in the perioperative period in order to determine the risk of complications and anticipate them.

  7. Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: Report of two cases.

    Science.gov (United States)

    García-Fontán, Eva; Blanco Ramos, Montserrat; García, Jose Soro; Carrasco, Rommel; Cañizares, Miguel Ángel; González Piñeiro, Ana

    2018-05-19

    Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) is a rare disorder characterized by a proliferation of neuroendocrine cells within the lung. It is classically described as a disease with persistent cough, dyspnea and wheezing in non-smoker middle aged females. CT of the chest reveals diffuse air trapping with mosaic pattern. We present two cases of DIPNECH that were sent to our department to perform a lung biopsy with the diagnostic suspicion of diffuse interstitial disease. Both cases were women with a history of chronic cough and moderate effort dyspnea. The aim of this paper is that physicians take into account this diagnostic entity before treating as an asthmatic a patient with these characteristics, not forgetting that they are prenoplastic lesions. Copyright © 2018 Elsevier España, S.L.U. All rights reserved.

  8. Autoimmune central diabetes insipidus in a patient with ureaplasma urealyticum infection and review on new triggers of immune response.

    Science.gov (United States)

    Murdaca, Giuseppe; Russo, Rodolfo; Spanò, Francesca; Ferone, Diego; Albertelli, Manuela; Schenone, Angelo; Contatore, Miriam; Guastalla, Andrea; De Bellis, Annamaria; Garibotto, Giacomo; Puppo, Francesco

    2015-12-01

    Diabetes insipidus is a disease in which large volumes of dilute urine (polyuria) are excreted due to vasopressin (AVP) deficiency [central diabetes insipidus (CDI)] or to AVP resistance (nephrogenic diabetes insipidus). In the majority of patients, the occurrence of CDI is related to the destruction or degeneration of neurons of the hypothalamic supraoptic and paraventricular nuclei. The most common and well recognized causes include local inflammatory or autoimmune diseases, vascular disorders, Langerhans cell histiocytosis (LCH), sarcoidosis, tumors such as germinoma/craniopharyngioma or metastases, traumatic brain injuries, intracranial surgery, and midline cerebral and cranial malformations. Here we have the opportunity to describe an unusual case of female patient who developed autoimmune CDI following ureaplasma urealyticum infection and to review the literature on this uncommon feature. Moreover, we also discussed the potential mechanisms by which ureaplasma urealyticum might favor the development of autoimmune CDI.

  9. Coexistence of Granular Cell Tumor with Squamous Cell Carcinoma on the Tongue: A Case Report

    Directory of Open Access Journals (Sweden)

    Recep Bedir

    2015-01-01

    Full Text Available Introduction: Granular cell tumors (GCTs are rare and mostly benign soft tissue tumors. Though they have been reported in all parts of body, they are generally located in the head and neck region, especially on the tongue. Some malign forms exist, but these have been rarely reported. Granular cell tumors have a neural origin and, in immunohistochemical evaluations, they express S-100 and neuron specific enolase (NSE. The treatment of these tumors is bulky surgical excision.   Case Report:   In this case, a cauliflower shaped lesion with a 1 cm diameter was excised from the midline tongue of a 65 year old woman. The histopathological evaluation indicated that it was squamous cell carcinoma (SCC covering GCT. Herein, the coexistence of GCT and SCC we describe on the same region of the tongue, in accordance with literature review, since this is a very rare condition.   Conclusion: Pseudoepitheliomatous hyperplasia may accompany GCTs on the tongue and this condition may mimic well-differentiated SCC. For this reason, with the help of Ki-67 and p63 expression, in addition to immunohistochemical markers, well-differentiated SCC should be differentiated from pseudoepitheliomatous hyperplasia through careful investigation.

  10. Mucinous tubular and spindle cell carcinoma of kidney: A clinicopathologic study of six cases

    Directory of Open Access Journals (Sweden)

    Mudassar Hussain

    2012-01-01

    Full Text Available Background: Mucinous tubular and spindle carcinoma (MTSCC of kidney is a rare, low-grade polymorphic tumor. Recent studies have described a wide morphology spectrum of this tumor. Aim: To report the clinico-pathologic features of six cases of MTSCC of kidney. Materials and Methods: Six cases of MTSCC of kidney were studied and literature was reviewed. Immunohistochemistry was done by Envision method. Results: The age of the patients ranged from 44 to 84 years (mean 58.5 years. Four patients were males and two were females. The tumor was located in the left kidney in four cases and in the right kidney in two cases. The tumor size ranged from 4.5 to 15 cm (mean 6.4 cm. All tumors exhibited an admixture of tubules, spindle cells, and mucinous stroma in variable proportions. Tubules were predominant in five cases and spindle cells in one case. Psammomatous calcifications, papillations, and necrosis were seen in two cases. Collections of foamy histiocytes were noted in four cases. Cytoplasmic vacuoles and osseous metaplasia were seen in one case each. All cases were Fuhrman′s nuclear grade II. Five cases were of stage pT1, and one was pT3. All cases stained positive for alcian blue at pH 2.5. Immunohistochemical stain CK7 was positive in all cases and CD10 was positive in 1/1 case. All patients were alive and well at follow-up of 12-59 months (mean 33.5 months. No metastases were detected. Conclusions: We report six cases of MTSCC of kidney, a rare distinct variant of RCC, with a favorable prognosis. A male predominance was seen in our cases. MTSCC shares histologic and immunohistochemical overlap with papillary renal cell carcinoma (PRCC and cytogenetic analysis should be performed in difficult cases to avoid a misdiagnosis.

  11. Clear-cell chondrosarcoma of the maxilla Report of a case

    NARCIS (Netherlands)

    Slootweg, P.J.

    Clear-cell chondrosarcoma is a variant of chondrosarcoma which is characterized by a typical histomorphology and a very slow rate of growth. A case is presented in which the tumor was located in the maxilla.

  12. A case of renal cell carcinoma and angiomyolipoma in an ...

    African Journals Online (AJOL)

    Abstract. We describe a case of renal cell carcinoma in the right kidney together with an angiomyolipoma in the left kidney, encountered in an adolescent girl at Potchefstroom Provincial Hospital, North West Province, South Africa.

  13. Spontaneous pneumothorax in diffuse cystic lung diseases.

    Science.gov (United States)

    Cooley, Joseph; Lee, Yun Chor Gary; Gupta, Nishant

    2017-07-01

    Diffuse cystic lung diseases (DCLDs) are a heterogeneous group of disorders with varying pathophysiologic mechanisms that are characterized by the presence of air-filled lung cysts. These cysts are prone to rupture, leading to the development of recurrent spontaneous pneumothoraces. In this article, we review the epidemiology, clinical features, and management DCLD-associated spontaneous pneumothorax, with a focus on lymphangioleiomyomatosis, Birt-Hogg-Dubé syndrome, and pulmonary Langerhans cell histiocytosis. DCLDs are responsible for approximately 10% of apparent primary spontaneous pneumothoraces. Computed tomography screening for DCLDs (Birt-Hogg-Dubé syndrome, lymphangioleiomyomatosis, and pulmonary Langerhans cell histiocytosis) following the first spontaneous pneumothorax has recently been shown to be cost-effective and can help facilitate early diagnosis of the underlying disorders. Patients with DCLD-associated spontaneous pneumothorax have a very high rate of recurrence, and thus pleurodesis should be considered following the first episode of spontaneous pneumothorax in these patients, rather than waiting for a recurrent episode. Prior pleurodesis is not a contraindication to future lung transplant. Although DCLDs are uncommon, spontaneous pneumothorax is often the sentinel event that provides an opportunity for diagnosis. By understanding the burden and implications of pneumothoraces in DCLDs, clinicians can facilitate early diagnosis and appropriate management of the underlying disorders.

  14. Giant cell tumour of tendon sheath and synovial membrane: A review of 26 cases.

    Science.gov (United States)

    Kant, Kumar Shashi; Manav, Ajoy Kumar; Kumar, Rakesh; Abhinav; Sinha, Vishvendra Kumar; Sharma, Akshat

    2017-11-01

    Aim of our study is to highlight the incidence and benign nature of Giant cell tumour of tendon sheath and need for complete removal, thus minimizing the chances of recurrence. A total of 26 cases of Giant cell tumour of tendon sheath operated in the department of Orthopaedics, Patna Medical College & Hospital, Patna from 2003 to 2010 were included in this study. The surgery was performed after clinical evaluation of the lesion and Fine Needle Aspiration Cytology (FNAC). The tumour underwent en bloc marginal excision. The patients were followed up for minimum two year. Our study population consisted of 18 females and 8 males. The mean age at the time of surgery was 38.3 years (range, 18-62 years). Twenty three cases were found in the 3rd and 4th decade. Twenty two cases involved upper extremity and only 4 cases in lower extremity. MRI was done in 2 cases where diagnosis was in doubt. Bony indentation on X-ray film was found in 7 cases and thorough curettage of cortical shell was done. All the cases were treated by marginal excision. Three cases developed post-operative stiffness but regained full range of movement with physiotherapy. Sensory impairment was seen in 3 cases. Recurrence occurred in 2 case and they were treated by repeat marginal excision. Meticulous en-masse marginal excision of the giant cell tumour of tendon sheath in blood less field using magnification is the treatment of choice.

  15. Amlodipine induced plasma cell granuloma of the gingiva: A novel case report.

    Science.gov (United States)

    Vishnudas, Bhandari; Sameer, Zope; Shriram, Bansode; Rekha, Kardile

    2014-07-01

    Drug-induced gingival overgrowth (DIGO) can be a serious concern for both patients and clinicians. DIGO is a well-documented side-effect of some pharmacologic agents, including, but not limited to, calcium channel blockers, phenytoin, and cyclosporine. Plasma cell granulomas (pseudotumors) are exceedingly rare, non-neoplastic, reactive tumor-like proliferation, primarily composed of plasma cells that manifest primarily in the lungs, but may occur in various anatomic locations. Intraoral plasma cell granulomas involving the lip, oral mucosa, tongue, and gingiva have been reported in the past. This is the first case report of amlodipine induced plasma cell granuloma of the gingiva in the medical literature presenting a 54 year-old female patient with hypertension, who received amlodipine (10 mg/day, single dose orally) for 2 years, sought medical attention because of developing maxillary anterior massive gingival overgrowth causing functional and esthetic problem, which was treated by excisional biopsy. Histologically, these lesions were composed of mature plasma cells, showing polyclonality for both lambda and kappa light chains and fibrovascular connective tissue stroma confirming a diagnosis of plasma cell granuloma. This case also highlights the need to biopsy for unusual lesions to rule out potential neoplasms.

  16. Osteoclastic Giant Cell Rich Squamous Cell Carcinoma of the Uterine Cervix: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Lucía Alemán-Meza

    2014-01-01

    Full Text Available Cervical carcinoma is the most common malignancy of the female genital tract and represents the second most common malignancy in women worldwide. Histologically 85 to 90% of cervical cancers are squamous cell carcinoma. Osteoclastic giant cell rich squamous cell carcinoma is an unusual histological variant of which only 4 cases have been reported. We present the case of a 49-year-old woman with a 6-month history of irregular vaginal bleeding. Examination revealed a 2.7 cm polypoid mass in the anterior lip of the uterine cervix. The patient underwent hysterectomy with bilateral salpingo-oophorectomy. Microscopically the tumor was composed of infiltrative nests of poorly differentiated nonkeratinizing squamous cell carcinoma. Interspersed in between these tumor cells were numerous osteoclastic giant cells with abundant eosinophilic cytoplasm devoid of nuclear atypia, hyperchromatism, or mitotic activity. Immunohistochemistry was performed; CK and P63 were strongly positive in the squamous component and negative in the osteoclastic giant cells, while CD68 and Vimentin were strongly positive in the giant cell population and negative in the squamous component. The patient received chemo- and radiotherapy for recurrent disease identified 3 months later on a follow-up CT scan; 7 months after the surgical procedure the patient is clinically and radiologically disease-free.

  17. Renal cell carcinoma with t(6,11: A case report and review of literature

    Directory of Open Access Journals (Sweden)

    K S Jansi Prema

    2017-01-01

    Full Text Available Renal cell carcinomas (RCCs with t(6,11 are very rare tumours. Only a few cases have been reported so far. t(6,11 results in fusion of alpha gene and transcription factor EB (TFEB gene resulting in the overexpression of TFEB. The specific light and immunohistochemical features help in the diagnosis of this rare type of tumor. We report a case of t(6,11 RCC in a 38-year-old female who was incidentally found to have a right renal mass. We present this case to emphasize the typical light microscopic picture of this extremely rare tumor. Two population of cells are seen: larger cells with abundant cytoplasm and smaller cells with scant cytoplasm. Smaller cells are arranged around hyaline nodules resulting in the formation of characteristic pseudorosettes. Immunohistochemically, these tumors are diffusely positive for vimentin and focally positive for HMB 45 and CD 117. Knowledge about the typical biphasic light microscopic appearance and the characteristic immunohistochemical features help in the diagnosis of this rare type of translocation associated RCC.

  18. Primary Signet Ring Cell Adenocarcinoma of the Urinary Bladder: A Report of 2 Cases

    Directory of Open Access Journals (Sweden)

    Wiem Boukettaya

    2014-05-01

    Full Text Available Primary signet ring cell carcinoma of the urinary bladder is a rare and aggressive histologic subtype of adenocarcinoma. In general, this tumor occurs in the middle age, and clinical presentation does not differ from transitional cell carcinomas. The prognosis is often poor, given the advanced stage at diagnosis. To our knowledge, <100 cases of signet ring cell adenocarcinoma of the urinary bladder have been reported. We report 2 cases with bladder linitis plastica primitive, and we draw attention to its pathologic, anatomoclinical, and evolution specificity to optimize its therapeutic management.

  19. SIADH Induced by Pharyngeal Squamous Cell Carcinoma: Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Hafiz Muhammad Sharjeel Arshad

    2016-01-01

    Full Text Available Background. The Syndrome of Inappropriate Antidiuretic Hormone (SIADH is considered to be the most common cause of euvolemic hyponatremia. The most common malignancy associated with SIADH is small cell lung cancer. We present a rare case of a patient with SIADH secondary to well differentiated squamous cell carcinoma of the naso-oropharynx. Case. A 46-year-old Caucasian woman presented to emergency department with four-week history of progressive dysphagia. On examination, she was found to have a pharyngeal mass. CT scan and MRI of neck confirmed a mass highly suspicious of carcinoma. Patient’s serum sodium level decreased to 118 mEq/L and other labs including serum and urine osmolality confirmed SIADH. She was started on fluid restriction and oral sodium tablets which gradually improved her serum sodium levels. Biopsy confirmed diagnosis of squamous cell carcinoma of pharynx. Conclusion. SIADH can be caused by squamous cell carcinoma. Appropriate management includes fluid restriction.

  20. [Coexisting systemic lupus erythematosus and sickle cell disease: case report and literature review].

    Science.gov (United States)

    Robazzi, Teresa Cristina M V; Alves, Crésio; Abreu, Laís; Lemos, Gabriela

    2015-01-01

    To report a case of coexisting systemic lupus erythematosus (SLE) and sickle cell disease (SCD) with a review of the literature on the topic. Report of case and research of the association between SLE and SCD in literature through scientific articles in health sciences databases, such as LILACS, MEDLINE/Pubmed and Scielo, until May 2012. Descriptors used: 1. Sickle cell anemia; 2. Sickle cell disease; 3. Systemic lupus erythematosus; 4. Hemoglobinopathies. The authors describe an association between SLE and SS hemoglobinopathy in an eight-year-old female patient displaying articular, hematologic and neuropsychiatric manifestations during clinical evolution. Forty-five cases of association between SLE and SCD are described in literature, mostly adult (62.2%), women (78%) and with the SS phenotype in 78% of the cases, and different clinical manifestations. Compared with our patient, articular, hematologic and neuropsychiatric manifestations were present in 76%, 36% and 27% of the cases, respectively. SLE and SCD are chronic diseases that have several clinical and laboratory findings in common, meaning difficult diagnosis and difficulty in finding the correct treatment. Although the association between these diseases is not common, it is described in literature, so it is imperative that physicians who treat such diseases be alert to this possibility. Copyright © 2012 Elsevier Editora Ltda. All rights reserved.

  1. A Case of Mature Natural Killer-Cell Neoplasm Manifesting Multiple Choroidal Lesions: Primary Intraocular Natural Killer-Cell Lymphoma

    Directory of Open Access Journals (Sweden)

    Yoshiaki Tagawa

    2015-11-01

    Full Text Available Purpose: Natural killer (NK cell neoplasm is a rare disease that follows an acute course and has a poor prognosis. It usually emerges from the nose and appears in the ocular tissue as a metastasis. Herein, we describe a case of NK-cell neoplasm in which the eye was considered to be the primary organ. Case: A 50-year-old female displayed bilateral anterior chamber cells, vitreous opacity, bullous retinal detachment, and multiple white choroidal mass lesions. Although malignant lymphoma or metastatic tumor was suspected, various systemic examinations failed to detect any positive results. A vitrectomy was performed OS; however, histocytological analyses from the vitreous sample showed no definite evidence of malignancy, and IL-10 concentration was low. Enlarged choroidal masses were fused together. Three weeks after the first visit, the patient suddenly developed an attack of fever, night sweat, and hepatic dysfunction, and 5 days later, she passed away due to multiple organ failure. Immunohistochemisty and in situ hybridization revealed the presence of atypical cells positive for CD3, CD56, and Epstein-Barr virus-encoded RNAs, resulting in the diagnosis of NK-cell neoplasm. With the characteristic clinical course, we concluded that this neoplasm was a primary intraocular NK-cell lymphoma. Conclusions: This is the first report to describe primary intraocular NK-cell neoplasm. When we encounter atypical choroidal lesions, we should consider the possibility of NK-cell lymphoma, even though it is a rare disease.

  2. Granular cell tumor of the esophagus. Report of three cases.

    Science.gov (United States)

    Cohle, S D; McKechnie, J C; Truong, L; Jurco, S

    1981-06-01

    Granular cell tumors, (formerly called myoblastomas) involving the esophagus were encountered in three patients. In all three the tumors were asymptomatic and in two they were multiple. The first published endoscopic photographs of such a tumor are presented. The successful total removal of this neoplasm using the endoscope is described. The pathologic, radiologic and therapeutic aspects of previously reported cases of granular cell tumor of the esophagus are reviewed and compared with the three reported herein.

  3. Blastic plasmacytoid dendritic cell neoplasm: report of two pediatric cases.

    Science.gov (United States)

    Dharmani, Preeti Ashok; Mittal, Neha Manish; Subramanian, P G; Galani, Komal; Badrinath, Yajamanam; Amare, Pratibha; Gujral, Sumeet

    2015-01-01

    Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare subtype of acute leukemia that typically follows a highly aggressive clinical course in adults, whereas experience in children with this disease is very limited. We report cases of two children in whom bone marrow showed infiltration by large atypical monocytoid 'blast-like' cells which on immunophenotyping expressed CD4, CD56, HLA-DR and CD33 while were negative for CD34 other T-cell, B-cell and myeloid markers. The differential diagnoses considered were AML, T/NK-cell leukemia and acute undifferentiated leukemia. Additional markers CD303/BDCA-2 and CD123 which are recently validated plasmacytoid dendritic cell markers were done which helped us clinch the diagnosis of this rare neoplasm. An accurate diagnosis of BPDCN is essential in order to provide prompt treatment. Due to its rarity and only recent recognition as a distinct clinicopathological entity, no standardized therapeutic approach has been established for BPDCN.

  4. Multiple Synchronous Central Giant Cell Granulomas of the Maxillofacial Region: A Case Report

    International Nuclear Information System (INIS)

    Kang, Min Seok; Kim, Hak Jin

    2010-01-01

    Multifocal central giant cell granulomas (CGCG) in the maxillofacial region are suggestive of systemic disease such as hyperparathyroidism or an inherited syndrome such as Noonan-like multiple giant cell lesion syndrome. Only 5 cases of multifocal CGCGs in the maxillofacial region without any concomitant systemic disease have currently been reported. We report here on an unusual case of 17-year-old man who presented with multifocal CGCGs of the bilateral posterior mandible and right maxilla and he was without any concomitant systemic disease

  5. Multiple Synchronous Central Giant Cell Granulomas of the Maxillofacial Region: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Min Seok; Kim, Hak Jin [Pusan National University Hospital, Busan (Korea, Republic of)

    2010-01-15

    Multifocal central giant cell granulomas (CGCG) in the maxillofacial region are suggestive of systemic disease such as hyperparathyroidism or an inherited syndrome such as Noonan-like multiple giant cell lesion syndrome. Only 5 cases of multifocal CGCGs in the maxillofacial region without any concomitant systemic disease have currently been reported. We report here on an unusual case of 17-year-old man who presented with multifocal CGCGs of the bilateral posterior mandible and right maxilla and he was without any concomitant systemic disease

  6. Retrospective analysis of nonendodontic periapical lesions misdiagnosed as endodontic apical periodontitis lesions in a population of Taiwanese patients.

    Science.gov (United States)

    Huang, Hsun-Yu; Chen, Yuk-Kwan; Ko, Edward Cheng-Chuan; Chuang, Fu-Hsiung; Chen, Ping-Ho; Chen, Ching-Yi; Wang, Wen-Chen

    2017-07-01

    We aimed to evaluate nonendodontic periapical lesions clinically misdiagnosed as endodontic periapical pathoses in a population of Taiwanese patients. Cases (2000-2014) of histopathological diagnoses of nonendodontic periapical lesions were retrieved from all cases with a clinical diagnosis of radicular cyst, apical granuloma, or apical periodontitis in the institution. These cases were regarded as misdiagnosed nonendodontic periapical lesions, of which the types and frequencies, in addition to the demographic data, were determined. Four thousand and four specimens were clinically diagnosed as endodontically associated pathoses, of which 118 cases (2.95%) received a histopathological diagnosis of a nonendodontic pathologic entity, the most frequent lesion being keratocystic odontogenic tumor (KCOT, n = 38, 32.20%), followed by fibro-osseous lesion (n = 18, 15.25%), and dentigerous cyst (n = 13, 11.02%). Nine malignant lesions in the periapical area [squamous cell carcinoma (n = 7, 5.93%), adenoid cystic carcinoma (n = 1, 0.85%), and Langerhans cell histiocytosis (n = 1, 0.85%)] were also noted. A wide variety of histopathological diagnoses, including benign odontogenic and non-odontogenic cystic and tumorous lesions and infectious diseases, as well as malignant lesions, was noted in these 118 cases of nonendodontic periapical lesions. Squamous cell carcinoma was the most predominant malignancy of nonendodontic periapical lesions misdiagnosed as apical periodontitis lesions from imaging examination overlooking the clinical findings. The current data form a useful basis for clinicopathological investigation and educational teaching regarding nonendodontic periapical lesions misdiagnosed as endodontic apical periodontitis lesions.

  7. T-cell receptor (TCR) phenotype of nodal Epstein-Barr virus (EBV)-positive cytotoxic T-cell lymphoma (CTL): a clinicopathologic study of 39 cases.

    Science.gov (United States)

    Kato, Seiichi; Asano, Naoko; Miyata-Takata, Tomoko; Takata, Katsuyoshi; Elsayed, Ahmed Ali; Satou, Akira; Takahashi, Emiko; Kinoshita, Tomohiro; Nakamura, Shigeo

    2015-04-01

    Among Epstein-Barr virus (EBV)-positive cytotoxic T/NK-cell lymphoma, there are only a few reports on the clinicopathologic features of patients with primary nodal presentation (nodal EBV cytotoxic T-cell lymphoma [CTL]). Here, we compared the clinicopathologic profiles of 39 patients with nodal EBV CTL with those of 27 cases of "extranasal" NK/T-cell lymphoma of nasal type (ENKTL), especially addressing their T-cell receptor (TCR) phenotype. Histologically, 22 of 39 nodal EBV CTL cases (56%) were unique in having centroblastoid appearance, which was contrasted with the lower incidence of this feature in ENKTL (15%, P=0.001). In contrast, pleomorphic appearance was more frequently seen in ENKTL than in nodal EBV CTL (67% vs. 23%, P=0.001). Thirty-three of 39 nodal EBV CTL cases (85%) were of T-cell lineage on the basis of TCR expression and/or TCRγ gene rearrangement; in detail, 18 cases (46%) were TCRβ positive (αβ T), 5 (13%) were TCRγ and/or δ positive (γδ T), and 10 (26%) were TCR-silent type with clonal TCRγ gene rearrangement but no expression of TCRβ, γ, or δ. These results were clearly contrasted by a lower incidence of T-cell lineage in ENKTL (7 cases, 26%, P<0.001). Notably, the survival time of the 5 nodal lymphoma patients with γδ T-cell phenotype was within 3 months, which was inferior to those of αβ T and TCR-silent types (P=0.003), and 3 of those with available clinical information were all found to be associated with autoimmune diseases. These data suggest that nodal EBV CTL is distinct from ENKTL.

  8. Basal cell carcinoma of the nipple-areola complex: a case report.

    Science.gov (United States)

    Huang, Ching-Wen; Pan, Ching-Kuen; Shih, Teng-Fu; Tsai, Cheng-Chien; Juan, Chung-Chou; Ker, Chen-Guo

    2005-10-01

    Basal cell carcinoma (BCC) of the nipple-areola complex is very rare. Only 24 cases were reported in the literature and 17 (70.8%) of these cases arose in men. Most of the cases were treated with simple excision. We report on a case of BCC of the nipple-areola complex in a 46-year-old woman, treated with partial mastectomy. Metastasis to the axillary lymph nodes had been noted in 3 (12.5%) of the 24 reported cases of BCC of the nipple-areola complex. Thus, we applied the concept of the sentinel lymph node to detect possible metastases of axillary lymph nodes, letting us avoid the unnecessary axillary lymph node dissection and possible future morbidity.

  9. A Case Report of Early Gastric Signet Ring Cell Carcinoma

    African Journals Online (AJOL)

    2018-02-07

    gmail.com. How to cite this article: Akabah PS, Mocan S, Molnar C, Dobru D. Importance of optical diagnosis in early gastric cancer: A case report of early gastric signet ring cell carcinoma. Niger J Clin Pract 2017;20:1342-5.

  10. Spindle Cell Carcinoma of the hypopharynx: a case report

    Directory of Open Access Journals (Sweden)

    Dillu Ram Kandel

    2018-03-01

    Full Text Available Spindle cell carcinoma of hpopharynx is a rare pathology. It is a poorly differentiated variant of squamous cell carcinoma and morphologically resembles sarcoma. This is a disease of old age. It is usually associated with smoking and alcohol abuse. When it is associated with radiation exposure history it behaves more aggressively. Surgery is considered as the main modality of treatment and adjuvant radiotherapy if necessary. Here we present a case of 79 year old male with spindle cell carcinomaof right piriform fossa with 2-month history of progressive dysphasia and hoarseness that has been affecting his ability to speak and swallow with history of weight loss and past history of radiotherapy. So possibility of spindle cell carcinoma of the hypopharynx should beconsidered in an old patient with rapidly developing swelling of the hypopharynx with past history of radiation exposure. As it is a highly aggressive disease it should be treated timely and more aggressively to prolong the survival of the patient.   

  11. The first case of acinic cell carcinoma of the breast within a fibroadenoma: case report.

    Science.gov (United States)

    Limite, G; Di Micco, R; Esposito, E; Sollazzo, V; Cervotti, M; Pettinato, G; Varone, V; Benassai, G; Monda, A; Luglio, G; Maisto, V; Izzo, G; Forestieri, P

    2014-01-01

    A case of acinic cell carcinoma of the breast is reported in a 26-year-old woman. She presented a lump in her right breast, that seemed to be a fibroadenoma. The open biopsy revealed a well-bordered fibroadenoma, together with a proliferation of cells characterized by serous acinar differentiation and eosinophilic cytoplasmic granules. Tumor cells stained for amylase, lysozyme, α-1-antichymotripsin, epithelial membrane antigen, S-100 protein, pan-cytokeratin, cytokeratin 7 and E-cadherin. Estrogen receptor, progesterone receptor, human epidermal growth factor receptor 2 overexpression, CD10, P63, smooth muscle actin, cytokeratin 5/6 were negative. The sentinel node was negative. 8 months after surgery she is in good clinical conditions without recurrence or metastases. Copyright © 2014 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved.

  12. A rare case of splenic littoral cell angioma in a child

    Directory of Open Access Journals (Sweden)

    Recep Bedir

    2014-01-01

    Full Text Available Littoral cell angioma (LCA is a rare, benign primary vascular neoplasm of the spleen. The tumor originates from the littoral cells lining the sinuses of the red pulp of the spleen. Preoperative distinction of this tumor from other benign or malign splenic lesions is difficult. Radiologically most cases present as multiple nodules. Definitive diagnosis can only be made histopathologically and immunohistochemically following splenectomy. This clinical situation can coexist with various malignancies and autoimmune disorders. Even though, it is mostly benign, since it has the potential to become malignant after splenectomy, long-term follow-up is required. We present an LCA case, which appeared as a solitary mass in the spleen of an 11-year-old girl with abdominal pain admitted to our hospital.

  13. Cytological and histological correlation of granular cell tumor in a series of three cases

    Directory of Open Access Journals (Sweden)

    Soutrik Das

    2018-01-01

    Full Text Available Granular cell tumor (GCT is an uncommon soft tissue tumor characterized by proliferation of cells with granular eosinophilic cytoplasm. We came across three such tumors, one in the tongue, one in the chest wall, and one in the right deltoid region, which were referred for fine-needle aspiration cytology. On cytological examination, the first two cases were diagnosed as GCT, and the mass in deltoid region was suggestive of proliferative myositis. The cytological details of these cases are discussed. The excision biopsies of the first two cases and Tru-cut biopsy of the deltoid mass confirmed the diagnosis of GCT.

  14. Multifocal Abrikossoff's granular cell tumor of the oesophagus: Case report

    Directory of Open Access Journals (Sweden)

    Ranđelović Tomislav D.

    2008-01-01

    Full Text Available INTRODUCTION Granular cell tumors, relatively uncommon soft tissue tumors, have been a matter of debate among pathologists regarding histogenesis for a long time. Less common locations are in the aerodigestive tract including the oesophagus. CASE OUTLINE We have recently treated a rare case, a 37-year old male, who was admitted due to dysphagia and a painful swallow with occasional pharyngo-nasal regurgitation followed with a mild loss of weight. Standard clinical examination including X-ray chest, ECG and laboratory tests did not show pathological findings. Barium contrast oesophagography demonstrated multiple ovoid defects in the wall of the oesophagus. CT scan of the chest confirmed luminal narrowing owing to the tumor of the upper oesophagus. Upper endoscopy showed unusual multifocal nodular lesions alongside the oesophageal axis covered by smooth mucosa. A primary biopsy specimen taken from the largest nodules confirmed an unusual pathological finding of the granular cell tumor. Subtotal, transpleural oesophagectomy was performed and reconstruction was derived by long colon segment interposition through the posterior mediastinum. The postoperative course was uneventful. The operative specimen consisted of four ovoid tumors alongside the oesophagus (the greatest diameter 0.5-1.8, average 1.25. All verified tumors histologicaly consisted of a spindle-shaped or polygonal cells containing small and large eosinophilic granules and central nuclei. Most tumor cells showed strongly positive immunohistochemical staining for S-100 protein. These tumor cells were partially positive for p-53 and Ki-67. No lymph node metastases were detected histologically. CONCLUSION Multifocal granular cell tumor of the oesophagus is an unusual finding with low incidence, and rarely caused symptoms. Pathological features and multiplicity of such tumors emphasized malignant predisposition requiring surgical resection of the oesophagus.

  15. [Oral squamous cell carcinoma and lichen planus vs. lichenoid lesions. Case report].

    Science.gov (United States)

    Esquivel-Pedraza, Lilly; Fernández-Cuevas, Laura; Ruelas-Villavicencio, Ana Lilia; Guerrero-Ramos, Brenda; Hernández-Salazar, Amparo; Milke-García, María Pilar; Méndez-Flores, Silvia

    2016-01-01

    The development of squamous cell carcinoma from oral lichen planus is controversial. We report a case of intraoral squamous cell carcinoma, which presents together with lesions of oral lichen planus. The aim of this report was to analyze the problem to distinguish between the incipient changes of squamous cell carcinoma from the features described in oral lichen planus, in order to establish an accurate diagnosis of both entities. A 57-year old man with a history of smoking and chronic alcohol intake, who had an ulcerated tumor mass located in the tongue, and bilateral white reticular patches on buccal mucosa and borders of the tongue. The histopathological report was moderately differentiated invasive squamous cell carcinoma and lichen planus respectively. The premalignant nature of OLP is still indeterminate and controversial, this is primarily due to inconsistency in the clinical and histological diagnostic criteria used to differentiate cases of oral lichen planus from lichenoid reactions or other lesions causing intraepithelial dysplasia with high potentially malignant transformation. Oral lichenoid reactions are possibly most likely to develop malignant transformation as compared to the classic OLP lesions.

  16. A rare case of breast carcinoma co-existing with axillary mantle cell lymphoma

    Directory of Open Access Journals (Sweden)

    Scally John

    2003-12-01

    Full Text Available Abstract Background Mantle cell lymphoma (MCL is a rare variety of non-Hodgkin's lymphoma which originates from CD5+ B-cell population in the mantle zones of lymphoid follicles. Coexistence of such tumours in the axillary lymph nodes with invasive breast cancers without prior history of adjuvant chemotherapy or radiotherapy has not been previously reported in literature. Case report We report a rare case of breast cancer co-existing with stage I mantle cell lymphoma of the ipsilateral axillary lymph node detected fortuitously by population screening. Conclusion Though some studies have tried to prove breast carcinomas and lymphomas to share a common molecular or viral link, more research needs to be done to establish whether such a link truly exists.

  17. Basal Cell Carcinoma With Matrical Differentiation: Clinicopathologic, Immunohistochemical, and Molecular Biological Study of 22 Cases.

    Science.gov (United States)

    Kyrpychova, Liubov; Carr, Richard A; Martinek, Petr; Vanecek, Tomas; Perret, Raul; Chottová-Dvořáková, Magdalena; Zamecnik, Michal; Hadravsky, Ladislav; Michal, Michal; Kazakov, Dmitry V

    2017-06-01

    Basal cell carcinoma (BCC) with matrical differentiation is a fairly rare neoplasm, with about 30 cases documented mainly as isolated case reports. We studied a series of this neoplasm, including cases with an atypical matrical component, a hitherto unreported feature. Lesions coded as BCC with matrical differentiation were reviewed; 22 cases were included. Immunohistochemical studies were performed using antibodies against BerEp4, β-catenin, and epithelial membrane antigen (EMA). Molecular genetic studies using Ion AmpliSeq Cancer Hotspot Panel v2 by massively parallel sequencing on Ion Torrent PGM were performed in 2 cases with an atypical matrical component (1 was previously subjected to microdissection to sample the matrical and BCC areas separately). There were 13 male and 9 female patients, ranging in age from 41 to 89 years. Microscopically, all lesions manifested at least 2 components, a BCC area (follicular germinative differentiation) and areas with matrical differentiation. A BCC component dominated in 14 cases, whereas a matrical component dominated in 4 cases. Matrical differentiation was recognized as matrical/supramatrical cells (n=21), shadow cells (n=21), bright red trichohyaline granules (n=18), and blue-gray corneocytes (n=18). In 2 cases, matrical areas manifested cytologic atypia, and a third case exhibited an infiltrative growth pattern, with the tumor metastasizing to a lymph node. BerEP4 labeled the follicular germinative cells, whereas it was markedly reduced or negative in matrical areas. The reverse pattern was seen with β-catenin. EMA was negative in BCC areas but stained a proportion of matrical/supramatrical cells. Genetic studies revealed mutations of the following genes: CTNNB1, KIT, CDKN2A, TP53, SMAD4, ERBB4, and PTCH1, with some differences between the matrical and BCC components. It is concluded that matrical differentiation in BCC in most cases occurs as multiple foci. Rare neoplasms manifest atypia in the matrical areas

  18. Clear-cell variant urothelial carcinoma of the bladder: a case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Hossein Tezval

    2012-10-01

    Full Text Available Clear cell variants of transitional cell carcinomas (TCC of the bladder are extremely rare tumors. Only 6 cases have been reported until now. We report of a 67 year old man who presented with fast growing tumor disease. While initial diagnosis showed localized bladder tumor, final histopathology revealed pT4, G3, L1 urothelial carcinoma with clear cell differentiation. No more than 14 weeks after initial diagnosis the patient died from multi-organ failure after unsuccessful salvage laparotomy which showed massive tumor burden within the pelvis and peritoneal carcinosis. This case demonstrated an extremely fast tumor growth. Therefore, patients with clear cell urothelial carcinoma should be treated vigorously and without time delay. We present a case of clear cell variant of TCC which exhibited an extremely aggressive behavior. To our knowledge this is the fifth report of this rare disease.

  19. Immunoglobulin derived depositions in the nervous system: novel mass spectrometry application for protein characterization in formalin-fixed tissues.

    Science.gov (United States)

    Rodriguez, Fausto J; Gamez, Jeffrey D; Vrana, Julie A; Theis, Jason D; Giannini, Caterina; Scheithauer, Bernd W; Parisi, Joseph E; Lucchinetti, Claudia F; Pendlebury, William W; Bergen, H Robert; Dogan, Ahmet

    2008-10-01

    Proteinaceous deposits are occasionally encountered in surgically obtained biopsies of the nervous system. Some of these are amyloidomas, although the precise nature of other cases remains uncertain. We studied 13 cases of proteinaceous aggregates in clinical specimens of the nervous system. Proteins contained within laser microdissected areas of interest were identified from tryptic peptide sequences by liquid chromatography-electrospray tandem mass spectrometry (LC-MS/MS). Immunohistochemical studies for immunoglobulin heavy and light chains and amyloidogenic proteins were performed in all cases. Histologically, the cases were classified into three groups: 'proteinaceous deposit not otherwise specified' (PDNOS) (n=6), amyloidoma (n=5), or 'intracellular crystals' (n=2). LC-MS/MS demonstrated the presence of lambda, but not kappa, light chain as well as serum amyloid P in all amyloidomas. lambda-Light-chain immunostaining was noted in amyloid (n=5), although demonstrable monotypic lymphoplasmacytic cells were seen in only one case. Conversely, in PDNOS kappa, but not lambda, was evident in five cases, both light chains being present in a single case. In three cases of PDNOS, a low-grade B-cell lymphoma consistent with marginal zone lymphoma was present in the brain specimen (n=2) or spleen (n=1). Lastly, in the 'intracellular crystals' group, the crystals were present within CD68+ macrophages in one case wherein kappa-light chain was found by LC-MS/MS only; the pathology was consistent with crystal-storing histiocytosis. In the second case, the crystals contained immunoglobulin G within CD138+ plasma cells. Our results show that proteinaceous deposits in the nervous system contain immunoglobulin components and LC-MS/MS accurately identifies the content of these deposits in clinical biopsy specimens. LC-MS/MS represents a novel application for characterization of these deposits and is of diagnostic utility in addition to standard immunohistochemical analyses.

  20. Oral Granular Cell Tumor: Report of Case Series and a Brief Review of the Literature

    Directory of Open Access Journals (Sweden)

    Karakostas Panayiotis

    2017-07-01

    Full Text Available Background/Aim: The present analysis focuses on examining a case series of eight patients diagnosed with a granular cell tumor located in the oral cavity. Case series: The patients’ clinical states were thoroughly studied, along with the histopathological and immunohistochemical examinations findings. Their surgical treatment and postoperative course are also within the scope of this analysis. Numerous histogenesis theories and the appropriate tumor treatment are mentioned within the article being always in accordance with the relative literature. Conclusions: Oral granular cell tumor is a benign oral disease of possible neural origin commonly located on the tongue. Surgical excision is the treatment of choice. In any case, histological and immunohistochemical examination confirm both the clinical diagnosis and the differential diagnosis between oral squamous cell carcinoma.

  1. A case of congenital Langerhans cell histiocytosis with skin and thymic lesions: Exploring the prognostic value of thymus involvement

    Directory of Open Access Journals (Sweden)

    M.M. Escudero-Góngora

    2016-10-01

    Full Text Available Thymus evaluation is not included in the guidelines of the Histiocyte Society, so its prevalence, management and prognosis are not well established. We present a newborn with self-healing cutaneous LCH and thymic involvement that was evaluated with a thoracic ultrasound. With the current evidence we are unable to predict the prognosis of the thymus association in neonatal LCH. We suggest that performing thymic ultrasound study, which is a non-invasive technique, would allow us to know the incidence of thymic involvement and its role on prognosis.

  2. Merkel cell carcinoma occurring in a black woman: a case report.

    Science.gov (United States)

    Kadiri, Selma; Aissa, Abdellah; Berhili, Soufiane; Khmou, Mouna; Elmajjaoui, Sanaa; Kebdani, Tayeb; El Khannoussi, Basma; Elkacemi, Hanan; Benjaafar, Noureddine

    2017-01-31

    Merkel cell carcinoma is a rare, very aggressive neuroectodermal tumor of the skin. It is typically located on sun-exposed skin and frequently found in white men aged between 70 and 80 years. We report a case of a 58-year-old black woman diagnosed with Merkel cell carcinoma of the posterior face of the right elbow. She had biopsy excision and was lost to follow-up. Four months later, she presented with recurrent disease on the inferior third of the right arm with three ipsilateral axillary lymph node metastases. Amputation of the right arm and ipsilateral axillary lymph node dissection were performed, followed by adjuvant radiotherapy. Six months later, the patient died as a result of respiratory failure caused by lung metastasis. To the best of our knowledge, no specific studies have been done comparing the course and the characteristics of Merkel cell carcinoma in white and black populations, and no similar case has been reported in the literature. The Merkel cell carcinoma is very rare in black people. As described elsewhere in the literature, our patient had a poor outcome despite radical management. To date, to the best of our knowledge, there has been no comparison of the prognosis of this tumor in white and black populations.

  3. Synchronous Oligometastatic Non-Small Cell Lung Cancer and Isolated Renal Cell Carcinoma: A Case Report and Literature Review.

    Science.gov (United States)

    Nguyen, Timothy K; Louie, Alexander V

    2015-10-27

    A 58-year-old gentleman presenting with a progressive headache, visual disturbance, decreased appetite, and weight loss was found to have a localized clear cell carcinoma of the kidney and synchronous Stage IV non-small cell lung cancer with a solitary brain metastasis. This case illustrates the challenges in distinguishing between primary and metastatic disease in a patient with both renal cell carcinoma and lung cancer. We highlight the uncertainties in the diagnosis and management of this unique clinical scenario and the potential implications on prognosis.

  4. Giant cell lesions with a Noonan-like phenotype: a case report.

    Science.gov (United States)

    Cancino, Claudia Marcela H; Gaião, Léonilson; Sant'Ana Filho, Manoel; Oliveira, Flavio Augusto Marsiaj

    2007-05-01

    The purpose of this article is to describe a case of multiple giant cell lesions of the mandible that occurred in a 14-year-old girl with phenotypic characteristics associated with Noonan Syndrome (NS). NS is a dysmorphic disorder characterized by hypertelorism, short stature, congenital heart defects, short and webbed neck, skeletal anomalies, and bleeding diathesis. A 14-year-old girl with a previous diagnosis of NS (sporadic case) presented with multiple radiolucent lesions in the body and ramus of her mandible. In terms of clinical behavior and the described radiographic characteristics, giant cells lesions with Noonan-like phenotype can be considered a form of cherubism. Therefore, surgical intervention is not necessary, but radiographic follow-up and observation is very important during the control and gradual regression of the lesions.

  5. Primary B cell lymphoma of the tongue base: a case report

    Science.gov (United States)

    Bechir, Achour; Asma, Achour; Haifa, Regaieg; Nesrine, Abdessayed; Yosra, Ben Youssef; Badreddine, Sriha; Abderrahim, Khelif

    2016-01-01

    Primary non-Hodgkin’s lymphoma’s of the tongue is very rare and accounts for 1% of all malignant tumor of the oral cavity. Clinical features are non-specific ulcerative lesions that do not heal. In the literature, the majority of cases are diffuse large B cell type however, T cell phenotype also may occur. We describe a 77 years old man, who presented with an ulcerative mass in the left margin of the tongue the diagnosis diffuse large B cell lymphoma was confirmed. The patient is actually on treatment R-mini CEOP and has favorable evolution. PMID:28292136

  6. Malignant primary germ-cell tumor of the brain: case report

    Energy Technology Data Exchange (ETDEWEB)

    Yamamoto, Toyoshiro; Sato, Shinichi; Nakao, Satoshi; Ban, Sadahiko; Namba, Koh (Kobe Municipal Central Hospital (Japan))

    1983-04-01

    The unusual case of a 15 year old boy with three discrete paraventricular germ-cell tumors is reported. The first tumor was located just lateral to the left thalamus and included a massive cystic part around it, the second tumor in the paraventricular region above the head of the left caudate nucleus and the third tumor in the medial part of the left parietal lobe. Total removal of all tumors was successfully accomplished in stages at four separate operations, namely, the first tumor was removed through the left transsylvian approach, the second tumor via left superior frontal gyrus and the third tumor via left superior frontal gyrus and left superior parietal lobule. Histological examination revealed that the first tumor was teratoma, the second was choriocarcinoma and the third was germinoma. Primary germ-cell tumors of the brain can be divided into 5 groups: 1) germinoma; 2) embryonal carcinoma; 3) choriocarcinoma; 4) yolk-sac tumor; or 5) teratoma. In this case, a combination of three different histological patterns was seen. If malignant germ-cell tumor is supected on CT, aggressive extirpation should be done, not only to determine the exact diagnosis, but also to provide the basis for subsequent adjunctive therapy.

  7. Giant cell granuloma of the maxilla - a case report and review of the literature

    International Nuclear Information System (INIS)

    Setubal, Roger; Menezes, Benedito; Carvalho, Marcos Brasilino de; Soares, Aldemir Humberto; Souza, Ricardo Pires de

    1997-01-01

    Giant cell granuloma is an uncommon lesion of the giant cell lesion's group, which includes brown tumor of hyperparathyroidism, true giant cell tumor, cherubism and aneurysmal bone cyst. their histologic features are very similar and make certain types indistinguishable from each other, remaining a considerable controversy on its classification. The authors report a case of giant cell maxillary granuloma and makes a review of the literature. (author)

  8. Primary mucinous adenocarcinoma of the bladder with signet-ring cells: case report

    Directory of Open Access Journals (Sweden)

    Marcelo Lorenzi Marques

    Full Text Available CONTEXT: Primary adenocarcinomas of the bladder are uncommon and usually occur by contiguity with or hematogenic dissemination of other adenocarcinomas such as colorectal, prostate and gynecological tract carcinomas. Mucinous and signet-ring cell histological patterns are even rarer and it is often difficult to morphologically distinguish them from metastatic colorectal adenocarcinoma. CASE REPORT: We present and discuss a rare case of primary mucinous adenocarcinoma of the bladder with signet-ring cells in a 57-year-old male patient. Other primary sites for the tumor had been excluded and, in the absence of digestive tract tumor and for confirmation that it was a primary bladder tumor, an immunohistochemistry study was performed.

  9. Case report 331: Small cell osteosarcoma of the tibia with diffuse metastatic disease

    International Nuclear Information System (INIS)

    Roessner, A.; Miebs, T.; Grundmann, E.; Immenkamp, M.; Hiddemann, W.; Althoff, J.

    1985-01-01

    In summary, the case is presented of a 29-year-old woman who developed a sclerosing small-cell osteosarcoma in the upper end of the tibia. The unique features in this case are reflected both in its morphology and protracted clinical course, while its histological pattern resembles in some features a small cell variant of the highly malignant osteosarcoma described by Sim and Martin. In addition to the unusual clinical course, the failure in response to chemotherapy underscores that this tumor differed in its biological behavior from other highly malignant types of osteosarcoma. The importance of DNA analysis is stressed. (orig./WU)

  10. A case of squamous cell carchinoma, basal cell epithelioma and Bowen's disease arising from chronic radiodermatitis

    International Nuclear Information System (INIS)

    Matsushima, Hironori; Yamasaki, Kenshi; Hatamochi, Atsushi; Shinkai, Hiroshi

    1997-01-01

    We report a case of multiple cancers developing in chronic radiodermatitis. A 43-year-old Japanese male presented with chronic radiodermatitis on both dorsal surfaces of his fingers, following a long-term irradiation by Grenz rays for the treatment of psoriasis vulgaris. At the same site, he developed squamous cell carcinoma and Bowen's disease 7 years after the last irradiation. Three years later, he noticed a black papule on the dorsal surface of his right middle finger. After a histological examination, this papule diagnosed to be basal cell epithelioma. (author)

  11. Cell therapy for the treatment of lower limb lymphedema. Case report

    International Nuclear Information System (INIS)

    Goicoechea Diaz, Pedro; Hernandez Ramirez, Porfirio; Artaza Sanz, Heriberto

    2010-01-01

    Although lymphedema is a common disabling disease causing significant morbidity for affected patients, treatment for this condition remains limited and largely ineffective. Some reported data suggest that some bone-marrow derived cells may play a role in lymphangiogenesis. It appears that blood vessels and lymphatic vessels might use the same population of cells for vasculogenesis and lymphangiogenesis. Therefore, adult stem cell therapy could be a new useful strategy for the treatment of lymphedema. We report a resolution of a severe lower limb bilateral lymphedema after implantation of autologous adult stem cells derived from bone marrow. As far as we know, this is the first reported case with chronic lower limb lymphedema treated successfully with autologous cell therapy. This procedure is a low-cost, relatively simple and easy to perform option that opens new ways for the treatment of lymphedema

  12. Radiation-induced spindle cell sarcoma: A rare case report

    Directory of Open Access Journals (Sweden)

    Khan Mubeen

    2009-01-01

    Full Text Available Ionizing radiation has been known to induce malignant transformation in human beings. Radiation-induced sarcomas are a late sequel of radiation therapy. Most sarcomas have been reported to occur after exposure to a radiation dose of 55 Gray (Gy and above, with a dose ranging from 16 to 112 Gys. Spindle cell sarcomas, arising after radiotherapy given to treat the carcinoma of head and neck region is a very uncommon sequel. This is a rare case report of spindle cell sarcoma of left maxilla, in a 24-year-old male, occurring as a late complication of radiotherapy with Cobalt-60 given for the treatment of retinoblastoma of the left eye 21 years back.

  13. Oral plasma cell granuloma: A case report of an ambiguous lesion

    Directory of Open Access Journals (Sweden)

    Manveen Kaur Jawanda

    2014-01-01

    Full Text Available Plasma cell granuloma (PCG is a rare reactive tumor such as proliferation composed chiefly of plasmacytic infiltrate. Both clinically and histopathologically, it may be misinterpreted as various pathological entities thus necessitating the complete evaluation of patient and proper histopathological and immunohistochemical analysis of the tissue to rule out other lesions with poor prognosis. Here, we present a case of PCG of gingiva in a female patient masquerading as pyogenic granuloma clinically and plasma cell neoplasms histopathologically.

  14. Acute T- cell lymphoblastic lymphoma - A case report | Sumba | East ...

    African Journals Online (AJOL)

    We highlight the case of a two year old female who presented with a two month history of left posterior auricular swelling. The swelling developed following trauma, was painless and progressively enlarging. After extensive evaluation the mass was noted to be an extramedullary presentation of Acute T cell lymphoblastic ...

  15. Clear cell hidradenocarcinoma of the eyelid: a case report with a review of the literature.

    Science.gov (United States)

    Singh, Gurcharan; Narasimha, Aparna; Kumar, Harendra; Datti, Narendra

    2013-04-01

    Clear cell hidradenocarcinomas are extremely rare neoplasms, with very few well-documented cases reported in the literature. The most common sites are the head and neck regions. These tumors are histologically malignant but are not always aggressive. They are known for recurrence and may metastasize widely. Treatment is wide local resection. We report on a case of clear cell hidradenocarcinoma occurring over the eyelid together with a review of the literature.

  16. Merkel Cell Carcinoma with Spontaneous Regression: A Case Report and Immunohistochemical Study

    Directory of Open Access Journals (Sweden)

    Hitoshi Terui

    2016-02-01

    Full Text Available Merkel cell carcinoma (MCC is an aggressive neuroendocrine carcinoma that only rarely regresses spontaneously. Since little is known about the immunological mechanisms involved in the spontaneous regression of MCC, we describe a case of MCC with spontaneous regression and employed immunohistochemical staining for cytotoxic and immunosuppressive molecules to investigate possible mechanisms involved in the spontaneous regression of MCC. Interestingly, compared to conventional MCC, tumor-infiltrating lymphocytes in MCC with spontaneous regression contained higher numbers of CD8+ cells and granulysin-bearing cells and lower numbers of CD206+ cells. Our present study suggests one of the possible reasons for the spontaneous regression of MCC.

  17. Early Onset Squamous Cell Carcinoma In A Case Of Lichen Planus

    Directory of Open Access Journals (Sweden)

    Singh Shri Nath

    1998-01-01

    Full Text Available Lichen planus, which is a very common condition, is being presented. However, the uncommon feature in this cases is its early onset and equally early development of squamous cell carcinoma on a lesion on the right thigh.

  18. Mesenchymal Stem Cells May Ameliorate Nephrotic Syndrome Post-Allogeneic Hematopoietic Stem Cell Transplantation-Case Report

    Directory of Open Access Journals (Sweden)

    Xin Zhang

    2017-08-01

    Full Text Available IntroductionBecause of their immunomodulatory and anti-inflammatory effects, mesenchymal stem cells (MSCs have been considered as potential therapeutic agents for treating immune-related or autoimmune diseases, such as graft-versus-host disease (GVHD. Nephrotic syndrome (NS after allogeneic hematopoietic stem cell transplantation (allo-HSCT is an uncommon complication with unclear etiology and pathogenesis. It may be an immune disorder involving immune complex deposition, B cells, regulatory T cells (Tregs, and Th1 cytokines and be a manifestation of chronic GVHD. Corticosteroids and calcium antagonists, alone or in combination, are the most common therapeutic agents in this setting. Rituximab is commonly administered as salvage treatment. However, treatment failure and progressive renal function deterioration has been reported to occur in approximately 20% of patients in a particular cohort.Case presentationWe present a patient who developed NS 10 months after allo-HSCT. After treatment failure with cyclosporine A, prednisone, and rituximab, she achieved a complete response with MSC treatment. The clinical improvement of this patient was accompanied by a decreased B cell population together with an increased frequency of regulatory B cells (Bregs and Tregs after MSC treatment.ConclusionMSCs could modulate NS after allo-HSCT by suppressing B cell proliferation, inducing Tregs and Bregs, and inhibiting inflammatory cytokine production by monocytes and NK cells. Among all these, Bregs might play an important role in ameliorating the NS of this patient.

  19. Two cases of breast carcinoma with osteoclastic giant cells: Are the osteoclastic giant cells pro-tumoural differentiation of macrophages?

    Directory of Open Access Journals (Sweden)

    Shishido-Hara Yukiko

    2010-08-01

    Full Text Available Abstract Breast carcinoma with osteoclastic giant cells (OGCs is characterized by multinucleated OGCs, and usually displays inflammatory hypervascular stroma. OGCs may derive from tumor-associated macrophages, but their nature remains controversial. We report two cases, in which OGCs appear in common microenvironment despite different tumoural histology. A 44-year-old woman (Case 1 had OGCs accompanying invasive ductal carcinoma, and an 83-year-old woman (Case 2 with carcinosarcoma. Immunohistochemically, in both cases, tumoural and non-tumoural cells strongly expressed VEGF and MMP12, which promote macrophage migration and angiogenesis. The Chalkley count on CD-31-stained sections revealed elevated angiogenesis in both cases. The OGCs expressed bone-osteoclast markers (MMP9, TRAP, cathepsin K and a histiocyte marker (CD68, but not an MHC class II antigen, HLA-DR. The results indicate a pathogenesis: regardless of tumoural histology, OGCs derive from macrophages, likely in response to hypervascular microenvironments with secretion of common cytokines. The OGCs have acquired bone-osteoclast-like characteristics, but lost antigen presentation abilities as an anti-cancer defense. Appearance of OGCs may not be anti-tumoural immunological reactions, but rather pro-tumoural differentiation of macrophage responding to hypervascular microenvironments induced by breast cancer.

  20. Humeral Metastasis in a case of Squamous Cell Carcinoma - a ...

    African Journals Online (AJOL)

    A rare case of squamous cell carcinoma with metastasis to distal acral skeleton – humerus within two months of diagnosis of the primary is being reported. The metastasis to the bones from carcinoma cervix is uncommon especially in the distal appendicular skeleton. A 47 years female came with spontaneous fracture of ...

  1. Mantle cell lymphoma of the gastrointestinal tract presenting with multiple intussusceptions – case report and review of literature

    Directory of Open Access Journals (Sweden)

    Abo Stephen M

    2009-07-01

    Full Text Available Abstract Background Mantle cell lymphoma (MCL is an aggressive type of B-cell non-Hodgkin's lymphoma that originates from small to medium sized lymphocytes located in the mantle zone of the lymph node. Extra nodal involvement is present in the majority of cases, with a peculiar tendency to invade the gastro-intestinal tract in the form of multiple lymphomatous polyposis. MCL can be accurately diagnosed with the use of the highly specific marker Cyclin D1. Few cases of mantle cell lymphoma presenting with intussuception have been reported. Here we present a rare case of multiple intussusceptions caused by mantle cell lymphoma and review the literature of this disease. Case presentation A 68-year-old male presented with pain, tenderness in the right lower abdomen, associated with nausea and non-bilious vomiting. CT scan of abdomen revealed ileo-colic intussusception. Laparoscopy confirmed multiple intussusceptions involving ileo-colic and ileo-ileal segments of gastrointestinal tract. A laparoscopically assisted right hemicolectomy and extended ileal resection was performed. Postoperative recovery was uneventful. The histology and immuno-histochemistry of the excised small and large bowel revealed mantle cell lymphoma with multiple lymphomatous polyposis and positivity to Cyclin D1 marker. The patient was successfully treated with Rituximab-CHOP chemotherapy and remains in complete remission at one-year follow-up. Conclusion This is a rare case of intestinal lymphomatous polyposis due to mantle cell lymphoma presenting with multiple small bowel intussusceptions. Our case highlights laparoscopic-assisted bowel resection as a potential and feasible option in the multi-disciplinary treatment of mantle cell lymphoma.

  2. Seven cases of radiation-induced cutaneous squamous cell carcinoma

    Energy Technology Data Exchange (ETDEWEB)

    Sugita, Kazunari; Yamamoto, Osamu; Suenaga, Yoshinori [Univ. of Occupational and Environmental Health, Kitakyushu, Fukuoka (Japan). School of Medicine

    2000-09-01

    We report 7 cases of radiation-induced skin cancer. The diagnosis was based on the history of radiotherapy for benign skin diseases (5 cases) and of occupational exposures to medical doctors (2 cases). All cases were squamous cell carcinomas which arose from chronic radiodermatitis. The estimated latent period of these tumors ranged from 6 to 64 years, with an average of 29.9 years. After surgical treatments of the lesions, no local recurrences were observed in all cases. Benign skin diseases had sometimes been treated with low-energy radiation before the 1960s. Considering the estimated latent period, the peak time point of developing risk of radiation-induced skin cancer by such treatment has been already passed, however, the danger of it should not be ignored in future. In association with multiplicity of radiation usage, occupational exposure of radiation may develop the risk of occurrence of skin cancer in future. Therefore, we should recognize that radiation-induced skin cancer is not in the past. In the cases of chronic skin diseases showing warty keratotic growth, erosion and ulcer, we should include chronic radio-dermatitis in the differential diagnosis. It is necessary to recall all patients about the history of radiotherapy or radiation exposure. Rapid histopathological examination is mandatory because of the suspicion of radiation-induced skin cancer. (author)

  3. Seven cases of radiation-induced cutaneous squamous cell carcinoma

    International Nuclear Information System (INIS)

    Sugita, Kazunari; Yamamoto, Osamu; Suenaga, Yoshinori

    2000-01-01

    We report 7 cases of radiation-induced skin cancer. The diagnosis was based on the history of radiotherapy for benign skin diseases (5 cases) and of occupational exposures to medical doctors (2 cases). All cases were squamous cell carcinomas which arose from chronic radiodermatitis. The estimated latent period of these tumors ranged from 6 to 64 years, with an average of 29.9 years. After surgical treatments of the lesions, no local recurrences were observed in all cases. Benign skin diseases had sometimes been treated with low-energy radiation before the 1960s. Considering the estimated latent period, the peak time point of developing risk of radiation-induced skin cancer by such treatment has been already passed, however, the danger of it should not be ignored in future. In association with multiplicity of radiation usage, occupational exposure of radiation may develop the risk of occurrence of skin cancer in future. Therefore, we should recognize that radiation-induced skin cancer is not in the past. In the cases of chronic skin diseases showing warty keratotic growth, erosion and ulcer, we should include chronic radio-dermatitis in the differential diagnosis. It is necessary to recall all patients about the history of radiotherapy or radiation exposure. Rapid histopathological examination is mandatory because of the suspicion of radiation-induced skin cancer. (author)

  4. [Nasal type natural killer/T cell lymphoma: case series and literature review].

    Science.gov (United States)

    Düzlü, Mehmet; Ant, Ayça; Tutar, Hakan; Karamert, Recep; Şahin, Melih; Sayar, Erolcan; Cesur, Nesibe

    2016-01-01

    Nasal type natural killer/T-cell lymphoma is a rare type of extranodal non-Hodgkin lymphoma which originates from nasal cavity and paranasal sinuses. Exact diagnosis of nasal natural killer/T-cell lymphoma, which is a rapidly progressive clinical condition, may be established by immunohistochemical analysis on biopsy material after clinical suspicion. In this article, we report four cases of nasal natural killer/T-cell lymphoma who were followed-up in our clinic and discuss the diagnosis and treatment of the disease in light of the literature data.

  5. Vitamin E Intake and Risk of Renal Cell Carcinoma: A Meta-Analysis of 7 Case-Control Studies.

    Science.gov (United States)

    Shang, Yonggang; Yi, Shanhong; Cui, Dong; Han, Guangwei; Liu, Chengcheng

    2015-07-01

    Vitamin E intake may reduce the risk of renal cell carcinoma, but the results were inconsistent. Hence, we conducted a meta-analysis to assess the association between dietary vitamin E intake and the risk of renal cell carcinoma. We searched PubMed to identify the relevant case-control studies up to June 2014. Reference lists of retrieved articles were also reviewed. Odds ratios and corresponding 95% confidence intervals were used to estimate the association between dietary vitamin E intake and the risk of renal cell carcinoma. We identified 7 case-control studies regarding dietary vitamin E intake and risk of renal cell carcinoma, involving 5789 cases and 14866 controls. The odds ratio of renal cell carcinoma for the highest compared with the lowest dietary vitamin E intake was 0.75 (95% confidence interval: 0.59-0.91), and heterogeneity was observed across studies. The association between dietary vitamin E intake and the risk of renal cell carcinoma was not significantly differed by gender, but this association were inconsistent in the North American and European populations. Our study provided a evidence that there was a significant inverse association of dietary vitamin E intake with risk of renal cell carcinoma. However, this finding was based on the case-control studies, more well-designed cohort studies are needed. Copyright © 2015 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.

  6. A rare occurrence of a steroid cell tumor of the pelvic mesentery: a case report

    OpenAIRE

    Louis Robert; Murhekar Kanchan; Majhi Urmila

    2011-01-01

    Abstract Introduction Steroid cell tumors are microscopically characterized by abundant eosinophilic or vacuolated cytoplasm that is often positive for fat stains. These tumors could be of ovarian or ectopic adrenal origin. We present a rare case of a steroid cell tumor arising from the pelvic mesentery. Case presentation A 31-year-old Asian woman was undergoing treatment for infertility and virilizing symptoms. She underwent laparoscopy followed by laprotomy for a suspected ovarian cyst/pelv...

  7. Central Granular Cell Odontogenic Tumor: Report of a Case with CBCT Features.

    Directory of Open Access Journals (Sweden)

    Najmeh Anbiaee

    2014-06-01

    Full Text Available Central granular cell odontogenic tumor CGCOT of the jaw is an exceedingly rare benign odontogenic neoplasm with 35 reported cases in the literature. Among these, very few studies have focused on the cone-beam CT features of CGCOT. Here, we report a case of an asymptomatic CGCOT in a 16-year-old girl and focus on the cone-beam CT features. Only 36 cases of this lesion, including this one, have been reported so far. The case presented is of special importance due to the young age of the patient, the posterior location of the lesion and the multilocular pattern in the cone beam CT images.

  8. Congenital granular cell epulis—a case report

    Directory of Open Access Journals (Sweden)

    Alexander Aresdahl, DDS

    2015-03-01

    Full Text Available Congenital granular cell epulis (CGCE is an uncommon benign lesion found in newborns. It has predominance for females with an 8:1 ratio in relation to males and is exclusively encountered in the oral cavity. The most affected oral site is located around the canine/incisor region of the maxillary alveolar ridge, where the lesion arises from the soft tissue as a solitary pedunculated mass. CGCE's histogenesis remains obscure and controversial. We present a rare case of 2 separate CGCE lesions adjacent to each other measuring 23 × 18 × 10 and 15 × 10 mm, positioned facially on the right maxillary alveolar process. The patient, a 2-day-old female newborn, did not experience any serious difficulty regarding breathing or deglutition. Complete surgical excision was the treatment of choice in this case, and the procedure was performed under both general and local anesthesia. Histologic and immunohistochemical analysis confirmed the diagnosis of CGCE. The patient showed satisfactory postoperative healing and excellent health at both the 10-day recall appointment and the 6-month follow-up.

  9. Dendritic cell neurofibroma sine pseudorosettes: report of a case with a granulomatous appearance.

    Science.gov (United States)

    Petersson, Fredrik

    2011-10-01

    An unusual variant of dendritic cell neurofibroma is reported. In contrast to previous cases, the formation of pseudorosettes was lacking. The tumor was located on the anterior aspect of the thigh in a previously healthy 71-year-old woman with no evidence of neurofibromatosis. The tumor was composed of type-1 and type-2 cells, which were immunoreactive for S-100 protein and CD57. The granulomatous appearance was due to the zonal accumulation of CD34-positive dendritic cells and type-1 cells in a serpiginous fashion surrounding large areas with lesser cellularity featuring type-2 cells with scattered type-1 cells arranged in a haphazard fashion. Intralesional small neurites positive for neurofilament and perilesional perineural cells positive for epithelial membrane antigen were documented immunohistochemically.

  10. A rare case of primary clear cell sarcoma of the pubic bone resembling small round cell tumor: an unusual morphological variant

    International Nuclear Information System (INIS)

    Nakayama, Shoko; Tsuji, Motomu; Hanafusa, Toshiaki; Yokote, Taiji; Iwaki, Kazuki; Akioka, Toshikazu; Miyoshi, Takuji; Hirata, Yuji; Takayama, Ayami; Nishiwaki, Uta; Masuda, Yuki

    2012-01-01

    Clear cell sarcoma (CCS) and malignant melanoma share overlapping immunohistochemistry with regard to the melanocytic markers HMB45, S100, and Melan-A. However, the translocation t(12; 22)(q13; q12) is specific to CCS. Therefore, although these neoplasms are closely related, they are now considered to be distinct entities. However, the translocation is apparently detectable only in 50%–70% of CCS cases. Therefore, the absence of a detectable EWS/AFT1 rearrangement may occasionally lead to erroneous exclusion of a translocation-negative CCS. Therefore, histological assessment is essential for the correct diagnosis of CCS. Primary CCS of the bone is exceedingly rare. Only a few cases of primary CCS arising in the ulna, metatarsals, ribs, radius, sacrum, and humerus have been reported, and primary CCS arising in the pubic bone has not been reported till date. We present the case of an 81-year-old man with primary CCS of the pubic bone. Histological examination of the pubic bone revealed monomorphic small-sized cells arranged predominantly as a diffuse sheet with round, hyperchromatic nuclei and inconspicuous nucleoli. The cells had scant cytoplasm, and the biopsy findings indicated small round cell tumor (SRCT). Immunohistochemical staining revealed the tumor cells to be positive for HMB45, S100, and Melan-A but negative for cytokeratin (AE1/AE3) and epithelial membrane antigen. To the best of our knowledge, this is the first case report of primary CCS of the pubic bone resembling SRCT. This ambiguous appearance underscores the difficulties encountered during the histological diagnosis of this rare variant of CCS. Awareness of primary CCS of the bone is clinically important for accurate diagnosis and management when the tumor is located in unusual locations such as the pubic bone and when the translocation t(12; 22)(q13; q12) is absent

  11. The reclusive patient-a case report & clinical review of Merkel cell carcinoma

    Directory of Open Access Journals (Sweden)

    Ramanathan C

    2005-01-01

    Full Text Available Advanced Merkel Cell Carcinoma with intra - hepatic metastases in a reclusive gentleman is described. We present an interesting case with learning points and a review of this uncommon malignancy.

  12. Metaplastic thymoma with myasthenia gravis presumably caused by an accumulation of intratumoral immature T cells: a case report.

    Science.gov (United States)

    Tajima, Shogo; Yanagiya, Masahiro; Sato, Masaaki; Nakajima, Jun; Fukayama, Masashi

    2015-01-01

    Among human neoplasms, thymomas are well known for their association with paraneoplastic autoimmune diseases such as myasthenia gravis. However, regarding rare metaplastic thymoma, only one case of an association with myasthenia gravis has been reported. Here, we present the second case of a 44-year-old woman with metaplastic thymoma associated with myasthenia gravis. In metaplastic thymoma, intratumoral terminal deoxynucleotidyl transferase-positive T-cells (immature T-cells) are generally scarce, while they were abundant in the present case. We believe that these immature T-cells could be related to the occurrence of myasthenia gravis.

  13. A case with primary signet ring cell adenocarcinoma of the prostate and review of the literature

    Directory of Open Access Journals (Sweden)

    Orcun Celik

    2014-06-01

    Full Text Available Primary signet cell carcinoma of the prostate is a rare histological variant of prostate malignancies. It is commonly originated from the stomach, colon, pancreas, and less commonly in the bladder. Prognosis of the classical type is worse than the adenocarcinoma of the prostate. Primary signet cell adenocarcinoma is diagnosed by eliminating the adenocarcinomas of other organs such as gastrointestinal tract organs. In this case report, we present a case with primary signet cell adenocarcinoma of the prostate who received docetaxel chemotherapy because of short prostate specific antigen doubling time.

  14. A rare case of extremely high counts of circulating tumor cells detected in a patient with an oral squamous cell carcinoma

    International Nuclear Information System (INIS)

    Wu, Xianglei; Mastronicola, Romina; Tu, Qian; Faure, Gilbert Charles; De Carvalho Bittencourt, Marcelo; Dolivet, Gilles

    2016-01-01

    Despite aggressive regimens, the clinical outcome of head and neck squamous cell carcinoma remains poor. The detection of circulating tumor cells could potentially improve the management of patients with disseminated cancer, including diagnosis, treatment strategies, and surveillance. Currently, CellSearch ® is the most widely used and the only Food and Drug Administration-cleared system for circulating tumor cells detection in patients with metastatic breast, colorectal, or prostate cancer. In most cases of head and neck squamous cell carcinoma, only low counts of circulating tumor cells have been reported. A 56-year-old white male with no particular medical history, was diagnosed with a squamous cell carcinoma of oral cavity. According to the imaging results (computed tomography and 18 F-fluorodeoxyglucose positron emission tomography / computed tomography) and panendoscopy, the TNM staging was classified as T4N2M0. A non-interruptive pelvimandibulectomy was conducted according to the multidisciplinary meeting advices and the postoperative observations were normal. The patient complained of a painful cervical edema and a trismus 6 weeks after the surgery. A relapse was found by computed tomography and the patient died two weeks later. The search for circulating tumor cells in peripheral venous blood by using the CellSearch ® system revealed a very high count compared with published reports at three time points (pre-operative: 400; intra-operative: 150 and post-operative day 7: 1400 circulating tumor cells). Of note, all detected circulating tumor cells were epidermal growth factor receptor negative. We report here for the first time a rare case of oral squamous cell carcinoma with extremely high circulating tumor cells counts using the CellSearch ® system. The absolute number of circulating tumor cells might predict a particular phase of cancer development as well as a poor survival, potentially contributing to a personalized healthcare

  15. A case of squamous cell lung cancer after treating with radiation for small cell lung cancer

    International Nuclear Information System (INIS)

    Hayashi, Toshinari; Ide, Hiroshi; Siomi, Katsuhiko; Nakamura, Yukinobu; Tada, Shinya; Kageyama, Hiroshi; Kido, Masamitsu

    1999-01-01

    A 77-year-old man was admitted due to an abnormal shadow on a chest X-ray film in September 1993. Small cell lung cancer was diagnosed by transbronchial lung biopsy of left S 3 . Because of his pulmonary and renal dysfunction, he received only 40 Gy irradiation alone, and the tumor shadow disappeared. After 38 months' observation, a new nodular shadow was detected in the left upper lung field in March 1997. A tumor was found in left B 3 by bronchoscopy, and biopsy revealed squamous cell carcinoma. Because of his advanced age and hypoxia, he has had no active treatment. This was a rare case of small cell lung cancer with long term survival, treated only by radiation, in which a different histologic type of carcinoma appeared in the same radiation field. (author)

  16. Biphasic papillary renal cell carcinoma is a rare morphological variant with frequent multifocality: a study of 28 cases.

    Science.gov (United States)

    Trpkov, Kiril; Athanazio, Daniel; Magi-Galluzzi, Cristina; Yilmaz, Helene; Clouston, David; Agaimy, Abbas; Williamson, Sean R; Brimo, Fadi; Lopez, Jose I; Ulamec, Monika; Rioux-Leclercq, Nathalie; Kassem, Maysoun; Gupta, Nilesh; Hartmann, Arndt; Leroy, Xavier; Bashir, Samir Al; Yilmaz, Asli; Hes, Ondřej

    2018-04-01

    To further characterise biphasic squamoid renal cell carcinoma (RCC), a recently proposed variant of papillary RCC. We identified 28 tumours from multiple institutions. They typically showed two cell populations-larger cells with eosinophilic cytoplasm and higher-grade nuclei, surrounded by smaller, amphophilic cells with scanty cytoplasm. The dual morphology was variable (median 72.5% of tumour, range 5-100%); emperipolesis was found in all cases. The male/female ratio was 2:1, and the median age was 55 years (range 39-86 years). The median tumour size was 20 mm (range 9-65 mm). Pathological stage pT1a was found in 21 cases, pT1b in three, and pT3a and pT3b in one each (two not available). Multifocality was found in 32%: multifocal biphasic RCC in one case, biphasic + papillary RCC in two cases, biphasic + clear cell RCC in three cases, biphasic + low-grade urothelial carcinoma of the renal pelvis in one case, and biphasic + Birt-Hogg-Dubé syndrome in one case. Positive immunostains included: PAX8, cytokeratin (CK) 7, α-methylacyl-CoA racemase, epithelial membrane antigen, and vimentin. Cyclin D1 was expressed only in the larger cells. The Ki67 index was higher in the larger cells (median 5% versus ≤1%). Negative stains included: carbonic anhydrase 9, CD117, GATA-3, WT1, CK5/6, and CK20; CD10 and 34βE12 were variably expressed. Gains of chromosomes 7 and 17 were found in two evaluated cases. Follow-up was available for 23 patients (median 24 months, range 1-244 months): 19 were alive without disease, one was alive with recurrence, and one had died of disease (two had died of other causes). Biphasic papillary RCC is a rare variant of papillary RCC, and is often multifocal. © 2017 John Wiley & Sons Ltd.

  17. CT findings of primary squamous cell carcinoma of the stomach: a case report

    International Nuclear Information System (INIS)

    Kim, Kyoung Min; Lee, Chang Hee; Kim, Kyeong Ah; Park, Cheol Min

    2008-01-01

    Primary squamous cell carcinoma is a rare tumor of the stomach with an incidence ranging from 0.04% to 0.4% of all diagnosed gastric cancers. We report a case of squamous cell carcinoma in the stomach associated with hypertrophic gastropathy and observed as a huge mass and wall thickening on the greater curvature site by a multidetector CT

  18. Recurrent nitrofurantoin-induced giant cell interstitial pneumonia: Case report and literature review

    Directory of Open Access Journals (Sweden)

    Boeun Lee

    2015-01-01

    Full Text Available Giant cell interstitial pneumonia (GIP is a rare form of chronic interstitial pneumonia typically associated with hard metal exposure. Only two cases of GIP induced by nitrofurantoin have been reported in the medical literature. We are reporting a case of recurrent nitrofurantoin-induced GIP. Although extremely rare, GIP needs to be included in the differential diagnosis in patients with chronic nitrofurantoin use who present with respiratory illness.

  19. Ovarian germ cell tumors with rhabdomyosarcomatous components and later development of growing teratoma syndrome: a case report

    Directory of Open Access Journals (Sweden)

    Al-Jumaily Usama

    2012-01-01

    Full Text Available Abstract Introduction Development of a sarcomatous component in a germ cell tumor is an uncommon phenomenon. Most cases reported have a grim prognosis. Growing teratoma syndrome is also an uncommon phenomenon and occurs in approximately 2% to 7% of non seminomatous germ cell tumors and should be treated surgically. Case presentation We report the case of a 12-year-old Asian girl with an ovarian mixed germ cell tumor containing a rhabdomyosarcomatous component. She was treated with a germ cell tumor chemotherapy regimen and rhabdomyosarcoma-specific chemotherapy. Towards the end of her treatment, she developed a retroperitoneal mass that was increasing in size. It was completely resected, revealing a mature teratoma, consistent with growing teratoma syndrome. She is still in complete remission approximately three years after presentation. Conclusion The presence of rhabdomyosarcoma in a germ cell tumor should be treated by a combined chemotherapy regimen (for germ cell tumor and rhabdomyosarcoma. In addition, development of a mass during or after therapy with normal serum markers should raise the possibility of growing teratoma syndrome that should be treated surgically.

  20. A fatal case of immune hyperhemolysis with bone marrow necrosis in a patient with sickle cell disease

    Directory of Open Access Journals (Sweden)

    Matthew S. Karafin

    2017-03-01

    Full Text Available In patients with sickle cell disease, hyperhemolysis is a rare but life-threatening complication of transfusion. In this case report, we describe a 61 year-old woman with hemoglobin sickle cell (SC disease and history of alloimmunization who developed hyperhemolysis associated with a transfusion. She was found to have a warm and a clinically-significant cold autoantibody. Severe anemia (Hb 2.7 g/dL with reticulocytopenia and thrombocytopenia prompted a bone marrow biopsy, which demonstrated extensive bone marrow necrosis. Despite treatment, the bone marrow failure did not improve and the patient died on hospital day 38. This case illustrates the potential risks of transfusion in a patient with sickle cell disease, especially one with previous hemolytic reactions. While uncommon, hyperhemolysis can cause death, in this case by extensive bone marrow necrosis. In patients with sickle cell disease, judicious use of red cell transfusions with phenotypically-matched units can diminish, but never completely abrogate, the risks associated with transfusion.

  1. Primary plasma cell leukemia: A report of two cases of a rare and aggressive variant of plasma cell myeloma with the review of literature

    Directory of Open Access Journals (Sweden)

    Prithal Gangadhar

    2016-01-01

    Full Text Available Plasma cell leukemia (PCL is a rare and aggressive variant of myeloma accounting for 2-3% of all plasma cell dyscrasias characterized by the presence of circulating plasma cells. The diagnosis is based on the % (≥20% and absolute number (≥2x10 9 /L of plasma cells in the peripheral blood. The incidence of primary PCL (pPCL is very rare and reported to occur in <1 in a million. It is classified as either pPCL occurring at diagnosis or as secondary PCL in patients with relapsed/refractory myeloma. pPCL is a distinct clinicopathological entity with different cytogenetic and molecular findings. The clinical course is aggressive with short remissions and survival duration. We report two cases of pPCL, both having acute onset of illness, varied clinical presentation with one of them showing "hairy cell morphology," with rapidly progressing renal failure, and was not suspected to be plasma cell dyscrasia clinically. A detailed hematopathological evaluation clinched the diagnosis in this case. It is recommended that techniques such as immunophenotyping by flow cytometry and protein electrophoresis must be performed for confirmatory diagnosis. A detailed report of two cases and a review of PCL are presented here.

  2. Squamous cell lung carcinoma presenting as melena: a case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Ibrahim Azar

    2017-10-01

    Full Text Available Lung cancer has a predilection to widely metastasize to the liver, bone, brain and adrenal glands. Metastasis of primary lung tumors to the stomach is infrequent, with only sporadic cases reported. Most cases are asymptomatic and diagnosed post-mortem on autopsy. The incidence of symptomatic gastrointestinal metastases is extremely rare. Herein, we describe a case of gastric metastasis by squamous cell lung carcinoma, presenting as melena and diagnosed by esophagogastroduodenoscopy. To the best of our knowledge, only twenty other cases in the English literature have reported symptomatic gastric metastasis of lung cancer diagnosed by endoscopic biopsy. A brief review of the literature shows gastric metastasis of lung cancer to have a predilection to occur most frequently in male smokers with the most common type of tumor likely to be squamous cell carcinoma.

  3. Immunoexpression of EGFR and EMMPRIN in a series of cases of head and neck squamous cell carcinoma.

    Science.gov (United States)

    de Andrade, Ana Luiza Dias Leite; Ferreira, Stefânia Jeronimo; Ferreira, Sonia Maria Soares; Ribeiro, Camila Maria Beder; Freitas, Roseana de Almeida; Galvão, Hébel Cavalcanti

    2015-10-01

    The epidermal growth factor receptor (EGFR) and the extracellular matrix metalloproteinase inducer (EMMPRIN) have been identified as oncologically important targets. This study aimed to evaluate the immunoexpression of EGFR and EMMPRIN in a series of cases of head and neck squamous cell carcinoma (HNSCC). Forty-five cases of HNSCC were selected for this study and evaluated with anti-EGFR and anti-EMMPRIN antibodies. The percentage of positive cells was determined assessing to the following categories: score 1 (staining in 0-50% of cells), score 2 (staining in 51-75% of cells), and score 3 (staining in >75% of cells). Immunostaining intensity was graded according to the following parameters: score 1 (absent/weak expression) and score 2 (strong expression). For EGFR, a predominance of high median scores was observed in cases of both histological grades of malignancy and in different clinical stages (p>0.05). For EMMPRIN, a statistically significant difference was observed between the histological grades of malignancy (p=0.030). Regarding the immunostaining intensity of EMMPRIN, it was observed a predominance of score 1 in cases with stages I/II, whereas most cases with stages III/IV presented score 2 (p=0.032). Considering the anatomical location, most cases of buccal floor presented higher median score of EMMPRIN in comparison with the other sites (p=0.015). These findings suggest that both proteins are potential targets for cancer therapy and EMMPRIN can be used as a prognostic marker of a more aggressive biological behavior in patients with HNSCC. Copyright © 2015 Elsevier GmbH. All rights reserved.

  4. A case of squamous cell carchinoma, basal cell epithelioma and Bowen`s disease arising from chronic radiodermatitis

    Energy Technology Data Exchange (ETDEWEB)

    Matsushima, Hironori; Yamasaki, Kenshi; Hatamochi, Atsushi; Shinkai, Hiroshi [Chiba Univ. (Japan). School of Medicine

    1997-10-01

    We report a case of multiple cancers developing in chronic radiodermatitis. A 43-year-old Japanese male presented with chronic radiodermatitis on both dorsal surfaces of his fingers, following a long-term irradiation by Grenz rays for the treatment of psoriasis vulgaris. At the same site, he developed squamous cell carcinoma and Bowen`s disease 7 years after the last irradiation. Three years later, he noticed a black papule on the dorsal surface of his right middle finger. After a histological examination, this papule diagnosed to be basal cell epithelioma. (author)

  5. Treatment with adipose stem cells in a patient with moderate Alzheimer's disease: case report

    Directory of Open Access Journals (Sweden)

    Tsolaki M

    2015-10-01

    Full Text Available Magda Tsolaki,1,2 Stelios Zygouris,1,3 Vassilis Tsoutsikas,2 Doxakis Anestakis,2,4,5 George Koliakos6,7 1Third Department of Neurology, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece; 2Greek Association of Alzheimer's Disease and Related Disorders, Thessaloniki, Greece; 3CND+, 4Laboratory of Forensic Medicine and Toxicology, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece; 5Laboratory of General Biology, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece; 6Biohellenika Stem Cells Bank, Thessaloniki, Greece; 7Department of Biological Chemistry, Medical School, Aristotle University of Thessaloniki, Thessaloniki, Greece Objective: This article presents the case of a female patient with Alzheimer's disease (AD. The patient was treated with cholinesterase inhibitors and also with intravenous administration of autologous adipose stem cells.Methods: The patient was assessed with a neuropsychological battery including measures of general cognition, functional problems, neuropsychiatric issues, memory (verbal, visual and episodic, verbal learning and visuospatial abilities. Magnetic resonance imaging (MRI scans were conducted before and after the treatment with stem cells.Results: A transient and mild improvement of scores in measures of general cognition and neuropsychiatric issues was evident. A rapid deterioration followed the initial improvement. The first MRI scan showed ischemic areas in periventricular white matter of both hemispheres, as well as in both temporal and parietal lobes. The second MRI scan revealed the same picture with no significant changes.Conclusion: This case report indicates that the administration of stem cells is feasible in a clinical setting however its effectiveness in the treatment of AD is uncertain. The improvement of the patient's condition highlights the potential therapeutic action of stem cells, however the rapid deterioration poses

  6. Primary clear cell ductal adenocarcinoma of the pancreas: A case report and clinicopathologic literature review

    Directory of Open Access Journals (Sweden)

    Yashpal Modi

    2014-01-01

    Full Text Available We present a very rare, interesting case of a carcinoma of the pancreas with predominantly abundant clear cell morphology. According to the WHO classification, primary clear cell carcinoma of the pancreas is classified as a rare "miscellaneous" carcinoma. The tumor was observed in the distal body and tail of the pancreas of a 74-year-old woman. The histopathology of tumor cells showed well-defined cell membranes, clear cytoplasm, and prominent cell boundaries. Immunohistochemical (IHC staining showed positive reactions to antibodies against vimentin, cytokeratin 7 (CK-7, mucicarmine (MUC-1, periodic acid-Schiff (PAS, periodic acid-Schiff with diastase (PASD, carcinoembryonic antigen (CEA, and Carbohydrate Antigen 19-9 (CA 19-9. On the other hand, IHC staining was negative for alpha-fetoprotein (AFP, cytokeratin 20 (CK-20, HMB45, chromogranin, and synaptophysin. The patient was subsequently diagnosed with a primary solid-type pancreatic clear cell carcinoma with hepatic metastasis. Herein, we report this rare case and include a review of the current literature of this tumor.

  7. Granular cell tumor on perianal region: a case report.

    Directory of Open Access Journals (Sweden)

    Kesici Ugur

    2013-07-01

    Full Text Available Granular cell tumor (GCT was first described by Abrikossoff in 1926. GCT is a rarely seen soft tissue tumor and is generally benign. While the tumor can be seen in all parts of the body it is generally located on the head and neck region, and especially on the tongue. GCT is rarely seen in the anal-perianal region. In accordance with literature this case was reported because it was thought to be the 27th anal-perianal located GCT case. In this case report, approximately 0,5-1 cm pedunculated polypoid lesion was determined in the perianal region during the physical examination of a 23 year old female patient who applied with palpable mass complaint in the perianal region. Lesion in the patient was totally excited with healthy skin-subcutaneous tissue under local anesthesia. A benign granular cell tumor was detected in the histopathological examination. Positive staining was monitored immunohistochemically with S-100 and neuron specific enolase (NSE. GCT is a rarely seen tumor in the anal-perianal region and its malign transformation rate is very low. Even lesions seen in the perianal region have clinically a benign appearance, a histopathological examination should be conducted and also GCT should be kept in mind during diagnosis. Malign-benign separation of these lesions is difficult so histopathological examination should be conducted with great care. Large local excision in the treatment provides curative treatment. But for those presenting malign transformation further examination must be performed for metastasis. After the treatment local recurrence and metastasis should be considered carefully. Prognosis of metastatic disease is very bad.

  8. An Unusual Case of Locally Advanced Glycogen-Rich Clear Cell Carcinoma of the Breast

    Directory of Open Access Journals (Sweden)

    Beatriz Martín-Martín

    2011-09-01

    Full Text Available Glycogen-rich clear cell (GRCC is a rare subtype of breast carcinoma characterized by carcinoma cells containing an optically clear cytoplasm and intracytoplasmic glycogen. We present the case of a 55-year-old woman with a palpable mass in the right breast and clinical signs of locally advanced breast cancer (LABC. The diagnosis of GRCC carcinoma was based on certain histopathological characteristics of the tumor and immunohistochemical analysis. To our knowledge, this is the first case of GRCC LABC with intratumoral calcifications. There is no evidence of recurrence or metastatic disease after 14 months’ follow-up.

  9. Metastatic renal cell carcinoma – a case report

    Directory of Open Access Journals (Sweden)

    Grzegorz Wróbel

    2017-09-01

    Full Text Available Renal cell carcinoma (RCC is not a single uniform entity but a group of related neoplasms in which the histologic findings, cytogenetic abnormalities, biologic behavior and imaging appearances of the tumors are subtype dependent. PET-CT is the fusion of functional and anatomic information acquired almost simultaneously that lets us see the body and disease in a way that is diagnostically very powerful. The case concerns the result imaging 18F-fluorodeoxyglucose positron emission tomography (PET-CT to patient 47 years old (women is diagnosed with numerous changes in both lungs, the liver and the skeletal system in the abdominal lymph nodes. Primary change in left kidney is indicated. Metastasis is a process consisting of cells spreading from the primary site of the cancer to distant parts of the body. Functional imaging, particularly with PET–CT, might improve the accuracy of diagnosis and provide essential information that could allow clinicians to make more appropriate therapeutic decisions than they previously could without this technique.

  10. Primary T cell central nervous system lymphoblastic lymphoma in a child: case report and literature review.

    Science.gov (United States)

    Mazur, Marcus D; Ravindra, Vijay M; Alashari, Mouied; Raetz, Elizabeth; Poppe, Matthew M; Bollo, Robert J

    2015-06-01

    Primary central nervous system lymphoma (PCNSL) of T cell origin is rare in pediatric patients. We report a case of T cell PCNSL in a 12-year-old boy and review the literature to highlight the importance of brain biopsy to definitively establish the diagnosis when PCNSL is suspected. A 12-year-old boy presented with worsening left-sided weakness, nausea, vomiting, headache, blurred vision, and diplopia. Magnetic resonance imaging revealed right parietal gyral thickening with faint meningeal contrast enhancement. No clear diagnosis was identified after serum testing, cerebrospinal fluid analysis, and cerebral angiography. To establish the diagnosis definitively, a right craniotomy and open, frameless stereotactic biopsy were performed, which yielded the diagnosis of lymphoblastic T cell lymphoma. PCNSL of T cell origin in children remains poorly studied, with only 18 detailed cases reported over the last three decades, including this case. Establishing a definitive diagnosis of PCNSL is challenging, and a brain biopsy is often required to obtain enough tissue for pathological analysis. Increasing awareness and identification of children diagnosed with T cell PCNSL is needed to better understand the molecular biology of this disease and develop more standardized treatment regimens.

  11. The 'grey area' between small cell and non-small cell lung carcinomas. Light and electron microscopy versus clinical data in 14 cases

    NARCIS (Netherlands)

    Mooi, W. J.; van Zandwijk, N.; Dingemans, K. P.; Koolen, M. G.; Wagenvoort, C. A.

    1986-01-01

    We studied 14 lung tumours which on light microscopy had posed difficulties on classification as either small cell or non-small cell carcinomas. The light and electron microscopical features were compared with patient follow-up data. Electron microscopy showed neuroendocrine granules in 12 cases,

  12. Multifocal Langerhans cell sarcoma involving epidermis: a case report and review

    Directory of Open Access Journals (Sweden)

    Wang Changsong

    2012-08-01

    Full Text Available Abstract Objective To study the clinico-pathological characteristics of Langerhans cell sarcoma (LCS which involving epidermis. Methods A case of primary multifocal LCS was analyzed in histopathology and immunophenotype. Results A 41-year-old man with multifocal cutaneous LCS involving the inguina and waist was reported. Clinical and pathology data were available. Neoplastic cells with markedly malignant cytological features were observed. Tumor cells exhibited irregular shape with abundant and eosinophilic red staining cytoplasm; large, irregular-shaped, showing lobulated or dented nucleus and some cells with a longitudinal nuclear groove and prominent nucleoli. The tumor cells expressed CD1a, Langerin (CD207, S-100 protein, CD68 and vimentin, and did not express pan-T or B cell markers and epithelial markers. The patient died less than 1 year after diagnosis due to local recurrence and metastasis to the lung, despite the administration of local radiation and chemotherapy. Conclusions LCS is a tumor with markedly malignant cytological features that originates from Langerhans cells. Primary multifocal neoplasms involving epidermis is even rare. Accurate diagnosis is based on the histopathological and immunohistochemical of the tumor cells. Virtual slide The virtual slide(s for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1182345104754765.

  13. Nevoid basal cell carcinoma syndrome—case report and genetic study

    Directory of Open Access Journals (Sweden)

    Yu-Feng Huang

    2010-09-01

    Full Text Available Nevoid basal cell carcinoma syndrome (also named Gorlin-Goltz syndrome is a rare disease. Commonly seen features include multiple odontogenic keratocysts (OKCs, nevus-like basal cell carcinoma, and bifid ribs. Genetic alterations of the PTCH1 gene are associated with the disease. Herein, we report the case of a 15-year-old girl who presented with multiple OKCs, a bifid rib, ectopic calcification of the falx cer-ebri, and an arachnoid cyst of the cerebrum. No basal cell carcinoma was identified. In addition, a search for genetic alterations was performed on the patient. We identified a genetic mutation of C→T in exon 12 (c.1686 bp and a G→C mutation in intron 13 (g.91665 bp of the PTCH1 gene. Although a similar mutation in exon 12 was reported in a literature search, the mutation in intron 13 has not previously been reported. The patient has continued to be followed-up almost 3 years after the surgery with no recurrence of the OKCs or development of basal cell carcinoma.

  14. A Rare Case of Diffuse Idiopathic Pulmonary Neuroendocrine Cell Hyperplasia

    Directory of Open Access Journals (Sweden)

    Godwin Ofikwu

    2015-01-01

    Full Text Available Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH is a rare clinical condition with only about 100 cases reported in the literature. It is characterized by primary hyperplasia of pulmonary neuroendocrine cells (PNECs which are specialized epithelial cells located throughout the entire respiratory tract, from the trachea to the terminal airways. DIPNECH appears in various forms that include diffuse proliferation of scattered neuroendocrine cells, small nodules, or a linear proliferation. It is usually seen in middle-aged, nonsmoking women with symptoms of cough, dyspnea, and wheezing. We present a 45-year-old, nonsmoking woman who presented with symptoms of DIPNECH associated with bilateral pulmonary nodules and left hilar adenopathy. Of interest, DIPNECH in our patient was associated with metastatic pulmonary carcinoids, papillary carcinoma of the left breast, oncocytoma and angiomyolipoma of her left kidney, and cortical nodules suggestive of tuberous sclerosis. She had video assisted thoracoscopic surgery (VATS, modified radical mastectomy with reconstruction, and radical nephrectomy. She is currently symptom-free most of the time with over two years of follow-up.

  15. Nevoid basal cell carcinoma syndrome with medulloblastoma and meningioma. Case report

    International Nuclear Information System (INIS)

    Fukushima, Yutaka; Oka, Hidehiro; Utsuki, Satoshi; Iwamoto, Kazuhisa; Fujii, Kiyotaka

    2004-01-01

    A 35-year-old man presented with a rare case of nevoid basal cell carcinoma syndrome, or Gorlin's syndrome, associated with both medulloblastoma and meningioma, manifesting as visual field constriction due to multiple parasellar tumors. He had undergone resection of a medulloblastoma at the age of 1 year 9 months, followed by adjunctive irradiation with a total dose of 40 Gy. He presented with multiple subcutaneous nodules on his face and neck. Histological examination of biopsy specimens established the diagnosis of nevoid basal cell carcinoma syndrome. Tuberculum sellae meningioma was removed through a craniotomy, and his symptoms improved. Meningioma is known to occur in the field of therapeutic irradiation, so chemotherapy may be a better option for medulloblastoma associated with nevoid basal cell carcinoma syndrome. (author)

  16. Squamous cell carcinoma of the canine nasal cavity and frontal sinus: eight cases

    International Nuclear Information System (INIS)

    Rogers, K.S.; Walker, M.A.; Helman, R.G.

    1996-01-01

    Squamous cell carcinoma of the canine nasal cavity and frontal sinus was diagnosed in eight cases between May 1988 and April 1994. The most common presenting complaints were nasal discharge, including epistaxis; sneezing; and facial deformity or exophthalmos. Metastasis was not identified in any case, but bone lysis and invasion into tissues outside the nasal cavity were noted in five cases. Computed tomograms were performed in five cases and were more useful than radiographs in determining the extent of neoplastic involvement. Euthanasia was performed within one week of diagnosis in three cases at the owner's request; one case died at home within one month; and the remaining four cases were euthanized within eight months due to progressive clinical signs. The mean survival time in these eight cases was three months, with a range of zero weeks to eight months

  17. Follicular dendritic cell sarcoma: a report of six cases and a review of the Chinese literature

    Directory of Open Access Journals (Sweden)

    Wen Jifang

    2010-10-01

    Full Text Available Abstract Goals The main purpose of this study is to broaden the clinicopathological spectrum and increase recognition of follicular dendritic cell sarcoma (FDCS through analysis of the clinical and pathological features of 50 cases. Methods The clinicopathological features of total 50 cases of FDCS were analyzed including a review of 44 cases reported in Chinese literature before October 2009 and six original cases from the pathology files conducted by the authors. Results The youngest patient came under observation in this study is only seven years old. Including the cases contributed by the authors, our literary review indicated that male dominated the tumor cases (M: F = 3: 2. 28 cases (56% present with this disease in extranodal sites. Tumor cells demonstrated positive staining for the follicular dendritic cell markers CD21 (47/49, CD35 (43/45, CD23 (20/23 and CD68 (23/25. In situ hybridization for Epstein-Barr virus-encoded RNA was performed in 10 cases. Nevertheless, EBV expression was absent in all these cases. The follow-up analysis of all cases shows that 26 (81.2% patients were alive and disease free; 6 (18.8% patients were alive with recurrent disease or metastasis; and nobody had died of this disease at the time of last follow-up. Conclusions The diagnosis of the FDCS is based on the findings of morphology and immunohistochemistry. The FDCS occurred in China should be viewed and treated as a low-grade sarcoma, and the role of the EBV in the pathogenesis of this tumor is still uncertain. There is a possibility that the tumor might be racial or geographic correlated, because most cases were reported from Eastern Asia area; it's particular the case of the liver or spleen tumor.

  18. Follicular dendritic cell sarcoma: a report of six cases and a review of the Chinese literature.

    Science.gov (United States)

    Wang, Haiwei; Su, Zhansan; Hu, Zhongliang; Wen, Jifang; Liu, Baoan

    2010-10-11

    The main purpose of this study is to broaden the clinicopathological spectrum and increase recognition of follicular dendritic cell sarcoma (FDCS) through analysis of the clinical and pathological features of 50 cases. The clinicopathological features of total 50 cases of FDCS were analyzed including a review of 44 cases reported in Chinese literature before October 2009 and six original cases from the pathology files conducted by the authors. The youngest patient came under observation in this study is only seven years old. Including the cases contributed by the authors, our literary review indicated that male dominated the tumor cases (M: F = 3: 2). 28 cases (56%) present with this disease in extranodal sites. Tumor cells demonstrated positive staining for the follicular dendritic cell markers CD21 (47/49), CD35 (43/45), CD23 (20/23) and CD68 (23/25). In situ hybridization for Epstein-Barr virus-encoded RNA was performed in 10 cases. Nevertheless, EBV expression was absent in all these cases. The follow-up analysis of all cases shows that 26 (81.2%) patients were alive and disease free; 6 (18.8%) patients were alive with recurrent disease or metastasis; and nobody had died of this disease at the time of last follow-up. The diagnosis of the FDCS is based on the findings of morphology and immunohistochemistry. The FDCS occurred in China should be viewed and treated as a low-grade sarcoma, and the role of the EBV in the pathogenesis of this tumor is still uncertain. There is a possibility that the tumor might be racial or geographic correlated, because most cases were reported from Eastern Asia area; it's particular the case of the liver or spleen tumor.

  19. A Case of Squamous Cell Carcinoma Developing Within a Red-Ink Tattoo.

    Science.gov (United States)

    Sherif, Sara; Blakeway, Elizabeth; Fenn, Chris; German, Alyn; Laws, Philip

    Cutaneous reactions to tattoos are well reported and include allergic reactions, infections, and foreign body granuloma or may be a presenting sign of sarcoidosis. There have been very few reported cases of squamous cell carcinoma (SCC) arising in tattoo-treated skin. We report a case of SCC arising within a red-ink tattoo and discuss the potential the role of chronic low-grade inflammation in pathogenesis. This should serve to raise awareness of potential tattoo-related serious adverse effects.

  20. Metastatic Small Cell Carcinoma of the Breast from Cancer of the Uterine Cervix: A Case Report

    Directory of Open Access Journals (Sweden)

    Beom Seok Kwak

    2018-01-01

    Full Text Available We report here on a case of 51-year-old woman with metastatic small cell carcinoma of the breast that came from her cancer of the uterine cervix. She underwent radical hysterectomy with bilateral salpingo-oophorectomy due to small cell carcinoma of the uterine cervix, and adjuvant radiotherapy was administered to the pelvis. Breast metastasis with a palpable mass then occurred 3 months after the primary surgery. Simple mastectomy and adjuvant chemotherapy were performed. She initially showed a good response to the therapy, yet she ultimately died of multiple metastases with a fulminating disease course. This is an extremely rare case, and only 1 similar case has been reported earlier, so we report on this case along with a review of the relevant literature.

  1. Basaloid squamous cell carcinoma of the penis with papillary features: a clinicopathologic study of 12 cases.

    Science.gov (United States)

    Cubilla, Antonio L; Lloveras, Belén; Alemany, Laia; Alejo, María; Vidal, August; Kasamatsu, Elena; Clavero, Omar; Alvarado-Cabrero, Isabel; Lynch, Charles; Velasco-Alonso, Julio; Ferrera, Annabelle; Chaux, Alcides; Klaustermeier, Joellen; Quint, Wim; de Sanjosé, Silvia; Muñoz, Nubia; Bosch, Francisco Xavier

    2012-06-01

    There are 3 distinct variants of penile squamous cell carcinoma frequently associated with human papillomavirus (HPV): basaloid, warty-basaloid, and warty carcinomas. Considering the high incidence rates of penile cancer in some countries, a large international study was designed to evaluate the presence of HPV, its genotype distribution, and its association with histologic types of penile cancer. In this international review of >900 cases, we found a group of highly distinct papillary neoplasms composed of basophilic cells resembling urothelial tumors but frequently associated with HPV. Macroscopically, tumors were exophytic or exoendophytic. Microscopically, there was a papillomatous pattern of growth with a central fibrovascular core and small basophilic cells lining the papillae. Positivity for HPV was present in 11 of 12 tumors (92%). Single genotypes found were HPV-16 in 9 tumors and HPV-51 in 1 tumor. Multiple genotypes (HPV-16 and HPV-45) were present in another case. Overexpression of p16 was observed in all cases. Uroplakin-III was negative in all cases. The differential diagnosis was with basaloid, warty-basaloid, warty, and papillary squamous cell carcinoma and with urothelial carcinomas. Local excision (4 cases), circumcision (3 cases), or partial penectomy (5 cases) were preferred treatment choices. Tumor thickness ranged from 1 to 15 mm (average, 7 mm). Two patients with tumors invading 11 and 15 mm into the corpus spongiosum developed inguinal nodal metastasis. Of 11 patients followed up (median 48 mo), 7 were alive with no evidence of metastatic disease, 3 died from causes other than penile cancer, and another died postoperatively. This morphologically distinct tumor probably represents a papillary variant of basaloid carcinomas (papillary-basaloid carcinomas). Unlike typical basaloid carcinomas, the overall prognosis was excellent. However, deeply invasive tumors were associated with regional nodal metastasis indicating a potential for tumor

  2. Fine needle aspiration biopsy of three cases of squamous cell carcinoma presenting as a thyroid mass: cytological findings and differential diagnosis.

    Science.gov (United States)

    Rosa, M; Toronczyk, K

    2012-02-01

    Primary squamous cell carcinomas of the thyroid gland are extremely rare, comprising about 1% of thyroid malignancies. Although squamous cell carcinomas are readily identified as such on aspiration cytology in the majority of cases, the differentiation of primary versus metastatic tumour might not always be easy. Herein, we report three cases of squamous cell carcinomas involving the thyroid gland. Fine needle aspiration cytology (FNAC) was performed in three patients with a thyroid mass using standard guidelines. Smears were stained with Diff-Quik and Papanicolaou stains. Two patients were male and one was female, aged 59, 45 and 35 years, respectively. In all three patients a thyroid mass was present. FNAC smears in all cases showed cytological features of squamous cell carcinoma including keratinization and necrosis. After clinical and cytological correlation, one case appeared to be primary, one case metastatic, and in the third case no additional clinical information or biopsy follow-up was available for further characterization. Because primary squamous cell carcinoma of the thyroid is a rare finding, metastatic squamous cell carcinoma should always be excluded first. Metastatic disease usually presents in the setting of widespread malignancy, therefore a dedicated clinical and radiological investigation is necessary in these cases. In both clinical scenarios the patient's prognosis is poor. © 2010 Blackwell Publishing Ltd.

  3. Endodontic misdiagnosis of periapical central giant cell granuloma: Report of case with 2 years of follow-up

    Directory of Open Access Journals (Sweden)

    Rahul Kumar

    2012-01-01

    Full Text Available Central giant cell granuloma is considered as reactive lesion of jaws possibly to intramedullary hemorrhage or trauma. It may manifest as radiolucencies anywhere in the mandible or maxilla. In rare cases, it can appear as a localized periapical area and mimic an endodontic lesion. This report presents a case where central giant cell granuloma was misdiagnosed as a periapical cyst in 20-year-old male and was treated by conventional endodontic treatment. However the lesion was refractory to endodontic treatment and proved to be central giant cell granuloma after surgical intervention and histopathological examination. The purpose of this case report is to emphasize on periodic follow-up of periapical lesions after endodontic treatment and surgical intervention if required.

  4. [Growth in children with diabetes insipidus].

    Science.gov (United States)

    Morla Báez, E; Dorantes Alvarez, L M; Chavarría Bonequi, C

    1980-01-01

    Commercial preparations of vasopressin for the treatment of diabetes insipidus are not available in Mexico. Besides, the hormone is useless in the nephrogenic variety. In the department of Endocrinology at the Hospital Infantil de Mexico, a preparation containing hydrochlorothiazide, aminopyrine and potassium chloride, which reduces urinary volumes in about two thirds, is employed in all varieties of the disease. Growth in stature was investigated in 44 patients under treatment, attending the Endocrine Outpatient Clinic since 1967 for a period of 2 to 12 years. Clinical material included 29 males and 15 females. There were 23 idiopathic, 7 histiocytosis, 5 nephrogenic, 4 craniopharyngiomas, 2 psychogenic polydipsia, 2 traumatic and 1, as a sequel of tuberculous meningoencephalitis. Six idiopathic, 2 nephrogenic, 2 traumatic, 1 histiocytosis, and 1 psychogenic proceeded between percentiles 3 and 97, parallel to the nearest line of reference along the whole period of study. Two nephrogenic, 2 histiocytosis, 1 psychogenic, 1 post-meningoencephalitis and 14 idiopathic, grew below the third percentile, but parallel to it. One nephrogenic, 4 histiocytosis, 4 craniopharyngioma and 3 idiopathic progressively departed from the initial centile. Two of the latter had growth hormone deficiency, and 1 had been very irregularly treated. It is concluded that the therapy employed limits stature impairment but does not produce catch-up growth. Accordingly, it is proposed that the treatment of diabetes insipidus should be started as early as possible, and that if progress in stature is appreciably deteriorated, the presence of additional pathology should be suspected.

  5. Systemic mast cell disease (SMCD) and bone pain. A case treated with radiotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Hesselmann, S.; Micke, O.; Schaefer, U.; Willich, N. [University Hospital Muenster (Germany). Dept. of Radiotherapy and Radiooncology

    2002-05-01

    Background: Systemic mast cell disease (SMCD) is a rare disease characterized by a multitopic proliferation of cytologically and/or functionally abnormal tissue mast cells. SMCD preferentially involves the skin, spleen, liver, lymph nodes and the bone marrow. The cause of SMCD is unknown. Bony pain, caused by mast cell infiltration of the marrow cavity, is present in up to 28% of cases and is frequently chronic and difficult to palliate with medical therapy. Case Report: We report one case of refractory bone pain in a 54-year-old female Caucasian patient with advanced SMCD and associated bony involvement, which was treated with radiotherapy for pain palliation. Between 1995 and 1998, the patient was irradiated at four different locations: 1) right shoulder and proximal right humerus, 2) both hands, 3) both knees, 4) left humerus with a total dose of 40 Gy in 2.0 or 2.5 Gy daily fractions. Results: Different results of pain palliation were achieved. In one location the pain was reduced for 55 months until her death due to disease progression, whereas in two other locations a pain control was maintained for 3 and 6 months after radiotherapy. In one location, no pain reduction was achieved. Severe side effects were not observed. Conclusion: Palliative radiotherapy has a role in the control of severe intractable bone pain in patients with advanced SMCD, though in some cases the effect may be short or incomplete. The observed palliation of pain can even differ in the same patient. (orig.)

  6. Systemic mast cell disease (SMCD) and bone pain. A case treated with radiotherapy

    International Nuclear Information System (INIS)

    Hesselmann, S.; Micke, O.; Schaefer, U.; Willich, N.

    2002-01-01

    Background: Systemic mast cell disease (SMCD) is a rare disease characterized by a multitopic proliferation of cytologically and/or functionally abnormal tissue mast cells. SMCD preferentially involves the skin, spleen, liver, lymph nodes and the bone marrow. The cause of SMCD is unknown. Bony pain, caused by mast cell infiltration of the marrow cavity, is present in up to 28% of cases and is frequently chronic and difficult to palliate with medical therapy. Case Report: We report one case of refractory bone pain in a 54-year-old female Caucasian patient with advanced SMCD and associated bony involvement, which was treated with radiotherapy for pain palliation. Between 1995 and 1998, the patient was irradiated at four different locations: 1) right shoulder and proximal right humerus, 2) both hands, 3) both knees, 4) left humerus with a total dose of 40 Gy in 2.0 or 2.5 Gy daily fractions. Results: Different results of pain palliation were achieved. In one location the pain was reduced for 55 months until her death due to disease progression, whereas in two other locations a pain control was maintained for 3 and 6 months after radiotherapy. In one location, no pain reduction was achieved. Severe side effects were not observed. Conclusion: Palliative radiotherapy has a role in the control of severe intractable bone pain in patients with advanced SMCD, though in some cases the effect may be short or incomplete. The observed palliation of pain can even differ in the same patient. (orig.)

  7. [Primary peripheral T-cell lymphoma of the penis: a case report and review of the literature].

    Science.gov (United States)

    Shi, Yan-Lin; Yin, Hong-Lin; Zhou, Xiao-Jun; Zhou, Hang-Bo; Lu, Zhen-Feng

    2008-11-01

    To report a case of primary peripheral T-cell lymphoma of the penis. We analyzed the clinicopathological characteristics of the case of primary peripheral T-cell lymphoma using histological, cytochemical and immunohistochemical methods and by review of the literature. The patient was a 65 years old man and presented with a diffuse enlargement of the penis as the initial sign, followed by erosive ulcer in the caput penis and inguinal lymphadenectasis. The tumor was pathohistologically manifested as an epidermal ulcer, with tumorous necrosis around the capillary, infiltrative growth and atypical changes of the neoplastic cells and proliferation of capillaries. Immunohistochemically, the tumor cells were positive for CD43 and CD3, but negative for CD20, CD79a, CD34, CD30, CD56 and CD34. Clinically it responded to the chemotherapy designed for peripheral T-cell lymphoma. Primary peripheral T-cell lymphoma of the penis is an extremely rare malignant tumor, the diagnosis of which relies on histopathological examination, immunohistochemical staining and differentiation between squamous cell carcinoma and other types of lymphoma.

  8. Immunoarchitectural patterns in nodal marginal zone B-cell lymphoma: a study of 51 cases.

    Science.gov (United States)

    Salama, Mohamed E; Lossos, Izidore S; Warnke, Roger A; Natkunam, Yasodha

    2009-07-01

    Nodal marginal zone lymphoma (NMZL) represents a rare and heterogeneous group that lacks markers specific for the diagnosis. We evaluated morphologic and immunoarchitectural features of 51 NMZLs, and the following immunostains were performed: CD20, CD21, CD23, CD5, CD3, CD43, CD10, Ki-67, BCL1, BCL2, BCL6, HGAL, and LMO2. Four immunoarchitectural patterns were evident: diffuse (38 [75%]), well-formed nodular/follicular (5 [10%]), interfollicular (7 [14%]), and perifollicular (1 [2%]). Additional features included a monocytoid component (36 [71%]), admixed large cells (20 [39%]), plasma cells (24 [47%]), compartmentalizing stromal sclerosis (13 [25%]), and prominent blood vessel sclerosis (10 [20%]). CD21 highlighted disrupted follicular dendritic cell meshwork in 35 (71%) of 49 cases, and CD43 coexpression was present in 10 (24%) of 42 cases. A panel of germinal center-associated markers was helpful in eliminating cases of diffuse follicle center lymphoma. Our results highlight the histologic and immunoarchitectural spectrum of NMZL and the usefulness of immunohistochemical analysis for CD43, CD23, CD21, BCL6, HGAL, and LMO2 in the diagnosis of NMZL.

  9. Esophageal Squamous Cell Carcinoma With Pancreatic Metastasis: A Case Report

    Directory of Open Access Journals (Sweden)

    Abbas Alibakhshi

    2011-11-01

    Full Text Available Malignant tumors of pancreas are usually primary neoplasms and pancreatic metastases are rare findings. We are reporting a case of squamous cell carcinoma (SCC of the esophagus with pancreatic metastasis. A 59-year old woman was admitted with chief complaint of abdominal pain and mass. She was a known case of esophageal SCC since 4 years before when she had undergone transthoracic esophagectomy and cervical esophago-gastrostomy. In order to evaluate recent abdominal mass, CT scan was done which revealed septated cystic lesion in the body and the tail of the pancreas. Palliative resection of the tumor was performed and its histological study showed SCC compatible with her previously diagnosed esophageal cancer.

  10. Bilateral sertoli-leydig cell tumor of the ovary: A rare case report

    OpenAIRE

    Alam Kiran; Maheshwari Veena; Rashid Seema; Bhargava Shruti

    2009-01-01

    Sertoli leydig cell tumors also known as arrhenoblastoma, are a rare member of the sex cord-stromal tumor group of ovarian and testicular cancers, comprising less than 1% of all ovarian tumors, which occur in young adults and are almost always unilateral. We hereby report a case of a 17-year-old female presenting with a short history of irregular menses and an abdominal lump, which was histologically proven to be a bilateral sertoli leydig cell tumor of the ovary, an exceptionally rare...

  11. Unusual echocardiographic features seen in a case of giant cell myocarditis.

    Science.gov (United States)

    Kochar, Minisha; López-Candales, Angel; Ramani, Gautam; Rajagopalan, Navin; Edelman, Kathy

    2008-11-01

    The case of an 18-year-old college football player with a recent history of streptococcal pharyngitis who was experiencing progressive disabling dyspnea on exertion with easy fatigability and lack of stamina, and was taken to the hospital after a syncopal episode is described. The patient was initially diagnosed with heart failure and treated accordingly. However, because of a fulminant clinical deterioration, an endomyocardial biopsy was recommended, which showed focal giant cell transformation consistent with giant cell myocarditis. Treatment with methylprednisolone and cyclosporine was promptly initiated. Several apical clots were noted during treatment, but the patient attained full recovery with treatment.

  12. Rectal Metastases from Squamous Cell Carcinoma: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    S. Cedrés

    2012-01-01

    Full Text Available Non-small-cell lung cancer (NSCLC represents 85% of lung cancer. The most frequent sites of distant metastasis are the liver, adrenal glands, bones and brain. Gastrointestinal metastases are uncommon and rectal metastases are extremely rare. Here we report a case of squamous cell carcinoma of the lung with rectal metastases.

  13. Searches of Students' Cell Phones: Case Analysis and Best Practices

    Science.gov (United States)

    Nowak, Benjamin; Glenn, William J.

    2017-01-01

    Lower courts are beginning to grapple with challenges to students' Fourth Amendment right to be free from unreasonable search and seizure as it relates to the digital environment, cell phones in particular. Recently, lower courts in several states have applied standards set forth decades ago to decide cases involving searches of students' mobile…

  14. Cure in a patient with multiple osseus metastases in non-small cell lung cancer: a case report.

    Science.gov (United States)

    Hawighorst, H; Gademann, G

    1993-10-01

    This case was reported to describe a case of cure in a 61-year old patient with squamous cell lung cancer and multiple extrathoracic metastasis. A left upper lobectomy of lung for a squamous cell carcinoma was performed on a 61-year old man with curative intent. Four months later two osseus metastases were irradiated with Cobalt 60 up to 40 Gy. The two irradiated lesions showed continuously shrinkage as well as signs of recalcification. Eleven years later the patient shows clinically absolute well being and on CT there are no signs of recurrent disease of the lung or bone anymore. To our knowledge has nobody so far reported of a case of as squamous cell lung cancer which was operated and irradiated on thus resulting in cure. Further on the authors discuss that it might well be worthwhile to define subgroups in stage 4 non-small cell lung cancer (presence of extrathoracic metastases) which might benefit from a more aggressive treatment approach than pure palliation.

  15. Primary cutaneous marginal zone B-cell lymphoma with amyloid deposition: report of two cases with review of literature.

    Science.gov (United States)

    Zhang, Hai-Yan; Liu, An-Li; Zhou, Ling-sheng; He, Miao-Xia; Wang, Jian-Xin

    2010-06-01

    Amyloid deposition is rare. If there was a great amount of amyloid depositions in the skin tissue, it would be considered to be amyloid deposition disease at first, and then primary cutaneous marginal zone B-cell lymphoma (PCMZL). This study was to analyze the diagnosis and differential diagnosis of two cases of PCMZL with amyloid deposition. Clinicopathologic characteristics and follow-up of two cases of PCMZL were analyzed. Immunohistochemical staining was performed by EnVision method using antibodies LCA, CD19, CD20, CD79a, CD3, CD7, MUM1, kappa, lambda, Ki-67. IgH and TCRgamma gene rearrangement was detected by polymerase chain reactive (PCR). Case 1, a 71-year-old Chinese male, had a subcutaneous mass on the right elbow that was initially diagnosed with "amyloidosis" in 2004. Three years after the initial diagnosis, he developed recurrences on the right para-auxillary that was still diagnosed with "probably amyloidosis". Four years after the first diagnosis, the patient presented a lesion on the right para-auxillary with a diameter of 2 cm and a lesion on the temporal-parietal dural with a size of 6.0 cmx3.0 cmx3.0 cm. Case 2, a 68-year-old Chinese male, had a subcutaneous mass next to back of the left ear with a size of 9.0 cmx5.0 cm, and he underwent a operation one year previously because of subcutaneous mass in the same site. Microscopically, the tumors of both cases were located in dermis and subcutaneous, tumor cells were medium size with a nodular or diffuse distribution, and some of tumor cells were plasmacytoid/plasma cells. Morphologically, the temporal-parietal dural lesion was similar to subcutaneous lesion and infiltrated into cranial (case 1). Juxtaposed the tumor cells of two cases, there were the large amyloid deposits of amorphous hyaline material and concentrically laminated hyaline spherules in case 1, while cord-like amyloid deposits in case 2. Reactive lymphoid follicles with germinal centers and foreign body giant cells in the stroma were

  16. Sinus histiocytosis with massive lymphadenopathy: MRI findings of osseous lesions

    Energy Technology Data Exchange (ETDEWEB)

    Lin, J. [Dept. of Radiology, Univ. of Illinois Hospital, Chicago, IL (United States); Lazarus, M. [Dept. of Radiology, Univ. of Illinois Hospital, Chicago, IL (United States); Wilbur, A. [Dept. of Radiology, Univ. of Illinois Hospital, Chicago, IL (United States)

    1996-04-01

    The case of a 20-year-old female with SHML and osseous involvement is presented. We describe three different patterns of involvement in the diametaphyseal portions of the long bones with similar MRI signal characteristics. (orig.)

  17. Intraepidermal Merkel cell carcinoma: A case series of a rare entity with clinical follow up.

    Science.gov (United States)

    Jour, George; Aung, Phyu P; Rozas-Muñoz, Eduardo; Curry, Johnathan L; Prieto, Victor; Ivan, Doina

    2017-08-01

    Merkel cell carcinoma (MCC) is a rare but aggressive cutaneous carcinoma. MCC typically involves dermis and although epidermotropism has been reported, MCC strictly intraepidermal or in situ (MCCIS) is exceedingly rare. Most of the cases of MCCIS described so far have other associated lesions, such as squamous or basal cell carcinoma, actinic keratosis and so on. Herein, we describe 3 patients with MCC strictly in situ, without a dermal component. Our patients were elderly. 2 of the lesions involved the head and neck area and 1 was on a finger. All tumors were strictly intraepidermal in the diagnostic biopsies, and had histomorphologic features and an immunohistochemical profile supporting the diagnosis of MCC. Excisional biopsies were performed in 2 cases and failed to reveal dermal involvement by MCC or other associated malignancies. Our findings raise the awareness that MCC strictly in situ does exist and it should be included in the differential diagnosis of Paget's or extramammary Paget's disease, pagetoid squamous cell carcinoma, melanoma and other neoplasms that typically show histologically pagetoid extension of neoplastic cells. Considering the limited number of cases reported to date, the diagnosis of isolated MCCIS should not warrant a change in management from the typical MCC. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  18. Case of radiation cancer associated with spinocellular carcinoma and basal cell epithelial tumor

    Energy Technology Data Exchange (ETDEWEB)

    Oohara, K.; Ootsuka, F. (Tokyo Univ. (Japan). Faculty of Medicine); Mizoguchi, M.

    1980-12-01

    The patient was a 66 year-old male who had received radiotherapy for psoriasis vulgaris in frontal plane for 10 years since the age of 19. This radiotherapy was carried out once a week for 5 to 6 weeks and stopped for following 5 to 6 weeks. The source and the dose were unknown. Multiple superficial basal cell epithelial tumor occurred 32 to 33 years after that in the region over which radiation had been given. Moreover, 37 years after that, spinocellular carcinoma occurred in the same region. Spinocellular carcinoma in this case increased rapidly and reached the depth of frontal plane. Atypic of cancer cells was marked, and various findings were observed. Characteristics of these tumor cells were mixture of spindle cells and cells with vacuoles. Partially, findings common to basal cell epithelial tumor were coexisted, and senile keratosis was also discovered.

  19. A case of severe encephalitis while on PD-1 immunotherapy for recurrent clear cell ovarian cancer

    Directory of Open Access Journals (Sweden)

    Morgan Burke

    2018-05-01

    Full Text Available Recurrent clear cell ovarian carcinoma is a difficult to treat condition and early trial data has suggested a possible role for immune checkpoint inhibitors. Nivolumab is an anti-PD-1 immunotherapy that has been used in this setting. While immune related toxicity of these agents has been well described, the occurrence of immune specific neurotoxicity is thought to be rare. We present a case of severe encephalitis while on PD-1 immunotherapy for a recurrent ovarian clear cell cancer and we believe this to be the first such reported case associated with the use of PD-1 inhibitor monotherapy. In this case, a 64-year-old woman with persistent clear cell ovarian cancer on Nivolumab presented with a severe fever of unknown origin and delirium; initial imaging and diagnostic work-up suggested a neurological etiology, but with no clear source. We concluded that this was a severe case of immune related encephalitis, thought to be brought about by the anti-PD-1 immunotherapy which responded well to systemic corticosteroids and plasmapheresis and the patient able to make a full recovery. We present a summary of the case and its management as well as a review of the literature on the previously reported neurotoxicity's of PD-1 inhibitors.

  20. Genome wide single cell analysis of chemotherapy resistant metastatic cells in a case of gastroesophageal adenocarcinoma

    International Nuclear Information System (INIS)

    Hjortland, Geir Olav; Fodstad, Oystein; Smeland, Sigbjorn; Hovig, Eivind; Meza-Zepeda, Leonardo A; Beiske, Klaus; Ree, Anne H; Tveito, Siri; Hoifodt, Hanne; Bohler, Per J; Hole, Knut H; Myklebost, Ola

    2011-01-01

    Metastatic progression due to development or enrichment of therapy-resistant tumor cells is eventually lethal. Molecular characterization of such chemotherapy resistant tumor cell clones may identify markers responsible for malignant progression and potential targets for new treatment. Here, in a case of stage IV adenocarcinoma of the gastroesophageal junction, we report the successful genome wide analysis using array comparative genomic hybridization (CGH) of DNA from only fourteen tumor cells using a bead-based single cell selection method from a bone metastasis progressing during chemotherapy. In a case of metastatic adenocarcinoma of the gastroesophageal junction, the progression of bone metastasis was observed during a chemotherapy regimen of epirubicin, oxaliplatin and capecitabine, whereas lung-, liver and lymph node metastases as well as the primary tumor were regressing. A bone marrow aspirate sampled at the site of progressing metastasis in the right iliac bone was performed, and single cell molecular analysis using array-CGH of Epithelial Specific Antigen (ESA)-positive metastatic cells, and revealed two distinct regions of amplification, 12p12.1 and 17q12-q21.2 amplicons, containing the KRAS (12p) and ERBB2 (HER2/NEU) (17q) oncogenes. Further intrapatient tumor heterogeneity of these highlighted gene copy number changes was analyzed by fluorescence in situ hybridization (FISH) in all available primary and metastatic tumor biopsies, and ErbB2 protein expression was investigated by immunohistochemistry. ERBB2 was heterogeneously amplified by FISH analysis in the primary tumor, as well as liver and bone metastasis, but homogenously amplified in biopsy specimens from a progressing bone metastasis after three initial cycles of chemotherapy, indicating a possible enrichment of erbB2 positive tumor cells in the progressing bone marrow metastasis during chemotherapy. A similar amplification profile was detected for wild-type KRAS, although more heterogeneously

  1. Primary intraosseous squamous cell carcinoma mimicking periapical disease: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Yoon Joo; Oh, Song Hee; Kang, Ju Han; Choi, Hwa Young; Kim, Gyu Tae; Choi, Yong Suk; Hwang, Eui Hwan [Dept. of Oral and Maxillofacial Radiology, School of Dentistry, Kyung Hee University, Seoul (Korea, Republic of); Yu, Jae June [Dept. of Oral and Maxillofacial Radiology, Kangdong Sacred Heart Hospital, Hallym Medical Center, Seoul (Korea, Republic of)

    2012-09-15

    Primary intraosseous squamous cell carcinoma (PIOSCC) is a rare carcinoma, which arises within the jaws without connection to the oral mucosa and presumably develops from a remnant of odontogenic epithelium. We present a case of solid type PIOSCC in a 52-year-old male patient complaining of dull pain on his left lower molar. In this case, early stage PIOSCC mimicking a periapical lesion might lead to a one-year delay in treatment due to the misdiagnosis of osteomyelitis after extraction of the third molar. The clinical, radiological, and histologic features are described. In this case, there was initial radiographic evidence for PIOSCC mimicking a periapical lesion. Incautious radiographic interpretation and treatment procedures had delayed the correct diagnosis and resulted in extensive bony destruction during the patient's disease progression.

  2. Radiographic features of plasma cell leukemia in the maxilla: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Wong, Phillip; Kashtwari, Deeba; Nair, Madhu K. [Dept. of Oral and Maxillofacial Radiology, Oral and Maxillofacial Diagnostic Sciences/Radiology, Colleges of Dentistry/Medicine, University of Florida, Gainesville (United States)

    2016-12-15

    Plasma cell leukemia (PCL) is an aggressive form of multiple myeloma where there is hematogenous spread of abnormal plasma cells into the periphery. This is opposed to multiple myeloma, where the abnormal plasma cells stay in the bone marrow. PCL is more common in males than females, and is also more common in African-Americans than Caucasians. Signs and symptoms of PCL include, but are not limited to, renal insufficiency, hypercalcemia, anemia, lytic bone lesions, thrombocytopenia, hepatomegaly, and splenomegaly. Here, we discussed a case of a 71-year-old Caucasian female recently diagnosed with primary PCL with radiographic features of this disease throughout the body, with an emphasis on the maxillofacial skeleton and relevance from a dental standpoint.

  3. Eukaryotic Cell Cycle as a Test Case for Modeling Cellular Regulation in a Collaborative Problem-Solving Environment

    Science.gov (United States)

    2007-03-01

    computer models of cell cycle regulation in a variety of organisms, including yeast cells, amphibian embryos, bacterial cells and human cells. These...and meiosis ), but they do not nullify the central role played by irreversible, alternating START and FINISH transitions in the cell cycle. 32...AFRL-IF-RS-TR-2007-69 Final Technical Report March 2007 EUKARYOTIC CELL CYCLE AS A TEST CASE FOR MODELING CELLULAR REGULATION IN A

  4. Iris juvenile xanthogranuloma in an infant - spontaneous hyphema and secondary glaucoma.

    Science.gov (United States)

    Pantalon, Anca; Ștefănache, Tudor; Danciu, Mihai; Zurac, Sabina; Chiseliță, Dorin

    2017-01-01

    Juvenile xanthogranuloma (JXG) is a benign histiocytic skin disorder mainly encountered during infancy and childhood. Although with multiple potential localizations, less than 1% of the cases exhibit ocular manifestations. Some of these might lead to serious complications, specifically, secondary glaucoma that can result in severe and blinding eye disease. The aim of the present case report was to demonstrate typical clinical features, emphasize the difficulties attributed when managing these patients and literature review. We present the case of 4 months old female baby with spontaneous hyphema and secondary unilateral glaucoma due to ocular JXG. The natural history and treatment of the condition were extremely difficult to handle due to multiple opinions in histopathology related to other severe conditions that resembled with the lesions detected in this case: myelomonocytic leukemia and Langerhans cell histiocytosis. Although a minority of patients with JXG have ocular involvement, recognition of this condition is important because a treatment delay can lead to serious complications, such as glaucoma and spontaneous hyphema, as in our case. A thorough differential diagnosis represents the key to a proper management plan in these patients, both on short and long term. "Triple disease" defined as JXG plus neurofibromatosis type 1 (NF-1) and juvenile chronic myelogenous leukemia (JCML) has been reported, but it was not confirmed in our patient.

  5. Pituitary disease in childhood: utility of magnetic resonance

    International Nuclear Information System (INIS)

    Vela, A. C.; Oleaga, L.; Ibanez, A. M.; Campo, M.; Grande, D.

    2000-01-01

    To assess the utility of magnetic resonance (MR) imaging in the study of pediatric patients with clinical suspicion of pituitary disease. We studied 18 patients aged 7 to 18 years.Fifteen had hormonal disturbances, two presented amenorrhea and 1 complained of headache, fever and symptoms of polyuria and polydipsia. All the patients were examined using a Siemens SP 42 1-Tesla MRI scanner. Sagittal and coronal T1-weighted spin-echo images were obtained; in addition T2-weighted spin-echo or fast spin-echo imaging was performed in ten cases and intravenous gadolinium was administered in nine. We found 9 patients with hypothalamic-pituitary dysgenesis, 2 with germinoma, 2 cases of pituitary hemosiderosis in patients with thalassemia, 2 cases of microadenoma, one abscess, one case of idiopathic central diabetes insipidus and one of Langerhans cell histiocytosis. MR enabled us to assess pituitary structural alterations in children with hypothalamic-pituitary hormone deficiencies. In our series of patients, hypothalamic-pituitary dysgenesiss was the most frequent cause of adenohypophyseal deficiencies, and most cases of central diabetes insipidus were secondary to masses in the sellar and suprasellar region. In patients with thalassemia, T2-weighted MR images showed the amount of iron deposited in adenophypophysis. Gadolinium-enhanced studies were useful in the study of masses and when the presence of microadenoma was suspected. (Author) 26 refs

  6. Pituitary disease in childhood: utility of magnetic resonance; Patologia hipofisaria en la edad pediatrica: unidad de la resonance magnetica

    Energy Technology Data Exchange (ETDEWEB)

    Vela, A. C.; Oleaga, L.; Ibanez, A. M.; Campo, M.; Grande, D. [Hospital de Basurto. Bilbao (Spain)

    2000-07-01

    To assess the utility of magnetic resonance (MR) imaging in the study of pediatric patients with clinical suspicion of pituitary disease. We studied 18 patients aged 7 to 18 years.Fifteen had hormonal disturbances, two presented amenorrhea and 1 complained of headache, fever and symptoms of polyuria and polydipsia. All the patients were examined using a Siemens SP 42 1-Tesla MRI scanner. Sagittal and coronal T1-weighted spin-echo images were obtained; in addition T2-weighted spin-echo or fast spin-echo imaging was performed in ten cases and intravenous gadolinium was administered in nine. We found 9 patients with hypothalamic-pituitary dysgenesis, 2 with germinoma, 2 cases of pituitary hemosiderosis in patients with thalassemia, 2 cases of microadenoma, one abscess, one case of idiopathic central diabetes insipidus and one of Langerhans cell histiocytosis. MR enabled us to assess pituitary structural alterations in children with hypothalamic-pituitary hormone deficiencies. In our series of patients, hypothalamic-pituitary dysgenesiss was the most frequent cause of adenohypophyseal deficiencies, and most cases of central diabetes insipidus were secondary to masses in the sellar and suprasellar region. In patients with thalassemia, T2-weighted MR images showed the amount of iron deposited in adenophypophysis. Gadolinium-enhanced studies were useful in the study of masses and when the presence of microadenoma was suspected. (Author) 26 refs.

  7. Mucinous tubular and spindle cell carcinoma of kidney: A rare case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Geramizadeh Bita

    2009-10-01

    Full Text Available Low grade mucinous tubular and spindle cell carcinoma of kidney was newly established as a distinct renal cell carcinoma in the World Health Organization (WHO classification of 2004. Until now, less than 60 cases have been reported and the largest series represented approximately 15 patients with this type of tumor. Herein, we report a case of mucinous tubular and spindle cell carcinoma in a 63-year-old male presented with right flank pain which was diagnosed after nephrectomy. Pathologists should consider this diagnosis and its spectrum of histopathologic features in mind to ensure an accurate diagnosis.

  8. Successful Treatment of Plasma Cell-Rich Acute Rejection Using Pulse Steroid Therapy Alone: A Case Report

    Directory of Open Access Journals (Sweden)

    Yo Komatsuzaki

    2017-01-01

    Full Text Available Despite the recent development of immunosuppressive agents, plasma cell-rich acute rejection (PCAR has remained refractory to treatment. Herein, we report an unusual case of PCAR that responded well to pulse steroid therapy alone. A 47-year-old man was admitted for a protocol biopsy three months after kidney transplantation, with a stable serum creatinine level of 1.6 mg/dL. Histological examination showed focal aggressive tubulointerstitial inflammatory cell infiltration of predominantly polyclonal mature plasma cells, leading to our diagnosis of PCAR. Three months following three consecutive days of high-dose methylprednisolone (mPSL therapy, an allograft biopsy performed for therapy evaluation showed persistent PCAR. We readministered mPSL therapy and successfully resolved the PCAR. Although PCAR generally develops more than six months after transplantation, we diagnosed this case early, at three months after transplantation, with focally infiltrated PCAR. This case demonstrates the importance of early diagnosis and prompt treatment of PCAR to manage the development and severity of allograft rejection.

  9. Primary Diffuse Large Cell Lymphoma of the Bladder: Case Report and Literature Review

    OpenAIRE

    Mansour Ansari; Hamid Nasrollahi; Majdaddin Rajaei; Maral Mokhtari; Seyed Hasan Hamedi; Mohammad Mohammadianpanah; Shapour Omidvari; Ahmad Mosalaei; Niloofar Ahmadloo

    2017-01-01

    Most bladder tumors are epithelial in origin. Nonepithelial cancers are rarely located in the bladder. Sarcomas are the most common malignancies among nonepithelial cancers. Primary bladder lymphoma is rare and mostly low grade. Here, we have reported a case of diffuse large cell lymphoma of the bladder. The patient, a 64-year-old man, had urinary frequency for 18 months. Abdominal sonography indicated a thick bladder wall and transurethral biopsy showed diffuse large cell lymp...

  10. MRI findings of small cell neuroendocrime carcinoma of the uterine cervix: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Won, You Sun; Yi, Boem Ha; Lee, Hae Kyung; Lee, Min Hee; Choi, Seo Youn; Kwak, Jeong Ja [Soonchunhyang University College of Medicine, Bucheon Hospital, Bucheon (Korea, Republic of)

    2015-10-15

    Small cell neuroendocrine carcinoma of the uterine cervix is a rare primary neoplasm, accounting for less than 5% of all uterine cervical cancers. The tumor is known to have an aggressive behavior and poor prognosis. In this article, we present the MRI findings of 5 cases of pathologically-proven small cell neuroendocrine carcinoma of the uterine cervix, including diffusion-weighted images.

  11. Jaw cyst-Basal cell nevus-Bifid rib syndrome: A case report

    Directory of Open Access Journals (Sweden)

    Rai S

    2007-09-01

    Full Text Available Jaw cyst-Basal cell nevus-Bifid rib syndrome or Gorlin-Goltz syndrome involves multiple organ system. The most common findings include multiple odontogenic keratocysts in the jaws and basal cell nevus on the skin that have an early age onset. These multiple odontogenic keratocysts warrant aggressive treatment at the earliest because of the damage and possible complications associated with them. Recurrence in these lesions is the most characteristic feature that has to be taken in consideration while explaining the prognosis to the patient. A case report of a child affected with Gorlin-Goltz syndrome diagnosed, treated and followed at this hospital is presented here.

  12. Erdheim-Chester Disease: Comprehensive Review of Molecular Profiling and Therapeutic Advances.

    Science.gov (United States)

    Haroun, Faysal; Millado, Kristen; Tabbara, Imad

    2017-06-01

    The revised 2016 World Health Organization classification introduced Erdheim-Chester disease (ECD) as a provisional entity within the histiocytic and dendritic cell neoplasms separate from the juvenile xanthogranuloma family based on distinct molecular features. However, evolving knowledge regarding the molecular and genetic aberrations in addition to common clinical features of ECD support the classification of ECD together with Langerhans cell histiocytosis (LCH). Accordingly, ECD can be thought of as an inflammatory myeloid clonal disorder based on the detection of various activating mutations along the mitogen activated protein kinase-extracellular signal regulated kinase (MAPK-ERK) pathway with most notable variant being a valine to a glutamic acid substitution at amino acid 600 in the B-rapidly accelerated fibrosarcoma protein (BRAFV600E). In this group, targeted therapy with a B-Raf inhibitor alone or combined with a MAPK-ERK (MEK) inhibitor has shown promising results based on several case reports. Currently, two phase II trials with BRAF inhibitors are underway and could potentially change the standard of care. MEK inhibitors may also be efficacious in ECD harboring mutations in MAP2K1; other potential targetable aberrations include programed cell death receptor 1 and mutations in phosphoinositide 3-kinase. Copyright© 2017, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.

  13. A Case of Diffuse Large B-Cell Lymphoma Mimicking Primary Effusion Lymphoma-Like Lymphoma

    Directory of Open Access Journals (Sweden)

    Daisuke Usuda

    2017-11-01

    Full Text Available A 93-year-old female was transferred to the emergency ward of our hospital due to disturbance of consciousness and hypotension. Computed tomography showed bilateral pleural and pericardial effusion without evidence of tumor masses or lymphadenopathy. Cytodiagnosis of pleural effusion revealed proliferation of atypical lymphoid-like cells with pan-B surface markers. We suspected primary effusion lymphoma-like lymphoma; however, the monoclonality of these cells was not confirmed. Cytodiagnosis of bone marrow revealed lymphoma cells with monoclonal B-cell markers. These findings prompted a diagnosis of diffuse large B-cell lymphoma with bone marrow invasion. In the case of pericardial or pleural effusion, clinicians should consider carefully both hematological malignancy and its classification.

  14. Semiinvasive Aspergillosis Case Coexisting to Metastatic Renal Cell Cancer

    Directory of Open Access Journals (Sweden)

    Hatice Kilic

    2013-10-01

    Full Text Available Chronic necrotizing pulmonary aspergillosis (CNPA is defined as an cavity or mass lesion in the lung due to invasion of lung tissue by a fungus of the Aspergillosis species. It was described also as semiinvasive aspergillosis. Semiinvasive pulmonary aspergillosis is generally seen in patients with primer immunocompromised and it can be fatal in the event of late diagnose. We present 60 years old patient who had had renal cell cancer admitted to hospital with metastatic nodules in his chest X-ray and Thorax computed tomography. We have seen yellow colour of bronchial secretion in his bronchoscopy. Multipl mantar hyphae by A. Fumigatus was detected in his bronchial lavage cytology. Itrakonazol was administred to this patient. We review to this cases due to a semiinvasive aspergillosis was detected randomly when this case who had not both symptomatic and clinical sign by Aspergillosis is investigated.

  15. Human papillomavirus (HPV) infection in a case-control study of oral squamous cell carcinoma and its increasing trend in northeastern Thailand.

    Science.gov (United States)

    Phusingha, Pensiri; Ekalaksananan, Tipaya; Vatanasapt, Patravoot; Loyha, Kulchaya; Promthet, Supannee; Kongyingyoes, Bunkerd; Patarapadungkit, Natcha; Chuerduangphui, Jureeporn; Pientong, Chamsai

    2017-06-01

    Human papillomavirus (HPV) is an independent risk factor for development of oral squamous cell carcinoma (OSCC). This study aimed to investigate the role of HPV infection and the trend in percentage of HPV-associated OSCC over a 5-year period in northeastern Thailand. In this case-control study, 91 exfoliated oral cell samples and 80 lesion cell samples from OSCC cases and exfoliated oral cells from 100 age/gender-matched controls were collected. HPV infection was investigated by PCR using GP5+/GP6+ primers followed by HPV genotyping using reverse line blot hybridization. Quantitative RT-PCR was used to evaluate HPV oncogene transcription. Temporal trends of HPV infection were evaluated in archived formalin-fixed paraffin-embedded (FFPE) OSCC tissues using in situ hybridization. HPV DNA was found in 17.5% (14/80) of lesion samples from OSCC cases and 29.7% (27/91) of exfoliated oral cell samples from the same cases. These values were significantly higher than in exfoliated oral cell samples from controls (13%, 13/100). HPV-16 was the genotype most frequently found in OSCC cases (92.8%, 13/14 infected cases). Interestingly, HPV oncogene mRNA expression was detected and correlated with OSCC cases (P cases were positive for HPV E6/E7 mRNA expression. There was a trend of increasing percentage of HPV-associated OSCC from 2005 to 2010. This was especially so for females with well-differentiated tumors in specific tongue sub-sites. We suggest that HPV infection plays an important role in oral carcinogenesis in northeastern Thailand. © 2016 Wiley Periodicals, Inc.

  16. CT and MR imaging findings of sphenoidal masses

    Energy Technology Data Exchange (ETDEWEB)

    Takahashi, Shoki; Higano, Shuichi (Tohoku Univ., Sendai (Japan). School of Medicine); Ishii, Kiyoshi (and others)

    1994-07-01

    CT and MR imaging findings of 57 sphenoidal masses were retrospectively reviewed to assess the possibility of differential diagnosis between them. Various kinds of masses such as pituitary adenoma, epipharyngeal cancer, mucocele, chordoma, chondroma, chondrosarcoma, distant metastasis, multiple myeloma, fibrous dysplasia, craniopharyngioma, hemangiopericytoma, giant cell tumor, primary sphenoidal cancer, malignant melanoma, leukemia, histiocytosis X, and giant cell tumor were included in this series. CT scanning was performed in all cases using a spin-echo pulse sequence. The relative density of the masses, bony changes and calcification were evaluated on CT, and on MR images, signal intensity of the masses relative to the normal gray matter, contrast enhancement and extension/contour were evaluated. Although no single feature appeared to be specific to the masses, detection of calcification on CT, identification of the normal pituitary gland as deformed or displaced on T1-weighted images, signal intensity on T2-weighted images, and extension of the masses seemed to be useful and should be examined in terms of their ability to assist in differential diagnosis. Finally, accommodative classification of sphenoidal masses primarily based on presumed origin or mode of extension was attempted. (author).

  17. Bilateral sertoli-leydig cell tumor of the ovary: A rare case report

    Directory of Open Access Journals (Sweden)

    Alam Kiran

    2009-01-01

    Full Text Available Sertoli leydig cell tumors also known as arrhenoblastoma, are a rare member of the sex cord-stromal tumor group of ovarian and testicular cancers, comprising less than 1% of all ovarian tumors, which occur in young adults and are almost always unilateral. We hereby report a case of a 17-year-old female presenting with a short history of irregular menses and an abdominal lump, which was histologically proven to be a bilateral sertoli leydig cell tumor of the ovary, an exceptionally rare entity in itself.

  18. Merkel cell carcinoma with fingolimod treatment for multiple sclerosis: A case report.

    Science.gov (United States)

    Mahajan, Kedar R; Ko, Jennifer S; Tetzlaff, Michael T; Hudgens, Courtney W; Billings, Steven D; Cohen, Jeffrey A

    2017-10-01

    Neoplasms and reactivation of latent viruses have been observed in individuals taking fingolimod. Merkel cell carcinoma (MCC), a rare neuroendocrine skin cancer, is associated with immunosuppression and can be triggered by the oncogenic Merkel cell polyoma virus (MCPyV). We report a case of a 61-year-old man with multiple sclerosis who developed MCPyV-positive MCC 4 years after starting fingolimod. This is the second report of MCC associated with MCPyV in an individual on fingolimod. Copyright © 2017 Elsevier B.V. All rights reserved.

  19. Basal cell carcinoma arising in association with a maxillary keratocyst in a patient with Gorlin-Goltz syndrome. Report of a case.

    Science.gov (United States)

    Lazaridou, Maria Nikolaou; Dimitrakopoulos, Ioannis; Tilaveridis, Ioannis; Iliopoulos, Christos; Heva, Antigoni

    2012-03-01

    Gorlin-Goltz syndrome, also known as nevoid basal cell carcinoma syndrome, is an autosomal dominant inherited disorder which is characterized by the presence of multiple basal cell carcinomas, maxillary keratocysts, and musculoskeletal anomalies. We present a case of a patient suffering from Gorlin-Goltz syndrome who developed an intraosseous basal cell carcinoma associated with a recurrent maxillary keratocyst. To our knowledge, this is the first case of malignant transformation of a keratocyst into a basal cell carcinoma described in the literature. This case highlights the importance of careful histologic examination of keratocysts excised in patients suffering from Gorlin-Goltz syndrome.

  20. Subungual squamous cell carcinoma: A case study

    Energy Technology Data Exchange (ETDEWEB)

    Neill, Cory J., E-mail: coryjneill@gmail.com

    2017-07-01

    The purpose of this case study is to describe a dosimetric delivery of radiation to a superficial disease process involving the skin and bone of the distal finger. A 76-year-old male patient presented with a subungual squamous cell carcinoma (SCC) of the left distal index finger with bony involvement. The patient refused conventional surgical treatment but agreed to external beam radiation therapy (EBRT). There is a gap in the current literature describing how to successfully immobilize fingers and which EBRT modality is dosimetrically advantageous in treating them. The construction of a simple immobilization method with the patient in a reproducible position is described. The use of photons and electrons were compared ultimately showing photons to be dosimetrically advantageous. Long-term efficacy of the treatment was not evaluated because of patient noncompliance.

  1. Cure in a patient with multiple osseus metastases in non-small cell lung cancer: a case report

    International Nuclear Information System (INIS)

    Hawighorst, H.; Gademann, G.

    1993-01-01

    Purpose: This case was reported to describe a case of cure in a 61-year old patient with squamous cell lung cancer and multiple extrathoracic metastasis. Methods and materials: A left upper lobectomy of lung for a squamous cell carcinoma was performed on a 61-year old man with curative intent. Fourt months later two osseous metastases were irradiated with Cobalt 60 up to 40 Gy. Results: The two irradiated lesions showed continuously shrinkage as well as signs of recalcification. Eleven years later the patient shows clinically absolut well being and on CT there are no signs of recurrent disease of the lung or bone anymore. Discussion: To our knowledge has nobody so far reported of a case of a squamous cell lung cancer which was operated and irradiated on thus resulting in cure. Furtheron the authors discuss that it might well be worthwile to define subgroups in stage 4 non-small cell lung cancer (presence of extrathoracic metastases) which might benefit from a more aggressive treatment approach than pure palliation. (orig.) [de

  2. Basal cell carcinoma of the skin with areas of squamous cell carcinoma: a basosquamous cell carcinoma?

    OpenAIRE

    de Faria, J

    1985-01-01

    The diagnosis of basosquamous cell carcinoma is controversial. A review of cases of basal cell carcinoma showed 23 cases that had conspicuous areas of squamous cell carcinoma. This was distinguished from squamous differentiation and keratotic basal cell carcinoma by a comparative study of 40 cases of compact lobular and 40 cases of keratotic basal cell carcinoma. Areas of intermediate tumour differentiation between basal cell and squamous cell carcinoma were found. Basal cell carcinomas with ...

  3. Nonsyndromic Synchronous Multifocal Central Giant Cell Granulomas of the Maxillofacial Region: Report of a Case.

    Directory of Open Access Journals (Sweden)

    Anita Munde

    2015-04-01

    Full Text Available Central giant cell granuloma (CGCG is a benign proliferation of fibroblasts and multinucleated giant cells that almost exclusively occurs in the jaws. It commonly occurs in young adults showing a female predilection in the anterior mandible. Multifocal CGCGs in maxillofacial region are very rare and suggestive of systemic diseases such as hyperparathyroidism, an inherited syndrome such as Noonan-like multiple giant cell lesion syndrome or other disorders. Only 10 cases of multifocal CGCGs in the maxillofacial region without any concomitant systemic disease have been reported in the English literature. Here, we report an unusual case of 36 year-old female presented with non-syndromic synchronous, multifocal CGCGs in the left posterior mandible and left posterior maxilla without any concomitant systemic disease. Relevant literature is reviewed and the incidence, clinical features, radiological features, differential diagnosis and management of CGCGs are discussed.

  4. Primary intraosseous squamous cell carcinoma arising in an odontogenic keratocyst previously treated with marsupialization: case report and immunohistochemical study.

    Science.gov (United States)

    Martínez-Martínez, Marisol; Mosqueda-Taylor, Adalberto; Delgado-Azañero, Wilson; Rumayor-Piña, Alicia; de Almeida, Oslei Paes

    2016-04-01

    A rare case of primary intraosseous squamous cell carcinoma arising in an odontogenic keratocyst (OKC) is presented here, with the clinical and histologic features of the first biopsy showing characteristics of OKC and the second biopsy disclosing a squamous cell carcinoma. Immunoprofile of this case was compared with five cases of classical OKC by using cytokeratins CK5, CK14, and CK19, CD138, p63, Ki-67, p53, and bcl-2. Classic OKCs showed expected positivity, mainly in the basal and/or suprabasal layers with most antibodies, except for p53, which was negative, whereas the present case showed irregular positivity in all layers, indicating that this can be useful for differential diagnosis and suggesting a possible role in malignant transformation into primary intraosseous squamous cell carcinoma. In conclusion, immunohistochemical differences between the first biopsy of the present case and classic OKC suggest that immunohistochemistry can be helpful in cases with areas of subtle initial malignant transformation. Copyright © 2016 Elsevier Inc. All rights reserved.

  5. Trisomy 4 in a case of acute undifferentiated myeloblastic leukemia with hand-mirror cells.

    Science.gov (United States)

    Kao, Y S; McCormick, C; Vial, R

    1990-04-01

    A case of acute undifferentiated myelocytic leukemic with trisomy 4 is described. The patient is a 61-year-old woman who developed leukemia 4 1/2 years after receiving radiation therapy for uterine carcinoma. Many leukemic cells exhibited hand-mirror configuration after the bone marrow aspirate was left at room temperature overnight. The relationship between trisomy 4 and hand-mirror cells in acute myelocytic leukemia is unknown.

  6. Central giant cell granuloma: A case report and review

    Directory of Open Access Journals (Sweden)

    Krishnaveni Buduru

    2017-01-01

    Full Text Available Central giant cell granuloma (CGCG is a benign intra-osseous lesion of unknown etiology, and occurs in jaws. Clinically and radiographically difference between its nature - aggressive and non-aggressive can be made. It is characterized histologically by cellular fibrous tissue containing multiple foci of hemorrhage, aggregations of multinucleated giant cells, and occasionally, trabeculae of woven bone. Histologically, identical lesions occur in patients with known genetic defects such as cherubism, Noonan syndrome, or neurofibromatosis type I. It has an increased predilection for mandible and females in younger age group. Surgical curettage or resection is the most common therapy in aggressive lesions. The drawback is undesirable damage to the jaw or teeth, tooth germs, and frequent recurrences. Non-aggressive tumors respond well to such treatments. We are presenting a case of an aggressive type of CGCG of mandible in a young patient, who presented with massive swelling associated with loss of teeth in just 6 months duration.

  7. Unusual case of spindle cell sarcoma metastases to right ventricle: a case report and a literature review.

    Science.gov (United States)

    Frakulli, Rezarta; Cammelli, Silvia; Salvi, Fabrizio; Balestrini, Damiano; Baldissera, Antonella; Degli Esposti, Claudio; Martelli, Ombretta; Abate, Massimo; Piaoli, Anna; Ferrari, Stefano; Morganti, Alessio G; Frezza, Giovanni Piero

    2017-09-01

    Cardiac metastases from sarcoma are uncommon. Due to their rarity there is not a standard of care. However, complete cardiac metastases resection is the best option but most of patients has widespread disease. In these patients palliative radiotherapy (RT) might improve symptoms and prevent further cardiac function decline. Here we present the case of a symptomatic 30-year-old woman with spindle cell sarcoma metastasis of right ventriculum and widespread disease. The patient received radiotherapy to the heart with palliative intent. Cardiac metastases represent a challenging clinic problem. Treatment should be individualized in a multidisciplinary setting, when possible surgery seems to be the best options. However, radiotherapy even in case of widespread disease can improve clinical control symptoms by reducing the mass effect.

  8. Primary Gastric Small Cell Carcinoma in Elderly Patients: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Jian-Han Lai

    2017-03-01

    Full Text Available We report the case of an 86-year-old man with primary gastric small cell carcinoma (SmCC. He was admitted to our hospital owing to gastrointestinal bleeding complicated by anemia. An upper gastrointestinal endoscopic examination revealed a large, irregularly ulcerated tumor on the upper to middle body of the stomach. Small cell carcinoma was diagnosed based on the results of histologic and immunohistochemical studies of an endoscopic biopsy specimen. According to previous reports of gastric SmCC, only one-sixth of cases have been correctly diagnosed preoperatively. In our case, it was an aggressive malignancy that had an extremely poor prognosis. We believe that careful endoscopic examination including immunohistochemical investigation is necessary to accurately diagnose gastric SmCC in clinical practice.

  9. Pitfalls and major issues in the histologic diagnosis of peripheral T-cell lymphomas: results of the central review of 573 cases from the T-Cell Project, an international, cooperative study.

    Science.gov (United States)

    Bellei, Monica; Sabattini, Elena; Pesce, Emanuela Anna; Ko, Young-Hyeh; Kim, Won Seog; Cabrera, Maria Elena; Martinez, Virginia; Dlouhy, Ivan; Paes, Roberto Pinto; Barrese, Tomas; Vassallo, Josè; Tarantino, Vittoria; Vose, Julie; Weisenburger, Dennis; Rüdiger, Thomas; Federico, Massimo; Pileri, Stefano

    2017-12-01

    Peripheral T-cell lymphomas (PTCLs) comprise a heterogeneous group of neoplasms that are derived from post-thymic lymphoid cells at different stages of differentiation with different morphological patterns, phenotypes and clinical presentations. PTCLs are highly diverse, reflecting the diverse cells from which they can originate and are currently sub-classified using World Health Organization (WHO) 2008 criteria. In 2006 the International T-Cell Lymphoma Project launched the T-Cell Project, building on the retrospective study previously carried on by the network, with the aim to prospectively collect accurate data to improve knowledge on this group of lymphomas. Based on previously published reports from International Study Groups it emerged that rendering a correct classification of PTCLs is quite difficult because the relatively low prevalence of these diseases results in a lack of confidence by most pathologists. This is the reason why the T-Cell Project requested the availability of diagnostic material from the initial biopsy of each patient registered in the study in order to have the initial diagnosis centrally reviewed by expert hematopathologists. In the present report the results of the review process performed on 573 cases are presented. Overall, an incorrect diagnosis was centrally recorded in 13.1% cases, including 8.5% cases centrally reclassified with a subtype eligible for the project and 4.6% cases misclassified and found to be disorders other than T-cell lymphomas; 2.1% cases were centrally classified as T-Cell disorders not included in the study population. Thus, the T-Cell Project confirmed the difficulties in providing an accurate classification when a diagnosis of PTCLs is suspected, singled out the major pitfalls that can bias a correct histologic categorization and confirmed that a centralized expert review with the application of adequate diagnostic algorithms is mandatory when dealing with these tumours. Copyright © 2016 John Wiley & Sons

  10. Primary testicular diffuse large B-cell lymphoma: A case report

    Directory of Open Access Journals (Sweden)

    Muhammad Sadiq

    2017-12-01

    Full Text Available Primary testicular diffuse large-B cell lymphoma (DLBCL is an uncommon and aggressive disease with predominant manifestation in the older age. Herein, we report a case of 47-year-old male patient who presented with three months history of left testis swelling. The patient underwent unilateral (left radical orchiectomy. Histopathological examination revealed extensive involvement and replacement of testicular parenchyma by a tumor composed of large discohesive sheets of cells with pleomorphic, hyperchromatic nuclei and prominent nucleoli. Immunohistochemical (IHC staining showed reactivity for LCA & Pan B (CD20 and negativity for OCT 3/4, SALL4 and Inhibin. Moreover, Pan T (CD3 highlighted reactive T-cells. These features rendered the diagnosis of DLBCL of testis. The hybrid 2-[fluorine-18] fluoro-2-deoxy-d-glucose (FDG positron emission tomography/computed tomography (PET/CT demonstrated two para-aortic FDG avid lymph nodes on the left side at the level of L2 vertebra. Presently, the patient has been planned for doxorubicin-based chemotherapy (i.e., cyclophosphamide, doxorubicin, vincristine and prednisone; CHOP along with intrathecal Methroxate (MTX, which would presumably improve the prognosis. Our study would expand the pool of this uncommon tumor towards its better understanding. Keywords: Primary testicular lymphoma, Diffuse large-B cell lymphoma, Orchiectomy, Doxorubicin-based chemotherapy

  11. Sarcomatoid carcinoma associated with small cell carcinoma of the urinary bladder: a series of 28 cases.

    Science.gov (United States)

    Urrea, Yuly Ramirez; Epstein, Jonathan I

    2017-09-01

    The association of sarcomatoid carcinoma (SC) with small cell carcinoma (SCC) has not been systematically studied. We identified 39 consult cases between 2001 and 2016 with available slides for review in 28 cases. There were 19 men and 9 women (mean age: 78 years [51-89]). In 26 (92.8%) cases, the sarcomatoid component had nonspecific malignant spindle cells, 4 (14%) chondrosarcoma, 2 (7%) myxoid sarcomatous, 1 (3.5%) osteosarcoma, and 1 (3.5%) rhabdomyosarcoma. The predominant component was SCC in 11 (39%) cases, urothelial carcinoma in 6 (21%), sarcomatoid in 3 (10%), and equal sarcomatoid and SCC in 8 (29%). There were 3 morphological groups: group 1 (18/28 [64%]) showed a gradual transition from SCC to other components; group 2 (5/28 [18%]) had an abrupt transition from SCC to other components; and in group 3 (5/28 [18%]), the SCC was separate from other components. In group 1, 12 (66%) cases of SCC showed a gradual transition to sarcomatoid areas; 3 (17%) to urothelial carcinoma; and 3 (17%) to multiple components including squamous cell carcinoma, urothelial carcinoma, and sarcomatoid. Mortality did not differ based on pathological groups. The 36-month actuarial risk of death was 64.3%. The multitude of different components in these tumors is further evidence of the remarkable ability of carcinoma of the bladder to show divergent differentiation with, in some cases, gradual transition between SCC and other elements including sarcomatoid. Greater recognition of this entity with chemotherapy targeted to the various histological elements may have important therapeutic implications. Copyright © 2017 Elsevier Inc. All rights reserved.

  12. Psychogenic fever in a patient with small cell lung cancer: a case report

    International Nuclear Information System (INIS)

    Xu, Mengdan; Zhang, Xiaoye; Xu, Zhaoguo; Cui, Guoyuan; Yu, Li; Qi, Xiaoying; Lin, Jia; Liu, Yan

    2015-01-01

    Fever is common in malignant tumors. We report an exceptional case of psychogenic fever in a patient with small cell lung cancer. This is the first case report of psychogenic fever in a patient with small cell lung cancer. A 61-year-old Chinese man diagnosed with small cell carcinoma on June 30, 2012, came to our department with a complaint of fever lasting more than 1 month. He had undergone chemoradiotherapy for lung cancer 6 months previously. After admission, his body temperature fluctuated in the range of 37 °C to 39 °C. Somatic symptoms associated with anxiety were obvious. A 24-item Hamilton Anxiety Scale was used to assess the patient’s condition. A score of 32 confirmed a diagnosis of severe anxiety. After a week of antianxiety treatment, the patient’s temperature returned to normal. Psychogenic fever is common in cancer patients and deserves more attention. Patients with psychogenic fever must be distinguished from patients with infectious fever (including neutropenic fever), and tumor fever. Additionally, antianxiety or antidepression treatment should be provided. A concern is that continual anxiety may adversely affect anticancer therapy

  13. A Case of Primary Gastric Small-Cell Carcinoma in an Elderly Patient

    Directory of Open Access Journals (Sweden)

    Fa-Chang Yu

    2012-03-01

    Full Text Available We report a case of primary small-cell carcinoma of the stomach in a 75-year-old man. The patient was admitted to our hospital with a 1-week history of intermittent tarry stool. An upper gastrointestinal examination revealed a large stage A2 ulcer in the greater curvature of the body of the stomach, and pathological findings from biopsy specimens revealed small-cell carcinoma. The tumor cells were small-sized, composed of hyperchromatic nuclei with scant cytoplasm, and stained positive for cytokeratin, synaptophysin, and chromogranin A. The patient was diagnosed with primary small-cell carcinoma of the stomach. He declined further evaluation and received palliative management. This is a rare carcinoma of the stomach, with aggressive manifestations and a poor prognosis. The mean survival of patients with primary gastric small-cell carcinoma is reported to be 7 months. The choice of treatment for this disease is still controversial. This rare gastric tumor should be listed in the differential diagnosis of gastric carcinoma in the elderly.

  14. GIANT CELL GRANULOMAS OF THE JAWS (ANALYSIS OF 1,083 CASES

    Directory of Open Access Journals (Sweden)

    Ismail Yazdi

    1995-07-01

    Full Text Available Giant cell granulomas of the jaws are rather common in [rail, however, more reliable data need to be published In this retrospecti ve study 1,083 cases ojPGCO and CGCG (817 peripheral and 203 central were extracted from 6800 oral biopsy archives of the Oral Pathology Department and were analyzed: Age and sex ofthe patients and type and location distribution were obtained Our results show that most cases ofPGCG and CGCG occurred before"nthe fifth and during fourth decade, respectively. Slight predominance offemales was notedfor both types. Mandible wa~ often more affected (60.3%, especially in the premolarmolar region. The results obtained in this study were in agreement with other independent reports.

  15. Diagnosis and Treatment of Esophageal Granular Cell Tumor: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Ramin Niknam

    2017-01-01

    Full Text Available Gastrointestinal granular cell tumors are uncommon. The most common site of gastrointestinal granular cell tumor (GCT is esophagus. We report a case of esophageal GCT incidentally diagnosed by endoscopy. The lesion was evaluated by endoscopic ultrasonography and resected using the endoscopic technique without complication.

  16. Naevi as a risk factor for basal cell carcinoma in Caucasians: a Danish case-control study

    DEFF Research Database (Denmark)

    Lock-Andersen, J; Drzewiecki, K T; Wulf, H C

    1999-01-01

    The number of melanocytic naevi in Caucasians is related to previous exposure to the sun and is a well-documented major risk factor for cutaneous malignant melanoma. Basal cell carcinoma, which is the most common form of skin cancer, has also been shown to be related to exposure to the sun....... To investigate whether the number of common naevi is a risk factor for basal cell carcinoma in Caucasians we performed whole-body counting of naevi > or =2 mm in a Danish case-control study with 145 cases of primary basal cell carcinoma and 119 controls matched on age, gender and place of residence. Naevi were...... recorded according to size and body region and the skin phototype was assessed. There was no correlation between self-reported skin type and the number of naevi. Females with basal cell carcinoma had more naevi than did female controls (median number of naevi: 65 and 32, respectively) while males...

  17. B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and classical Burkitt's lymphoma: A case report and review

    OpenAIRE

    Chettiankandy, Tabita Joy; Tupkari, Jagdish Vishnu; Kumar, Keshav; Ahire, Manisha Sandeep

    2016-01-01

    B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma (DLBCL) and classical Burkitt's lymphoma (BL), is a diagnostic provisional category in the World Health Organization 2008 classification of lymphomas. This category was designed as a measure to accommodate borderline cases that cannot be reliably classified into a single distinct disease entity after all available morphological, immunophenotypical and molecular studies have been performed. Typica...

  18. An intraventricular clear cell meningioma revealed by an inflammatory syndrome in a male adult: a case report.

    Science.gov (United States)

    Cassereau, J; Lavigne, C; Michalak-Provost, S; Ghali, A; Dubas, F; Fournier, H D

    2008-07-01

    Intraventricular meningiomas are infrequent intracranial tumors. Clinical symptoms are mainly due to an increased intracranial pressure or a direct pressure on the surrounding brain structures. Inflammatory syndrome was described in some patients with chordoid meningiomas. Here we report a case of right intraventricular clear cell meningioma in a 50-year-old man who presented with fever, headache, and inflammatory syndrome. Clinical and biological normalization was rapidly obtained after tumor removal. Immunohistochemical examination showed tumor cells and lymphocytes positivity for the pyrogenic cytokine interleukin-6, with a same intensity. To our knowledge, this is the first case described in the literature concerning an adult man with an intraventricular clear cell meningioma associated with a systemic inflammatory syndrome.

  19. Nevoid basal cell carcinoma syndrome (Gorlin-Goltz syndrome). Case report.

    Science.gov (United States)

    Fini, G; Belli, E; Mici, E; Virciglio, P; Moricca, L M; D'Itri, L; Leonardi, A; Malavenda, M S; Krizzuk, D; Merola, R; Maturo, A; Pasta, V

    2013-01-01

    Gorlin-Goltz syndrome or nevoid basal cell carcinoma syndrome (NBCCS) comprises multiple basal cell carcinomas, keratocysts of the jaw, palmar/plantar pits, spine and rib anomalies, calcifications of the falx cerebri etc. The diagnosis is made according to clinical criteria (Kimonis Criteria) and genetic ones. We studied one family where father and then his sun resulted affected by each syndrome. Gorlin-Goltz syndrome is a rare disease diagnosed according to clinical criteria sometimes difficult to integrate. The family case we presented shows how you can get diagnosis even in older age and after numerous surgeries. Patients should be given special attention and therefore should be monitorized and need multidisciplinary treatments continued in time, even a trivial change of signs and symptoms may be an important indicator of a precipitating event which puts the patient's life under threat.

  20. An Extremely Rare and Unusual Case of Retroperitoneal and Pelvic Metastasis from Squamous Cell Carcinoma of Vallecula

    Directory of Open Access Journals (Sweden)

    Abhishek Purkayastha

    2016-06-01

    Full Text Available We report an extremely rare and unusual case of retroperitoneal and pelvic metastasis from primary squamous cell carcinoma of vallecula. Generally carcinoma oropharynx metastasizes to lungs, liver and bone while retroperitoneal and pelvic metastasis is rarely heard of. To the best of our knowledge this case is one of the scantly reported cases ever of this kind in the world.  A 60-year-old male presented with dysphagia and hoarseness of voice of four month duration.  Computed tomography (CT scan face and neck showed growth right vallecula. Biopsy of lesion showed squamous cell carcinoma. Metastatic work up was negative. He received definitive chemo-radiation. Patient during follow up presented with dyspepsia, abdominal discomfort and weight loss. Whole body positron emission tomography (WB PET scan revealed retroperitoneal and pelvic lymph node deposits which were confirmed as metastasis of squamous cell carcinoma by CT guided fine needle aspiration cytology (FNAC. Patient was exhibited palliative chemotherapy but his general condition deteriorated and he finally succumbed to his metastatic illness. This case is being reported to highlight its extreme rarity, the diagnostic and therapeutic challenges it presented and its overall dismal prognosis.

  1. Cutaneous Clear Cell Sarcoma: a clinicopathologic, immunohistochemical and molecular analysis of 12 cases emphasizing its distinction from dermal melanoma

    Science.gov (United States)

    Hantschke, Markus; Mentzel, Thomas; Rütten, Arno; Palmedo, Gabriele; Calonje, Eduardo; Lazar, Alexander J.; Kutzner, Heinz

    2010-01-01

    Clear cell sarcoma (CCS) of tendons and aponeuroses / malignant melanoma of soft parts is a rare tumor and in the majority of cases presents a characteristic reciprocal translocation t(12;22)(q13;q12) that results in fusion of the EWS and ATF1 genes. Although the melanocytic differentiation of CCS is indisputable, its precise lineage remains unclear. Typically, the slowly growing tumor affects the extremities of adolescents or young adults, especially around the ankle and foot. CCS is classically regarded as a deep soft tissue tumor associated with tendons or aponeuroses. This traditional view is put into perspective by the description of primary CCS of the gastrointestinal tract, which may have a variant fusion gene EWSR1-CREB1. We describe 12 cases of cutaneous CCS and discuss the differential diagnoses. These 12 cases share an identical immunohistochemical profile with malignant melanoma (MM) and thus can easily be confused with a dermal variant of spindle cell MM or metastasis of MM. The patients' ages ranged from 6 to 74 years (median: 25 y), and there was a female predominance (10 females, 2 males). Most tumors (n = 9) were located on the extremities, 2 tumors arose on the back, and 1 on the abdomen. The mean tumor size was 0,97 cm (range, 0,4 to 1,7 cm). Six cases showed invasion of the subcutis, the other 6 cases were entirely dermal. Tumor necrosis was evident in 2 cases, melanin pigment in 2 cases, and ulceration in 1 tumor. All cases showed uniform nests and fascicles of pale spindled or slightly epitheloid cells with finely granular eosinophilic or clear cytoplasm. There was fair pleomorphism with plump spindled nuclei and significantly prominent nucleoli. Multinucleated wreath-like tumor giant cells were observed in two-thirds of cases, but were usually present only focally. The dense cellular aggregates were encased by delicate fibrous septa. The stroma showed a sclerotic reticulated pattern. Partly, the nests of spindle cells bordered the epidermis

  2. Primary cutaneous large B-cell lymphoma of scalp: Case report of a rare variant

    Directory of Open Access Journals (Sweden)

    Yasmeen Khatib

    2017-01-01

    Full Text Available Primary cutaneous large B-cell lymphoma (Bcl is defined as a lymphoma composed of large cells constituting more than 80% of the infiltrate and absence of extracutaneous involvement after staging investigations. In the new World Health Organization/European Organization for Research and Treatment of Cancer classification, cutaneous Bcls with large cells are of three types - primary cutaneous large Bcl leg type (PCLBCLLT, primary cutaneous follicle center lymphoma diffuse type (PCFCLDT, and primary cutaneous large Bcls other (PCLBCLO. These three different types are distinct in terms of their clinicopathological features and survival. The PCLBCLO has intermediate features between those of PCLBCLLT and PCFCLDT. We present a case of PCLBCLO in a 57-year-old male who presented with a scalp swelling. Ultrasonography examination was suggestive of a sebaceous cyst. Computed tomography scan revealed the presence of an ill-defined hyperdense region in the soft tissue of the scalp region extending into the deeper layers of the scalp. Fine-needle aspiration cytology (FNAC revealed the presence of atypical lymphoid cells. Diagnosis was confirmed by biopsy and immunohistochemistry. Patient received rituximab combined with doxorubicin, vincristine, cyclophosphamide, and prednisolone regimen with complete resolution of the lesion. We present this case for its rarity, the utility of FNAC in early diagnosis, and to discuss the differential diagnosis.

  3. Unusual Upper Gastrointestinal Bleeding due to Late Metastasis from Renal Cell Carcinoma: A Case Report

    Directory of Open Access Journals (Sweden)

    Wen-Tsan Chang

    2004-03-01

    Full Text Available A case of recurrent massive upper gastrointestinal bleeding originating from metastatic renal cell carcinoma is reported. A 63-year-old woman underwent right nephrectomy 9 years previously and experienced no recurrence during follow-up. A gradually enlarging ulcerative tumor over the bulb of the duodenum and four subsequent episodes of massive bleeding from this tumor occurred between June 2001 and March 2002. The patient underwent surgery in April 2002 for intractable bleeding from the tumor. Renal cell carcinoma metastasis to the duodenum was confirmed from the surgical specimen. Upper gastrointestinal bleeding due to malignancy is very rare and the duodenum is the least frequently involved site. Furthermore, a solitary late renal cell carcinoma metastasis 9 years after a nephrectomy is extremely uncommon. This case suggests that life-long follow-up of renal cell carcinoma patients is necessary, owing to unpredictable behavior and the possibility of long disease-free intervals. In nephrectomized patients suffering from gastrointestinal bleeding, complete evaluation, especially endoscopic examination, is indicated. The possibility of late recurrent renal cell carcinoma metastasis to the gastrointestinal tract should be kept in mind, although it is rare. If the patient is fit for surgery, metastatectomy is the first choice of treatment.

  4. A case of primary diffuse large B-cell non-Hodgkin's lymphoma misdiagnosed as chronic periapical periodontitis.

    Science.gov (United States)

    Jessri, M; AbdulMajeed, A A; Matias, M A; Farah, C S

    2013-06-01

    Lymphoma is a malignant neoplasm of component cells of the lymphoid system which is very rare in the jaws. Here we report a case of primary diffuse large B-cell lymphoma located in the periapical region of a mandibular molar which was misdiagnosed as chronic periapical periodontitis. The present case was diagnosed at an early stage and effectively managed by chemotherapy. Although lymphoma of the mandible is rare, it must be considered in the differential diagnosis of radiolucent lesions in this region. Lack of knowledge of this rare presentation may lead to delays in diagnosis and poor prognosis. © 2013 Australian Dental Association.

  5. Alpha-fetoprotein-producing ovarian clear cell adenocarcinoma with fetal gut differentiation: a rare case report and literature review.

    Science.gov (United States)

    Chao, Wei-Ting; Liu, Chia-Hao; Lai, Chiung-Ru; Chen, Yi-Jen; Chuang, Chi-Mu; Wang, Peng-Hui

    2018-06-22

    Alpha-fetoprotein (AFP) is a useful tumor marker for ovarian germ cell tumors, particularly yolk sac tumor (YST). It is valuable for both diagnosis and further follow-up. Epithelial ovarian carcinoma (EOC) rarely secretes AFP, especially for clear cell type and in the postmenopausal women. Based on the limited knowledge about AFP-producing clear cell type EOC, a case and literature review on this topic is extensively reviewed. We report a 55-year-old postmenopausal woman experienced vaginal spotting for one month, and serum level of AFP was 60,721 ng/ml initially. Histological examination was clear cell type EOC. Tumor cells revealed strong immunoreactivity for glypican-3 (GPC3) and AFP and weak for hepatocyte nuclear factor-1 beta (HNF-1 beta), but negative for CD30, making the diagnosis of AFP-producing clear cell type EOC with fetal gut differentiation in focal areas, FIGO (International Federation of Gynecology and Obstetrics) IIIc. Although the patient underwent an intensive treatment, including optimal debulking surgery and multi-agent chemotherapy, the patient died of disease. To provide a better understanding of clinical and molecular characteristics of the AFP-producing clear cell type EOC, we conducted a systematic literature review. A total of three papers described the AFP-producing clear cell type EOC are available. The overall survival rate of these cases, including the current case is 50%. Although immunohistochemical examination is not always needed in routine for the diagnosis of clear cell type EOC, to distinguish from other tumors, especially germ cell tumors, or to provide the better way to monitor therapeutic response or to evaluate the disease status, immunostaining, including GPC3, HNF-1 beta, CD30, cytokeratin 7 or 20, and AFP is taken into account. Due to rarity, the appropriate chemotherapy regimen and the biological behavior of AFP-producing clear cell type EOC are still unclear.

  6. Environmental Technology Assessment of Introducing Fuel Cell City Buses. A Case Study of Fuel Cell Buses in Goeteborg

    Energy Technology Data Exchange (ETDEWEB)

    Karlstroem, Magnus

    2002-07-01

    Over the last several years, fuel cell systems have improved. These advancements have increased the expectations that fuel cells are a feasible option for several applications such as transportation and stationary use. There are several reasons why fuel cell buses in city centres appear to be the most beneficial market niche to begin introducing the technology in. The goal of the report is to compile information about fuel cell buses relevant for city administrators working with public transport and environmental issues. A literature review of the fuel cells in buses is included. This study also consists of an environmental assessment of using fuel cell buses with hydrogen produced in various ways for buses on bus route 60 in Goeteborg by 2006. The fuel cell buses are compared with other bus and fuel alternatives. There are two goals of the case study: 1. The first goal is to describe the technical system, the methodology, and the problem for the intended audience. In the future, this study could help frame future investment decisions. 2. The second goal is to present environmental performance results---emission, health, monetary---relative the alternative bus technologies. The model calculations showed that the social benefits were approximately SEK 910,000 each year if all buses were fuel cell buses compared with developed diesel buses. If the fuel cell buses were compared to natural gas buses, then the benefits were SEK 860,000 each year. The benefits were SEK 1.39/bus/km compared with diesel buses or SEK 1.30/bus/km compared with natural gas buses.

  7. A case of hirsutism due to bilateral diffuse ovarian Leydig cell hyperplasia in a post-menopausal woman.

    Science.gov (United States)

    Ali, F S.M.; Stanaway, S E.R.S.; Zakhour, H D.; Spearing, G; Bowen-Jones, D

    2003-11-01

    Hyperandrogenism in females usually results from ovarian or adrenal pathology. We present a case of virilizaton due to very rare bilateral ovarian diffuse interstitial proliferation of Leydig cells with no tumour or hilar cell hyperplasia identified. Interestingly, the case was further complicated by the finding of high levels of testosterone in one adrenal vein on selective venous sampling (SVS), resulting in an unnecessary unilateral adrenalectomy. Further sampling found high levels also in the ovarian veins, and the condition was finally cured by bilateral oophorectomy.

  8. Dramatic Response of a Case ofRecurrent Basal Cell Carcinoma toSystemic Chemotherapy

    Directory of Open Access Journals (Sweden)

    Mohammad Mohammadianpanah

    2010-10-01

    Full Text Available Basal cell carcinoma (BCC is the most common cancer among humans, and the standard treatment is surgery. Other modalities are reserved as a second line of treatment. Topical chemotherapy may be used in primary BCC. Systemic chemotherapy has no role in the primary treatment of BCC, although it may be efficacious in metastatic cases. We report the case of a patient with persistent recurrent BCC following multiple surgeries and radiotherapy, who achieved a dramatic response with a cisplatinand 5-flourouracil chemotherapy regimen.

  9. Metastatic ghost cell odontogenic carcinoma: description of a case and search for actionable targets

    Directory of Open Access Journals (Sweden)

    Maximilien J. Rappaport

    2015-09-01

    Full Text Available Ghost cell odontogenic carcinoma (GCOC is an exceedingly rare malignant tumor on the spectrum of already uncommon odontogenic or dentinogenic tumors. We describe here the case of metastatic GCOC in a patient with a history of recurrent dentinogenic ghost cell tumor of the mandible, now presenting with bilateral pleural effusions. We will discuss typical histopathologic and histochemical features of GCOC, along with results of genomic testing and their role in directing therapy.

  10. Case report of precursor B-cell lymphoblastic lymphoma presenting as syncope and cardiac mass in a nonimmunocompromised child.

    Science.gov (United States)

    Hahn, Barry; Rao, Sudha; Shah, Binita

    2007-08-01

    We report the case of a previously healthy, 10-year-old boy who presented to the emergency department with a syncopal episode. In the emergency department, the patient was diagnosed with a right atrial mass, later identified as a precursor B-cell lymphoblastic lymphoma (LL). Most causes of syncope in children are not life threatening. In most cases, it indicates a predisposition to vasovagal episodes. Lymphomas account for approximately 7% of malignancies among children younger than 20 years, are more common in white males and immunocompromised patients, and are predominantly tumors of T-cell origin. Children with non-Hodgkin lymphoma usually present with extranodal disease, most frequently involving the abdomen (31%), mediastinum (26%), or head and neck (29%). Our patient was unique in that he was a nonimmunocompromised, black boy, presenting with syncope in the setting of a large atrial mass identified as a precursor B-cell LL. To our knowledge, there are no reported cases of precursor B-cell LL presenting as syncope and a cardiac mass.

  11. Squamous cell carcinoma of the scrotum in a Nigerian: case report

    Directory of Open Access Journals (Sweden)

    Temple Oguike

    2009-07-01

    Full Text Available Squamous cell carcinoma of the scrotum is rare and to the best of our knowledge has never been reported from Nigeria. We report on a case thought to be occupation-related in a 42-year-old Nigerian taxi driver who had previously been an automobile mechanic and later a long-haul truck driver. He presented with a stage D disease and only palliation was feasible.

  12. A case of radiation cancer associated with spinocellular carcinoma and basal cell epithelial tumor

    International Nuclear Information System (INIS)

    Oohara, Kuniaki; Ootsuka, Fujio; Mizoguchi, Masako.

    1980-01-01

    The patient was a 66 year-old male who had received radiotherapy for psoriasis vulgaris in frontal plane for 10 years since the age of 19. This radiotherapy was carried out once a week for 5 to 6 weeks and stopped for following 5 to 6 weeks. The source and the dose were unknown. Multiple superficial basal cell epithelial tumor occurred 32 to 33 years after that in the region over which radiation had been given. Moreover, 37 years after that, spinocellular carcinoma occurred in the same region. Spinocellular carcinoma in this case increased rapidly and reached the depth of frontal plane. Atypic of cancer cells was marked, and various findings were observed. Characteristics of these tumor cells were mixture of spindle cells and cells with vacuoles. Partially, findings common to basal cell epithelial tumor were coexisted, and senile keratosis was also discovered. (Tsunoda, M.)

  13. Testicular Metastasis from Renal Cell Carcinoma: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Keren Rouvinov

    2017-04-01

    Full Text Available Testicular metastases from renal cell carcinoma (RCC are extremely rare. To the best of our knowledge, only 33 cases have been described in the literature. Most of the reported cases are of unilateral testicular metastasis from RCC. We report a case of metachronous ipsilateral testicular metastasis from RCC in a 78-year-old man 6 years after nephrectomy. Scrotal ultrasonography showed a 4 × 5 cm mass in the right testis. Right inguinal orchiectomy was performed for diagnosis. Computed tomography revealed liver and lung metastases. First-line therapy with sunitinib was started in November 2016 for metastatic RCC.

  14. A Rare Case of Intussusception Associated with Metastasize Small Cell Carcinoma of Lung

    Directory of Open Access Journals (Sweden)

    Razrim Rahim

    2012-11-01

    Full Text Available Intussusception is common cause of bowel obstruction in the paediatric age group compared to the elderly population. Many times, The diagnosis may be difficult because of asymptomatic nature of thisbowel disorder. We hereby describe the case of a 75-year-old male who presented with lethargy, weakness,loss of movement in the joints and was found to be anemic. The haemoglobin level was low so he wastransfused with packed cells. On gastrointestinal (GI endoscopy, upper GI bleed was observed. A mass was observed beyond ampulla at the 2nd and 3rd part of the duodenal junction. Computerized tomography (CTscan also showed a mass at the head of pancreas and the lesion at the left lung. In view of persistent bleed,‘Whipple’s procedure’ was performed. Histopathological examination showed small cell carcinoma of the lungs with metastasis to the pancreas and the jejunum. We here discuss the case of intussusception with intestinal metastasis which presented with gastrointestinal bleeding.

  15. Imaging in a case of giant cell tumor of tendon sheath in foot: A case report with re-view of literature

    Directory of Open Access Journals (Sweden)

    Sujata Patnaik

    2014-07-01

    Full Text Available Large sized Giant cell tumors (GCT of the tendon sheaths of the foot are rare. We present a case with a large tumor over the dor-sum of foot which was diagnosed and studied by plain radiog-raphy, Ultrasound, CT and MRI scans. It was histologically con-firmed on biopsy. When the size of the tumor (like Giant cell tu-mor is too large and spread over multiple bones of the foot MRI is the imaging modality of choice to precisely define the anatomy to help in taking surgical decisions.

  16. Chemoresistance, Cancer Stem Cells, and miRNA Influences: The Case for Neuroblastoma

    Directory of Open Access Journals (Sweden)

    Alfred Buhagiar

    2015-01-01

    Full Text Available Neuroblastoma is a type of cancer that develops most often in infants and children under the age of five years. Neuroblastoma originates within the peripheral sympathetic ganglia, with 30% of the cases developing within the adrenal medulla, although it can also occur within other regions of the body such as nerve tissue in the spinal cord, neck, chest, abdomen, and pelvis. MicroRNAs (miRNAs regulate cellular pathways, differentiation, apoptosis, and stem cell maintenance. Such miRNAs regulate genes involved in cellular processes. Consequently, they are implicated in the regulation of a spectrum of signaling pathways within the cell. In essence, the role of miRNAs in the development of cancer is of utmost importance for the understanding of dysfunctional cellular pathways that lead to the conversion of normal cells into cancer cells. This review focuses on highlighting the recent, important implications of miRNAs within the context of neuroblastoma basic research efforts, particularly concerning miRNA influences on cancer stem cell pathology and chemoresistance pathology for this condition, together with development of translational medicine approaches for novel diagnostic tools and therapies for this neuroblastoma.

  17. Small cell carcinoma arising in Barrett's esophagus: a case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Markogiannakis Haridimos

    2008-01-01

    Full Text Available Abstract Introduction Gastrointestinal tract small cell carcinoma is an infrequent and aggressive neoplasm that represents 0.1–1% of gastrointestinal malignancies. Very few cases of small cell esophageal carcinoma arising in Barrett's esophagus have been reported in the literature. An extremely rare case of primary small cell carcinoma of the distal third of the esophagus arising from dysplastic Barrett's esophagus is herein presented. Case presentation A 62-year-old man with gastroesophageal reflux history presented with epigastric pain, epigastric fullness, dysphagia, anorexia, and weight loss. Esophagogastroscopy revealed an ulceroproliferative, intraluminar mass in the distal esophagus obstructing the esophageal lumen. Biopsy showed small cell esophageal carcinoma. Contrast-enhanced chest and abdominal computed tomography demonstrated a large tumor of the distal third of the esophagus without any lymphadenopathy or distant metastasis. Preoperative chemotherapy with cisplatine and etoposide for 3 months resulted in a significant reduction of the tumor. After en block esophagectomy with two field lymph node dissection, proximal gastrectomy, and cervical esophagogastric anastomosis, the patient was discharged on the 14th postoperative day. Histopathology revealed a primary small cell carcinoma of the distal third of the esophagus arising from dysplastic Barrett's esophagus. The patient received another 3 month course of postoperative chemotherapy with the same agents and remained free of disease at 12 month review. Conclusion Although small cell esophageal carcinoma is rare and its association with dysplastic Barrett's esophagus is extremely infrequent, the high carcinogenic risk of Barrett's epithelium should be kept in mind. Prognosis is quite unfavorable; a better prognosis might be possible with early diagnosis and treatment strategies incorporating chemotherapy along with oncological radical surgery and/or radiotherapy as part of a

  18. Primary clear cell carcinoma of parotid gland: Case report and review of literature.

    Science.gov (United States)

    Rodríguez, Marta Saldaña; Reija, Maria Fe García; Rodilla, Irene González

    2013-01-01

    Clear cell carcinoma (CCC) is a rare low-grade carcinoma that represents only 1% to 2% of all salivary glands tumors. The finding of a clear cell tumor in a parotid gland involves the necessity of differential diagnosis between primary clear cell parotid tumors and metastases, mainly from kidney. The biological behavior is not very aggressive and development, which is very slow, is usually asymptomatic and indeed, the tumor often reaches considerable dimensions before being diagnosed. The treatment of choice is the surgical excision. There are rare cases of local recurrence and distant metastases. The aim of this article is to report a primary CCC in the parotid gland that microscopically closely resembled a metastatic CCC of renal origin, making microscopic differentiation difficult.

  19. [Small cell neuroendocrine tumour of the bladder: with reference to a case and bibliographical revision].

    Science.gov (United States)

    Lahoz Tornos, A; Marrón Penón, Maria C; Pardo López, Maria L; Nogueras Gimeno, M A; Pujol Obis, E; Del Villar Sordo, V

    2006-09-01

    The small cell neuroendocrine tumour is an infrecuent neoplasia, with inmunohistochemistry being the key to diagnosis. We present a new case making reference to treatment and its evolution there after. The clinic, diagnosis and treatment of this tumour is described. Bibliographical revision follours. The neuroendocrine tumour of small cell is an infrecuent neoplasia, in which the inmunohistochemistry study is key in the diagnosis. The differential diagnosis includes the high degree diferentiation transitionals cells carcinoma and primary and secondary linfoma. The standard treatment is based on chemotherapy plus surgery.

  20. Malignant granular cell tumour on the thoracic wall: a case report and literature review

    International Nuclear Information System (INIS)

    Perez, E.; Esteban, R.; Alcalaya, R.; Albors, L.; Jimenez, C.; Ovelar, Y.; Cantera, M.G.

    1993-01-01

    A case is presented of a malignant granular cell tumour in a 52-year-old patient the initial location of which was the thoracic wall. After the tumour's removal there was recurrence in the lymph nodes, retroperitoneum, bone, lung and orbits. The important features of this case are its extraordinary rarity, the unusual location in the thoracic wall, and the tumour's infrequent malignancy. The radiological and histological findings are discussed, and the literature on the subject is reviewed. (orig.)

  1. Hairy B-cell lymphoproliferative disorder and its differential diagnosis: a case with long-term follow-up

    Directory of Open Access Journals (Sweden)

    Kensuke Matsuda

    2017-09-01

    Full Text Available Hairy B-cell lymphoproliferative disorder (HBLD is one of chronic polyclonal B-cell lymphocytosis. We report a 47-year-old female Japanese patient diagnosed as having HBLD based on lymphocytosis with hairy cell appearance and characteristic phenotypes including CD11c+, and without B-cell monoclonalities. She was a non-smoker, and possessed HLA-DR4. She has been closely followed up without treatment and lymphoma development for over five years. Although this disease is quite rare and has been reported, to our knowledge, in only 13 Japanese cases, an accurate diagnosis, particularly differential diagnosis from persistent polyclonal B-cell lymphocytosis or hairy cell leukemia-Japanese variant is essential for the prevention of unnecessary treatments.

  2. Usefulness of (18)F-FDG PET/CT in recurrent basal cell carcinoma: Report of a case.

    Science.gov (United States)

    Ayala, S; Perlaza, P; Puig, S; Prats, E; Vidal-Sicart, S

    2016-01-01

    We analyze the case of a patient with left periorbital infiltrating basal cell carcinoma treated with surgical excision in October 2010. Surgery included orbital exenteration and reconstruction using skin graft and radiotherapy. In May 2013 a MR imaging showed a mass in the left orbital fossa, suggesting a recurrence in the graft. A basal cell carcinoma recurrence with perineural invasion was confirmed in the biopsy. On (18)F-FDG PET/CT performed, a hypermetabolic activity was observed in the left periorbital area with extension to surrounding sinus and bones. The use of (18)F-FDG PET/CT in patients with advanced basal cell carcinoma has not been fully explored due to the rarity of this entity. This case demonstrates the usefulness of this technique to determine the extent of non-melanocytic recurrent skin tumors, and its value in the staging and treatment control, supporting the incorporation of (18)F-FDG PET/CT in the management of advanced basal cell carcinoma. Copyright © 2015 Elsevier España, S.L.U. and SEMNIM. All rights reserved.

  3. True precocious puberty following treatment of a Leydig cell tumour: two case reports and literature review

    Directory of Open Access Journals (Sweden)

    Alberto eVerrotti

    2015-11-01

    Full Text Available Leydig cell testicular tumours are a rare cause of precocious pseudopuberty in boys. Surgery is the main therapy and shows good overall prognosis. The physical signs of precocious puberty are expected to disappear shortly after surgical removal of the mass. We report two children, 7.5 and 7.7 year-old boys, who underwent testis-sparing surgery for a Leydig cell testicular tumour causing precocious pseudopuberty. During follow-up, after an immediate clinical and laboratory regression, both boys presented signs of precocious puberty and ultimately developed central precocious puberty. They were successfully treated with gonadotropin releasing hormone analogues. Only 6 other cases have been described regarding the development of central precocious puberty after successful treatment of a Leydig cell tumour causing precocious pseudo puberty. Gonadotropin-dependent precocious puberty should be considered in children treated for a Leydig cell tumour presenting persistent or recurrent physical signs of puberty activation. In such cases, therapy with gonadotropin releasing hormone analogues appears to be the most effective medical treatment.

  4. Intra-abdominal desmoplastic small cell tumor in a girl : a case report

    International Nuclear Information System (INIS)

    Lee, Young Seok

    1998-01-01

    Desmoplastic small cell tumor is a rare primary intra-abdominal disease with a predilection for the adolescent boy. This aggressive tumor involved multiple intraperitoneal organs, peritoneal, mesentery and omental seedings, and sometimes retroperitoneal extension. The author reports a case of this rare tumor, demonstrated by CT, MRI and US, in a 14-year-old girl. (author). 9 refs., 2 figs

  5. Squamous cell carcinoma of the finger masquerading as an abscess. Case report.

    LENUS (Irish Health Repository)

    O'Sullivan, S T

    2012-02-03

    A 43-year-old man presented with an abscess on his left ring finger, which recurred despite multiple drainage procedures. Histological examination of the lesion was unhelpful; it was only on histopathological examination of the finger after ray amputation that the diagnosis of cutaneous squamous cell carcinoma was established. This case illustrates the need to consider malignancy when dealing with chronic finger infections.

  6. Primary Cutaneous CD4-Positive Small/Medium Pleomorphic T-cell Lymphoma – A Case Report

    Directory of Open Access Journals (Sweden)

    Micković Milena

    2016-12-01

    Full Text Available Primary cutaneous CD4-positive small- to medium-sized pleomorphic T-cell lymphoma is a provisional entity in the 2005 WHO-EORTC classification for cutaneous lymphomas. It is a rare condition and, in most cases, it has a favorable clinical course and prognosis. Primary cutaneous CD4-positive small/medium pleomorphic T-cell lymphoma (PCSM-TCL is defined as a cutaneous T-cell lymphoma with predominantly small- to medium-sized CD4-positive pleomorphic T-cells without a history of patches and plaques typical of mycosis fungoides. PCSM-TCL usually presents as a solitary plaque or tumor on the head, neck, trunk or upper extremities and it is considered to have indolent clinical behavior. Histologically, it is characterized by a dense infiltration of small/medium-sized pleomorphic T-cells that involves the entire dermal thickness, often with nodular extension into the hypodermis. Using immunohistochemical staining, the majority of the reported cases proved to be CD3, CD4 positive and CD8, CD30 negative. However, due to the rarity and heterogeneity of the PCSM-TCL, precise clinicopathologic characteristics of PCSM-TCL have not been well characterized and the optimal treatment for this group of lymphomas is yet to be defined. Dermatologists and pathologists should be aware of this entity in order to avoid unnecessary aggressive treatments.

  7. Retiform Sertoli-Leydig Cell Tumor in a 38-Year-Old Woman: A Case Report, Retrospective Review, and Review of Current Literature

    Directory of Open Access Journals (Sweden)

    Laura C. Nwogu

    2017-01-01

    Full Text Available Ovarian sex cord-stromal tumors arise from the stromal cells that surround and support the oocytes. Sertoli-Leydig cell tumors belong to this category of ovarian neoplasms. We present the case of a 38-year-old woman who was found to have a right ovarian mass. The mass was resected and diagnosed as Stage I Sertoli-Leydig cell tumor, retiform variant, following histopathologic and immunohistochemical examination. This case is unusual given the rarity of the retiform variant of Sertoli-Leydig cell tumor and the atypically older age of 38 years at presentation.

  8. Adult T-cell leukemia on the east coast of Kii Peninsula--presentation of an anti-ATLA-negative case.

    Science.gov (United States)

    Karitani, Y; Kobayashi, T; Koh, T; Iwata, Y; Tanaka, I; Minami, N; Shirakawa, S

    1983-01-01

    Nineteen patients with adult T-cell leukemia (ATL) have been found in the last seven years along the east coast of Kii Peninsula in Japan. The leukemic cells were of the immunologically inducer/helper T-cell phenotype. The prognosis was very poor (median survival time, 85 days), and most of the patients had fatal complications of pulmonary infections. Antibody against ATL-associated antigen (anti-ATLA) was detected in sera from 9 of 10 patients who were born along the coast. However, it was not detected in one patient who was born in a district surrounded by mountains. Although he had neither superficial lymphadenopathy nor skin lesions, he showed rapid clinical deterioration. His leukemic cells appeared to be extremely bizarre with marked nuclear deformation compared with those of the other patients. In surface marker studies the leukemic cells reacted positively with OKT3, OKT4 and OKIa-1 monoclonal antibodies. The characteristics of the anti-ATLA-negative case are discussed in comparison with the other ATL cases.

  9. Importance of gallium-67 scintigraphy in primary cutaneous B-cell lymphoma: report of two cases

    International Nuclear Information System (INIS)

    Attab, Cyomara Sanches; Moriguchi, Sonia Marta; Rocha, Euclides Timoteo da

    2010-01-01

    The authors describe two cases of cutaneous B-cell lymphoma where correct staging, treatment and follow-up could be achieved through a combination of conventional imaging studies and gallium-67 scintigraphy. (author)

  10. The nested variant of bladder transitional cell carcinoma: Review of 12 cases and review of the literature.

    Science.gov (United States)

    Carrion-Ballardo, C J; Gala-Solana, L; Portillo-Martin, J A; Azueta-Etxebarria, A; Truan-Cacho, D; Campos-Juanatey, F

    2015-01-01

    The nested variant of bladder transitional cell carcinoma is extremely rare and has a different biological behavior to other bladder tumors. The aim of this study is to analize if their behavior is as aggressive as has been described in the literature. Review of 12 diagnosed cases with nested variant of bladder transitional cell carcinoma and analysis of demographic characteristics, clinical presentation, tumor characteristics, treatment options, analysis of recurrence and cancer-specific survival between January 1997 and December 2010 in our hospital. 50% of the cases had a pathologic stage ≥T2, with grade of differentiation G2 (50%) or G3 (50%). After the pathological result of the TUR (transurethral resection) Bladder, 5 cases underwent radical cystoprostatectomy, 3 a second TUR bladder and 4 cases with treatment chemotherapy and/or radiotherapy (RT). Five out of 12 cases (41.7%) died due to bladder cancer and 3 died (25%) of other causes (urinary sepsis, respiratory failure, renal failure). With a median follow up of 40 months, the overall survival was 50% and cancer-specific survival of 65.6%. The nested variant of bladder transitional cell carcinoma is a disease with an advanced-stage presentation, with high recurrence and mortality rates despite the use of different treatments. So far there is not a clinical practice guideline for this variety of urothelial tumor. Copyright © 2014 AEU. Publicado por Elsevier España, S.L.U. All rights reserved.

  11. Clear cell urothelial carcinoma of the urinary bladder: a case report and review of the literature.

    Science.gov (United States)

    Knez, Virginia M; Barrow, Willis; Lucia, M Scott; Wilson, Shandra; La Rosa, Francisco G

    2014-08-14

    The occurrence of clear cell tumors in the bladder is not uncommon. Clear cell dysplasia is well-described and characterized by focal replacement of transitional mucosa by cells with abundant clear cytoplasm, nuclear enlargement, and a granular chromatin pattern. Clear cells can also be seen in clear cell adenocarcinoma, which is rare, comprising 0.5% to 2.0% of the reported bladder carcinomas. Other clear cell tumors found in the bladder to be considered in the differential diagnosis are tumors of Müllerian origin and metastatic lesions, such as renal cell carcinoma, clear cell sarcoma, and malignant melanoma. Clear cell urothelial carcinoma is exceedingly rare, with only nine clinical cases described in the literature. We report the case of a 75-year-old Caucasian man who presented with intermittent hematuria, in whom a bladder tumor was identified. A final histopathology examination of a cystoprostatectomy specimen revealed a pT3b, G3 urothelial carcinoma of clear cell type (>90% clear cells) and a prostatic adenocarcinoma of Gleason grade 3+3 (score=6). The bladder tumor consisted of sheets of malignant cells with severe nuclear atypia and abundant clear cytoplasm; no glandular or tubular structures were identified. Tumor cells were periodic acid-Schiff positive and negative after diastase treatment; additional mucicarmine and oil red O stains were negative. Immunohistochemical stains showed the tumor cells positive for cytokeratin 7 (CK7), p63 (>80% nuclei), p53 (about 30% nuclei), vimentin, E-cadherin, cluster of differentiation (CD10), and Ki-67 (>70% nuclei). Stains for cell adhesion molecule 5.2 (CAM 5.2), CD117, cytokeratin 20 (CK20), human melanoma black 45 (HMB-45), paired box protein (PAX 8), placental alkaline phosphatase (PLAP), prostate specific antigen (PSA), renal cell carcinoma (RCC), cancer antigen 25 (CA25), leukocyte common antigen (LC), S-100 protein, and uroplakin III were all negative. The tumor marker profile was consistent with clear

  12. Production cell implementation in the facade execution: a construction case study

    Directory of Open Access Journals (Sweden)

    Renato Nunes Mariz

    2014-05-01

    Full Text Available The construction industry is an environment with much waste. An alternative that could minimize this problem is Lean Thinking, as it focuses on eliminating waste. A widely used tool in lean manufacturing is the production cell, whose main goal is to keep production in continuo flow. In the construction context, the applications of this tool are still incipient, arousing the interest to be further investigated. The aim of this study is to analyze how a production cell was applied in a facade service execution and evaluate this result. We used the case study method in a contractor with experience in facades execution. We collected qualitative data (in situ observations and interviews with employees and quantitative (time and cost data supplied by the contractor. After the data were analyzed (before and after cell implementation Through analysis and literature review we decided to follow those steps: a methods b materials c people d equipments and machines. The results point out improvement in the employee relationship with the administration, increased employee commitment, cost savings with direct labor and increase the pay received by employees. This paper contributes to expanding the use of this tool (production cell in the construction and shows that through this application, many benefits can be achieved.

  13. Human Embryonic Stem Cell Therapy in Crohn’s Disease: A Case Report

    Science.gov (United States)

    Shroff, Geeta

    2016-01-01

    Patient: Male, 21 Final Diagnosis: Crohn’s disease Symptoms: Intolerance to specific foods • abdominal pain and diarrhea Medication: Human embryonic stem cell therapy Clinical Procedure: Human embryonic stem cell transplantation Specialty: Gastroenterology Objective: Unusual or unexpected effect of treatment Background: Crohn’s disease is a chronic inflammatory disease of the intestines, mainly the colon and ileum, related with ulcers and fistulae. It is estimated to affect 565 000 people in the United States. Currently available therapies, such as antibiotics, thiopurines, and anti-tumor necrosis factor-alpha agents, are only observed to reduce the complications associated with Crohn’s disease and to improve quality of life, but cannot cure the disease. Stem cell therapy appears to have certain advantages over conventional therapies. Our study aimed to evaluate the efficacy of human embryonic stem cell therapy in a patient with Crohn’s disease. Case Report: A 21-year-old male with chief complaints of intolerance to specific foods, abdominal pain, and diarrhea underwent human embryonic stem cell therapy for two months. After undergoing human embryonic stem cell therapy, the patient showed symptomatic relief. He had no complaints of back pain, abdominal pain, or diarrhea and had improved digestion. The patient had no signs and symptoms of skin infection, and had improved limb stamina, strength, and endurance. The condition of patient was stable after the therapy. Conclusions: Human embryonic stem cell therapy might serve as a new optimistic treatment approach for Crohn’s disease. PMID:26923312

  14. Circulating Tumor Cells and Cardiac Metastasis from Esophageal Cancer: A Case Report

    Directory of Open Access Journals (Sweden)

    Francesca Consoli

    2011-05-01

    Full Text Available We report the case of a 67-year-old man affected by metastatic esophageal cancer. The patient developed a symptomatic heart metastasis presenting as mimicking ST-segment elevation myocardial infarction. Cardiac magnetic resonance imaging (MRI documented the presence of a mass in the apex and septum of the left ventriculum. The dissemination of cancer was confirmed by the detection of circulating tumor cells (CTCs in the peripheral blood, measured by the CellSearch System (Veridex, LLC, Raritan, N.J., USA. The blood sample drawn at cardiac disease progression revealed the presence of 2 CTCs per 7.5 ml of blood. This report highlights the potential role of CTCs as markers of metastatic spread.

  15. Primary Diffuse Large Cell Lymphoma of the Bladder: Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Mansour Ansari

    2017-01-01

    Full Text Available Most bladder tumors are epithelial in origin. Nonepithelial cancers are rarely located in the bladder. Sarcomas are the most common malignancies among nonepithelial cancers. Primary bladder lymphoma is rare and mostly low grade. Here, we have reported a case of diffuse large cell lymphoma of the bladder. The patient, a 64-year-old man, had urinary frequency for 18 months. Abdominal sonography indicated a thick bladder wall and transurethral biopsy showed diffuse large cell lymphoma. Immunohistochemistry (IHC results showed that the tumor was positive for CD20, CD45, and Pax-5 and negative for BCL-2, cytokeratin, and S100. He had a normal bone marrow biopsy, abdominal, pelvic and chest CT scans. He had no B symptoms. The patient received 6 cycles of R-CHOP followed by radiotherapy (36 Gy to the pelvis. Six months after treatment, the patient is well and has returned to work. We have searched PubMed for primary diffuse large cell lymphoma. Primary diffuse large cell lymphoma of the bladder is best treated according to treatment for diffuse large cell lymphoma of other sites, which includes chemotherapy and radiotherapy. As seen in our review, primary diffuse large cell lymphoma of the bladder has a similar clinical course to diffuse large cell lymphoma of other sites.

  16. Papillary Squamotransitional Cell Carcinoma of the Uterine Cervix: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Shomaila Aamir M. Akbar

    2016-01-01

    Full Text Available Introduction. Papillary squamotransitional cell carcinoma (PSTCC is an uncommon histopathological variant of squamous cell carcinoma (SCC of the uterine cervix, which occurs in postmenopausal women. Presentation of Case. Herein, we describe a case of a 63-year-old woman who presented with 4-month history of postmenopausal vaginal bleeding. Vaginal examination revealed a fragile lesion of size 1×1 cm invading left posterior vaginal fornice and parametrium. Biopsy showed the presence of papillae containing fibrovascular cores lined by multilayered atypical epithelial cells resembling squamous and transitional cell epithelium, confirming the diagnosis of PSTCC of the uterine cervix. After staging work-up she was staged according to the International Federation of Gynecology and Obstetrics (FIGO staging system 2009 as FIGO IIB, and she was started on extended field concurrent chemoradiation. Discussion. PSTCC of the uterine cervix is an extremely rare and aggressive entity. PSTCC is often characterized by the presence of papillary structures with prominent fibrovascular cores. PSTCC of the uterine cervix should be differentiated from transitional cell carcinoma, squamous papilloma, papillary adenocarcinoma, and cervical intraepithelial neoplasia with papillary features. Conclusion. PSTCC of the uterine cervix is a diagnostic challenge; further studies regarding the mechanism underlying the development of PSCC are warranted.

  17. Symptomatic Cushing's syndrome and hyperandrogenemia in a steroid cell ovarian neoplasm: a case report.

    Science.gov (United States)

    Sedhom, Ramy; Hu, Sophia; Ohri, Anupam; Infantino, Dorian; Lubitz, Sara

    2016-10-12

    Malignant steroid cell tumors of the ovary are rare and frequently associated with hormonal abnormalities. There are no guidelines on how to treat rapidly progressive Cushing's syndrome, a medical emergency. A 67-year-old white woman presented to our hospital with rapidly developing signs and symptoms of Cushing's syndrome secondary to a steroid-secreting tumor. Her physical and biochemical manifestations of Cushing's syndrome progressed, and she was not amenable to undergoing conventional chemotherapy secondary to the debilitating effects of high cortisol. Her rapidly progressive Cushing's syndrome ultimately led to her death, despite aggressive medical management with spironolactone, ketoconazole, mitotane, and mifepristone. We report an unusual and rare case of Cushing's syndrome secondary to a malignant steroid cell tumor of the ovary. The case is highlighted to discuss the complications of rapidly progressive Cushing's syndrome, an underreported and often unrecognized endocrine emergency, and the best available evidence for treatment.

  18. Giant Cell Tumor of the Thoracic Spine Presenting as a Posterior Mediastinal Tumor with Benign Pulmonary Metastases: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Tae Hun [Daegu Fatima Hospital College of Medicine, Daegu (Korea, Republic of); Rho, Byung Hak; Bahn, Young Eun; Choi, Won Il [Dongsan Medical Center, Keimyung University School of Medicine, Daegu (Korea, Republic of)

    2010-11-15

    Giant cell tumor of bone is a benign, but potentially aggressive lesion that can show local recurrence and metastases. We report here on a case of a 29-year-old man who presented with an incidentally found mediastinal mass. Chest radiography and computed tomography showed a huge mediastinal mass with bilateral pulmonary nodules and the diagnosis of giant cell tumor with benign pulmonary metastasis was confirmed. To the best of our knowledge, this is the first reported case of primary thoracic spinal giant cell tumor manifesting as a huge mediastinal mass with pulmonary metastases

  19. Pseudocarcinomatous hyperplasia in anaplastic large cell lymphoma, a mimicker of poorly differentiated squamous cell carcinoma: report of a case and review of the literature.

    Science.gov (United States)

    Price, Alexandra; Miller, Jason H; Junkins-Hopkins, Jacqueline M

    2015-11-01

    Pseudocarcinomatous hyperplasia can occasionally be observed in biopsies of CD30-positive lymphoproliferative disorders. It is important to be cognizant of this association, because epithelial hyperproliferation can overshadow large atypical lymphoid cells, leading to an erroneous diagnosis of squamous cell carcinoma (SCC) or keratoacanthoma. Herein, we present a case of anaplastic large cell lymphoma (ALCL) with pseudocarcinomatous hyperplasia simulating a poorly differentiated carcinoma and review the literature on this subject. Immunohistochemical staining with p63 helped delineate the infiltrating tongues of pseudocarcinomatous hyperplasia from the malignant infiltrate. We present this case to raise awareness of the potential for pseudocarcinomatous hyperplasia to occur in the setting of CD30+ lymphoproliferative disorders. Clinicians and dermatopathologists should consider the possibility of ALCL or lymphomatoid papulosis when examining lesions with features of inflamed SCC, especially if the tumor presents on a site or in a patient that is not typical of SCC. © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

  20. Primary leiomyoma of ureter coexisting with renal cell carcinoma: A case report

    International Nuclear Information System (INIS)

    Baek, Seung Hwan; Kim, Hee Jin; Han, Hyun Young

    2014-01-01

    Mesenchymal origin of ureter tumors account for less than 3 percent of all primary ureteral tumors. Among mesenchymal tumors, primary leiomyoma of ureter is extremely rare. Here, we present a case of primary leiomyoma of ureter coexisting with renal cell carcinoma. When encountering well-defined homogeneously enhanced mass of ureter on computed tomography, radiologist should keep in mind that ureteral leiomyoma should be considered as differential diagnosis.

  1. Primary leiomyoma of ureter coexisting with renal cell carcinoma: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Baek, Seung Hwan; Kim, Hee Jin; Han, Hyun Young [Dept. of Radiology, Eulji University Hospital, Daejeon (Korea, Republic of)

    2014-12-15

    Mesenchymal origin of ureter tumors account for less than 3 percent of all primary ureteral tumors. Among mesenchymal tumors, primary leiomyoma of ureter is extremely rare. Here, we present a case of primary leiomyoma of ureter coexisting with renal cell carcinoma. When encountering well-defined homogeneously enhanced mass of ureter on computed tomography, radiologist should keep in mind that ureteral leiomyoma should be considered as differential diagnosis.

  2. Radiographically Negative, Asymptomatic, Sentinel Lymph Node Positive Cutaneous T-Cell Lymphoma in a 3-Year-Old Male: A Case Report

    Directory of Open Access Journals (Sweden)

    Jeffrey Carson

    2012-01-01

    Full Text Available We present a case of a 3-year-old male originally diagnosed with a CD30+ anaplastic cutaneous T-cell lymphoma with no evidence of systemic disease after CT scan, PET scan, and bone marrow aspiration. Sentinel lymph node biopsy (SLNB was performed as an additional step in the workup and showed microscopic disease. Current management/recommendations for cutaneous T-cell lymphoma do not include SLNB. Medical and surgical management of cutaneous malignancies is dramatically different for local versus advanced disease. Therefore adequate evaluation is necessary to properly stage patients for specific treatment. Such distinction in extent of disease suggests more extensive therapy including locoregional radiation and systemic chemotherapy versus local excision only. Two international case reports have described SLNB in cutaneous T-cell lymphoma with one demonstrating evidence of node positive microscopic disease despite a negative metastatic disease workup. This case is being presented as a novel case in a child with implications including lymphoscintigraphy and SLNB as a routine procedure for evaluation and staging of cutaneous T-cell lymphoma if the patient does not demonstrate evidence of metastatic disease on routine workup.

  3. Case report on xeroderma pigmentosum with squamous cell carcinoma in a ten year old child

    Directory of Open Access Journals (Sweden)

    Uday Kumar Sonnappa

    2018-04-01

    Full Text Available Xeroderma pigmentosum (XPis a rare inherited skin disorder characterized by a heightened sensitivity to the DNA damaging effects of ultraviolet radiation (UV. The main source of UV is the sun. The symptoms of XP can be seen in any sun-exposed area of the body. The effects are greatest on the skin, the eyelids and the surface of the eyes but the tip of the tongue may also be damaged. In addition, approximately 25% of XP patients also develop abnormalities of the nervous system manifesting as progressive neuro-degeneration with hearing loss. People with XP have a 10,000-fold increased risk for developing skin cancer including basal cell carcinoma, squamous cell carcinoma and melanoma. They also have a 2000-fold increased risk for cancer of the eye and surrounding ocular tissues. These symptoms appear early in life, typically before age 10 years. This case is being presented to highlight the rarity of a case of xeroderma pigmentosum with squamous cell carcinoma in a ten year old child.

  4. Giant cell arteritis complicated by acute pancreatitis: a case report

    Directory of Open Access Journals (Sweden)

    Seneviratne Deepthi

    2008-11-01

    Full Text Available Abstract Introduction We describe a case of giant cell arteritis in a woman who was treated with high-dose systemic corticosteroids and subsequently developed acute pancreatitis. Case presentation A 78-year-old Caucasian woman presented with four weeks of progressive headache and scalp tenderness. One day before ophthalmology assessment, she had experienced visual obscurations in both eyes. Her visual acuity was 6/9 in both eyes, with a right afferent pupillary defect and right swollen optic nerve. She was diagnosed as having temporal arteritis and was urgently treated with high-dose pulsed intravenous and oral corticosteroids. Her previous diet-controlled diabetes needed insulin and oral hyperglycaemic therapy to control erratic blood sugars. On day 8 of treatment with steroids, she became unwell with epigastric pain and vomiting. She was diagnosed with acute pancreatitis and was treated conservatively. Conclusion Acute pancreatitis, a potentially life-threatening condition, is a rare but important side effect of systemic corticosteroids.

  5. Primary Squamous Cell Carcinoma of the Breast during Pregnancy: A Case Report

    Directory of Open Access Journals (Sweden)

    John Adi Ashindoitiang

    2011-01-01

    Full Text Available Primary squamous cell carcinoma of the breast (SCCB is a very rare malignancy of the breast and is generally aggressive. It is even rarer during the gestational period. Only few cases have been reported during pregnancy and lactation (Rokutanda et al., 2000. SCCB seen within the gestational period tends to be very aggressive and has a larger size than other breast carcinomas. Pure SCCB is derived from the epidermis of the breast, nipple, or metaplasia on chronic inflammatory background (Bige et al., 2007, such as complicated breast cyst, dermoid cyst, or abscess. We report a case of SCCB in a 30-year-old primigravida that had an aggressive propensity and fatal outcome.

  6. Current perspective on the pathogenesis of central diabetes insipidus.

    Science.gov (United States)

    Ghirardello, Stefano; Malattia, Clara; Scagnelli, Paola; Maghnie, Mohamad

    2005-07-01

    Diabetes insipidus is a heterogeneous condition characterised by polyuria and polydipsia caused by a lack of secretion of vasopressin, its physiological suppression following excessive water intake, or kidney resistance to its action. The clinical and laboratory diagnosis is confirmed by standard tests, but recent advances in molecular biology and imaging techniques have shed new light on the pathophysiology of this disease. In many patients, central diabetes insipidus is caused by a germinoma or craniopharyngioma; Langerhans' cell histiocytosis and sarcoidosis of the central nervous system; local inflammatory, autoimmune or vascular diseases; trauma from surgery or accident; and, rarely, genetic defects in vasopressin biosynthesis inherited as autosomal dominant or X-linked recessive traits. Thirty to fifty percent of cases are considered idiopathic. Tumour-associated central diabetes insipidus is uncommon in children younger than 5 years old. Biopsy of enlarged pituitary stalk should be reserved for patients with hypothalamic-pituitary mass and progressive thickening of the pituitary stalk since spontaneous recovery may occur. Molecular biology in selected patients may identify those with apparently idiopathic diabetes insipidus carrying the vasopressin-neurophysin II gene mutation.

  7. Expansion of peripheral and intratumoral regulatory T-cells in hepatocellular carcinoma: A case-control study

    Directory of Open Access Journals (Sweden)

    Sapna Thakur

    2011-01-01

    Full Text Available Background: Hepatocellular carcinoma (HCC is notorious for poor prognosis with limited therapeutic options. A better understanding of the role of regulatory T-cells (Tregs in HCC is important for design of immunotherapy based clinical protocol. The objective of the present study was to evaluate the presence of Tregs in tumor microenvironment in patients with HCC compared to chronic hepatitis (CH. Materials and Methods: The frequency of CD4 + CD25 + Treg cells was evaluated from peripheral blood (PB of 28 patients of HCC and 30 controls including CH cases and healthy donors using flowcytometry. Intratumoral Treg were also analyzed in tissue samples from 17 HCC cases and 15 CH cases. In addition the expression of FOXP3 and CTLA-4 was also studied by RT-PCR. Results: Frequency of CD4 + CD25 + cells in the PBMCs of HCC cases was significantly higher than in HC (10.8 ± 7.64 vs 3.05 ± 1.30, P < 0.005 and CH patients (2.88 ± 1.92, P < 0.005. Also Treg population was significantly higher in HCC tumor microenvironment compared to CH biopsies (15.8 ± 5.32 vs 5.51 ± 3.40, P < 0.05. Expression of FOXP3 and CTLA-4 was also significantly higher in HCC patients ( P < 0.05 compared to CH group. Conclusions: We provide evidence of an increased population of Treg not only in the PB but also in tumor microenvironment of HCC patients, suggesting association of enhanced Treg activity with poor immune responses to tumor antigens. These findings may in future play a significant role in designing immunotherapeutic approaches in HCC.

  8. Case Reports of Adipose-derived Stem Cell Therapy for Nasal Skin Necrosis after Filler Injection

    Directory of Open Access Journals (Sweden)

    Ha Min Sung

    2012-01-01

    Full Text Available With the gradual increase of cases using fillers, cases of patients treated by non-medical professionals or inexperienced physicians resulting in complications are also increasing. We herein report 2 patients who experienced acute complications after receiving filler injections and were successfully treated with adipose-derived stem cell (ADSCs therapy. Case 1 was a 23-year-old female patient who received a filler (Restylane injection in her forehead, glabella, and nose by a non-medical professional. The day after her injection, inflammation was observed with a 3×3 cm skin necrosis. Case 2 was a 30-year-old woman who received a filler injection of hyaluronic acid gel (Juvederm on her nasal dorsum and tip at a private clinic. She developed erythema and swelling in the filler-injected area A solution containing ADSCs harvested from each patient's abdominal subcutaneous tissue was injected into the lesion at the subcutaneous and dermis levels. The wounds healed without additional treatment. With continuous follow-up, both patients experienced only fine linear scars 6 months postoperatively. By using adipose-derived stem cells, we successfully treated the acute complications of skin necrosis after the filler injection, resulting in much less scarring, and more satisfactory results were achieved not only in wound healing, but also in esthetics.

  9. A case of limbic encephalitis presenting as a paraneoplastic manifestation of limited stage small cell lung cancer: a case report

    Directory of Open Access Journals (Sweden)

    Butt Mohammad

    2010-12-01

    Full Text Available Abstract Introduction The differential diagnosis of altered mental status and behavioral change is very extensive. Paraneoplastic limbic encephalitis is a rare cause of cognitive impairment, which should be considered in the differential diagnosis. Case presentation A 64-year-old British Caucasian woman presented to our hospital with a 12-week history of confusion and short-term memory loss. She was hyponatremic with a serum sodium level of 128mmol/L. Moreover, there was evidence of left hilar prominence on the chest radiograph. A thoracic computed tomography scan showed left hilar opacity with confluent lymphadenopathy. A percutaneous biopsy confirmed a diagnosis of small cell lung cancer. There was no radiological evidence of brain metastasis on the computed tomography scan. In view of continued cognitive impairment, which was felt to be disproportionate to hyponatremia, a magnetic resonance imaging scan of the brain was undertaken. It showed hyperintense signals from both hippocampi, highly suggestive of limbic encephalitis presenting as a paraneoplastic manifestation of small cell lung cancer. She had a significant radiological and clinical response following chemotherapy and radiotherapy. Conclusion This case highlights the importance of considering paraneoplastic syndromes in patients with neurological symptoms in the context of lung malignancy. If initial investigations fail to reveal the cause of cognitive impairment in a patient with malignancy, magnetic resonance imaging may be invaluable in the diagnosis of limbic encephalitis. The clinical presentation, diagnostic techniques and management of paraneoplastic limbic encephalitis are discussed in this case report.

  10. Cell Phone Video Recording Feature as a Language Learning Tool: A Case Study

    Science.gov (United States)

    Gromik, Nicolas A.

    2012-01-01

    This paper reports on a case study conducted at a Japanese national university. Nine participants used the video recording feature on their cell phones to produce weekly video productions. The task required that participants produce one 30-second video on a teacher-selected topic. Observations revealed the process of video creation with a cell…

  11. A dural metastatic small cell carcinoma of the gallbladder as the first manifestation: a case report.

    Science.gov (United States)

    Tonomura, Shuichi; Kitaichi, Tomoko; Onishi, Rina; Kakehi, Yoshiaki; Shimizu, Hisao; Shimada, Keiji; Kanemasa, Kazuyuki; Fukusumi, Akio; Takahashi, Nobuyuki

    2018-03-16

    A dural metastasis is one of the essential differential diagnoses of meningioma. In general, carcinomas of the breast and lung in females and prostate in males have been the most commonly reported primary lesions of dural metastases. However, dural metastasis of gallbladder carcinoma is extremely rare. Here, we report a unique case of a dural matter metastasis of gallbladder carcinoma as the first manifestation, which was autopsy-defined as small cell carcinoma. A 78-year-old man came to our hospital complaining of left hemianopia. Brain computed tomography (CT) revealed a sizeable parasagittal dural-based extra-axial tumor. However, the findings for meningioma were atypical by magnetic resonance imaging, suggesting a meningioma mimic. A contrast-enhanced CT scan of the abdomen revealed a large gallbladder carcinoma. The patient opted for the best supportive care and died 2 months later. The post-mortem examination revealed small cell carcinoma in gallbladder carcinoma. Moreover, an immunologically similar carcinoma was detected in the dural metastasis. To the best of our knowledge, this is the first case of a dural metastasis of gallbladder small cell carcinoma. A systemic examination is essential for clinicians when atypical findings of meningioma are observed, suggesting a meningioma mimic. We present this rare case with a review of the literature.

  12. Acinic cell carcinoma of parotid gland metastasis to left cavernous sinus: a case report and review of literature

    Directory of Open Access Journals (Sweden)

    LIN Xiao-yan

    2013-12-01

    Full Text Available Objective To investigate the clinical manifestations and pathological features of parotid gland papillary acinic cell carcinoma metastasis to left cavernous sinus. Methods The clinical manifestations, pathological features and differential diagnosis were studied in one case of parotid papillary acinic cell carcinoma metastasis to left cavernous sinus. Related literatures were also reviewed. Results The patient was a 50-year-old female who presented paroxysmal dizziness for 5 months and blurred vision in her left eye for 10 months. The MRI examination showed left parasellar space-occupying mass in the cavernous sinus. In operation, the tumor was located in the superior wall of left cavernous sinus, soft and red-grey in color, with abundant blood supply. The histomorphological examination revealed the tumor cells were arranged in solid, acinar or papillary pattern. The tumor cells were large, with eosinophilic cytoplasm, round or oval nuclei and small nucleoli. Immunohistochemical staining found that the tumor cells expressed cytokeratin (CK, epithelial membrane antigen (EMA, vimentin (Vim and S-100 protein (S-100, and showed weak positive expression of glial fibrillary acidic protein (GFAP and focal positive expression of P53 protein. Ki-67 labeling index was about 5%-10% . The tumor cells were negative for neuroendocrine markers and pituitary hormone protein markers. This case was difficult to differentiate from other primary intracalvarium tumors, including papillary meningioma, papillary tumor of choroid plexus, papillary ependymoma, papillary glioneuronal tumors as well as chordoma. According to the medical history and the comparison of histomorphology and immunophenotyping between parotid gland tumor cells and left cavernous sinus tumor cells, the final diagnosis was metastatic papillary parotid acinar cell carcinoma of the left cavernous sinus. The patient was followed for 21 months and no recurrence was seen. Conclusion It is very rare and

  13. A Rare Case of a Renal Cell Carcinoma Confined to the Isthmus of a Horseshoe Kidney

    Directory of Open Access Journals (Sweden)

    Michael Kongnyuy

    2015-01-01

    Full Text Available Horseshoe kidney (HSK is the most common renal anomaly. Reports of the incidence of renal cell carcinoma (RCC in HSK are conflicting. Very few cases of isthmus-located RCC have been reported in the literature. We report a unique case of an isthmus-located RCC. Proper vascular and tumor imaging prior to surgery is key to successful tumor removal.

  14. Peripheral odontogenic keratocyst associated with nevoid basal cell carcinoma syndrome: a case report.

    Science.gov (United States)

    Sakamoto, Kei; Morita, Kei-Ichi; Shimada, Yasuyuki; Omura, Ken; Izumo, Toshiyuki; Yamaguchi, Akira

    2014-07-01

    Peripheral odontogenic keratocyst (POKC) is a rare gingival cyst showing histologic features identical to those of keratocystic odontogenic tumor. A rare case of POKC associated with nevoid basal cell carcinoma syndrome (NBCCS) is presented. A 24-year-old woman with NBCCS presented with a pigmented papule, 3 mm in size, involving the lingual gingiva of the right canine area of the mandible. Based on a clinical diagnosis of benign pigmentation, an excisional biopsy was performed, and a histopathologic diagnosis of POKC was rendered. The lining cells were positive for the proteins GLI2, BCL2, keratin 8, keratin 17, and mTOR. TP53 and Ber-EP4 were also weakly positive. Gene mutational analysis on a buccal swab sample revealed 2 missense mutations in the PTCH1 gene. This case is a distinctive example of a genuine soft tissue counterpart of keratocystic odontogenic tumor, in which an aberrant PTCH1-GLI pathway played a considerable role in the pathogenesis. Copyright © 2014 Elsevier Inc. All rights reserved.

  15. Metastatic Mantle Cell Lymphoma to the Pituitary Gland: Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Arthur Wang

    2016-01-01

    Full Text Available We present an unusual case of a metastatic mantle cell lymphoma (MCL to the pituitary gland. The patient had a known history of MCL for which she previously received chemotherapy. She presented with new-onset diplopia and confusion, and reported a history of progressive vision blurriness associated with headache, nausea, and vomiting. MRI of the brain showed an enhancing lesion within the sella turcica involving the cavernous sinuses bilaterally, extending into Meckel's cave on the left, and abutting the optic nerves bilaterally. Following surgical excision, histopathology revealed the tumor to be a MCL. Metastatic pituitary tumors are rare and have been estimated to make up 1% of tumors discovered in the sellar region. The two most common secondary metastatic lesions to the sella are breast and lung carcinoma followed by prostate, renal cell, and gastrointestinal carcinoma. Metastatic lymphoma to the pituitary gland is especially rare and is estimated to constitute 0.5% of all metastatic tumors to the sella turcica. To our knowledge, this is the first reported case of MCL metastasizing to the pituitary gland.

  16. A Rare Case of Gastric Variceal Hemorrhage Secondary to Infiltrative B-Cell Lymphoma

    Directory of Open Access Journals (Sweden)

    Adrienne Lenhart

    2016-10-01

    Full Text Available Portal hypertension commonly arises in the setting of advanced liver cirrhosis and is the consequence of increased resistance within the portal vasculature. Less commonly, left-sided noncirrhotic portal hypertension can develop in a patient secondary to isolated obstruction of the splenic vein. We present a rare case of left-sided portal hypertension and isolated gastric varices in a patient with large B-cell lymphoma, who was treated with splenic artery embolization. The patient is a 73-year-old male with no previous history of liver disease, who presented with coffee ground emesis and melena. On admission to hospital, he was found to have a hemoglobin level of 3.4 g/l. Emergent esophagogastroduodenoscopy showed isolated bleeding gastric varices (IGV1 by Sarin classification in the fundus and cardia with subsequent argon plasma coagulation injection. He was transferred to our tertiary center where work-up revealed normal liver function tests, and abdominal ultrasound showed patent hepatic/portal vasculature without cirrhosis. MRI demonstrated a large heterogeneously enhancing mass in the pancreatic tail, with invasion into the spleen and associated splenic vein thrombosis. Surgery consultation was obtained, but urgent splenectomy was not recommended. The patient instead underwent splenic artery embolization to prevent future bleeding from his known gastric varices. Pathology from a CT-guided biopsy was consistent with diffuse large B-cell lymphoma. PET imaging showed uptake in the splenic hilum/pancreatic tail region with no additional metastatic involvement. He was evaluated by the Hematology Department to initiate R-CHOP chemotherapy. During his outpatient follow-up, he reported no further episodes of melena or hematemesis. To the best of our knowledge, there have only been two published case reports of large B-cell lymphoma causing upper gastrointestinal bleeding from isolated gastric varices. These cases were treated with splenectomy or

  17. Primary multifocal osseous lymphoma in a child

    Energy Technology Data Exchange (ETDEWEB)

    Sato, Takashi S.P. [University of Iowa, Carver College of Medicine, Iowa City, IA (United States); Ferguson, Polly J. [University of Iowa, Department of Pediatrics, Iowa City, IA (United States); Khanna, Geetika [Washington University, Mallinckrodt Institute of Radiology, St Louis, MO (United States)

    2008-12-15

    We report a case of primary multifocal osseous lymphoma in a 6-year-old girl presenting with multifocal osteolytic lesions without systemic symptoms or identifiable non-osseous primary tumor. The differential diagnoses for such a presentation include histiocytosis X, chronic recurrent multifocal osteomyelitis, acute lymphoblastic leukemia, metastatic disease, and primary bone lymphoma. Although non-Hodgkin lymphoma is common in the pediatric population, its presentation as a primary bone tumor, especially with multifocal disease, is extremely rare and is frequently misdiagnosed. We hope that awareness of this entity will help radiologists achieve timely diagnosis and intervention. (orig.)

  18. Primary "cutaneous" T-cell anaplastic large cell lymphoma, CD30+, neutrophil-rich variant with subcutaneous panniculitic lesions, in a post-renal transplant patient: report of unusual case and literature review.

    Science.gov (United States)

    Salama, S

    2005-06-01

    Posttransplantation lymphoproliferative disorders (PTLD) presenting clinically in the skin are rare and usually of B-cell phenotype. Only 7 cases of cutaneous T-cell PTLD have been previously reported, mostly mycosis fungoides type, with no known cases of "cutaneous" presentation by CD30 (Ki-1) anaplastic large cell lymphoma (ALCL). The case reported is a 59-year-old male who developed multiple skin nodules on the right leg, 6 years following renal transplantation. Initial biopsy showed ALCL involving the dermis with a background rich in neutrophils. The neoplastic cells were of T-cell phenotype, strongly CD30 with typical staining, and BCL-2 positive, but P53 negative. No EBV was detected by IHC, ISH, or DNA analysis. One year later, he developed painful subcutaneous nodules with surrounding erythema, resembling deep pustules or panniculitis, which on biopsy showed preferential involvement of the subcutaneous fat and prominent component of neutrophils. Twenty-two months following diagnosis, he died of cardiac failure with terminal myocardial infarct. There was however no clinical evidence of systemic spread of the lymphoma.This report adds to the clinical and morphologic spectrum of these rare "cutaneous" lymphomas of T-cell lineage arising in the posttransplantation setting, and suggests that EBV does not play a role in their pathogenesis.

  19. Mastoid cells myiasis in a Saudi man: A case report | Al-Abidi | West ...

    African Journals Online (AJOL)

    We report here the case of myiasis of the mastoid cells in a 50-year old Saudi farmer. Eight larvae of suspected Calliphorid fly were extracted from his right mastoid at examination in the clinic. The larvae almost ate into his brain, using their powerful screw-shaped mouth parts. It is the first report of Calliphorid larvae affecting ...

  20. Post-transfusion purpura treated with plasma exchange by haemonetics cell separator. A case report

    DEFF Research Database (Denmark)

    Laursen, B; Morling, N; Rosenkvist, J

    1978-01-01

    A case of post-transfusion purpura in a 61-year-old, multiparous female with a platelet alloantibody (anti-Zwa) in her serum is reported. The patient was successfully treated with plasma exchange by means of a Haemonetics 30 cell separator and corticosteroids. Compared with other therapeutic...