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Sample records for extremely rare tumor

  1. Malignant Hidradenocarcinoma in the Lower Extremity: A Case Report of a Rare Tumor.

    Science.gov (United States)

    Kane, Brendan; Adler, Evan; Bhandari, Tarun; Rose, Michael; DiGuglielmo, Nicola; Sun, Xiu

    Malignant hidradenocarcinomas are rare soft tissue tumors of sweat gland origin. We present the case of a soft tissue, fungating tumor of 15 years' duration of the medial ankle in an 85-year-old male that exhibited malignant features clinically and radiographically. Subsequent punch biopsy revealed a diagnosis of malignant hidradenocarcinoma. Given the risk of recurrence and the poor radiation and chemotherapy options, the patient initially decided to leave the lesion untreated. However, he soon developed lower extremity cellulitis from the exposed lesion and decided to have the tumor excised, eliminating the source of the infection. In the present case study, we discuss the etiology, clinical and radiographic characteristics, and treatment options for this rare lesion. At the 18-month follow-up visit, he had had no recurrence of the lesion. Copyright © 2017 The American College of Foot and Ankle Surgeons. Published by Elsevier Inc. All rights reserved.

  2. Cryptococcoma mimicking a brain tumor in an immunocompetent patient: case report of an extremely rare presentation.

    Science.gov (United States)

    Paiva, Aline Lariessy Campos; Aguiar, Guilherme Brasileiro de; Lovato, Renan Maximilian; Zanetti, Arthus Vilar Deolindo; Panagopoulos, Alexandros Theodoros; Veiga, José Carlos Esteves

    2017-11-06

    Central nervous system (CNS) infectious diseases have high prevalence in developing countries and their proper diagnosis and treatment are very important for public health planning. Cryptococcus neoformans is a fungus that may cause several CNS manifestations, especially in immunocompromised patients. Cryptococcal meningitis is the most common type of involvement. Mass-effect lesions are uncommon: they are described as cryptococcomas and their prevalence is even lower among immunocompetent patients. The aim here was to report an extremely rare case of cryptococcoma causing a mass effect and mimicking a brain tumor in an immunocompetent patient. The literature on CNS cryptococcal infections was reviewed with emphasis on cryptococcomas. Clinical, surgical and radiological data on a female patient with this rare presentation of cryptococcoma mimicking a brain tumor are described. A 54-year-old female patient presented to the emergency department with a rapid-onset progressive history of confusion and completely dependency for basic activities. Neuroimaging showed a left occipital lesion and neurosurgical treatment was proposed. From histopathological evaluation, a diagnosis of cryptococcoma was established. She received clinical support with antifungals, but despite optimal clinical treatment, her condition evolved to death. Cryptococcal infections have several forms of presentation and, in immunocompetent patients, their manifestation may be even more different. Cryptococcoma is an extremely rare presentation in which proper surgical and clinical treatment should be instituted as quickly as possible, but even so, there is a high mortality rate.

  3. Teratoid Wilms′ tumor - A rare renal tumor

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    Biswanath Mukhopadhyay

    2011-01-01

    Full Text Available Teratoid Wilms′ tumor is an extremely rare renal tumor. We report a case of unilateral teratoid Wilms′ tumor in a 4-year-old girl. The patient was admitted with a right-sided abdominal mass. The mass was arising from the right kidney. Radical nephrectomy was done and the patient had an uneventful recovery. Histopathology report showed teratoid Wilms′ tumor.

  4. Glomus tumor of penis- A rare case

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    Madhuri S. Kate

    2013-01-01

    Full Text Available Glomus tumors are rare, painful, and benign soft tissue tumors of the skin arising from the glomus body, an arteriovenous thermoregulatory structure. This lesion is usually found on the extremities. Glomus tumor involving the external genital organs including penis is extremely rare. Until now, only few cases have been reported in the available literature. A 22 year old male patient presented with a painful nodule over the penis. The pathologic diagnosis was glomus tumor of the glans penis. We report herewith a case of glomus tumor of penis diagnosed on histopathologic examination. Glomus tumor, being a benign neoplasm, complete extirpation of the glomus tumor is the treatment of choice.

  5. 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

  6. Rare case of gastrointestinal stromal tumor of the anal canal

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    Madhu Kumar

    2013-01-01

    Full Text Available Gastrointestinal stromal tumor (GIST is a rare mesenchymal neoplasm of the gastrointestinal tract. GIST of anal canal is very rare representing only 3% of all anorectal mesenchymal tumors. We report an extremely rare case of GIST of the anal canal in 60-years-old man with history of irregular bowel habits with dark colored stool mixed with blood and constipation from 6 month. Diagnosis was made on the basis of histomorphological and immunohistochemical examination.

  7. Angiomyolipoma and Malignant PEComa: Discussion of Two Rare Adrenal Tumors

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    Douglas Kwazneski II

    2016-01-01

    Full Text Available Angiomyolipoma and PEComa are rare tumors descending from perivascular epithelial cells (PECs, with distinctive IHC, morphological, and ultrastructural features. The kidney is the most frequent site of origin, but not the only one; however, adrenal gland angiomyolipomas are extremely rare. We describe two cases being found in the adrenal glands. Given the paucity of literature on the subject, more information on this disease is necessary for diagnosis and treatment. Here, we describe two complete case reports, from presentation to treatment and follow-up, along with imaging and microscopic pathology samples, and provide a comprehensive review as to the history and current literature available regarding these extremely rare tumors.

  8. Rare, but challenging tumors: NET

    International Nuclear Information System (INIS)

    Ivanova, D.; Balev, B.

    2013-01-01

    Full text: Introduction: Gastroenteropancreatic Neuroendocrine Tumors (GEP - NET) are a heterogeneous group of tumors with different locations and many different clinical, histological, and imaging performance. In a part of them a secretion of various organic substances is present. The morbidity of GEP - NET in the EU is growing, and this leads to increase the attention to them. What you will learn: Imaging methods used for localization and staging of GEP - NET, characteristics of the study’s protocols; Classification of GEP - NET; Demonstration of typical and atypical imaging features of GEP - NET in patients registered at the NET Center at University Hospital ‘St. Marina’, Varna; Features of metastatic NET, The role of imaging in the evaluation of treatment response and follow-up of the patients. Discussion: The image semiotics analysis is based on 19 cases of GEP - NET registered NET Center at University Hospital ‘St. Marina’. The main imaging method is multidetector CT (MDCT), and magnetic resonance imaging (MRI ) has advantages in the evaluation of liver lesions and the local prevalence of anorectal tumors. In patients with advanced disease and liver lesions the assessment of skeletal involvement (MRI/ nuclear medical method) is mandatory. The majority of GEP - NET have not any specific imaging findings. Therefore it is extremely important proper planning and conducting of the study (MDCT and MR enterography; accurate assessment phase of scanning, positive and negative contrast). Conclusion: GEP - NET is a major diagnostic challenge due to the absence of typical imaging characteristics and often an overlap with those of the tumors of different origin can be observed. Therefore, a good knowledge of clinical and imaging changes occurring at different locations is needed. MDCT is the basis for the diagnosis, staging and follow-up of these neoplasms

  9. Papilliferous Keratoameloblastoma: An Extremely Rare Case Report

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    Neeta Mohanty

    2013-01-01

    Full Text Available Odontogenic tumors develop in the jaw bones from the odontogenic tissue-oral epithelium in tooth germ, enamel organ, dental papilla, reduced enamel epithelium, remnants of Hertwig’s root sheath or dental lamina, and so forth. Hence, a bewildering variety of tumors are encountered in the maxilla and mandible. Ameloblastoma is the second most common odontogenic neoplasm after odontomes, and it has numerous clinical and histologic variants. We report a very rare histologic variant: the papilliferous keratoameloblastoma which is the fifth reported case in the English literature.

  10. Bronchial carcinoid tumors: A rare malignant tumor

    African Journals Online (AJOL)

    2015-02-03

    Feb 3, 2015 ... Nigerian Journal of Clinical Practice • Sep-Oct 2015 • Vol 18 • Issue 5. Abstract. Bronchial carcinoid tumors (BCTs) are an uncommon group of lung tumors. They commonly affect the young adults and the middle aged, the same age group affected by other more common chronic lung conditions such as ...

  11. Neuroendocrine tumor of the inguinal node: A very rare presentation

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    Niharika Bisht

    2017-12-01

    Full Text Available Neuroendocrine tumors are a broad family of tumors arising most commonly in the gastrointestinal tract and the bronchus pulmonary tree. The other common sounds are the parathyroid, pituitary and adrenal gland. Inguinal node as a primary presentation of a neuroendocrine tumor is an extremely rare presentation. We present the case of a 43-year-old-male who presented with the complaints of an inguinal node swelling without any other symptoms and on further evaluation was diagnosed to have a non-metastatic neuroendocrine tumor of the inguinal node. He was treated with a combination of chemotherapy and surgery and is presently awaiting completion chemotherapy.

  12. Rare tumors of the rectum. Narrative review.

    Science.gov (United States)

    Errasti Alustiza, José; Espín Basany, Eloy; Reina Duarte, Angel

    2014-11-01

    Most rectal neoplasms are adenocarcinomas, but there is a small percentage of tumors which are of other histological cell lines such as neuroendocrine tumors, sarcomas, lymphomas and squamous cell carcinomas, which have special characteristics and different treatments. We have reviewed these rare tumors of the rectum from a clinical and surgical point of view. Copyright © 2013 AEC. Published by Elsevier Espana. All rights reserved.

  13. A Rare Case of Malignant Glomus Tumor of the Esophagus

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    Gurvinder Singh Bali

    2013-01-01

    Full Text Available Glomus tumors are rare neoplasms that usually occur on the hands in a subungual location, or sometimes in palms, wrists or soles of the feet. They are described as purple/pink tiny painful lesions with a triad of pain, local point tenderness, and cold hypersensitivity. They are almost always benign, but rare malignant variants have been reported. They have also been reported to be present at unusual locations, like the lung, stomach, or liver. Gastrointestinal glomus tumors are extremely rare tumors and very few cases have been reported in the literature. Most that have been reported were usually benign in nature. A rare esophageal glomangioma, mimicking a papilloma, was reported in 2006. We report a case of glomangiosarcoma (malignant glomus tumor in a 49-year-old female, who presented with symptoms of dysphagia including some spasm and hoarseness and subjective unintentional weight loss. On endoscopic exam, she was found to have a distal esophageal mass with malignant features. Radiologically, the mass had a size of about 8 cm on the CT scan without evidence of metastases. Pathology and immunostaining of the biopsy showed features resembling a malignant glomus tumor. She underwent an endoscopic and laparoscopic staging of the tumor along with ultrasound. Based on the laparoscopic findings, which were consistent with the preoperative diagnosis, she was scheduled for an esophagectomy. Histopathology and immunophenotypic features of the excised mass were consistent with a diagnosis of malignant glomus tumor.

  14. Extraosseous Ewing′s tumor of larynx: A rare presentation

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    Vinod Shinde

    2015-01-01

    Full Text Available Primitive neuroectodermal tumors (PNETs are a group of highly malignant tumors composed of small round cells of neuroectodermal origin that affect soft tissue and bone. PNET of the larynx is extremely rare. We report a case of a 41-year-old male who presented with the complaints of progressively increasing stridor of 3 months duration, which was diagnosed as a case of neuroectodermal tumor in the subglottis. Patient was subjected to microlaryngeal surgery and the tumor was excised. Postoperatively, patient was given three cycles of chemotherapy comprising of ifosfamide, etoposide, and mesna, along with granulocyte colony stimulating factor, with 21 days interval. After chemotherapy repeat computed tomography scan showed no evidence of the tumor and no lymphadenopathy. Patient is symptom free for 18 months following completion of treatment. He is under regular follow-up and is undergoing monthly serial endoscopic evaluation.

  15. Rare giant cell tumor involvement of the olecranon bone

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    Chen Yang

    2014-01-01

    Full Text Available Giant cell tumor (GCT of bone is a relatively common benign bone lesion and is usually located in long bones, but involvement of the olecranon is extremely rare. Here, we present a case of solitary GCT of bone in the olecranon that was confirmed by preoperative needle biopsy and postoperative histological examination. The treatment included intralesional curettage, allogeneic bone grafting, and plating. At 26 months follow-up, the patient had no local recurrence.

  16. Synovial Sarcoma-A Rare Tumor of the Larynx

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    Ghodrat Mohammadi

    2016-05-01

    Full Text Available Introduction: Malignant mesenchymal tumors of the larynx are rare. One type of malignant mesenchymal tumor is synovial sarcoma with unknown histogenesis, which occurs predominantly in the lower extremities of young adults. The head and neck region is a relatively rare location. There are few cases of malignant mesenchymal tumors with laryngeal localization in literature.  Case Report: In this report, a new case in a 23-year-old man, which was referred with increasing hoarseness for eight months, and dysphagia, odynophagia, and dyspnea since nearly one year ago, is reported. Indirect laryngoscopy revealed a laryngeal submucosal mass. The patient was operated and the histopathological diagnosis of synovial sarcoma was confirmed by IHC (Immunohistochemisry.  Conclusion:  Synovial sarcoma occurs predominantly in the lower extremities of young adults. Because very few cases of laryngeal synovial sarcoma are reported, every new case will bring some new information about diagnosis and therapy. It is of utmost importance to get to know new aspects and therapeutical modalities of this rare tumor.

  17. Malignant rhabdoid tumor of the tongue: A rare occurrence.

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    Hazarika, Munlima; Rahman, Tashnin; Sarma, Anupam; Krishnatreya, Manigreeva

    2014-05-01

    Malignant rhabdoid tumors (MRTs) are highly aggressive neoplasms that most commonly occur in the kidneys of young children. Malignant rhabdoid tumor of the tongue is an extremely rare entity and very few have been reported in the literature. The course of extra-renal MRT is short and its prognosis is very poor. A 19-year-old female presented with a progressive swelling and restricted mobility of the tongue for over 3 months duration. We present here a locally advanced case of MRT of the tongue, its diagnosis, management and review of the literature related to it.

  18. Ewing's sarcoma, a rare but dangerous tumor

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    Theophilus Maksha Dabkana

    2015-01-01

    Full Text Available Ewing's sarcoma or Ewing tumor is a rare primary bone tumor that affects mainly children and adolescents. It belongs to a group of cancers known collectively as Ewing sarcoma family tumors or Ewing family tumors. By the time, the patients present and diagnosis is made, the disease is usually advanced. We reviewed the case files of two patients managed in our hospital within one (2013. Fine-needle aspirations for cytology (FNAC and tissue biopsy were used for diagnosis in the two patients we had. The two patients, both males aged 20 years and 38 years presented late and FNAC and tissue biopsy revealed Ewing's sarcoma. They were referred for radio- and chemotherapy. However, due to poor socioeconomic status, they died of their primary disease. Unless diagnosed early, and in the absence of a multidisciplinary approach, Ewing's sarcoma is a fatal disease.

  19. A Rare Tumor of Nasal Cavity: Glomangiopericytoma

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    Aysegul Verim

    2014-01-01

    Full Text Available Glomangiopericytoma is a rare vascular neoplasm characterized by a pattern of prominent perivascular growth. A 72-year-old woman was admitted to our clinic complaining of nasal obstruction, frequent epistaxis, and facial pain. A reddish tumor filling the left nasal cavity was observed on endoscopy and treated with endoscopic excision. Microscopically, closely packed cells interspersed with numerous thin-walled, branching staghorn vessels were seen. Glomangiopericytoma is categorized as a borderline low malignancy tumor by WHO classification. Long-term follow-up with systemic examination is necessary due to high risk of recurrence.

  20. [Pott's puffy tumor: a rare complication of frontal sinusitis].

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    Aínsa Laguna, D; Pons Morales, S; Muñoz Tormo-Figueres, A; Vega Senra, M I; Otero Reigada, M C

    2014-05-01

    Pott's puffy tumor is a rare complication of frontal sinusitis characterized by swelling and edema in the brow due to a subperiosteal abscess associated with frontal osteomyelitis. Added complications are cellulitis by extension to the orbit and intracranial infection by posterior extension, with high risk of meningitis, intracranial abscess, and venous sinus thrombosis. Early diagnosis and aggressive medical or surgical treatment are essential for optimal recovery of affected patients. In the antibiotic age it is extremely rare, with very few cases described in the recent literature. A case is presented of a Pott inflammatory tumor in a 7 year-old boy, as a complication of acute pansinusitis who presented with front preseptal swelling and intracranial involvement with thrombosis of ophthalmic and superior orbital veins and frontal epidural abscess extending to the subarachnoid space. Copyright © 2013 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.

  1. Recurrence of Solid Pseudopapillary Tumor: A Rare Pancreatic Tumor

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    Chandra Punch

    2016-01-01

    Full Text Available Solid pseudopapillary tumor of the pancreas (SPTP is a rare disease of young females that does not usually recur after resection. Here we report a case of an elderly female with history of SPTP ten years ago who presented with anorexia and a palpable left lower quadrant abdominal mass. Imaging revealed metastatic disease and US-guided biopsy of the liver confirmed the diagnosis of SPTP. Due to her advanced age and comorbidities, she elected to undergo hospice care. The objective of this case report is to increase awareness of this tumor and its possibility of recurrence, necessitating further guidelines for follow-up.

  2. Bronchial carcinoid tumors: A rare malignant tumor | Orakwe ...

    African Journals Online (AJOL)

    There are many reports of this rare group of tumors in the Western and Asian regions. The only report around our sub‑region is a post mortem report of an atypical variant. We wish to report a case of the typical variant and increase our index of suspicion. A 25‑year‑old male presented with a 4 years history of cough and ...

  3. A Rare Cutaneous Adnexal Tumor: Malignant Proliferating Trichilemmal Tumor

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    Omer Alici

    2015-01-01

    Full Text Available Proliferating trichilemmal tumors (PTTs are neoplasms derived from the outer root sheath of the hair follicle. These tumors, which commonly affect the scalp of elderly women, rarely demonstrate malignant transformation. Although invasion of the tumors into neighboring tissues and being accompanied with anaplasia and necrosis are accepted as findings of malignancy, histological features may not always be sufficient to identify these tumors. The clinical behavior of the tumor may be incompatible with its histological characteristics. Squamous-cell carcinoma should certainly be considered in differential diagnosis because of its similarity in morphological appearance with PTT. Immunostaining for CD34, P53, and Ki-67 is a useful adjuvant diagnostic method that can be used in differential diagnosis aside from morphological findings. In this study, we aimed to present the case of a 52-year-old female patient with clinicopathological features. We reported a low-grade malignant proliferating trichilemmal tumor in this patient and detected no relapse or metastasis in a 24-month period of follow-up.

  4. Surgical treatment of tumor-induced osteomalacia: a retrospective review of 40 cases with extremity tumors.

    Science.gov (United States)

    Sun, Zhi-jian; Jin, Jin; Qiu, Gui-xing; Gao, Peng; Liu, Yong

    2015-02-26

    Tumor-induced osteomalacia (TIO) is a rare syndrome typically caused by mesenchymal tumors. It has been shown that complete tumor resection may be curative. However, to our knowledge, there has been no report of a large cohort to exam different surgical approaches. This study was aimed to assess outcomes of different surgical options of patients with tumor-induced osteomalacia at a single institution. Patients with extremity tumors treated in our hospital from January, 2004 to July, 2012 were identified. The minimum follow-up period was 12 months. Patient's demography, tumor location, preoperative preparation, type of surgeries were summarized, and clinical outcomes were recorded. Successful treatment was defined as significant symptom improvement, normal serum phosphorus and significant improvement or normalization of bone mineral density at the last follow-up. Differences between patients with soft tissue tumors and bone tumors were compared. There were 40 (24 male and 16 female) patients identified, with an average age of 44 years. The tumors were isolated in either soft tissue (25 patients) or bone (12 patients) and combined soft tissue and bone invasion was observed in 3 patients. For the primary surgery, tumor resection and tumor curettage were performed. After initial surgical treatment, six patients then received a second surgery. Four patients were found to have malignant tumors base on histopathology. With a minimum follow-up period of 12 months, 80% of patients (32/40) were treated successfully, including 50% of patients (2/4) with malignant tumors. Compared to patients with bone tumor, surgical results were better in patient with soft tissue tumor. Surgical treatment was an effective way for TIO. Other than tumor curettage surgery, tumor resection is the preferred options for these tumors.

  5. A very rare, petro-clival, neurothekeoma tumor. Case Report

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    Iacob G.

    2015-09-01

    Full Text Available Known as nerve sheath myxoma too, neurothekeoma are benign tumors, usually arise in the skin of the head, neck region and upper extremities, in young females. Cerebral neurothekeoma are very rare, a few cases were already described in the parasellar area, in the middle cranial and posterior fossa. We present a petro-clival neurotekoma. A 78-year-old male was admitted for two years left fronto-temporal headache completed in the last 6 months with left trigeminal V1 neuralgia, left facial hypoesthesia, diplopia, swallowing disorders for liquid foods, balance disorders. From his medical records we noticed: stage 2 chronic kidney disease, hypertension, prostate adenoma, dyslipidemia hypercholesterolemia. The MRI showed a macronodular petroclival mass in hiper T2, hipo T1, flair iso signal; normal cerebral angiography. The patient was operated on using a left retrosigmoid, retromastoidian approach. A 4/3/3 cm tumor, gray, encapsulated, soft consistency, partially cystic, less bleeding, attached to the dura, displacing the basilar artery and brain stem controlateral, encasing the trigeminal nerve. The tumor was totally removed with a good postop evolution. Six months follow up he had no more facial pain, but only persistant left facial hypoesthesia. Histologically the tumor had lobular appearance with spindle or stellate cells embedded in abundant myxoid background. The tumor cells were diffusely positive for S100, PGP9.5’, CD 34” positive in vessels, Ki67’positive in 5%. Cranial MRI performed one month after surgery did not show any residual tumor. Also known as nerve sheath myxomas, neurothekoma are rare benign tumors. For intracranial neurothekoma surgical indication is mandatory

  6. A rare case with synchronous gastric gastrointestinal stromal tumor, pancreatic neuroendocrine tumor, and uterine leiomyoma.

    Science.gov (United States)

    Arabadzhieva, Elena; Yonkov, Atanas; Bonev, Sasho; Bulanov, Dimitar; Taneva, Ivanka; Vlahova, Alexandrina; Dikov, Tihomir; Dimitrova, Violeta

    2016-11-15

    Although gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract, they comprise less than 1% of all gastrointestinal tumors. Neuroendocrine tumors (NET) of the gastro-enteropancreatic system are also rare, representing about 2% of all gastrointestinal neoplasms. Pancreatic localization of NET is extremely uncommon-these tumors are only 1-5% of all pancreatic cancers. We describe an unusual case with triple tumor localization-a gastric tumor, a formation in the pancreas, which involves the retroperitoneal space, and a uterine leiomyoma. The exact diagnosis was confirmed with immunohistochemical study after surgical treatment of the patient. Distal pancreatic resection, splenectomy, partial gastrectomy, omentectomy, and hysterectomy were performed. The histological examination proved an epithelioid type of gastric GIST. Immunostaining showed focal positive expression of c-kit and no mitotic figures per 50 HPF. Histology of the pancreatic and retroperitoneal formation proved a well-differentiated NET with origin from the islets of Langerhans. The immunohistochemical study demonstrated co-expression of chromogranin A and synaptophysin. This is the fourth case published so far of a patient with synchronous pancreatic NET and gastric GIST. The main objective of the study is to present a unique case because we have not found any reports for coexistence of the described three types of neoplasm, as in our patient, and we hope that it will be valuable in the future investigations about the genesis, diagnosis, and treatment of these types of tumors.

  7. Laws of small numbers extremes and rare events

    CERN Document Server

    Falk, Michael; Hüsler, Jürg

    2004-01-01

    Since the publication of the first edition of this seminar book in 1994, the theory and applications of extremes and rare events have enjoyed an enormous and still increasing interest. The intention of the book is to give a mathematically oriented development of the theory of rare events underlying various applications. This characteristic of the book was strengthened in the second edition by incorporating various new results on about 130 additional pages. Part II, which has been added in the second edition, discusses recent developments in multivariate extreme value theory. Particularly notable is a new spectral decomposition of multivariate distributions in univariate ones which makes multivariate questions more accessible in theory and practice. One of the most innovative and fruitful topics during the last decades was the introduction of generalized Pareto distributions in the univariate extreme value theory. Such a statistical modelling of extremes is now systematically developed in the multivariate fram...

  8. Laws of small numbers extremes and rare events

    CERN Document Server

    Falk, Michael; Reiss, Rolf-Dieter

    2011-01-01

    Since the publication of the first edition of this seminar book in 1994, the theory and applications of extremes and rare events have enjoyed an enormous and still increasing interest. The intention of the book is to give a mathematically oriented development of the theory of rare events underlying various applications. This characteristic of the book was strengthened in the second edition by incorporating various new results. In this third edition, the dramatic change of focus of extreme value theory has been taken into account: from concentrating on maxima of observations it has shifted to l

  9. A Rare Gestational Trophoblastic Disease: Placental Site Trophoblastic Tumor

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    Senem Yaman Tunç

    2016-12-01

    PSTT is a rare tumor. In contrast to other trophoblastic tumors, PSTT produces a small amount of ß-HCG and it is relatively insensitive to chemotherapy. Adjuvant chemotherapy is suggested to follow surgical treatment in the cases with metastasis.

  10. Carcinoma ex basal cell adenoma of the parotid gland: A report of an extremely rare case.

    Science.gov (United States)

    Kusafuka, Kimihide; Kawasaki, Takuya; Nakajima, Takashi; Sugino, Takashi

    2017-07-01

    Malignant non-basaloid tumors that arise from basal cell adenoma (BCA) are extremely rare. The patient was a 72-year-old Japanese male, who had noticed swelling of the left parotid region 21 years ago. A superficial lobectomy was performed. About 60% of the tumor was made up of cribriform and trabecular tissue composed of basaloid cells, which exhibited mild atypia and nuclear expression of β-catenin. This portion of the tumor was considered to be a BCA. In the other part of the tumor, the proliferation of large eosinophilic atypical cells, most of which formed intraductal structures, was observed. These tumor cells displayed cellular atypia, and some of them formed Roman bridge structures or contributed to intracapsular invasion. Immunohistochemically, these cells were positive for cytokeratin 7, gross cystic disease fluid proten-15 (GCDFP-15), androgen receptor (AR), and mammaglobin (MMG) and exhibited a high Ki-67 labeling index. So, this portion of the tumor was considered to be a salivary duct carcinoma (SDC). The tumor's final diagnosis was SDC ex BCA (intracapsular type), which is extremely rare. GCDFP-15, AR, MMG, and Ki-67 are useful immunohistochemical markers for diagnosing SDC ex BCA. © 2017 The Authors. Pathology International Published by Japanese Society of Pathology and John Wiley & Sons Australia, Ltd.

  11. Knee glomangioma: a rare location for a glomus tumor

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    Ricardo Gonçalves

    2014-12-01

    Full Text Available Glomus tumor is a rare, benign neoplasm rising from the glomus apparatus of the skin. It occurs most frequently on fingers and toes and accounts for 1.6% of all soft tissue tumors. Clinical diagnosis may prove difficult if the tumor occurs on an extra digital location. We report a case of a vascular-type glomus tumor (glomangioma found in an atypical location, namely the lateral aspect of the knee joint.

  12. A rare case of malignant triton tumor in the cerebellopontine angle

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    Gong Li

    2012-04-01

    Full Text Available Abstract Malignant triton tumor (MTT is defined as malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation. Intracranial MTT is extremely rare, and only four cases have been reported in the literature. Here, we report a case of MTT occurring in the cerebellopontine angle, and describe its histopathological characteristics, immunohistochemical features, and prognosis. Virtual slides The virtual slide(s for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1336227313684480

  13. A rare case of malignant triton tumor in the cerebellopontine angle.

    Science.gov (United States)

    Gong, Li; Liu, Xiao-Yan; Zhang, Wen-Dong; Han, Xiu-Juan; Yao, Li; Zhu, Shao-Jun; Lan, Miao; Li, Yan-Hong; Zhang, Wei

    2012-04-19

    Malignant triton tumor (MTT) is defined as malignant peripheral nerve sheath tumor with rhabdomyoblastic differentiation. Intracranial MTT is extremely rare, and only four cases have been reported in the literature. Here, we report a case of MTT occurring in the cerebellopontine angle, and describe its histopathological characteristics, immunohistochemical features, and prognosis. The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1336227313684480.

  14. Malignant mandibular tumors: two case reports of rare mandibular ...

    African Journals Online (AJOL)

    Arun Kumar Agnihotri

    2014-02-26

    Feb 26, 2014 ... present two cases of rare malignant mandibular tumors in a single institution. KEY WORDS: .... Spiculated osteoblastic periosteal reaction was noted with mild ... displacement of right 2nd premolar and 2nd molar teeth were ...

  15. Extreme Quantum Memory Advantage for Rare-Event Sampling

    Science.gov (United States)

    Aghamohammadi, Cina; Loomis, Samuel P.; Mahoney, John R.; Crutchfield, James P.

    2018-02-01

    We introduce a quantum algorithm for memory-efficient biased sampling of rare events generated by classical memoryful stochastic processes. Two efficiency metrics are used to compare quantum and classical resources for rare-event sampling. For a fixed stochastic process, the first is the classical-to-quantum ratio of required memory. We show for two example processes that there exists an infinite number of rare-event classes for which the memory ratio for sampling is larger than r , for any large real number r . Then, for a sequence of processes each labeled by an integer size N , we compare how the classical and quantum required memories scale with N . In this setting, since both memories can diverge as N →∞ , the efficiency metric tracks how fast they diverge. An extreme quantum memory advantage exists when the classical memory diverges in the limit N →∞ , but the quantum memory has a finite bound. We then show that finite-state Markov processes and spin chains exhibit memory advantage for sampling of almost all of their rare-event classes.

  16. Extreme Quantum Memory Advantage for Rare-Event Sampling

    Directory of Open Access Journals (Sweden)

    Cina Aghamohammadi

    2018-02-01

    Full Text Available We introduce a quantum algorithm for memory-efficient biased sampling of rare events generated by classical memoryful stochastic processes. Two efficiency metrics are used to compare quantum and classical resources for rare-event sampling. For a fixed stochastic process, the first is the classical-to-quantum ratio of required memory. We show for two example processes that there exists an infinite number of rare-event classes for which the memory ratio for sampling is larger than r, for any large real number r. Then, for a sequence of processes each labeled by an integer size N, we compare how the classical and quantum required memories scale with N. In this setting, since both memories can diverge as N→∞, the efficiency metric tracks how fast they diverge. An extreme quantum memory advantage exists when the classical memory diverges in the limit N→∞, but the quantum memory has a finite bound. We then show that finite-state Markov processes and spin chains exhibit memory advantage for sampling of almost all of their rare-event classes.

  17. Plexiform Neurofibroma: A Rare Tumor of Submandibular Salivary ...

    African Journals Online (AJOL)

    A provisional diagnosis of sialadenitis with sialolithiasis was made. Excised mass was reported histopathologically as plexiform neurofibroma of submandibular salivary gland. Plexiform neurofibroma of the salivary gland is a rare benign tumor often present in the parotid gland. It is very rare in submandibular salivary gland.

  18. A Rare Cause of Prepubertal Gynecomastia: Sertoli Cell Tumor

    Directory of Open Access Journals (Sweden)

    Fatma Dursun

    2015-01-01

    Full Text Available Prepubertal gynecomastia due to testis tumors is a very rare condition. Nearly 5% of the patients with testicular mass present with gynecomastia. Sertoli cell tumors are sporadic in 60% of the reported cases, while the remaining is a component of multiple neoplasia syndromes such as Peutz-Jeghers syndrome and Carney complex. We present a 4-year-old boy with gynecomastia due to Sertoli cell tumor with no evidence of Peutz-Jeghers syndrome or Carney complex.

  19. Krukenberg Tumor: A Rare Cause of Ovarian Torsion

    Directory of Open Access Journals (Sweden)

    Sameer Sandhu

    2012-01-01

    Full Text Available Ovarian torsion is the fifth most common gynecological surgical emergency. Ovarian torsion is usually associated with a cyst or a tumor, which is typically benign. The most common is mature cystic teratoma. We report the case of a 43-year-old woman who came to the Emergency Department with rare acute presentation of bilateral Krukenberg tumors, due to unilateral ovarian torsion. In this case report, we highlight the specific computed tomography (CT features of ovarian torsion and demonstrate the unique radiological findings on CT imaging. Metastasis to the ovary is not rare and 5 to 10% of all ovarian malignancies are metastatic. The stomach is the common primary site in most Krukenberg tumors (70%; an acute presentation of metastatic Krukenberg tumors with ovarian torsion is rare and not previously reported in radiology literature.

  20. An Extremely Rare Case of Advanced Metastatic Small Cell Neuroendocrine Carcinoma of Sinonasal Tract

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    Yu Yu Thar

    2016-01-01

    Full Text Available Small cell neuroendocrine carcinoma (SNEC is a rare form of malignancy. It mainly presents as bronchogenic neoplasm, and the extrapulmonary form accounts for only 0.1% to 0.4% of all cancers. These extrapulmonary tumors have been described most frequently in the urinary bladder, prostate, esophagus, stomach, colon and rectum, gall bladder, head and neck, cervix, and skin. Primary SNEC of the sinonasal tract is extremely rare with only less than 100 cases reported in the literature. Because of extreme rarity and aggressiveness of the tumor, the management for this entity varies considerably mandating multimodality approach. In this paper, we report a patient presented with left-sided facial swelling, and the histopathologic examination confirmed primary SNEC of left sinonasal tract. The tumor involved multiple paranasal sinuses with invasion into the left orbit and left infratemporal fossa and metastasized to cervical lymph nodes and bone. The patient encountered devastating outcome in spite of optimal medical management and treatment with palliative chemotherapy highlighting the necessity for further research of primary SNEC of head and neck.

  1. Solitary Fibrous Tumor of Retromolar Pad; a Rare Challenging Case

    Science.gov (United States)

    Lotfi, Ali; Mokhtari, Sepideh; Moshref, Mohammad; Shahla, Maryam; Atarbashi Moghadam, Saede

    2017-01-01

    Solitary fibrous tumor has a wide spectrum of histopathologic features and many tumors show similar microscopic features. This similarity poses diagnostic challenges to the pathologists and immunohistochemical analysis is required in many cases. Moreover, it is a rare entity in orofacial region which consequently would make its diagnosis more challenging in oral cavity. The knowledge of various microscopic patterns of this tumor contributes to a proper diagnosis and prevents unnecessary treatment. This study reports a case of solitary fibrous tumor in the retromolar pad area and discusses its various histological features and differential diagnoses. PMID:28620640

  2. Clear cell hidradenocarcinoma of the breast: a very rare breast skin tumor.

    Science.gov (United States)

    Mezzabotta, Maurizio; Declich, Paolo; Cardarelli, Mery; Bellone, Stefano; Pacilli, Paolo; Riggio, Eliana; Pallino, Antonio

    2012-01-01

    Hidradenocarcinoma is an uncommon malignant intradermal tumor of sweat gland origin with a predilection for the face and extremities. It is encountered equally in males and females, usually in the second half of life. These tumors tend to be locally aggressive. In our case, the tumor was located relatively superficially but without any apparent connection to the overlying skin. The typical disease course includes local and sometimes multiple recurrences, and some patients develop regional lymph node and distant metastases. These type of tumors in the parenchyma of the breast are extremely rare. We report a case of hidradenocarcinoma in a 77-year-old woman who presented with a palpable inflammatory nodule in the right breast.

  3. Rare primary malignant tumors of the liver

    International Nuclear Information System (INIS)

    Dahan, H.; Zoppardo, P.; Chagnon, S.; Vilgrain, V.; Blery, M.

    1991-01-01

    Angiosarcoma, epithelioid hemangio-endothelioma (EHE) and fibrolamellar carcinoma (FLC) are far less frequent malignant primary tumors of the liver than liver-cell carcinoma, and usually do not occur in a chronic liver disease. Their diagnosis is histological but a few radiological criteria are suggestive: in younger subjects, a solitary, hypervascularized mass containing calcifications and/or a central fibrous scar suggests an FLC; nodular lesions merging into patches, scattered about the periphery, containing calcified clusters and showing a low and late contrast enhancement after injections suggest an EHE; lastly, in case of occupational exposure, an heterogeneous, hypervascularized mass with a centripetal blush but containing central areas that are opacified early should suggest angiosarcoma. (4 figs) [fr

  4. Pancreatoblastoma, a Rare Childhood Tumor: A Case Report

    Directory of Open Access Journals (Sweden)

    Asuman ARGON

    2017-05-01

    Full Text Available Pancreatoblastoma, rarely encountered in the literature, is a malignant exocrine tumor seen in the pancreas. A 5-year-old boy suffering from abdominal pain was sent to our institute for further examination and treatment. Clinical examination was normal but for a palpable abdominal tumor mass. Abdominal Doppler ultrasonography showed a mass with well-defined margins within the body of the pancreas. Laboratory tests, including lactic dehydrogenase, alpha-fetoprotein and cancer antigen 125 were abnormal. The tumor invading the splenic vein and transverse colon was removed totally. We observed a hypercellular tumor in histopathological examination. The tumor had epithelial acinar cells and squamoid morules (corpuscles separated by stromal bands. Adjuvant chemotherapy was used after surgery. However, the patient died 14 months later. All data about pancreatoblastoma have to be collected in order to choose the treatment to elucidate the molecular pathogenesis of the tumor, to diagnose it early and to develop target-specific treatments.

  5. Lipofibromatous Hamartoma of the Plantar Nerve An Extremely Rare Localization.

    Science.gov (United States)

    Mert, Murat; Hacısalihoglu, Payam

    2018-03-01

    Lipofibromatous hamartoma (LFH) is a rare, benign, tumor-like soft-tissue lesion that affects the peripheral nerves and forms a palpable neurogenic mass. Lipofibromatous hamartoma is associated with pain and sensory and/or motor deficits in the area of innervation of the affected nerve. This report describes a rare case of LFH of the plantar nerve. A 48-year-old woman presented to our outpatient orthopedic clinic with pain and a burning sensation on her left foot. The patient had a history of Morton's neuroma and had undergone a tarsal tunnel operation 2 years earlier at another center. None of her symptoms was alleviated by two previous operations. Magnetic resonance imaging with contrast revealed tenosynovitis of the flexor hallucis longus tendon and signal changes at deep tissue planes of the foot at the levels of the second and third toes, on the dorsal site and subcutaneous soft-tissue planes, suggesting edema and Morton's neuroma. The lesion was excised under spinal anesthesia, and histopathologic examination of the specimen revealed a diagnosis of LFH. The patient was discharged without any symptoms and her foot was normal at 8-month outpatient follow-up, with no indications of postoperative complications and/or recurrence.

  6. Rare post-operative complications of large mediastinal tumor resection

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    Mohsen Mirmohammadsadeghi

    2015-03-01

    Full Text Available Background: There are some reports in the literature, which suggest that cardiac tamponade drainage may transiently affect systolic function and also cause acute respiratory distress syndrome (ARDS. We did not find any reports of acute ventricular failure and ARDS secondary to mediastinal tumor resection without tamponade. Case Report: Here we report a 48-year-old woman presenting with massive pericardial effusion without tamponade in whom tumor was resected through median sternotomy using cardiopulmonary bypass. ARDS and acute heart failure were two rare complications that happened at the end of the operation secondary to a sudden decompression of the heart from tumor pressure. Conclusion: ARDS and acute heart failure are two rare complications, which can happen after large mediastinal tumor resection.

  7. Extreme magnetoresistance in magnetic rare-earth monopnictides

    Science.gov (United States)

    Ye, Linda; Suzuki, Takehito; Wicker, Christina R.; Checkelsky, Joseph G.

    2018-02-01

    The acute sensitivity of the electrical resistance of certain systems to magnetic fields known as extreme magnetoresistance (XMR) has recently been explored in a new materials context with topological semimetals. Exemplified by WTe2 and rare-earth monopnictide La(Sb,Bi), these systems tend to be nonmagnetic, nearly compensated semimetals and represent a platform for large magnetoresistance driven by intrinsic electronic structure. Here we explore electronic transport in magnetic members of the latter family of semimetals and find that XMR is strongly modulated by magnetic order. In particular, CeSb exhibits XMR in excess of 1.6 ×106% at fields of 9 T whereas the magnetoresistance itself is nonmonotonic across the various magnetic phases and shows a transition from negative magnetoresistance to XMR with fields above magnetic ordering temperature TN. The magnitude of the XMR is larger than in other rare-earth monopnictides including the nonmagnetic members and follows a nonsaturating power law to fields above 30 T. We show that the overall response can be understood as the modulation of conductivity by the Ce orbital state and for intermediate temperatures can be characterized by an effective medium model. Comparison to the orbitally quenched compound GdBi supports the correlation of XMR with the onset of magnetic ordering and compensation and highlights the unique combination of orbital inversion and type-I magnetic ordering in CeSb in determining its large response. These findings suggest a paradigm for magneto-orbital control of XMR and are relevant to the understanding of rare-earth-based correlated topological materials.

  8. Malignant bone tumors of the pelvis and of the extremities

    International Nuclear Information System (INIS)

    Gullotta, U.; Reiser, M.; Feuerbach, S.; Biehl, T.; Technische Univ. Muenchen

    1981-01-01

    Bone tumors of the extremities are usually diagnosed by conventional radiography. A good angiogram may render information not only about intra- and extraosseous extension of the tumor, but often also about the biological dignity. CT is usually not necessary, especially since it is sometimes difficult to define the extraosseous borders of these extremity tumors with this method. In bone tumors of the pelvis, however, neither conventional radiography nor angiography render reliable information about the extent of the tumor, which CT is very well able to do. Therefore CT is primarily indicated for evaluation of bone tumors in this region. Angiography is done only for preoperative evaluation of the vascular architecture or for potential therapeutic embolisation. (orig.) [de

  9. Primary intraspinal extradural primitive neuroectodermal tumor: A rare case.

    Science.gov (United States)

    Rege, Shrikant V; Tadghare, Jitendra; Patil, Harshad; Narayan, Sharadendu

    2016-01-01

    Primitive neuroectodermal tumors (PNETs) are aggressive childhood malignancies and are difficult to treat. Primary intraspinal PNETs are rare. These patients have poor prognosis with short survival time even after surgery and chemoradiation. As there are no standard guidelines exist for the management of these tumors, a multidisciplinary approach has been employed with varying success. According to the review of literature, only few cases of primary intraspinal extradural PNETs have been reported. Herein, author has described a case of intraspinal, extradural PNET.

  10. Krukenberg tumor in a young woman: A rare presentation

    Directory of Open Access Journals (Sweden)

    Deepa Hatwal

    2014-01-01

    Full Text Available Krukenberg tumors mostly occur after 40 years. Metastatic ovarian tumors in young age are very rare and reported to be 2% of all the cases. Thirty percent of all ovarian neoplasms occurring during childhood and adolescence are malignant. A 25-year-old woman, parity- 2, presented with abdominal distension, pain in abdomen and amenorrhea. On examination, 18 weeks lump was palpable, firm to hard in consistency, non-tender and mobile. On ultrasonography bilateral ovarian tumors were reported, without any peritoneal free fluid. Total abdominal hysterectomy with bilateral salpingo-oophorectomy was performed. Microscopic examination revealed signet ring cells with glandular differentiation, diffusely invading the ovarian parenchyma. Tumor cells exhibited strong, diffuse immunopositivity for CEA with focal strong immunopositivity for CK7 and CK20 and immunonegativity for SATB2. Diagnosis of Krukenberg tumor was made. Endoscopic biopsy confirmed the diagnosis of adenocarcinoma stomach. This case is reported because of its rarity in younger age group.

  11. Inflammatory angiomyolipoma of the liver: a rare hepatic tumor

    Directory of Open Access Journals (Sweden)

    Liu Yang

    2012-09-01

    Full Text Available Abstract Angiomyolipoma (AML is a rare mesenchymal neoplasm of the tumor, composed of a varying heterogeneous mixture of three tissue components: blood vessels, smooth muscle and adipose cells. Hepatic AML may demonstrate a marked histological diversity. We herein present one case of hepatic AML exhibiting prominent inflammatory cells in the background, which happened in a 61-year-old Chinese female patient, without signs of tuberous sclerosis. Histologically, the striking feature was the infiltration of numerous inflammatory cells in the background, including small lymphocytes, plasma cells, and eosnophils. The tumor cells were spindled and histiocytoid in shape, with slightly eosinophilic cytoplasm, and arranged along the vessels or scattered among the inflammatory background. Sinusoid structure was obviously seen in the tumor. Mature adipocytes and thick-walled blood vessels were focally observed at the boundaries between the tumor and surrounding liver tissues. The tumor cells were positive immunostaining for HMB-45, Melan-A, and smooth muscle actin. The inflammatory AML should be distinguished from other tumors with inflammatory background such as inflammatory myofibroblastic tumor and follicular dendritic cell tumor and deserves wider recognition for its occurrence as a primary hepatic tumor. Virtual slides The virtual slide(s for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1828633072762370

  12. Syringomatous carcinoma: Case report of a rare tumor entity | El ...

    African Journals Online (AJOL)

    Syringomatous carcinoma is an extremely invasive tumor, locally destructive and slowly growing adnexal tumour, derived from eccrine sweat glands. It is often mistaken, both clinically and microscopically, for other benign and malignant entities. The tumour recurrence is high due to extensive perineural invasion, but

  13. Networking for ovarian rare tumors: a significant breakthrough improving disease management.

    Science.gov (United States)

    Chiannilkulchai, N; Pautier, P; Genestie, C; Bats, A S; Vacher-Lavenu, M C; Devouassoux-Shisheboran, M; Treilleux, I; Floquet, A; Croce, S; Ferron, G; Mery, E; Pomel, C; Penault-Llorca, F; Lefeuvre-Plesse, C; Henno, S; Leblanc, E; Lemaire, A S; Averous, G; Kurtz, J E; Ray-Coquard, I

    2017-06-01

    Rare ovarian tumors represent >20% of all ovarian cancers. Given the rarity of these tumors, natural history, prognostic factors are not clearly identified. The extreme variability of patients (age, histological subtypes, stage) induces multiple and complex therapeutic strategies. Since 2011, a national network with a dedicated system for referral, up to 22 regional and three national reference centers (RC) has been supported by the French National Cancer Institute (INCa). The network aims to prospectively monitor the management of rare ovarian tumors and provide an equal access to medical expertise and innovative treatments to all French patients through a dedicated website, www.ovaire-rare.org. Over a 5-year activity, 4612 patients have been included. Patients' inclusions increased from 553 in 2011 to 1202 in 2015. Expert pathology review and patients' files discussion in dedicated multidisciplinary tumor boards increased from 166 cases in 2011 (25%) to 538 (45%) in 2015. Pathology review consistently modified the medical strategy in 5-9% every year. The rate of patients' files discussed in RC similarly increased from 294 (53%) to 789 (66%). An increasing number (357 in 5 years) of gynecologic (non-ovarian) rare tumors were also registered by physicians seeking for pathological or medical advice from expert tumor boards. Such a nation-wide organization for rare gynecological tumors has invaluable benefits, not only for patients, but also for epidemiological, clinical and biological research. © The Author 2017. Published by Oxford University Press on behalf of the European Society for Medical Oncology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

  14. Primitive neuroectodermal tumor presenting as a presacral mass: A rare case report with review of literature

    Directory of Open Access Journals (Sweden)

    Pradnya S Bhadarge

    2017-01-01

    Full Text Available Primitive neuroectodermal tumors (PNETs are a group of highly malignant small round cell tumor (SRCT of neuroectodermal origin. They exhibit a great diversity in their clinical manifestations and pathologic similarities with other SRCTs. PNET commonly occurs in the central nervous system, head and neck region, paravertebral region, pelvis, and lower extremities. PNET presenting as a presacral mass is very rare. We present a case of 65-year-old female patient presented with a mass in the abdomen. Exploratory laparotomy with excision of mass was carried out. Histopathology revealed the diagnosis of PNET. The rarity of PNET at presacral region prompted the description of this case.

  15. Orbital Chondroma: A rare mesenchymal tumor of orbit

    Directory of Open Access Journals (Sweden)

    Ruchi S Kabra

    2015-01-01

    Full Text Available While relatively common in the skeletal system, cartilaginous tumors are rarely seen originating from the orbit. Here, we report a rare case of an orbital chondroma. A 27-year-old male patient presented with a painless hard mass in the superonasal quadrant (SNQ of left orbit since 3 months. On examination, best-corrected visual acuity of both eyes was 20/20, with normal anterior and posterior segment with full movements of eyeballs and normal intraocular pressure. Computerized tomography scan revealed well defined soft tissue density lesion in SNQ of left orbit. Patient was operated for anteromedial orbitotomy under general anesthesia. Mass was excised intact and sent for histopathological examination (HPE. HPE report showed lobular aggregates of benign cartilaginous cells with mild atypia suggesting of benign cartilaginous tumor - chondroma. Very few cases of orbital chondroma have been reported in literature so far.

  16. Lymphoepithelial carcinoma: a case report of a rare tumor of the larynx.

    Science.gov (United States)

    Hammas, Nawal; Benmansour, Najib; El Alami El Amine, Mohamed Nour-Dine; Chbani, Laila; El Fatemi, Hind

    2017-01-01

    Lymphoepithelial carcinoma is a tumor mostly diagnosed in the nasopharynx, but it has also been described in a variety of nonnasopharyngeal sites. It is extremely rare in the larynx and should be distinguished from squamous cell carcinoma. Therefore, it must be known by clinicians, pathologists and oncologists. In this case report, we discuss its etiopathogeny, its epidemiological, clinical, pathological and therapeutic aspects, and its outcome. An 81-year-old Morrocan man, smoker for 40 years, presented with a 1 year history of dysphonia, dyspnea and dysphagia. Laryngoscopy showed a mass occupying supraglottic, glottic and subglottic levels of the larynx. Cervico-thoracic computed tomography scan showed a laryngeal wall thickening with cervical lymphadenopathy. Laryngeal biopsy was performed. Microscopic analysis and immunohistochemistry confirmed the diagnosis of laryngeal lymphoepithelial carcinoma. Immunostaining for LMP1 was negative. Laryngeal lymphoepithelial carcinoma is an extremely rare and an aggressive tumor. It is rarely associated with the EBV. It must be regarded as a distinct entity. Radiotherapy is advisable as the unique therapy for local tumor. A correct diagnosis and a close collaboration between the pathologist and clinicians is mandatory for an optimal treatment strategy.

  17. Nivolumab and Ipilimumab in Treating Patients With Rare Tumors

    Science.gov (United States)

    2018-05-14

    ; Pseudomyxoma Peritonei; Rare Disorder; Scrotal Squamous Cell Carcinoma; Seminal Vesicle Adenocarcinoma; Seminoma; Serous Cystadenocarcinoma; Small Intestinal Adenocarcinoma; Small Intestinal Squamous Cell Carcinoma; Spindle Cell Neoplasm; Squamous Cell Carcinoma of the Penis; Teratoma With Malignant Transformation; Testicular Non-Seminomatous Germ Cell Tumor; Thyroid Gland Carcinoma; Tracheal Carcinoma; Transitional Cell Carcinoma; Undifferentiated Gastric Carcinoma; Ureter Adenocarcinoma; Ureter Squamous Cell Carcinoma; Urethral Adenocarcinoma; Urethral Squamous Cell Carcinoma; Vaginal Adenocarcinoma; Vaginal Squamous Cell Carcinoma, Not Otherwise Specified; Vulvar Carcinoma

  18. A rare case of retroperitoneal malignant triton tumor invading renal vein and small intestine

    Directory of Open Access Journals (Sweden)

    Mijović Žaklina

    2013-01-01

    Full Text Available Introduction. Malignant Triton tumor is a very rare malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation. Most of those tumors occur in patients with von Recklinghausen’s disease or as a late complication of irradiation and commonly seen in the head, neck, extremities and trunk. Case report. We reported retroperitoneal malignant Triton tumor in a 57-year-old female patient. Skin lesions were not present, and there was no family history of neurofibromatosis or previous irradiation. The presented case is one of a few recorded in the specialized literature that occurs in the retroperitoneal space in sporadic form. In this case, tumor consisted of a multilobular mass was in close relation with the abdominal aorta and inferior vena cava and involved the renal vein with gross invasion of the small intestine. The patient underwent total resection of the tumor and left nefrectomy was performed. The small intestine 10 cm in length was also resected and end-to-end anastomosis was conducted. The postoperative course was uneventful and the patient was discharged from the hospital ten days after the surgery. Conclusion. Diagnostically, it is crucial to recognize this uncommon histological variant because malignant Triton tumor has a worse prognosis than classic malignant peripheral nerve sheath tumor does. The use of the immunohistochemistry is essential in making the correct diagnosis. Only appropriate pathological evaluation supported by immunostaining with S-100 protein and desmin confirmed the diagnosis. Aggressive surgical management treatment improves the prognosis of such cases with adjuvant radiotherapy.

  19. Giant cell tumor of soft tissues of low malignant potential: A rare diagnosis on fine needle aspiration cytology

    Directory of Open Access Journals (Sweden)

    Maithili M Kulkarni

    2016-01-01

    Full Text Available Primary giant cell tumors of soft tissues (GCT-ST are extremely rare soft tissue tumors, located in both superficial and deep soft tissues. They resemble osseous giant cell tumors morphologically and immunohistochemically. The tumor exhibits strong positive immunoreactivity for cluster of differentiation 68 (CD68 within multinucleated osteoclast-like giant cells and focal staining of mononuclear cells. Case reports describing the cytohistological features of this entity are very few. We report a case of GCT-ST of low malignant potential diagnosed on fine needle aspiration (FNA and confirmed on histological and immunohistochemical studies.

  20. Malignant phyllodes tumor of the breast metastasizing to the vulva: {sup 18}F FDG PET CT Demonstrating rare metastasis from a rare tumor

    Energy Technology Data Exchange (ETDEWEB)

    Khangembam, Bang Kim Chand Ra; Sharma, Punit; Singla, Su Has; Singhal, Abinav; Dhull, Varun Singh; Bal, Chand Rasek Har; Kumar, Rakesh [All India Institute of Medical Sciences, New Delhi (India)

    2012-09-15

    Phyllodes tumors are extremely rare fibroepithelial neoplasms accounting for 0.3 to 0.5% of all female breast tumors with an incidence of 2.1 per 1 million women. They are classified histologically into benign, borderline and malignant varieties. The majority of them are benign, with only 25% being malignant. Surgery remains the mainstay of treatment. One characteristic is that although the malignant variety tends to metastasize and recur, the benign form has also been found to behave in a similar manner. Benign phyllodes tumor has a 21% risk of local recurrence, while that of the malignant variety ranges from 20 to 32%. In patients with malignant phyllodes tumor, the rate of distant metastases ranges from 25 to 40%. The most frequent sites of distant metastasis is uncommon as this tumor spreads by hematogeneous route. Other sites for distant metastasis have been reported sporadically, including the duodenum, pancreas, brain, nasal cavity, forearm, parotid, skin, oral cavity, skeletal muscle, mandible and maxilla. We present a rare case of recurrent malignant phyllodes tumor with metastasis to the vulva, which has not been reported in the literature to the best of our knowledge. A 49 year old female who had undergone lumpectomy and locoregional radiotherapy 1 year previously for malignant phyllodes tumor of the right breast presented with difficulty in breathing and cervical lymphadenopathy. Chest X ray showed multiple pulmonary nodules suggestive of metastasis. She was referred for restaging with 18F fluorodeoxyglucose (FDG)positron emission tomography computed tomography (PET CT)FDG PET CT. Maximum intensity projection (MIP)PET images revealed multiple FDG avid enlarged cervical lymph nodes, bilateral pulmonary nodules along with left pleural effusion and extensive bone marrow metastases. The interesting finding was an intensely FDG avid (SUV{sup max}-21.4)subcutaneous soft tissue density lesion (measuring 2.0x2.2x2.0cm)in the vulva, which was later proved to be

  1. Hiccup: An Extremely Rare Presentation of Thyrotoxicosis of Graves’ Disease

    Directory of Open Access Journals (Sweden)

    Irshad Parray

    2011-03-01

    Full Text Available Persistent hiccup is a rare but potentially severe condition that can be symptomatic of a variety of diseases or idiopathic. Most episodes last only a few minutes and are self-limited, but hiccup can get persistent and become a real problem for physician and patient alike. The center of hiccup may be activated by a great variety of stimuli travelling along different nerve pathways and bring different effecter responses. We report a case of persistent hiccup as a presentation of impending thyroid storm of Graves’ disease. Though the condition is rare, clinicians should remain alert to the possibility of this diagnosis.

  2. Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Kidney: Two Unusual Presentations of a Rare Tumor

    Directory of Open Access Journals (Sweden)

    E. C. Castro

    2012-01-01

    Full Text Available Only few cases of primary renal Ewing's sarcoma have been reported in the literature to date. We present here two cases of renal ES/PNET with an uncanny presentation. The first case was discovered after the patient presented clinically with irradiating flank pain, mimicking the pain related with kidney stones. The second case had clinical presentation of pulmonary thromboembolism after the patient was involved in an automobilist accident. The tumors were mainly composed of small blue cells which by immunohistochemical were positive for neural markers, and FISH revealed the translocation 22q12 for the EWSR1 gene. The diagnosis of renal primitive neuroectodermal tumor/EWING tumor is very rare and usually involves several different diagnostic techniques. The differential diagnosis is usually broad with frequent overlapping features between the entities. The cases presented in this paper illustrated the difficulties with which routine anatomical pathologist is faced when dealing with rare renal poorly differentiated neoplasm in adults.

  3. A rare ovarian tumor, leydig stromal cell tumor, presenting with virilization: a case report

    Directory of Open Access Journals (Sweden)

    Soheila Aminimoghaddam

    2012-11-01

    Full Text Available  Abstract Leydig stromal cell tumor is a rare ovarian tumor that belongs to the group of sex-cord stromal tumors. They produce testosterone leading to hyperandrogenism. We present a 41yr old woman with symptoms of virilization and a mass of right adenex via ultra Sonography, and a rise of total and free serum testosterone. An ovarian source of androgen was suspected and a surgery performed. A diagnosis of leydig-stromal cell tumor was confirmed. Our report is a reminder that although idiopathic hirsutism and other benign androgen excess disorder like Polycystic Ovarian Syndrome (PCOs are common, ovarian mass should be considered in differential diagnosis. 

  4. An extremely rare neoplasm, histiocytic sarcoma: A report of two cases with an aggressive clinical course

    Directory of Open Access Journals (Sweden)

    Erkan Kayikcioglu

    2017-07-01

    Conclusions: HS is an extremely rare malignant neoplasm of the monocytic/macrophage lineage, with no standardized chemotherapy regimen for multisystemic disease. Metastatic patients have a more aggressive clinical course than those with unifocal disease.

  5. Computer tomography for rare soft tissue tumours of the extremities

    International Nuclear Information System (INIS)

    Boettger, E.; Semerak, M.; Stoltze, D.; Rossak, K.

    1979-01-01

    Five patients with undiagnosed soft tissue masses in the extremities were examined and in two a pathological diagnosis could be made. One was an extensive, invasive fibroma (desmoid) 22 cm long which could be followed from the thigh almost into the pelvis. It was sharply demarkated form the surrounding muscles and of higher density. The second case was a 12 cm long cavernous haemangioma in the semi-membranosus muscle. This was originally hypo-dense, but showed marked increase in its density after the administration of contrast. (orig.) [de

  6. Soft tissue chondroma: a rare tumor presenting as a cutaneous nodule

    Directory of Open Access Journals (Sweden)

    Dibakar Podder

    2015-04-01

    Full Text Available Soft tissue chondroma (STC, also known as extraskeletal chondroma or chondroma of soft parts is a benign cartilaginous tumor which arise de novo from soft tissue. Also, it is an extremely rare entity predominantly involving extremities, especially fingers. A 26 year old male presented with 3 year history of swelling in left index finger. On local examination a hard 2 × 2 cm swelling was seen over the volar aspect of left 2nd proximal phalanx. Swelling was mobile on contraction of tendons. X-ray showed a soft tissue shadow on volar aspect of left second proximal phalanx. Histopathology showed a well encapsulated, hypo cellular nodule composed of benign chondrocytes surrounded by hyaline chondroid matrix. Nuclear pleomorphism, mitosis or necrosis was not seen. Based on radiological and histopathological findings a diagnosis of STC was made. STC should be considered in patients with slow growing, soft tissue masses.

  7. A rare tumor of trachea: Inflammatory myofibroblastic tumor diagnosis and endoscopic treatment

    Directory of Open Access Journals (Sweden)

    Mehmet Akif Özgül

    2014-01-01

    Full Text Available Inflammatory myofibroblastic tumors (IMTs are rare childhood neoplasms, with benign clinical course. Although etiology of IMTs are not clear, recent studies have reported that IMT is a true neoplasm rather than a reactive or inflammatory lesion. IMTs are rarely seen in adults and tracheal involvement is also rare both in adults and also in children. We describe a 16-year old female patient who was misdiagnosed and treated as asthma in another center for a few months and presented with acute respiratory distress due to upper airway obstruction. Computerized tomography (CT of the chest and rigid bronchoscopy revealed a mass lesion that was nearly totally obliterating tracheal lumen. Bronchoscopic resection was performed under general anesthesia and the final pathological diagnosis was tracheal IMT.

  8. Sporadic Endolymphatic Sac Tumor-A Very Rare Cause of Hearing Loss, Tinnitus, and Dizziness

    DEFF Research Database (Denmark)

    Schnack, Didde Trærup; Kiss, Katalin; Hansen, Søren

    2017-01-01

    Sporadic endolymphatic sac tumor is a very rare neoplasm. It is low malignant, locally destructive and expansive, but non-metastasizing. The tumor is very rare in the sporadic form, but more often associated with Von Hippel-Lindau disease. A 65-year old man with left sided tinnitus and hearing loss......-operative freeze-microscopy showed inflammation tissue, whereas subsequent microscopy showed papillary-cystic endolymphatic sac tumor. Endolymphatic sac tumor is a rare neoplasm. The tumor may present with asymmetrically sensory neural hearing loss with or without tinnitus, dizziness and facial nerve paresis...

  9. A rare disease in an atypical location - Kimura's Disease of the upper extremity

    Energy Technology Data Exchange (ETDEWEB)

    Lam, Alan Cheuk Si; Lau, Vince Wing Hang [Queen Mary Hospital, Department of Radiology, Hong Kong (China); Au Yeung, Rex Kwok Him [University of Hong Kong, Department of Pathology, Li Ka Shing Faculty of Medicine, Hong Kong (China)

    2015-12-15

    Kimura's disease is a rare chronic inflammatory disorder predominantly affecting young Asian male patients, occurring mainly in the head and neck regions. Kimura's disease of the upper extremity is extremely rare, and previous case reports in the literature show similar imaging characteristics with consistent location at the medial epitrochlear region, predominantly with unilateral involvement. We present the first reported case of Kimura's disease affecting the anterolateral aspect of the upper arm, sparing the medial epitrochlear region, illustrating that with typical MR appearance and serology, the involvement of this rare disease in an atypical location still warrants consideration of this diagnosis. There was also bilateral asymmetrical involvement in our patient, suggesting the possibility of a propensity for Kimura's disease affecting the upper extremities to have bilateral involvement, which may necessitate imaging of the clinically asymptomatic contralateral limb in these patients for early lesion identification and treatment. (orig.)

  10. A rare disease in an atypical location - Kimura's Disease of the upper extremity

    International Nuclear Information System (INIS)

    Lam, Alan Cheuk Si; Lau, Vince Wing Hang; Au Yeung, Rex Kwok Him

    2015-01-01

    Kimura's disease is a rare chronic inflammatory disorder predominantly affecting young Asian male patients, occurring mainly in the head and neck regions. Kimura's disease of the upper extremity is extremely rare, and previous case reports in the literature show similar imaging characteristics with consistent location at the medial epitrochlear region, predominantly with unilateral involvement. We present the first reported case of Kimura's disease affecting the anterolateral aspect of the upper arm, sparing the medial epitrochlear region, illustrating that with typical MR appearance and serology, the involvement of this rare disease in an atypical location still warrants consideration of this diagnosis. There was also bilateral asymmetrical involvement in our patient, suggesting the possibility of a propensity for Kimura's disease affecting the upper extremities to have bilateral involvement, which may necessitate imaging of the clinically asymptomatic contralateral limb in these patients for early lesion identification and treatment. (orig.)

  11. SERTOLI-LEYDIG CELL TUMOR; A RARE CASE IN A POSTMENOPAUSAL PATIENT – CASE REPORT

    Directory of Open Access Journals (Sweden)

    Petra Krajnc

    2018-02-01

    Full Text Available Background. Sertoli-Leydig cell tumors belong to the group of sex cord stromal tumors of the ovary. They account for less than 0.5 % of all ovarian tumors and occur primarily in young women between 20 and 30 years of age. This type of tumors can secrete androgens, causing virilisation, and are extremely rarely presented in postmenopausis. Methods. A 73-year old multiparous woman was presented to our institution with complaints of abdominal distention and abdominal pain in her lower abdomen. On physical examination, she had a large, fixed palpable abdominal mass, approximately 20 cm in diameter, arising from the pelvis. The laboratoric tests revealed an elevated level of CA125 of 221.3 U/ml of serum. The ultrasound showed a complex cystic and solid pelvic tumor. There was no sign of ascites. Her hormonal status was within normal range and she also showed no signs of virilisation. On laparotomy a complex left ovarian mass, measuring 30 × 27 × 15 cm was found and sent to frozen section. The result of frozen section was a malignant tumor of unknown origin, therefore a radical surgical procedure was performed. The histopathological examination established the diagnosis of a malignant Sertoli-Leydig cell tumor of the left ovary, of intermediate differentiation. Other removed tissue was free of malignant cells. The early postoperative course was uneventful and the patient was released from hospital 10 days after surgery. However, she returned to our institution 16 days after surgery due to a proximal thrombosis of v. saphena magna. The patient was treated with low-molecularweight heparin and later warfarin for 6 weeks post operation. 16 months after the operation she was symptomatically treated for severe microcytic anemia. She showed no signs of a relapse. 27 months after primary surgery she was operated for the second time due to acute bowel obstruction. She had large masses of necrotic tumor removed from abdomen and transversostomia was performed

  12. RARE CASE OF DESMOID TUMOR OF URINARY BLADDER

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    Chalapathy

    2015-08-01

    Full Text Available Desmoid tumor is a benign soft tissue tumor which belongs to a family of myofibroblastic fibromatoses. Occasionally, desmoid tumors have an unusual site of occurrence . We describe a case of incisional hernia in postmenopausal women with an intra operative incidental finding of a desmoid tumor from anterior wall of urinary bladder for which a wide excision was performed

  13. Using Extreme Phenotype Sampling to Identify the Rare Causal Variants of Quantitative Traits in Association Studies

    OpenAIRE

    Li, Dalin; Lewinger, Juan Pablo; Gauderman, William J.; Murcray, Cassandra Elizabeth; Conti, David

    2011-01-01

    Variants identified in recent genome-wide association studies based on the common-disease common-variant hypothesis are far from fully explaining the hereditability of complex traits. Rare variants may, in part, explain some of the missing hereditability. Here, we explored the advantage of the extreme phenotype sampling in rare-variant analysis and refined this design framework for future large-scale association studies on quantitative traits. We first proposed a power calculation approach fo...

  14. CARCINOID TUMOR OF THE DUODENUM: a rare tumor at an unusual site. Case series from a single institution

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    Jaques WAISBERG

    2013-03-01

    Full Text Available Context Duodenal carcinoids are extremely rare, and their characteristics and biological behavior have not been fully elucidated. Objective To analyze the clinicopathological characteristics of patients with resected duodenal carcinoids. Methods Twenty patients (12 females and 8 males were investigated. Their average age was 66.4 ± 5.8 years old (43 to 88 years old. The data corresponding to the clinical picture, diagnosis, treatment, and prognosis of patients with duodenal carcinoid tumors subjected to resection over a period of 18 years (1993-2011 were analyzed. Results The most common symptoms were dyspepsia (50% and epigastric pain (45% followed by weight loss (10% and vomiting (5%. Carcinoid syndrome was not observed in any patient. The lesion was located on the first part of the duodenum in 15 (75% patients, the second part in 4 (20% patients, and the third part in 1 (5% patient. The diagnosis of a carcinoid tumor was established through an endoscopic excision biopsy in 19 (95% patients and an histopathological examination of the surgical specimen in 1 (5% patient. The average tumor size was 1.1 cm ± 0.4 cm (0.3 cm to 6.0 cm. Nineteen (95% patients were initially treated by endoscopic resection of the duodenal lesion. One patient (5%, whose tumor was on the third part of the duodenum underwent a duodenectomy of the third and fourth duodenal parts and duodenojejunal anastomosis. The duodenal carcinoid resection margin was involved in four (20% patients. Four (20% patients were subjected to a partial gastrectomy to fully remove the lesion. The tumor was restricted to the submucosal layer in 16 (80% cases, and it penetrated into the muscular layer in 4 (20% cases. All patients exhibited positive chromogranin A, neuron-specific enolase, and/or synaptophysin immunostaining. The average duration of the follow-up period was 39.6 months (3 to 96 months. Twelve (60% of the 20 cases in this series are alive without any evidence of active

  15. A rare tumoral combination, synchronous lung adenocarcinoma and mantle cell lymphoma of the pleura

    Directory of Open Access Journals (Sweden)

    Foroulis Christophoros N

    2008-12-01

    Full Text Available Abstract Background Coexistence of adenocarcinoma and mantle cell lymphoma in the same or different anatomical sites is extremely rare. We present a case of incidental discovery of primary lung adenocarcinoma and mantle cell lymphoma involving the pleura, during an axillary thoracotomy performed for a benign condition. Case presentation A 73-year old male underwent bullectomy and apical pleurectomy for persistent pneumothorax. A bulla of the lung apex was resected en bloc with a scar-like lesion of the lung, which was located in proximity with the bulla origin, by a wide wedge resection. Histologic examination of the stripped-off parietal pleura and of the bullectomy specimen revealed the synchronous occurrence of two distinct neoplasms, a lymphoma infiltrating the pleura and a primary, early lung adenocarcinoma. Immunohistochemical and fluorescence in situ hybridization assays were performed. The morphologic, immunophenotypic and genetic findings supported the diagnosis of primary lung adenocarcinoma (papillary subtype coexisting with a non-Hodgkin, B-cell lineage, mantle cell lymphoma involving both, visceral and parietal pleura and without mediastinal lymph node involvement. The neoplastic lymphoid cells showed the characteristic immunophenotype of mantle cell lymphoma and the translocation t(11;14. The patient received 6 cycles of chemotherapy, while pulmonary function tests precluded further pulmonary parenchyma resection (lobectomy for his adenocarcinoma. The patient is alive and without clinical and radiological findings of local recurrence or distant relapse from both tumors 14 months later. Conclusion This is the first reported case of a rare tumoral combination involving simultaneously lung and pleura, emphasizing at the incidental discovery of the two coexisting neoplasms during a procedure performed for a benign condition. Any tissue specimen resected during operations performed for non-tumoral conditions should be routinely sent for

  16. Epithelioid hemangioendothelioma: an overview and update on a rare vascular tumor

    Directory of Open Access Journals (Sweden)

    Angela Sardaro

    2014-10-01

    Full Text Available Epithelioid hemangioendothelioma is a rare vascular tumor, described for the first time in 1975 by Dail and Liebow as an aggressive bronchoalveolar cell carcinoma. The etiology is still a dilemma. Studies about suggestive hypothesis are ongoing. Most of the times it affects lung, liver and bones, although this kind of tumor may involve the head and neck area, breast, lymph nodes, mediastinum, brain and meninges, the spine, skin, abdomen and many other sites. Because of its heterogeneous presentation, as it represents less than 1% of all the vascular tumors, it is often misdiagnosed and not suitably treated, leading to a poor prognosis in some cases. Over 50-76% of the patients are asymptomatic. A small number of them complains respiratory symptoms. Bone metastases might cause pathological fractures or spine compression, if they arise in vertebrae. Imaging is necessary to determine morphological data, the involvement of surrounding tissues, and potentially the cleavage plan. It is important to recognize the expression of vascular markers (Fli-1 and CD31 are endothelial-specific markers, and the microscopic evidence of vascular differentiation to make a correct diagnosis, as many pulmonary diseases show multiple nodular lesions. Because of its rarity, there is no standard for treatment. We focused on radiotherapy as a good therapeutic option: despite the poor prognosis, evidence is in favor of radiotherapy which offers local pain control with good tolerance and better quality of life at least at a one-year follow-up in most of cases. Further studies are needed to establish the standard radiation dose to be used for locoregional control of such a complex and extremely rare disease.

  17. Gastric Glomus Tumor: A Rare Cause of Upper Gastrointestinal Bleeding

    Directory of Open Access Journals (Sweden)

    Yoshinori Handa

    2015-01-01

    Full Text Available A 24-year-old woman was referred to our department because of melena. These symptoms combined with severe anemia prompted us to perform an emergency upper endoscopy, which showed bleeding from an ulcerated 30 mm submucosal tumor in the gastric antrum. A computed tomography scan revealed a homogeneously enhanced mass, and endoscopic ultrasonography identified a well-demarcated mass in the third and fourth layers of the gastric wall. Because analysis of the possible medical causes remained inconclusive and the risk of rebleeding, laparoscopy-assisted gastric wedge resection was performed after administration of 10 units of red cell concentrate. Histological and immunohistological analysis revealed the tumor to be a gastric glomus tumor. Gastric submucosal tumors remain challenging to diagnose preoperatively as they show a variety of radiologic and clinicopathologic features and are associated with the risk of bleeding upon biopsy, as is indicated in the guidelines for gastric submucosal tumors. Gastric glomus tumors characteristically present with exsanguinating gastrointestinal hemorrhaging that often requires blood transfusion. Additionally, gastric submucosal tumors typically occur in elderly patients; however, this case involved a young patient who was 24 years old. Here, we describe this case in order to identify features that may aid in early differentiation of gastric submucosal tumors.

  18. Simulation of the 23 July 2012 Extreme Space Weather Event: What if This Extremely Rare CME Was Earth Directed?

    Science.gov (United States)

    Ngwira, Chigomezyo M.; Pulkkinen, Antti; Mays, M. Leila; Kuznetsova, Maria M.; Galvin, A. B.; Simunac, Kristin; Baker, Daniel N.; Li, Xinlin; Zheng, Yihua; Glocer, Alex

    2013-01-01

    Extreme space weather events are known to cause adverse impacts on critical modern day technological infrastructure such as high-voltage electric power transmission grids. On 23 July 2012, NASA's Solar Terrestrial Relations Observatory-Ahead (STEREO-A) spacecraft observed in situ an extremely fast coronal mass ejection (CME) that traveled 0.96 astronomical units (approx. 1 AU) in about 19 h. Here we use the SpaceWeather Modeling Framework (SWMF) to perform a simulation of this rare CME.We consider STEREO-A in situ observations to represent the upstream L1 solar wind boundary conditions. The goal of this study is to examine what would have happened if this Rare-type CME was Earth-bound. Global SWMF-generated ground geomagnetic field perturbations are used to compute the simulated induced geoelectric field at specific ground-based active INTERMAGNET magnetometer sites. Simulation results show that while modeled global SYM-H index, a high-resolution equivalent of the Dst index, was comparable to previously observed severe geomagnetic storms such as the Halloween 2003 storm, the 23 July CME would have produced some of the largest geomagnetically induced electric fields, making it very geoeffective. These results have important practical applications for risk management of electrical power grids.

  19. Rare incidence of tumor lysis syndrome in metastatic prostate cancer following treatment with docetaxel.

    Science.gov (United States)

    Bhardwaj, Sharonlin; Varma, Seema

    2018-03-01

    Tumor lysis syndrome is a serious and sometimes lethal complication of cancer treatment that is comprised of a set of metabolic disturbances along with clinical manifestations. Initiating chemotherapy in bulky, rapidly proliferating tumors causes rapid cell turnover that in turn releases metabolites into circulation that give rise to metabolic derangements that can be dangerous. This syndrome is usually seen in high-grade hematological malignancies. Less commonly, tumor lysis syndrome can present in solid tumors and even rarely in genitourinary tumors. In this report, the authors describe a specific case of tumor lysis syndrome in a patient with metastatic prostate cancer following treatment with docetaxel.

  20. 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...

  1. 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.

  2. Primary histiocytic sarcoma of the uterine cervix: an extremely rare entity

    Directory of Open Access Journals (Sweden)

    Binesh Fariba

    2017-03-01

    Full Text Available Objective. Histiocytic sarcoma (HS is an extraordinary rare tumor and it has an offensive clinical course. HS of the uterine cervix is a far uncommon tumor with just a few cases described so far. Here we presented a case of primary HS of the uterine cervix in a 62-year-old female initially misdiagnosed as large cell non-keratinizing squamous cell carcinoma. To the best of our knowledge, this is the first reported case of HS of the uterine cervix from Iran.

  3. Rare adrenal gland incidentaloma: an unusual Ewing's sarcoma family of tumor presentation and literature review.

    Science.gov (United States)

    Guo, Hui; Chen, Shuaiqi; Liu, Shukun; Wang, Kaixuan; Liu, Erpeng; Li, Faping; Hou, Yuchuan

    2017-04-04

    Members of the Ewing's sarcoma family of tumor (ESFT) are malignant neoplasms and rarely observed in the adrenal gland. We report an extremely exceptional case of ESFT rising from the adrenal gland in a 57-year-old Chinese man. The patient was hospitalized with abdominal swelling for 2 months. Computed tomography (CT) scan revealed a nearly-circular mass measuring about 8.1 × 10.6 cm in the right adrenal region. The patient underwent right adrenal resection. Histopathologic examination found the tumor was composed of small round blue cells forming typical Homer-Wright rosettes in focal area. The immunohistochemical analysis confirmed the case to be ESFT, which was positive for membranous CD99 and nuclear FLI-1. The patient was scheduled for four courses of large doses of chemotherapy and died for cancer metastasis one year later after surgery. Histopathological evidence of Homer-Wright rosettes and immunohistochemical markers positivity, such as CD99 and FLI-1, are valuable factors for ESFT diagnosis, although cytogenetic analysis is considered as the gold standard. Complete surgery is the treatment of choice for ESFT and adjuvant radiotherapy and combination chemotherapy can significantly improve the survival rate of postoperative patients.

  4. Bronchoscopic management of a rare benign endobronchial tumor

    Directory of Open Access Journals (Sweden)

    K. Madan

    2012-09-01

    Full Text Available Benign endobronchial tumors are uncommon. Bronchoscopic removal is the preferred modality of treatment although surgery may be required in some cases. Rigid bronchoscopy is usually recommended in the management of these tumors. However, flexible bronchoscopy is also used in many centers. We present a case of endobronchial lipoma, where an unusual complication during flexible bronchoscopic resection using snare forceps necessitated urgent rigid bronchoscopy. This case highlights the importance of rigid bronchoscopy in the management of endobronchial tumors. We believe that with a large benign endobronchial tumor in tracheal or main-stem bronchus, physicians should initially employ rigid bronchoscopy, switching to flexible if more peripheral treatment is required. Resumo: Os tumores endobrônquicos benignos são raros. A remoção broncoscópica é a modalidade de tratamento preferida, embora a cirurgia possa ser necessária em alguns casos. A broncoscopia rígida é geralmente considerada a modalidade preferencial na abordagem destes tumores. No entanto, a broncoscopia flexível também é utilizada em muitos centros. Apresentamos um caso de lipoma endobrônquico, onde uma complicação invulgar durante a ressecção broncoscópica flexível utilizando pinças de laço necessitou de uma urgente broncoscopia rígida. Neste caso, destaca-se a importância da broncoscopia rígida na abordagem dos tumores endobrônquicos. Acreditamos que com um tumor endobrônquico benigno de grandes dimensões da traqueia ou do brônquio principal, os médicos devem inicialmente utilizar a broncoscopia rígida, alternando para a flexível, se for necessário um tratamento mais periférico. Keywords: Bronchoscopy, Lipoma, Endobronchial tumor, Electrocautery, Interventional pulmonology, Palavras-chave: Broncoscopia, Lipoma, Tumor endobrônquico, Electrocautério, Pneumologia interventiva

  5. Relationship between accumulation of rare earth element in tumor and ionic radius

    Energy Technology Data Exchange (ETDEWEB)

    Kataoka, N [Kanazawa Univ. (Japan). School of Medicine

    1975-02-01

    The accumulation of rare earth elements in the different organs of Ehrlich's tumor-bearing mice at 48 hours after intraperitoneal administration was measured by a Ge(Li) semiconductor detector. Accumulation of all the rare earth elements was the highest in the pancreas. Accumulation of /sup 152/Eu in the different organs of Ehrlich's tumor-bearing mice was very high. The accumulation of rare earth elements in Ehrlich's tumor was lower than the accumulation of /sup 67/Ga and /sup 46/Sc. The tumor-organ concentration ratio of rare earth elements was remarkably lower than the accumulation of /sup 67/Ga and /sup 46/Sc. However, the accumulation of /sup 152/Eu in Ehrlich's tumor was somewhat higher than that of /sup 67/Ga. The relationship between the accumulation and the carrier content was examined. The lower the carrier content was, the higher was the accumulation in different organs. However, the carrier effect of rare earth on the uptake in different organs elements was slight. The author postulated that the elements in which the ionic radius is similar to that of Mg(0.62 A) or Ca(0.99 A) are abundant in the tumor cell membrane, and they might pass through the tumor cell membrane much more easily than would the other elements. However, the result was negative.

  6. Melanotic neuroectodermal tumor of infancy: A rare case report

    Directory of Open Access Journals (Sweden)

    E Rajendra Reddy

    2013-01-01

    Full Text Available Melanotic neuroectodermal tumor of infancy (MNTI is a relatively uncommon osteolytic-pigmented neoplasm that primarily affects the jaws of infants. The early onset and its rapid disfiguring spread necessitate early diagnosis. A 4-month-old male child reported with the complaint of swelling in the right back tooth region of the upper jaw, which rapidly increased in size causing disfigurement of the face. Radiographic examination showed a diffuse osteolytic radiolucent lesion in the right maxilla and displacement and dysmorphic changes in the developing primary tooth buds. Wide surgical excision was performed under general anesthesia. Histopathological report revealed characteristic large pigmented epitheloid cells (melanocyte like cells. The biphasic tumor cell population arranged in a background of fibrous connective tissue stroma is suggestive of MNTI involving the cancellous bone. Early diagnosis and management of such aggressive tumors precludes significant morbidity of the patient.

  7. Calcifying epithelial odontogenic tumor, a rare presentation in children: Two case reports

    Directory of Open Access Journals (Sweden)

    Susant Mohanty

    2014-01-01

    Full Text Available Calcifying epithelial odontogenic tumor (CEOT is a rare and benign odontogenic neoplasm that affects the jaws. It is certainly an atypical instance to find this tumor in children. Here, we present two case reports of CEOT presenting in mandible of a 12- and 13-year-old female child, respectively. CEOT have been reported to show features of malignant transformation also.

  8. Gastrointestinal stromal tumor of Meckel's diverticulum: a rare cause of intestinal volvulus.

    Science.gov (United States)

    Cengız, Fevzi; Sun, Mehmet Ali; Esen, Özgür Sipahi; Erkan, Nazif

    2012-08-01

    Meckel's diverticulum is the most common congenital abnormality of the gastrointestinal tract. Most cases are asymptomatic; however, when symptomatic, it is often misdiagnosed at presentation. Common complications presenting in adults include bleeding, obstruction, diverticulitis, and perforation. Tumors within a Meckel's diverticulum are rare. Herein, we present a gastrointestinal stromal tumor arising from the Meckel's diverticulum that led to intestinal obstruction by volvulus.

  9. An Unusual Site of Adenomatoid Odontogenic Tumor Presenting as Periapical (Radicular Cyst: A Rare Case Report

    Directory of Open Access Journals (Sweden)

    C Anand Kumar

    2010-01-01

    Here we are presenting a rare case report of an unusual site of extrafollicular adenomatoid odontogenic tumor in the mandible w.r.t 32, 33, 34 and 35 mimicking periapical disease clinical and radiographically. However, diagnosis of adenomatoid odontogenic tumor should be considered when the clinician is presented with a corticated radiolucency in the anterior lower jaw, especially in teens and young adults.

  10. Rare kidney tumor provides insight on metabolic changes

    Science.gov (United States)

    Researchers in The Cancer Genome Atlas (TCGA) Network have uncovered a number of new findings about the biology and development of a rare form of kidney cancer. They found that the disease – chromophobe renal cell carcinoma – stems in part from alteratio

  11. 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...

  12. Pediatric hepatic rhabdoid tumor: A rare cause of abdominal mass in children

    Directory of Open Access Journals (Sweden)

    Nicole Kapral, MD

    2018-06-01

    Full Text Available Pediatric hepatic rhabdoid tumors are rare tumors of the liver, with few cases reported in the literature. These aggressive tumors can be difficult to differentiate from hepatoblastomas on imaging alone, and surgical biopsy combined with special immunohistochemical stains can assist in differentiating these 2 tumor types. We present a case of hepatic rhabdoid tumor in a 7-month-old female infant, which was originally thought to be a hepatoblastoma; however, using BAF47 staining for INI-1 we were able to diagnose a rhabdoid tumor and affect the patient's medical oncologic therapy. Earlier detection and a better understanding of the imaging features of hepatic rhabdoid tumor may aid in improved patient management and treatment planning. Keywords: Rhabdoid tumor, INI-1, Hepatoblastoma, Pediatric, Rhabdomyosarcoma

  13. Myxoid Neurothekeoma: A Rare Soft Tissue Tumor of Hand in a ...

    African Journals Online (AJOL)

    Nerve sheath myxoma (neurothekeoma) is an uncommon benign soft tissue tumor of the peripheral nerves with fairly distinctive histological features. It is commonly located on the upper extremities or the head and the neck.[1] Histologic variants of neurothekeoma include myxoid, cellular, and mixed tumors. A recent ...

  14. A genome-wide study reveals rare CNVs exclusive to extreme phenotypes of Alzheimer disease.

    Science.gov (United States)

    Rovelet-Lecrux, Anne; Legallic, Solenn; Wallon, David; Flaman, Jean-Michel; Martinaud, Olivier; Bombois, Stéphanie; Rollin-Sillaire, Adeline; Michon, Agnès; Le Ber, Isabelle; Pariente, Jérémie; Puel, Michèle; Paquet, Claire; Croisile, Bernard; Thomas-Antérion, Catherine; Vercelletto, Martine; Lévy, Richard; Frébourg, Thierry; Hannequin, Didier; Campion, Dominique

    2012-06-01

    Studying rare extreme forms of Alzheimer disease (AD) may prove to be a useful strategy in identifying new genes involved in monogenic determinism of AD. Amyloid precursor protein (APP), PSEN1, and PSEN2 mutations account for only 85% of autosomal dominant early-onset AD (ADEOAD) families. We hypothesised that rare copy number variants (CNVs) could be involved in ADEOAD families without mutations in known genes, as well as in rare sporadic young-onset AD cases. Using high-resolution array comparative genomic hybridisation, we assessed the presence of rare CNVs in 21 unrelated ADEOAD cases, having no alteration on known genes, and 12 sporadic AD cases, with an age of onset younger than 55 years. The analysis revealed the presence of 7 singleton CNVs (4 in ADEOAD and 3 in sporadic cases) absent in 1078 controls and 912 late-onset AD cases. Strikingly, 4 out of 7 rearrangements target genes (KLK6, SLC30A3, MEOX2, and FPR2) encoding proteins that are tightly related to amyloid-β peptide metabolism or signalling. Although these variants are individually rare and restricted to particular subgroups of patients, these findings support the causal role, in human pathology, of a set of genes coding for molecules suspected for a long time to modify Aβ metabolism or signalling, and for which animal or cellular models have already been developed.

  15. Solid pseudopapillary tumor of pancreas with sickle cell trait: A rare case report

    Directory of Open Access Journals (Sweden)

    Harish S Permi

    2013-01-01

    Full Text Available Solid pseudopapillary tumor of pancreas is a rare pancreatic neoplasm affecting young women, has low malignant potential and amenable for surgical excision with good long-term survival. Sickle cell trait is benign condition, which involves one normal beta-globin chain and one HbS chain. Although it is a benign condition, individuals are prone to have rare complications that may predispose to death under certain circumstances. We report a rare coexistence of solid pseudopapillary tumor of pancreas with sickle cell trait in an 18-year-old female who underwent distal pancreatectomy with splenectomy. Histopathological examination and haemoglobin electrophoresis confirmed the diagnosis.

  16. Higher order magnetic modulation structures in rare earth metal, alloys and compounds under extreme conditions

    International Nuclear Information System (INIS)

    Kawano, S.

    2003-01-01

    Magnetic materials consisting of rare earth ions form modulation structures such as a helical or sinusoidal structure caused by the oscillating magnetic interaction between rare earth ions due to RKKY magnetic interaction. These modulation structures, in some cases, develop further to higher order modulation structures by additional modulations caused by higher order crystalline electric field, magnetic interactions such as spin-lattice interaction, external magnetic field and pressure. The higher order modulation structures are observed in a spin-slip structure or a helifan structure in Ho, and a tilt helix structure in a TbEr alloy. Paramagnetic ions originated from frustration generate many magnetic phases under applied external magnetic field. KUR neutron diffraction groups have performed the development and adjustment of high-pressure instruments and external magnetic fields for neutron diffraction spectrometers. The studies of 'neutron diffraction under extreme conditions' by the seven groups are described in this report. (Y. Kazumata)

  17. Metastatic hepatocellular carcinoma in the maxilla and mandible, an extremely rare presentation

    Directory of Open Access Journals (Sweden)

    Satya Ranjan Misra

    2015-01-01

    Full Text Available Malignancy is characterized by anaplasia, invasiveness, and metastasis. Primary oral squamous cell carcinoma is the most prevalent oral malignancy, but secondary malignancy from distant sites have also been reported. Hepatocellular carcinoma (HCC is a common primary liver malignancy that frequently metastasizes during the course of the disease, but < 1% of cases show oral involvement. Such secondary neoplasms do not have any pathognomonic clinical or radiologic findings, and therefore they pose a diagnostic challenge. Hence, in the differential diagnosis of malignant tumors of the oral cavity, it is essential to consider the occurrence of both primary as well as metastatic tumors despite the low incidence of the latter. A rare case of HCC metastasizing to both the maxilla and mandible is presented, in which the patient succumbed to the disease as a result of the delay in diagnosis.

  18. [The hidradenocarcinoma of the wrist - an extremely rare malignant carcinoma: case presentation and literature review].

    Science.gov (United States)

    Arsalan-Werner, A; Mentzel, T; Kempf, B; Sauerbier, M

    2013-10-01

    Hidradenocarcinomas are rare, yet highly malignant tumors of eccrine sweat gland origin. Due to its locally aggressive growth and likelihood for metastasis it should be considered as a differential diagnosis especially in case of suspicious intraoperative findings. We report the case of a 73-year-old female patient presenting with a hidradenocarcinoma of the wrist. Treatment of hidradenocarcinomas is similar to the treatment of sarcomas: The first step is an incisional biopsy and obtaining an expert second opinion on the histopathological diagnosis as well as staging. The second step is a clear margin resection of the tumor and the plastic-surgical reconstruction. A long-term follow-up is mandatory to detect potential recurrence or metastasis. © Georg Thieme Verlag KG Stuttgart · New York.

  19. Arterial tortuosity syndrome: An extremely rare disease presenting as a mimic of pulmonary sling

    Directory of Open Access Journals (Sweden)

    Amy Farkas, MD

    2018-02-01

    Full Text Available Pulmonary sling is the anatomic variant defined by the aberrant origin of the left pulmonary artery from the right pulmonary artery. This patient presented with a mimic of pulmonary sling as a result of an extremely rare condition, arterial tortuosity syndrome (ATS. The patient was first diagnosed with pulmonary sling on prenatal echocardiogram performed by cardiology. Computed tomography angiography of the chest obtained at birth to evaluate respiratory depression demonstrated ATS. The early detection of ATS has been demonstrated to improve patient outcome. This case provides an overview of the typical imaging features of ATS to aid radiologists in making this uncommon diagnosis.

  20. Primary carcinoid tumor arising within mature teratoma of the kidney: report of a rare entity and review of the literature

    Directory of Open Access Journals (Sweden)

    Parwani Anil V

    2007-05-01

    Full Text Available Abstract Background Primary carcinoid tumor arising within mature teratoma of the kidney is extremely rare, and their clinicopathologic features are not well described. Our objective was to further define the clinical features and pathologic spectra of primary carcinoid tumor arising within mature teratoma of the kidney. Methods Six previously reported case reports were identified using MEDLINE and a subsequent bibliographic search of all pertinent reports and reviews was performed. We also searched the electronic medical archival records of our institution and identified one additional unreported case. Data were extracted on the demographics, predisposing factors, clinical presentation, radiographic features, gross pathology, microscopic pathology, immunophenotype, therapy, and outcome of each of these seven cases. Results Primary carcinoid tumor arising within mature teratoma of the kidney was found at a mean age of 41.4 years. Of the 7 cases, 3 were female and 4 were male. Two of the 7 cases (28.6% were associated with horseshoe kidney. It typically presented with abdominal pain without carcinoid syndrome. It typical radiologic appearance was well circumscribed partly calcified Bosniak II-III lesion. Histologically, the carcinoid tumor showed monotonous small round cells arranged in classic anastomosing cords/ribbons intermixed with solid nests. Surgery was curative, no additional treatment was required, no local recurrences occurred, and no metastases occurred in all 7 cases. The 3 cases with available outcome data were alive at the time of publication of their respective cases (mean, 5 months. Conclusion Primary carcinoid tumor arising within mature teratoma of the kidney is a rare tumor that typically presents with abdominal pain without carcinoid syndrome. It is not associated with local recurrence and metastasis, is surgically curable, and has excellent prognosis.

  1. Genetics of rare mesenchymal tumors: implications for targeted treatment in DFSP, ASPS, CCS, GCTB and PEComa.

    Science.gov (United States)

    Rutkowski, Piotr; Przybył, Joanna; Świtaj, Tomasz

    2014-08-01

    Soft tissue and bone sarcomas comprise a heterogeneous group of mesenchymal tumors that include roughly 130 distinct diagnostic entities. Many of them are exceptionally rare, with only few cases diagnosed worldwide each year. Development of novel targeted treatment in this group of tumors is of special importance since many sarcoma subtypes are resistant to conventional chemotherapy and the effective therapeutic options are limited. In this review we aim to discuss the molecular implications for targeted therapy in selected rare soft tissue and bone sarcoma subtypes, including dermatofibrosarcoma protuberans (DFSP), alveolar soft part sarcoma (ASPS), clear cell sarcoma (CCS), giant cell tumor of bone (GCTB) and perivascular epithelioid cell neoplasms (PEComas). This article is part of a Directed Issue entitled: Rare cancers. Copyright © 2014 Elsevier Ltd. All rights reserved.

  2. A rare complication of secondary hyperparathyroidism: Brown tumor of the maxilla and mandible

    International Nuclear Information System (INIS)

    Sumer, Pinar A.; Sumer, Mahmut; Arik, Nurol; Karogoz, Filiz

    2004-01-01

    Brown tumors are focal bone lesions caused by increased osteoclastic activity and fibroblastic proliferation encountered in primary or more rarely secondary hyperparathyroidism. Ninety-two percent of the patients undergoing dialysis develop secondary hyperparathyroidism. Of these, approximately 1.5% develops brown tumors. Brown tumors of hyperparathyroidism may appear in any bone but are frequently found in the facial bones and jaws, particularly in long-standing cases of the disease. As it becomes common for hyperparathyroidism to be detected earlier during the disease, the bony manifestations of the disease are rarely seen. The following report describes a case of brown tumor of the maxilla and mandible in a patient with renal insufficiency. This patient presented multiple skeletal lesions, which are uncommonly seen now a days. (author)

  3. Gliosarcoma: un tumor cerebral poco común (Gliosarcoma, a rare brain tumor

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    Edison Vega

    2014-08-01

    Full Text Available Resumen (español El Gliosarcoma (GS es una neoplasia primaria agresiva y poco frecuente del Sistema Nervioso Central (SNC, compuesta de elementos astrocíticos anaplásicos y mesenquimales claramente malignos. El cuadro clínico se caracteriza por cefalea y convulsiones, que se presentan como parte de los síntomas iniciales en el 30% de los pacientes y cuyo porcentaje aumenta conforme avanza la enfermedad. Dentro de las manifestaciones secundarias, se describen cambios como alteración de la concentración y la personalidad; de manera particular las lesiones que afectan al lóbulo parietal se asocian a déficit motor o sensitivo. En cuanto al diagnóstico imagenológico por RMN, los gliomas malignos se observan bien circunscritos con edema focal y captan el contraste en la periferia con un centro hipointenso. Se reporta el caso de paciente Masculino de 45 años de edad, cuyos hallazgos clínico-imagenológicos concuerdan con los previamente descritos por lo que se plantea el diagnóstico de glioma parietooccipital derecho. Al realizar exeresis del tumor, se practicó estudio histopatológico, el cual fue reportado como un glioblastoma multiforme, y posterior al análisis inmunohistoquímico, se concluyó definitivamente como un gliosarcoma. La baja frecuencia de esta entidad patológica y la repercusión en la morbimortalidad de los pacientes que la padecen motivaron a la presentación de este caso. Abstract (english The Gliosarcoma (GS is an aggressive primary neoplasm and rare in central nervous system (CNS, composed of anaplastic astrocytic and mesenchymal elements clearly malignant. The clinical picture is characterized by headaches and seizures, presented as part of the initial symptoms in 30% of patients whose percentage increases as the disease progresses. Among the secondary manifestations, described changes as impaired concentration and personality of lesions particularly affecting the parietal lobe is associated with motor or sensory

  4. Clinicopathological study of rare invasive epithelial tumors of breast: An institutional study

    Directory of Open Access Journals (Sweden)

    Karthik Kasireddy

    2016-01-01

    Full Text Available Introduction: Invasive breast cancer (BC is the most common carcinoma in women. It accounts for 22% of all female cancers. Most tumors are derived from mammary duct epithelium, and up to 75% of BCs are ductal carcinomas. The second most common tumor is invasive lobular carcinoma. However, there are many variants which are less common but well defined by the World Health Organization classification. They comprise <10% of breast tumors. Their clinical behavior differs greatly. Hence, it is important to know their main histomorphological features to make the best treatment of choice and to foresee prognosis. Aims and Objectives: To study the histomorphological features, incidence, and clinical features of rare invasive epithelial tumors of the breast. Materials and Methods: This study was done in the department of pathology, Sri Devaraj Urs Medical College, Kolar. All the neoplastic breast lesions over a period of 5 years (July 2010-September 2015 are included in the study. Clinical features and other details (estrogen receptor/progesterone receptor, human epidermal receptor-2, lymph nodes are obtained from the department (surgery records. Specimens are received and preserved in 10% formalin and are subjected to routine histopathological processing. Hematoxylin and eosin sections are studied, and a morphological diagnosis is given. All rare invasive epithelial breast tumors will be reviewed meticulously. Results and Conclusion: A total number of invasive epithelial tumors of breast were 105. The most common presenting symptom was breast lump. Rare invasive epithelial breast tumors account to 28.5%. The age range from 15 to 70 years. Most common, rare invasive epithelial tumor in our study is medullary carcinoma. Hence, it is imperative to always maintain a Hawks vigil during microscopic diagnosis to know prognosis of the condition and to facilitate early and prompt treatment to the patient.

  5. A Rare Case of Breast Malignant Phyllodes Tumor With Metastases to the Kidney

    OpenAIRE

    Karczmarek-Borowska, Bożenna; Bukala, Agnieszka; Syrek-Kaplita, Karolina; Ksiazek, Mariusz; Filipowska, Justyna; Gradalska-Lampart, Monika

    2015-01-01

    Abstract Phyllodes tumors are rare breast neoplasms. Surgery is the treatment of choice. The role of postoperative radiotherapy and chemotherapy is still under dispute, as there are no equivocal prognostic factors. Treatment failure results in the occurrence of distant metastasis—mainly to the lungs, bones, liver, and brain. We have described the case of a woman with a malignant phyllodes tumor of the breast that was surgically treated. She did not receive adjuvant therapy because there is no...

  6. Epithelioid hemangioendothelioma – A rare pulmonary tumor

    Directory of Open Access Journals (Sweden)

    Sandra Saleiro

    2008-05-01

    Full Text Available The authors report a case of a rare pulmonary neoplasm – epithelioid hemangioendothelioma, in a 39 year-old woman, asymptomatic until December 2003, when she developed pleuritic and right-sided chest pain. The patient presented a previous chest radiograph, performed 13 years before, which showed multiple small bilateral pulmonary nodules attributed to tuberculous sequelae. The definitive histological diagnosis was made by lung biopsy through thoracotomy. The patient developed a clinical and imagiological worsening and then therapy with interferon α-2a was started. Even with imagiological stability of pulmonary lesions the patient remained symptomatic and died nine months after the diagnosis had been established.The authors emphasise the rarity of this type of pulmonary neoplasm and discuss its clinical presentation, histological features, treatment and prognosis. Resumo: Os autores descrevem um caso de uma neoplasia pulmonar rara – hemangioendotelioma epitelióide – numa doente do sexo feminino, de 39 anos, assintomática até Dezembro de 2003, altura em que apresentou toracalgia direita de características pleuríticas. A doente era portadora de uma radiografia torácica antiga, efectuada há 13 anos, que revelava múltiplos pequenos nódulos pulmonares bilaterais, atribuídos a sequelas de tuberculose pulmonar. O diagnóstico histológico definitivo foi efectuado através de biópsia pulmonar por toracotomia. Dado a doente ter apresentado agravamento clínico e imagiológico foi iniciada terapêutica com interferão α-2a. Apesar da estabilidade imagiológica das lesões pulmonares, a doente manteve-se sintomática e faleceu nove meses depois do diagnóstico ter sido estabelecido.Os autores realçam a raridade deste tipo de neoplasia pulmonar e discutem a sua apresentação clínica, características histológicas, tratamento e prognóstico. Key-words: Epithelioid

  7. Multifunctional biocompatible graphene oxide quantum dots decorated magnetic nanoplatform for efficient capture and two-photon imaging of rare tumor cells.

    Science.gov (United States)

    Shi, Yongliang; Pramanik, Avijit; Tchounwou, Christine; Pedraza, Francisco; Crouch, Rebecca A; Chavva, Suhash Reddy; Vangara, Aruna; Sinha, Sudarson Sekhar; Jones, Stacy; Sardar, Dhiraj; Hawker, Craig; Ray, Paresh Chandra

    2015-05-27

    Circulating tumor cells (CTCs) are extremely rare cells in blood containing billions of other cells. The selective capture and identification of rare cells with sufficient sensitivity is a real challenge. Driven by this need, this manuscript reports the development of a multifunctional biocompatible graphene oxide quantum dots (GOQDs) coated, high-luminescence magnetic nanoplatform for the selective separation and diagnosis of Glypican-3 (GPC3)-expressed Hep G2 liver cancer tumor CTCs from infected blood. Experimental data show that an anti-GPC3-antibody-attached multifunctional nanoplatform can be used for selective Hep G2 hepatocellular carcinoma tumor cell separation from infected blood containing 10 tumor cells/mL of blood in a 15 mL sample. Reported data indicate that, because of an extremely high two-photon absorption cross section (40530 GM), an anti-GPC3-antibody-attached GOQDs-coated magnetic nanoplatform can be used as a two-photon luminescence platform for selective and very bright imaging of a Hep G2 tumor cell in a biological transparency window using 960 nm light. Experimental results with nontargeted GPC3(-) and SK-BR-3 breast cancer cells show that multifunctional-nanoplatform-based cell separation, followed by two-photon imaging, is highly selective for Hep G2 hepatocellular carcinoma tumor cells.

  8. Kidney fibroxanthoma (malignant fibrous xanthoma): a rare tumor and an unusual cause of retroperitoneal hemorrhage.

    Science.gov (United States)

    Witz, M; Bernheim, J; Dinbar, A; Griffel, B

    1984-06-01

    A case of kidney fibroxanthoma (malignant fibrous xanthoma, malignant variant of xanthogranuloma), a rare malignant neoplasm of kidney, is described. In addition to the typical histologic features of retroperitoneal xanthogranuloma, this tumor showed obvious pleomorphism and mitotic activity of the histiocytes. We present this case in view of the rarity of this neoplasm and the unusual presentation as massive retroperitoneal hemorrhage.

  9. Hurthle cell tumor of the thyroid gland: Report of a rare case and ...

    African Journals Online (AJOL)

    2013-07-09

    Jul 9, 2013 ... Hurthle cell neoplasms are rare tumors. Their biology is confounding and behavior unpredictable. Tumor‑size of Hurthle cell neoplasms is an important preoperative index for predicting benignity. This index impacts on the choice doing either a total or hemithyroidectomy. While HCCs grow to comparatively.

  10. Thyroid Gland Metastasis from Cancer of the Uterine Cervix: An Extremely Rare Case Report.

    Science.gov (United States)

    Celik, Suleyman Utku; Besli, Dilara; Sak, Serpil Dizbay; Genc, Volkan

    The thyroid gland is a relatively uncommon site for a metastatic disease, although it is richly supplied with blood. The metastases may originate from various primary sites, mainly kidney, lung, head and neck, and breast. Thyroid metastasis from cervical carcinomas is extremely rare; and only a few cases have been previously reported in the literature. In patient with thyroid nodules and an oncological history, the possibility of thyroid metastasis should be seriously considered. Despite the rarity of the metastasis of cervical carcinoma to the thyroid, it is difficult to say appropriate treatment approach for these lesions. When managing such patients, decision-making should balance the possibility of gaining long-term survival against estimation of the aggressiveness of the disease and its possible complications. Here, a case of thyroid metastasis from a squamous cell carcinoma of the uterine cervix presenting with cervical mass and difficulty in swallowing and its treatment is reported.

  11. [Rare tumors of the head and neck; on behalf of the REFCOR, the French Network of rare head and neck tumors].

    Science.gov (United States)

    Baujat, Bertrand; Thariat, Juliette; Baglin, Anne Catherine; Costes, Valérie; Testelin, Sylvie; Reyt, Emile; Janot, François

    2014-05-01

    Malignant tumors of the upper aerodigestive tract may be rare by their histology (sarcoma, variants of conventional squamous cell carcinomas) and/or location (sinuses, salivary glands, ear, of various histologies themselves). They represent less than 10% of head and neck neoplasms. The confirmation of their diagnosis often requires a medical expertise and sometimes biomolecular techniques complementary to classical histology and immunohistochemistry. Due to their location, their treatment often requires a specific surgical technique. Radiation therapy is indicated based on histoclinical characteristics common to other head and neck neoplasms but also incorporate grade. Further, the technique must often be adapted to take into account the proximity of organs at risk. For most histologies, chemotherapy is relatively inefficient but current molecular advances may allow to consider pharmaceutical developments in the coming years. The REFCOR, the French Network of head and neck cancers aims to organize and promote the optimal management of these rare and heterogeneous diseases, to promote research and clinical trials.

  12. Adenomatoid odontogenic tumor of the mandible: review of the literature and report of a rare case

    Directory of Open Access Journals (Sweden)

    Braunstein Stefan

    2005-08-01

    Full Text Available Abstract Adenomatoid odontogenic tumor (AOT is a rare odontogenic tumor which is often misdiagnosed as odontogenic cyst. To acquire additional information about AOT, all reports regarding AOT and cited in "pubmed" since 1990 onward were reviewed. AOT accounts for about 1% until 9% of all odontogenic tumors. It is predominantly found in young and female patients, located more often in the maxilla in most cases associated with an uneruppted permanent tooth. For radiological diagnose the intraoral periapical radiograph seems to be more useful than panoramic. However, AOT frequently resemble other odontogenic lesions such as dentigerous cysts or ameloblastoma. Immunohistochemically AOT is characterized by positive reactions with certain cytokeratins. Treatment is conservative and the prognosis is excellent. For illustration a rare case of an AOT in the mandible is presented.

  13. Hidradenocarcinoma: A Rare Sweat Gland Neoplasm Presenting as Small Turban Tumor of the Scalp.

    Science.gov (United States)

    Asati, Dinesh P; Brahmachari, Swagata; Kudligi, Chandramohan; Gupta, Chandramohan

    2015-01-01

    Hidradenocarcinomas are very rare malignant sweat gland tumors that possess an infiltrative and/or low metastatic potential. Here we describe an interesting case of hidradenoma on the fronto-parietal region of the scalp of an elderly female, part of which had developed carcinomatous changes, infiltrating up to the pericranium. She developed intense itching, pain, spontaneous ulceration and rapid increase in the size of the tumor correlating with the expression of malignant behavior of the neoplasm. An initial incision biopsy suggested features of benign poroid hidradenoma, while the histology from the excised tumor exhibited a fairly well circumscribed epithelial neoplasm in dermis consisting of interconnected nodules as well as differentiated ducts, the neoplastic cells showing mild pleomorphism of nuclei, mitotic figures and abundant pale cytoplasm. Clefts, sclerotic stroma and foci of necrosis en mass were also seen. The final diagnosis was a well differentiated and slow growing hidradenocarcinoma. The tumor recurred locally despite total excision.

  14. Hidradenocarcinoma: A rare sweat gland neoplasm presenting as small turban tumor of the scalp

    Directory of Open Access Journals (Sweden)

    Dinesh P Asati

    2015-01-01

    Full Text Available Hidradenocarcinomas are very rare malignant sweat gland tumors that possess an infiltrative and/or low metastatic potential. Here we describe an interesting case of hidradenoma on the fronto-parietal region of the scalp of an elderly female, part of which had developed carcinomatous changes, infiltrating up to the pericranium. She developed intense itching, pain, spontaneous ulceration and rapid increase in the size of the tumor correlating with the expression of malignant behavior of the neoplasm. An initial incision biopsy suggested features of benign poroid hidradenoma, while the histology from the excised tumor exhibited a fairly well circumscribed epithelial neoplasm in dermis consisting of interconnected nodules as well as differentiated ducts, the neoplastic cells showing mild pleomorphism of nuclei, mitotic figures and abundant pale cytoplasm. Clefts, sclerotic stroma and foci of necrosis en mass were also seen. The final diagnosis was a well differentiated and slow growing hidradenocarcinoma. The tumor recurred locally despite total excision.

  15. Rare Presentation of Metastatic Cystic Trophoblastic Tumor in a Patient Without Prior Chemotherapy

    Directory of Open Access Journals (Sweden)

    Michael L. Wang

    2017-07-01

    Full Text Available Cystic trophoblastic tumor (CTT is a rare testicular germ cell tumor (GCT predominantly seen in post-chemotherapy patients. It is prognostically similar to teratoma and requires no additional chemotherapy in the absence of a nonteratomatous GCT component. We report a case of metastatic CTT in a patient with primary testicular teratoma without prior chemotherapy. Retroperitoneal lymph node metastases contained teratoma, embryonal carcinoma, and CTT. The CTT was β-hCG positive and SALL4 negative by immunohistochemistry (IHC. CTT can arise in metastatic testicular GCT in treatment naïve patients. An important differential diagnosis is choriocarcinoma due to treatment implications, and SALL4 IHC may help.

  16. Meningeous sarcoma: a rare tumor among the central nervous system neoplasia in childhood

    International Nuclear Information System (INIS)

    Rondinelli, Patricia Imperatriz Porto; Salvajoli, Joao Victor; Sredni, Simone Treiger; Araujo, Maria Betania Mahler

    2003-01-01

    We describe a case of meningeous malignancy in childhood, diagnosed by the Pediatric Department of the Cancer Hospital in Sao Paulo, Brazil, and do revise the world literature as well. The meningeous sarcoma (M S) is an extremely aggressive tumor, which appears in the central nervous system, at any age, but mainly in children. They represent a tiny percentage of brain tumors in children and sporadic cases are related in the world literature. Consequently, there are not enough clinical experiences about this distinct entity to allow the conclusion about which is the best therapeutic approach. (author)

  17. Recurrent multifocal cutaneous Kaposiform hemangioendothelioma: A rare vascular tumor of infancy and childhood

    Directory of Open Access Journals (Sweden)

    Bhagyalakshmi Atla

    2016-01-01

    Full Text Available Kaposiform hemangioendothelioma (KHE is a locally aggressive vascular tumor of childhood although cases occurring in adulthood are also described. The features overlap with juvenile capillary hemangioma and Kaposi sarcoma. We report a rare case of recurrent, multifocal (nose and chin cutaneous KHE initially occurring in a 3-year-old female child, uncomplicated by Kasabach–Merritt syndrome. Recurrences occurred over the next 6 years and resulted in complete distortion of the nose, requiring plastic repair.

  18. Recurrent multifocal cutaneous Kaposiform hemangioendothelioma: A rare vascular tumor of infancy and childhood.

    Science.gov (United States)

    Atla, Bhagyalakshmi; Sudhakar, P V; Rao, Nagarjun; Prasad, Uma

    2016-01-01

    Kaposiform hemangioendothelioma (KHE) is a locally aggressive vascular tumor of childhood although cases occurring in adulthood are also described. The features overlap with juvenile capillary hemangioma and Kaposi sarcoma. We report a rare case of recurrent, multifocal (nose and chin) cutaneous KHE initially occurring in a 3-year-old female child, uncomplicated by Kasabach-Merritt syndrome. Recurrences occurred over the next 6 years and resulted in complete distortion of the nose, requiring plastic repair.

  19. A Rare Renal Epithelial Tumor: Mucinous Cystadenocarcinoma Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Abdulkadir Tepeler

    2011-01-01

    Full Text Available Primary renal mucinous cystadenocarcinoma is a very rare lesion of kidney which originates from the metaplasia of the renal pelvic uroepithelium. Only one case with primary mucinous cystadenocarcinoma has been reported in the English literature. We report second case of mucinous cystadenocarcinoma which was radiologically classified as type-IIF Bosniak cyst in peripheral localization. We aimed to present this extreme and unusual entity with its radiological, surgical, and pathologic aspects under the light of literature.

  20. Serous Carcinoma of the Uterine Cervix, an Extremely Rare Aggressive Entity: A Literature Review.

    Science.gov (United States)

    Jonska-Gmyrek, Joanna; Zolciak-Siwinska, Agnieszka; Gmyrek, Leszek; Michalski, Wojciech; Poniatowska, Grazyna; Fuksiewicz, Malgorzata; Wiechno, Pawel; Kucharz, Jakub; Kowalska, Maria; Kotowicz, Beata

    2018-01-23

    Serous carcinoma of the uterine cervix (USCC) is an extremely rare subtype. To establish the treatment strategy in patients with USCC is an important issue. MEDLINE (PubMed) was searched for all articles published after the first publication by Lurie et al. [Eur J Obstet Gynecol Reprod Biol 1991; 40: 79-81], reporting woman diagnosed with USCC. Because of limited numbers of studies on the topic of the study, we could not keep a restriction of eliminating smaller sample sizes. A search of PubMed demonstrated that 113 cases of USCC have been reported in the literature since the first publication. The current treatment modality adopted for early cervical cancer is hysterectomy with bilateral iliac-obturator lymphadenectomy and postoperative radiotherapy (RT) or radiochemotherapy (RT-CT) if risk factors for cervical carcinoma appear. The treatment strategy for locally advanced USCC is preoperative RT-CT or chemotherapy (CHTH) with the intention to treat the patient surgically. The treatment option for disseminated disease is CHTH with paclitaxel and carboplatin. Risk factors and a more advanced clinical stage of USCC have an impact on poor outcomes despite the use of standard treatment methods, adapted for cervical cancer. The outside-pelvic failures tend to seek effective systemic treatment. © 2018 S. Karger AG, Basel.

  1. Onychomycosis: A Rare Presentation of Fungal Urinary Tract Infection in an Extremely Preterm Neonate

    Directory of Open Access Journals (Sweden)

    Shilpa Kalane

    2015-06-01

    Full Text Available Onychomycosis refers to nail infections, caused by fungi including yeasts and non-dermatophyte moulds. One or several toenails or fingernails (seldom all may be involved in this condition. Many cases of fingernail onychomycosis are due to yeasts. Fungal infection has emerged as an important cause of neonatal infection, associated with significant morbidity and mortality, especially in very low birth weight (< 1500 g and extremely low birth weight (< 1000 g infants. Herein, we report a case of a 24-day-old male infant, who presented with onychomycosis on the left ring fingernail, associated with fungal urinary tract infection (UTI. The evaluation of nails helped us detect fungal UTI. To date, there have been no reports suggesting onychomycosis as a presentation of fungal UTI. We could not find the association between onychomycosis and neonatal fungal UTI. Hence, retrospectively, it can be said that onychomycosis was a presentation of fungal UTI. Further studies are required to evaluate the etiology and treatment of neonatal onychomycosis. Moreover, dermatologists should pay particular attention to this rare event.

  2. RARE BENIGN EYELID TUMOR IN CHILDREN (EPITHELIOMA OF MALHERBE, PILOMATRIXOMA, OR TRICHELEMMOMA

    Directory of Open Access Journals (Sweden)

    A. A. Ryabtseva

    2015-01-01

    Full Text Available Aim. To describe clinical manifestations of rare eyelid tumor (epithelioma Malherbe and to improve differential diagnosis of benign eyelid tumors in children. Patients and methods. We observed 8 children aged 3,5‑8 years (sex ratio was 1:1. In all cases, examination, palpation, surgical excision of the tumor with histological examination were performed. Results. Trichilemmoma, or pilomatricoma, was suggested from clinical manifestations. Epithelioma Malherbe was diagnosed by histology only. Microscopically, the tumor is surrounded by a capsule which includes two cell types. Peripheral basophilic cells are small cells with poor cytoplasm, indistinct borders, and deeply basophilic nucleus. Central shadow cells have a distinct border and a central unstained area. Islands of small basaloid epithelial cells with squamous cell focuses and cornification are embedded in the stroma. Epithelial lesions are often necrotized. Epithelial mass is surrounded by granulations with giant cells. Osseous trabeculae are often adjacent to necrotic lesions. Further follow-up revealed no complications or recurrences. Conclusions. Our observations and literature data suggest that epithelioma Malherbe is occured in 1.3 % of benign eyelid tumors in childern. Tumor growth is slow and non-invasive. 

  3. Nephron-sparing surgery for treatment of reninoma: a rare renin secreting tumor causing secondary hypertension.

    Science.gov (United States)

    Torricelli, Fabio Cesar Miranda; Marchini, Giovanni Scala; Colombo, Jose Roberto; Coelho, Rafael Ferreira; Nahas, Willian Carlos; Srougi, Miguel

    2015-01-01

    A 25-year-old hypertensive female patient was referred to our institution. Initial workup exams demonstrated a 2.8 cm cortical lower pole tumor in the right kidney. She underwent laparoscopic partial nephrectomy without complications. Histopathologic examination revealed a rare juxtaglomerular cell tumor known as reninoma. After surgery, she recovered uneventfully and all medications were withdrawn. Case hypothesis: Secondary arterial hypertension is a matter of great interest to urologists and nephrologists. Renovascular hypertension, primary hyperadosteronism and pheocromocytoma are potential diagnosis that must not be forgotten and should be excluded. Although rare, chronic pyelonephritis and renal tumors as rennin-producing tumors, nephroblastoma, hypernephroma, and renal cell carcinoma might also induce hypertension and should be in the diagnostic list of clinicians. Promising future implications: Approximately 5% of patients with high blood pressure have specific causes and medical investigation may usually identify such patients. Furthermore, these patients can be successfully treated and cured, most times by minimally invasive techniques. This interesting case might expand knowledge of physicians and aid better diagnostic care in future medical practice.

  4. Amenorrhea as a rare drug-related adverse event associated with everolimus for pancreatic neuroendocrine tumors.

    Science.gov (United States)

    Kawaguchi, Yoshiaki; Maruno, Atsuko; Kawashima, Yohei; Ito, Hiroyuki; Ogawa, Masami; Mine, Tetsuya

    2014-11-14

    The patient was an asymptomatic 43-year-old woman. Abdominal ultrasonography and enhanced computed tomography showed a tumor lesion accompanied by multiple cystic changes in the liver and the pancreatic tail. Endoscopic ultrasound-fine needle aspiration was performed on the pancreatic tumor lesion and revealed pancreatic neuroendocrine tumor (PNET). As it was unresectable due to multiple liver metastases, the decision was made to initiate treatment with everolimus and transcatheter arterial chemoembolization. The patient ceased menstruating after the start of everolimus administration. When the administration was discontinued due to interstitial lung disease, menstruation resumed, but then again stopped with everolimus resumption. An association between everolimus and amenorrhea was highly suspected. Amenorrhea occurred as a rare adverse event of everolimus. As the younger women might be included in PNETs patients, we should put this adverse event into consideration.

  5. Clinical implications of a rare renal entity: Pleomorphic Hyalinizing Angiectatic Tumor (PHAT).

    Science.gov (United States)

    Scalici Gesolfo, Cristina; Serretta, Vincenzo; Di Maida, Fabrizio; Giannone, Giulio; Barresi, Elisabetta; Franco, Vito; Montironi, Rodolfo

    2017-02-01

    Pleomorphic Hyalinizing Angiectatic Tumor (PHAT) is a rare benign lesion characterized by slow growth, infiltrative behavior and high rate of local recurrences. Only one case has been described in retroperitoneum, at renal hilum, but not involving pelvis or parenchyma. Here we present the first case of PHAT arising in the renal parenchyma. A nodular lesion in right kidney lower pole was diagnosed to a 61 year old woman. The patient underwent right nephrectomy. Microscopically, the lesion showed solid and pseudo-cystic components with hemorrhagic areas characterized by aggregates of ectatic blood vessels. Pleomorphic cells were characterized by large eosinophilic cytoplasm with irregular and hyperchromatic nuclei. Immunohistochemistry was performed and the lesion was classified as a Pleomorphic Hyalinizing Angiectatic Tumor (PHAT). Due to the clinical behavior of this tumor, in spite of its benign nature, review of the surgical margins and close follow up after partial nephrectomy are mandatory. Copyright © 2016. Published by Elsevier GmbH.

  6. Extreme ultraviolet fluorescence spectroscopy of pure and core-shell rare gas clusters at FLASH

    Energy Technology Data Exchange (ETDEWEB)

    Schroedter, Lasse

    2013-08-15

    The interaction of rare gas clusters with short-wavelength radiation of free-electron lasers (FELs) has been studied extensively over the last decade by means of electron and ion time-of-flight spectroscopy. This thesis describes the design and construction of a fluorescence spectrometer for the extreme ultraviolet (XUV) spectral range and discusses the cluster experiments performed at FLASH, the Free-electron LAser in Hamburg. Fluorescence of xenon and of argon clusters was studied, both in dependence on the FEL pulse intensity and on the cluster size. The FEL wavelength was set to the giant 4d-resonance of xenon at 13.5 nm and the FEL pulse intensity reached peak values of 2.7.10{sup 15} W/cm{sup 2}. For xenon clusters, charge states of at least 11+ were identified. For argon, charge states up to 7+ were detected. The cluster-size dependent study revealed a decrease of the fluorescence yield per atom with increasing cluster size. This decrease is explained with the help of a geometric model. It assumes that virtually the entire fluorescence yield stems from shells of ions on the cluster surface, whereas ions in the cluster core predominantly recombine non-radiatively with electrons. However, the detailed analysis of fluorescence spectra from clusters consisting of a core of Xe atoms and a surrounding shell of argon atoms shows that, in fact, a small fraction of the fluorescence signal comes from Xe ions in the cluster core. Interestingly, these ions are as highly charged as the ions in the shells of a pure Xe cluster. This result goes beyond the current understanding of charge and energy transfer processes in these systems and points toward the observation of ultrafast charging dynamics in a time window where mass spectrometry is inherently blind. (orig.)

  7. Extreme ultraviolet fluorescence spectroscopy of pure and core-shell rare gas clusters at FLASH

    International Nuclear Information System (INIS)

    Schroedter, Lasse

    2013-08-01

    The interaction of rare gas clusters with short-wavelength radiation of free-electron lasers (FELs) has been studied extensively over the last decade by means of electron and ion time-of-flight spectroscopy. This thesis describes the design and construction of a fluorescence spectrometer for the extreme ultraviolet (XUV) spectral range and discusses the cluster experiments performed at FLASH, the Free-electron LAser in Hamburg. Fluorescence of xenon and of argon clusters was studied, both in dependence on the FEL pulse intensity and on the cluster size. The FEL wavelength was set to the giant 4d-resonance of xenon at 13.5 nm and the FEL pulse intensity reached peak values of 2.7.10 15 W/cm 2 . For xenon clusters, charge states of at least 11+ were identified. For argon, charge states up to 7+ were detected. The cluster-size dependent study revealed a decrease of the fluorescence yield per atom with increasing cluster size. This decrease is explained with the help of a geometric model. It assumes that virtually the entire fluorescence yield stems from shells of ions on the cluster surface, whereas ions in the cluster core predominantly recombine non-radiatively with electrons. However, the detailed analysis of fluorescence spectra from clusters consisting of a core of Xe atoms and a surrounding shell of argon atoms shows that, in fact, a small fraction of the fluorescence signal comes from Xe ions in the cluster core. Interestingly, these ions are as highly charged as the ions in the shells of a pure Xe cluster. This result goes beyond the current understanding of charge and energy transfer processes in these systems and points toward the observation of ultrafast charging dynamics in a time window where mass spectrometry is inherently blind. (orig.)

  8. Surgical treatment of a rare primary renal carcinoid tumor with liver metastasis

    Directory of Open Access Journals (Sweden)

    Rowland Randall G

    2008-04-01

    Full Text Available Abstract Background Carcinoid tumors are characteristically low grade malignant neoplasms with neuroendocrine differentiation that arise in various body sites, most commonly the lung and gastrointestinal tract, but less frequently the kidneys, breasts, ovaries, testes, prostate and other locations. We report a case of a carcinoid of renal origin with synchronous single liver metastases on radiological studies. Case presentation A 45 year-old patient who presented with abdominal pain was found on CT scan to have lesions in the right ovary, right kidney, and left hepatic lobe. CA-125, CEA, and CA 19-9 were within normal limits, as were preoperative liver function tests and renal function. Biopsy of the liver mass demonstrated metastatic neuroendocrine tumor. At laparotomy, the patient underwent total abdominal hysterectomy with bilateral salpingo-oophorectomy, radical right nephrectomy with lymphadenectomy, and left hepatectomy. Pathology evaluation reported a right ovarian borderline serous tumor, well-differentiated neuroendocrine carcinoma of the kidney (carcinoid with 2 positive retroperitoneal lymph nodes, and a single liver metastasis. Immunohistochemistry revealed that this lesion was positive for synaptophysin and CD56, but negative for chromogranin as well as CD10, CD7, and CD20, consistent with a well-differentiated neuroendocrine tumor. She is doing well one year after her initial surgery, with no evidence of tumor recurrence. Conclusion Early surgical intervention, together with careful surveillance and follow-up, can achieve successful long-term outcomes in patients with this rare malignancy.

  9. A Rare Case of Breast Malignant Phyllodes Tumor With Metastases to the Kidney: Case Report.

    Science.gov (United States)

    Karczmarek-Borowska, Bożenna; Bukala, Agnieszka; Syrek-Kaplita, Karolina; Ksiazek, Mariusz; Filipowska, Justyna; Gradalska-Lampart, Monika

    2015-08-01

    Phyllodes tumors are rare breast neoplasms. Surgery is the treatment of choice. The role of postoperative radiotherapy and chemotherapy is still under dispute, as there are no equivocal prognostic factors. Treatment failure results in the occurrence of distant metastasis-mainly to the lungs, bones, liver, and brain. We have described the case of a woman with a malignant phyllodes tumor of the breast that was surgically treated. She did not receive adjuvant therapy because there is no consensus on the role of postoperative chemotherapy and radiotherapy. One year following the surgery, the patient had left-sided nephrectomy performed because of a rapidly growing tumor of the kidney. Renal cancer was suspected; however, a histopathological examination revealed that it was a metastatic phyllodes tumor. At the same time, the patient was diagnosed as having metastases in the other kidney, the lungs, liver, and bones.Our case report describes not only an unusual localization of the metastases (in the kidneys), but also failure of the chemotherapy and the aggressive course of malignant phyllodes tumor. Identification of patients with high risk for distant metastasis and the introduction of uniform rules for the management of adjuvant chemotherapy and radiotherapy would make planning treatment as efficacious as possible.

  10. Mesonephric adenocarcinoma of the vagina. Diagnosis and multimodal treatment of a rare tumor and analysis of worldwide experience

    International Nuclear Information System (INIS)

    Mueller, Iris; Jacobs, Volker R.; Bogner, Gerhard; Staudach, Alfons; Koch, Horst; Wolfrum-Ristau, Pia; Schausberger, Christiane; Fischer, Thorsten; Kametriser, Gerhard; Sedlmayer, Felix

    2016-01-01

    Mesonephric adenocarcinoma of the vagina is an extremely rare tumor of the female genital tract, with only a few cases reported so far worldwide. Consequently, there is no established standard treatment and limited knowledge about the prognosis and biologic behavior of vaginal mesonephric adenocarcinoma. This report documents a new case of vaginal mesonephric adenocarcinoma diagnosed in a 54-year-old woman, and analyzes this in the context of all previously published cases. MRI demonstrated that the 2.5 x 1.8 cm tumor of the vaginal wall was invading urethra and bladder. Following surgical excision, histologic analysis determined mesonephric adenocarcinoma of the vagina, stage pT2 R1. In order to avoid the mutilating extended surgery which would be required to reach R0 and considerable impairment of quality of life, adjuvant radiochemotherapy was administered with external radiation and brachytherapy, including 5 cycles of cisplatin (40 mg/m 2 ) for radiosensitization. After 4 years of continuous oncologic follow-up, the patient is alive and clinically free of disease. In this case it was shown that adjuvant radiochemotherapy with radiation and brachytherapy was effective to manage the surgical R1 situation and maintain the patient's life quality. More published cases reports are needed to gradually substantiate optimal treatment strategies. (orig.) [de

  11. Isolated peritoneal hydatidosis clinically mimicking ovarian tumor: A rare case report

    Directory of Open Access Journals (Sweden)

    Pradhan M Pagaro

    2014-01-01

    Full Text Available Hydatid cyst disease is rare and it is a parasitic infection where humans accidentally get infected by ingesting larval forms of parasite whereas, the definitive hosts are dog. The common sites of hydatid cyst are liver, lungs, spleen. Unusual sites of the hydatid cyst is reported in subcutaneous tissue of anterior abdominal wall, peritoneum. We report an unusual form of the primary hydatid cyst disease involving peritoneum in a 65-year-old female, presenting as swelling in the abdomen since 3 months. Sonography revealed a cystic mass and diagnosis of ovarian tumor was considered. The Cancer Antigen 125 (CA--125, an ovarian malignant marker was normal. Exploratory laprotomy was carried out. Cytological examination, gross, and the histopathological findings suggested the diagnosis of hydatid cyst disease involving only peritoneum. Primary isolated hydatidosis involving peritoneum is very rare and only few cases have been reported. Moreover, it mimics other tumors of the abdomen like in our case we considered it as an ovarian tumor.

  12. Mesonephric adenocarcinoma of the vagina. Diagnosis and multimodal treatment of a rare tumor and analysis of worldwide experience

    Energy Technology Data Exchange (ETDEWEB)

    Mueller, Iris; Jacobs, Volker R.; Bogner, Gerhard; Staudach, Alfons; Koch, Horst; Wolfrum-Ristau, Pia; Schausberger, Christiane; Fischer, Thorsten [Paracelsus Medical University (PMU), Department of Obstetrics and Gynecology (OB/GYN), Salzburg (Austria); Kametriser, Gerhard; Sedlmayer, Felix [Paracelsus Medical University (PMU), Department of Radiology and Radiation Oncology, 5020 Salzburg (Austria)

    2016-09-15

    Mesonephric adenocarcinoma of the vagina is an extremely rare tumor of the female genital tract, with only a few cases reported so far worldwide. Consequently, there is no established standard treatment and limited knowledge about the prognosis and biologic behavior of vaginal mesonephric adenocarcinoma. This report documents a new case of vaginal mesonephric adenocarcinoma diagnosed in a 54-year-old woman, and analyzes this in the context of all previously published cases. MRI demonstrated that the 2.5 x 1.8 cm tumor of the vaginal wall was invading urethra and bladder. Following surgical excision, histologic analysis determined mesonephric adenocarcinoma of the vagina, stage pT2 R1. In order to avoid the mutilating extended surgery which would be required to reach R0 and considerable impairment of quality of life, adjuvant radiochemotherapy was administered with external radiation and brachytherapy, including 5 cycles of cisplatin (40 mg/m{sup 2}) for radiosensitization. After 4 years of continuous oncologic follow-up, the patient is alive and clinically free of disease. In this case it was shown that adjuvant radiochemotherapy with radiation and brachytherapy was effective to manage the surgical R1 situation and maintain the patient's life quality. More published cases reports are needed to gradually substantiate optimal treatment strategies. (orig.) [German] Das mesonephrische Adenokarzinom der Vagina ist ein aeusserst seltener Tumor des weiblichen Genitaltrakts. In der internationalen Literatur finden sich nur wenig gut dokumentierte Faelle. Das biologische Verhaltensmuster dieses Tumors und dessen Prognose sind weitgehend unbekannt. In diesem Bericht wird ein neuer Fall einer 54-jaehrigen Frau mit einem mesonephrischen Adenokarzinom der Vagina vorgestellt und unter Beruecksichtigung aller bisher publizierten Faelle analysiert. Bei dem 2,5 x 1,8 cm grossen Tumor der Vaginalwand zeigte sich in der Magnetresonanztomographie eine Infiltration der Urethra und

  13. Non-syndromic multiple keratocyst odontogenic tumor: A rare case report

    Directory of Open Access Journals (Sweden)

    Abhijeet Alok

    2015-01-01

    Full Text Available Keratocystic odontogenic tumors (KCOTs are one of the most frequent features of nevoid basal cell carcinoma syndrome (NBCCS. The condition is linked with mutation in the PTCH gene. Partial expression of the gene may result in occurrence of multiple recurring odontogenic keratocysts (OKCs. Although KCOTs are common in clinical practice, simultaneous occurrence of multiple cysts in both the maxilla and mandible of a patient is rare. These patients have early propensity to develop multiple neoplasms like basal cell carcinoma and medulloblastoma. Hence, early diagnosis and treatment is of utmost importance in reducing the severity of the long-term sequelae of NBCCS. We report a rare case of multiple KCOTs in a non-syndromic male patient, with emphasis on its diagnosis, radiographic features, and treatment.

  14. Inflammatory Myofibroblastic Tumor: A Rarely Seen Submucosal Lesion of the Stomach

    Directory of Open Access Journals (Sweden)

    Deniz Arslan

    2013-01-01

    Full Text Available Inflammatory myofibroblastic tumor (IMT is a rare mesenchymal benign tumor which is generally seen in children and in young adults. It is especially located in the lungs. In histopathological examination, neoplastic fusiform cells originating from a subtype of accessory immune system cells which are called fibroblastic reticulum cells are seen (Kouichi and Youichirou, 2008. Although IMT is histopathologically benign, imaging methods show its tendency for local recurrence and invasion. In most of the cases, it may not be possible to make a distinction whether it is malign or benign. Complete surgical resection is the most important treatment method. In this study, we have discussed the findings of our case having a gastric submucosal located IMT in light of the current literatures.

  15. A rare case of cystic subepithelial tumor in the stomach: Gastric adenomyoma

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Ho Seok; Jang, Yun Jin; Heo, Jun [Kyungpook National University Hospital, Daegu (Korea, Republic of)

    2015-12-15

    Gastric adenomyoma is a rare benign subepithelial tumor, characteristically composed of mucosal structures and a prominent smooth muscle stroma. Because of rarity and the nonspecific computed tomography (CT) features, it is difficult to diagnose gastric adenomyoma before operation. In our case, gastric adenomyoma showed a well-circumscribed cystic subepithelial mass with uneven wall thickness on a CT scan, similar to the findings of former reports. The radiologic differential diagnosis can be narrowed down to several diseases, including duplication cysts, gastritis cystica profunda, brunner's gland hyperplasia and solid tumors with cystic degeneration. Also, adenomyoma could be included in the differential diagnosis of gastric cystic subepithelial masses, especially in the distal part of the stomach.

  16. Radiologic diagnosis of malignant soft-tissue tumors of the extremities

    International Nuclear Information System (INIS)

    Peters, P.E.; Friedmann, G.

    1983-01-01

    In malignant soft-tissue tumors of the extremities the radiologist is asked to define size and extent of the lesion and it's relationship to adjacent structures. The assessment of the nature of the lesion is of utmost importance, however, the contribution of the different imaging modalities varies considerably. In a review article the current roles of conventional radiography, xeroradiography, real-time ultrasonography, computed tomography and arteriography in the diagnostic workup of malignant soft-tissue tumors of the extremities are discussed. The statements made are based upon own comparative studies as well as on a review of the literature. In the assessment of the nature of a soft-tissue mass the contribution of all radiologic imaging methods is rather limited, although arteriography may add valuable information if performed complementary to CT. Real-time ultrasonography is well suited to define size, location and extent of peripheral soft-tissue masses. It is therefore recommended as the first imaging method and for follow-up studies. Equivocal findings by real-time sonography and new cases for treatment planning must be confirmed by computed tomography which proved to be the most reliable and the best reproducible imaging method for soft-tissue tumors of the extremities. (orig.)

  17. GIANT HAND LIPOMA-CASE REPORT OF A RARE LOCALIZATION OF A COMMON TYPE OF TUMOR

    Directory of Open Access Journals (Sweden)

    Radivojcevic Uros

    2016-07-01

    Full Text Available Introduction: A lipoma is a benign tumor of the adipose tissue and the most common tumor of the subcutaneous tissue which is most commonly found on the trunk. It appears as a round or oval subcutaneous mass of soft consistency, which is not attached to the skin or to the deeper tissues. Its growth can cause compressive symptoms, the most common being pain and paresthesia. Case report: Considering the fact that lipomas very rarely occur in the hand and foot, in this paper we present a case of a large lipoma in the hand of a 70-year-old female patient, with a subcutaneous tumor, in the area of the ulnar side of the left palm, and the pain and tingling in the fourth and fifth finger. Longitudinal incision on the medial side of the palm and transversal incision up to the proximal palmar crease were performed under general endotracheal anesthesia. The extirpated tumor was, due to its dimensions (5.6 x 3.4 x 2.5 cm, categorized as a giant hand lipoma. A histopathological analysis confirmed the diagnosis of lipoma. Conclusion: A hand lipoma requires surgical treatment exclusively, involving a qualified hand surgeon, which is important because of the high functional and aesthetic importance of the hand.

  18. Retiform hemangioendothelioma over forehead: A rare tumor treated with chemoradiation and a review of literature

    Directory of Open Access Journals (Sweden)

    Anup Sunil Tamhankar

    2015-01-01

    Full Text Available Retiform hemangioendothelioma (RH is low grade tumor of skin and subcutaneous tissue. It needs to be differentiated from angiosarcoma as RH has excellent prognosis. It is usually seen in young adults on extremities. Sometimes it may mimic benign conditions and can delay treatment. Surgery has been mainstay of its treatment with or without adjuvant radiation. We present first case of RH on face. This is only second case being treated with definitive chemoradiation. So it′s important to distinguish RH from angiosarcoma due to treatment implications as well.

  19. Solitary fibrous tumor: A center's experience and an overview of the symptomatology, the diagnostic and therapeutic procedures of this rare tumor

    Directory of Open Access Journals (Sweden)

    Wolfgang Hohenforst-Schmidt

    2017-01-01

    Full Text Available Solitary Fibrous Tumor of the Pleura (SFTP is a rare tumor of the pleura. Worldwide about 800 patients diagnosed with this oncological entity have been described in the existing literature. We report our center's 13 year experience. During this time three patients suffering from this rare disease have been treated in our department. All patients were asymptomatic and their diagnosis was initially triggered by a random finding in a routine chest x-ray. The diagnosis was set preoperatively through a needle biopsy under computer tomography (CT guidance. The tumors were resected surgically though video-assisted thoracoscopic surgery (VATS or thoracotomy. Because of the lack of specific guidelines due to the rarity of the disease a long-term, systematic follow-up was recommended and performed. Parallel an overview of the diagnostic and therapeutic procedures of the rare tumor is made.

  20. Ewing tumors in infants

    NARCIS (Netherlands)

    van den Berg, Henk; Dirksen, Uta; Ranft, Andreas; Jürgens, Heribert

    2008-01-01

    Malignancies in infancy are extremely rare. Ewing tumors are hardly ever noted in these children. Since it is generally assumed that malignancies in infancy have an extremely poor outcome, we wanted to investigate whether this was also the case in Ewing tumors. We identified in the Munster data

  1. RRx-001 Priming of PD-1 Inhibition in the Treatment of Small Cell Carcinoma of the Vagina: A Rare Gynecological Tumor

    Directory of Open Access Journals (Sweden)

    Christina Brzezniak

    2017-03-01

    Full Text Available Small cell carcinoma of the vagina is rare, so rare in fact that the total number reported in English-language journals is less than 30. Due to this extremely low incidence, no specific treatment guidelines have been established, and most of what is clinically known is derived from a handful of single case reports. However, as befitting its highly aggressive histologic features, which are reminiscent of small cell lung cancer (SCLC, first-line treatment is modeled after SCLC. Herein is reported the case of a 51-year-old African-American patient with metastatic biopsy-proven small cell carcinoma of the vagina that progressed through multiple therapies: first-line cisplatin and etoposide (making it platinum-resistant and radiotherapy, followed by the tumor macrophage-stimulating agent RRx-001 in a clinical trial called QUADRUPLE THREAT, which per protocol preceded a mandated rechallenge with cisplatin and etoposide. RECIST v.1.1 tumor progression on both RRx-001 and cisplatin/etoposide was accompanied by central necrosis in several of the enlarged lymph nodes and hepatic metastases, which may have been evidence of pseudoprogression, accounting for her ongoing longer-than-expected survival, since the necrotic tissue may have primed the activity of the PD-1 inhibitor. The lack of response to RRx-001 is hypothesized to have correlated with sparse tumor macrophage infiltration, seen on pre- and post-treatment biopsies, since the mechanism of action of RRx-001 relates to stimulation of tumor-associated macrophages.

  2. Extremely rare collapse and build-up of turbulence in stochastic models of transitional wall flows.

    Science.gov (United States)

    Rolland, Joran

    2018-02-01

    This paper presents a numerical and theoretical study of multistability in two stochastic models of transitional wall flows. An algorithm dedicated to the computation of rare events is adapted on these two stochastic models. The main focus is placed on a stochastic partial differential equation model proposed by Barkley. Three types of events are computed in a systematic and reproducible manner: (i) the collapse of isolated puffs and domains initially containing their steady turbulent fraction; (ii) the puff splitting; (iii) the build-up of turbulence from the laminar base flow under a noise perturbation of vanishing variance. For build-up events, an extreme realization of the vanishing variance noise pushes the state from the laminar base flow to the most probable germ of turbulence which in turn develops into a full blown puff. For collapse events, the Reynolds number and length ranges of the two regimes of collapse of laminar-turbulent pipes, independent collapse or global collapse of puffs, is determined. The mean first passage time before each event is then systematically computed as a function of the Reynolds number r and pipe length L in the laminar-turbulent coexistence range of Reynolds number. In the case of isolated puffs, the faster-than-linear growth with Reynolds number of the logarithm of mean first passage time T before collapse is separated in two. One finds that ln(T)=A_{p}r-B_{p}, with A_{p} and B_{p} positive. Moreover, A_{p} and B_{p} are affine in the spatial integral of turbulence intensity of the puff, with the same slope. In the case of pipes initially containing the steady turbulent fraction, the length L and Reynolds number r dependence of the mean first passage time T before collapse is also separated. The author finds that T≍exp[L(Ar-B)] with A and B positive. The length and Reynolds number dependence of T are then discussed in view of the large deviations theoretical approaches of the study of mean first passage times and

  3. Extremely rare collapse and build-up of turbulence in stochastic models of transitional wall flows

    Science.gov (United States)

    Rolland, Joran

    2018-02-01

    This paper presents a numerical and theoretical study of multistability in two stochastic models of transitional wall flows. An algorithm dedicated to the computation of rare events is adapted on these two stochastic models. The main focus is placed on a stochastic partial differential equation model proposed by Barkley. Three types of events are computed in a systematic and reproducible manner: (i) the collapse of isolated puffs and domains initially containing their steady turbulent fraction; (ii) the puff splitting; (iii) the build-up of turbulence from the laminar base flow under a noise perturbation of vanishing variance. For build-up events, an extreme realization of the vanishing variance noise pushes the state from the laminar base flow to the most probable germ of turbulence which in turn develops into a full blown puff. For collapse events, the Reynolds number and length ranges of the two regimes of collapse of laminar-turbulent pipes, independent collapse or global collapse of puffs, is determined. The mean first passage time before each event is then systematically computed as a function of the Reynolds number r and pipe length L in the laminar-turbulent coexistence range of Reynolds number. In the case of isolated puffs, the faster-than-linear growth with Reynolds number of the logarithm of mean first passage time T before collapse is separated in two. One finds that ln(T ) =Apr -Bp , with Ap and Bp positive. Moreover, Ap and Bp are affine in the spatial integral of turbulence intensity of the puff, with the same slope. In the case of pipes initially containing the steady turbulent fraction, the length L and Reynolds number r dependence of the mean first passage time T before collapse is also separated. The author finds that T ≍exp[L (A r -B )] with A and B positive. The length and Reynolds number dependence of T are then discussed in view of the large deviations theoretical approaches of the study of mean first passage times and multistability

  4. Malignant Glomus Tumor of the Peritoneum: Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Baleato-González, Sandra; García-Figueiras, Roberto; Trujillo-Ariza, Maria Virginia [Department of Radiology, Complexo Hospitalario Universitario de Santiago de Compostela, Choupana s/n, 15701 Santiago de Compostela (A Coruña) (Spain); Carrera-Álvarez, Juan Jose [Department of Pathology, Complexo Hospitalario Universitario de Santiago de Compostela, Choupana s/n, 15701 Santiago de Compostela (A Coruña) (Spain)

    2014-07-01

    Glomus tumors are usually benign tumors that occur in the skin and soft tissues of the extremities. Visceral locations, such as stomach, intestines or lung, are extremely rare because glomus bodies are rare or absent in these organs. This report describes our experience in a 47-year-old woman diagnosed with a peritoneal malignant glomus tumor. This finding has not been previously reported.

  5. Malignant Glomus Tumor of the Peritoneum: Case Report

    International Nuclear Information System (INIS)

    Baleato-González, Sandra; García-Figueiras, Roberto; Trujillo-Ariza, Maria Virginia; Carrera-Álvarez, Juan Jose

    2014-01-01

    Glomus tumors are usually benign tumors that occur in the skin and soft tissues of the extremities. Visceral locations, such as stomach, intestines or lung, are extremely rare because glomus bodies are rare or absent in these organs. This report describes our experience in a 47-year-old woman diagnosed with a peritoneal malignant glomus tumor. This finding has not been previously reported

  6. Prognostic factors in urothelial renal pelvis and ureter tumors: a multicenter rare cancer network study

    International Nuclear Information System (INIS)

    Ozsahin, M.; Zouhair, A.; Villa, S.; Storme, G.; Chauvet, B.; Taussky, D.; Houtte, P. van; Ries, G.; Bontemps, P.; Coucke, P.; Mirimanoff, R.O.

    1997-01-01

    Purpose: To assess the prognostic factors and the outcome in patients with transitional-cell carcinoma of the renal pelvis and/or ureter. Materials and Methods: A series of 138 patients treated between 1971 and 1996 for transitional-cell carcinoma of the renal pelvis and/or ureter was collected in a retrospective multicenter study of the Rare Cancer Network. Twelve patients with distant metastases were excluded from the statistical evaluation. In the remaining 126 patients, median age was 66 years (range: 41-87). The male to female ratio was 2.5 ((90(36))). All but 3 patients underwent a radical surgery: nephroureterectomy (n = 71), nephroureterectomy and lymphadenectomy (n = 20), nephroureterectomy and partial bladder resection or transurethral resection (n = 20), nephrectomy (n = 8), and ureterectomy (n = 4). There were 6 stage pTa, 22 pT1, 17 pT2, 37 pT3, 37 pT4, and 7 pTx tumors. The pN-stage distribution was as follows: 69 pN0, 8 pN1, 14 pN2, 4 pN3, and 31 pNx. Sixty-one percent (n = 77) of the tumors were located in the renal pelvis, and 21% (n = 27) in the ureter. Renal pelvis and ureter localization was present together in 22 (17%) patients. There were 4 grade 1, 37 grade 2, 42 grade 3 tumors (grade was not registered in 43). Following surgery, microscopic (n = 16) or macroscopic (n = 17) tumor rest was detected in 33 patients. Postoperative radiotherapy was given in 45 (36%) patients with a median total dose of 50 Gy (range: 20-66) in median 25 fractions (range: 4-33). Adjuvant systemic chemotherapy was administered in 12 (10%) patients. The median follow-up period was 39 months (range: 5-220). Results: In a median period of 9 months (range: 1-141), 66% (n = 81) of the patients relapsed (local in 34, locoregional in 7, regional in 16, and distant in 24). The 5- and 10-year overall survival (Kaplan-Meier product-limit estimates) was respectively 29% (± 5) and 19% (± 5) in all patients. In univariate analyses (logrank test), statistically significant

  7. Definitive Radiotherapy for Ewing Tumors of Extremities and Pelvis: Long-Term Disease Control, Limb Function, and Treatment Toxicity

    International Nuclear Information System (INIS)

    Indelicato, Daniel J.; Keole, Sameer R.; Shahlaee, Amir H.; Shi Wenyin; Morris, Christopher G.; Marcus, Robert B.

    2008-01-01

    Purpose: More than 70% of Ewing tumors occur in the extremities and pelvis. This study identified factors influencing local control and functional outcomes after management with definitive radiotherapy (RT). Patients and Methods: A total of 75 patients with a localized Ewing tumor of the extremity or pelvis were treated with definitive RT at the University of Florida between 1970 and 2006 (lower extremity tumors in 30, pelvic tumors in 26, and upper extremity tumors in 19). RT was performed on a once-daily (40%) or twice-daily (60%) basis. The median dose was 55.2 Gy in 1.8-Gy daily fractions or 55.0 Gy in 1.2-Gy twice-daily fractions. The median observed follow-up was 4.7 years. Functional outcome was assessed using the Toronto Extremity Salvage Score. Results: The 10-year actuarial overall survival, cause-specific survival, freedom from relapse, and local control rate was 48%, 48%, 42%, and 71%, respectively. Of the 72 patients, 3 required salvage amputation. Inferior cause-specific survival was associated with larger tumors (81% for tumors 3 . Conclusions: Limb preservation was effectively achieved through definitive RT. Treating limited field sizes with hyperfractionated high-energy RT could minimize long-term complications and provides superior functional outcomes

  8. Excisional biopsy of suspected benign soft tissue tumors of the upper extremity: correlation between preoperative diagnosis and actual pathology

    NARCIS (Netherlands)

    Sluijmer, Heleen C. E.; Becker, Stéphanie J. E.; Bossen, Jeroen K. J.; Ring, David

    2014-01-01

    Tumors of the upper extremity are common and mostly benign. However, the prevalence of discordant diagnosis of a solid hand tumor is less studied. The objectives of this retrospective study were (1) to determine the proportion of patients with a different (discrepant or discordant) pathological

  9. Cellular Angiofibroma of the Prostate: A Rare Tumor in an Unusual Location

    Directory of Open Access Journals (Sweden)

    Inez Wyn

    2014-01-01

    Full Text Available We report the unusual occurrence of a cellular angiofibroma in prostatic tissue. In this case, a 84-year-old man presented in the emergency room with urinary retention. Ultrasound revealed an enlarged prostate, which was suggestive for benign prostatic hyperplasia. The patient was treated with a Millin retropubic prostatectomy. Macroscopically the prostate contained multiple circumscribed nodules. Microscopic examination of the tumor showed the appearance of cellular angiofibroma, consisting of bland spindle cells and prominent, hyalinized vessels. The diagnosis was supported by FISH, which revealed monoallelic loss of RB1/13q14 region, as seen in spindle cell lipoma, (extra- mammary myofibroblastoma, and cellular angiofibroma. Cellular angiofibromas are rare, benign soft tissue tumours and were never reported in the prostatic gland.

  10. Benign mixed tumor of the lacrimal sac

    Directory of Open Access Journals (Sweden)

    Jong-Suk Lee

    2015-01-01

    Full Text Available Neoplasms of the lacrimal drainage system are uncommon, but potentially life-threatening and are often difficult to diagnose. Among primary lacrimal sac tumors, benign mixed tumors are extremely rare. Histologically, benign mixed tumors have been classified as a type of benign epithelial tumor. Here we report a case of benign mixed tumor of the lacrimal sac.

  11. Melanotic Schwannomas Are Rarely Seen Pigmented Tumors with Unpredictable Prognosis and Challenging Diagnosis.

    Science.gov (United States)

    Keskin, Elif; Ekmekci, Sumeyye; Oztekin, Ozgur; Diniz, Gulden

    2017-01-01

    Melanotic Schwannoma (MS) is rarely seen and potentially malignant neoplasm that is categorized as a variant of Schwannoma. MS most frequently involves intracranial structures followed by posterior nerve roots in the spinal canal. Approximately 50% of the cases with MS have psammomatous calcifications and this type of MS is related to Carney complex with autosomal dominant inheritance. Most cases of MS are benign, though 10% of them are malignant with metastatic potential. MS mimics melanoma and the differential diagnosis should be made excluding other melanin producing neoplasms especially melanoma. Case 1 . A 42-year-old hypertensive male presented for checkup. He had a well-defined extraspinal oval lesion measuring 3.5 × 2.5 cm near right adrenal. Case 2 . A 22-year-old female presented with neurofibromatosis-2, bilateral acoustic schwannomas and café au lait lesions on sacrococcygeal region. She had an intradural extramedullary lesion measuring 6.1 × 2.0 cm at L1-2 level. MS is a rare neoplasm composed of Schwann cells and melanin pigment. These tumors are usually benign but they may become aggressive. The biologic behavior of MS is difficult to predict; the patients have to be followed up for a longer period due to its malignant potential.

  12. Rare cancers in children - The EXPeRT Initiative: a report from the European Cooperative Study Group on Pediatric Rare Tumors.

    Science.gov (United States)

    Bisogno, G; Ferrari, A; Bien, E; Brecht, I B; Brennan, B; Cecchetto, G; Godzinski, J; Orbach, D; Reguerre, Y; Stachowicz-Stencel, T; Schneider, D T

    2012-10-01

    The low incidence and the heterogeneity of very rare tumors (VRTs) demand for international cooperation. In 2008, EXPeRT (European Cooperative Study Group for Pediatric Rare Tumors) was founded by national groups from Italy, France, United Kingdom, Poland and Germany. The first aims of EXPeRT were to agree on a uniform definition of VRTs and to develop the currently most relevant scientific questions. Current initiatives include international data exchange, retrospective and prospective studies of specific entities, and the development of harmonized and internationally recognized guidelines. Moreover, EXPeRT established a network for expert consultation to assist in clinical decision in VRTs. © Georg Thieme Verlag KG Stuttgart · New York.

  13. Primary (Poorly Differentiated Sclerosing Liposarcoma of Temporal Region. An Uncommon Tumor in a Rare Site: A Case Report

    Directory of Open Access Journals (Sweden)

    Anuradha CK Rao

    2015-02-01

    Full Text Available Liposarcoma (LS in the head and neck region is a rare tumor. The sclerosing variant of LS is a subtype of well-differentiated LS characterized by areas of conventional LS admixed with hypocellular areas of stromal sclerosis that show atypical lipomatous cells. The (poorly differentiated sclerosing LS, on the other hand, is more cellular with atypical, pleomorphic and often bizarre giant tumor cells admixed with atypical lipoblasts. We report a case of poorly differentiated sclerosing LS of temporal region in a 49-year-old man. Radiologically, the tumor was dumbbell shaped with intra and extra cranial extension. In this case, we discuss the clinico-radiological and pathological findings of an unusual tumor in a rare location. [J Interdiscipl Histopathol 2015; 3(1.000: 33-35

  14. A squamous odontogenic tumor following an orthodontic micro-screw: A rare case report and review of the literature.

    Science.gov (United States)

    Tu, Jingqiu; Yin, Ping; Yuan, Yunyi; Chen, Jie; Yuan, Yongxiang; Lei, Yonghua

    2018-02-01

    We reported a very rare case of squamous odontogenic tumor(SOT) in a 23-year-old female. The tumor arose after an implanting operation of an orthodontic micro-screw, and was definitely diagnosed by the histopathological examination. Based on the case report and a review of the literature, we discussed about the general features, differential diagnosis and pathogenesis of SOT. Copyright © 2017 Elsevier Ltd. All rights reserved.

  15. Polyostotic fibrous dysplasia with gigantism and huge pelvic tumor: a rare case of McCune-Albright syndrome.

    Science.gov (United States)

    Sakayama, Kenshi; Sugawara, Yoshifumi; Kidani, Teruki; Fujibuchi, Taketsugu; Kito, Katsumi; Tanji, Nozomu; Nakamura, Atsushi

    2011-06-01

    We report a rare case of polyostotic fibrous dysplasia on endocrine hyperfunction with elevated human growth hormone and normal serum level of prolactin. There were some differential points of gender, gigantism, endocrine function, and GNAS gene from McCune-Albright syndrome. Malignant transformation was suspected in the pelvic tumor from imaging because rapid growth of the tumor by imaging was observed; however, no malignant change occurred in this case.

  16. Irradiated large segment allografts in limb saving surgery for extremity tumor - Philippine experience

    International Nuclear Information System (INIS)

    Wang, E.H.M.; Agcaoili, N.; Turqueza, M.S.

    1999-01-01

    Limb saving surgery has only recently become an option in the Phillipines. This has given a better comprehension of oncologic principles and from the refinement of bone-reconstruction procedures. Foremost among the latter is the use of large segment bone allografts. Large-segment allografts (LSA) are available from the Tissue and Bone Bank of the University of the Philippines (UP). After harvest, these bones are processed at the Bank, radiation-sterilized at the Philippine Nuclear Research Institute, and then stored in a -80 degree C deep freezer. We present our 4-year experience (Jan 93 - Dec 96) with LSA for limb saving surgery in musculoskeletal tumors. All patients included had: (1) malignant or aggressive extremity tumors; (2) surgery performed by the UP - Musculoskeletal Tumor Unit (UP-MUST Unit); (3) reconstructions utilizing irradiated large-segment allografts from the UP Tissue and Bone Bank; and (4) follow-up of at least one year or until death. Tumors included osteosarcoma (6) giant cell tumors (11), and metastatic lesions (3). Age ranged from 16-64 years old; 13 males and 7 females. Bones involved were the femur (12) tibia (5) and humerus (3). Average defect length was 15 cm and surgeries performed were intercalary replacement (5), resection arthrodesis (11), hemicondylar allograft (3), and allograft-prosthesis-composite (1). Follow-up ranged was from 17- 60 months or until death. Fifteen (1 5) were alive with NED (no evidence of disease), 3 were dead (2 of disease 1 of other causes), and 2 were AWED (alive with evidence of disease). Functional evaluation using the criteria of the International Society of Limb Salvage (ISOLS) was performed on 18 patients. This averaged 27.5 out of 30 points (92%) for 15 patients. Many having returned to their previous work and recreation. The 3 failures were due to infections in 2 cases (both of whom opted for amputations but who have not been fit with prostheses), and a fracture (secondary to a fall) in one case. Limb

  17. 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.

  18. Large cell/anaplastic medulloblastoma with myogenic, melanotic and neuronal differentiation: A case report of a rare tumor

    Directory of Open Access Journals (Sweden)

    Amany A. Fathaddin

    2014-01-01

    Full Text Available Medulloblastoma is an embryonal neuroepithelial tumor of the cerebellum and is the most common malignant central nervous system tumor in children. Different histological variants and patterns have been described. The classic variant represents the majority of cases. This report describes a rare case of large cell/anaplastic medulloblastoma with myogenic, melanotic and neuronal differentiation arising in the cerebellum of a 3-year-old boy who presented with headache and vomiting. Magnetic resonance imaging demonstrated a heterogeneously enhanced lesion in the fourth ventricle. Surgical resection of the tumor was accomplished, but a residual tumor was left behind because of the involvement of the brainstem. Postoperatively, the patient received chemotherapy and radiotherapy. Currently, 20 months after treatment, the patient has survived without further progression. Pathological examination revealed a high grade primitive neuronal tumor with foci of myogenic features, melanin containing epithelial elements and ganglion-like cells, which were confirmed by immunohistochemistry.

  19. Single coronary artery; extremely rare coronary anomaly successfully treated surgically in young adult male.

    LENUS (Irish Health Repository)

    Shah, A R

    2010-05-01

    Single coronary artery arising from aortic root, is a rare congenital anomaly. A 30-year-old male presented with acute myocardial infarction (MI) complaining of chest pain and raised troponin levels. Emergency angiography showed no coronary lesions but both left and right coronary arteries arising from single ostium. Patient was operated electively and perioperative findings confirmed the diagnosis of single coronary artery, as left coronary artery after taking origin from right sinus of valsalva runs through the septum, before dividing into left anterior descending and circumflex branches. The single coronary ostium opened with a slit like incision over the course of left main coronary, making the size of ostium three to four times bigger than the native one. In addition left internal mammary artery was harvested and grafted to the left anterior descending branch distally. Patient made successful recovery. Four months follow up dobutamine stress echo showed no inducible ischemia.

  20. Gynecomastia during imatinib mesylate treatment for gastrointestinal stromal tumor: a rare adverse event

    Directory of Open Access Journals (Sweden)

    Yan ZhongShu

    2011-11-01

    Full Text Available Abstract Background Imatinib mesylate has been the standard therapeutic treatment for chronic myeloid leukemia, advanced and metastatic gastrointestinal stromal tumor (GIST. It is well tolerated with mild adverse effects. Gynecomastia development during the course of treatment has been rarely reported. Methods Ninety-eight patients with advanced or recurrent GIST were treated with imatinib mesylate. Among the fifty-seven male patients six developed gynecomastia during the treatment. The lesions were confirmed by sonography. Sex hormone levels were determined in six patients with and without the presence of gynecomastia respectively. The patients with gynecomatia were treated with tamoxifene and the sex hormones were assayed before and after tamoxifene treatment. Results In patients with gynecomastia the lump underneath the bilateral nipples was 2.5 to 5 centimeters in diameter. Their serum free testosterone levels ranged between 356.61 and 574.60 ng/dl with a mean ± SD of 408.64 ± 82.06 ng/dl (95% CI 343.03~474.25 ng/dl, which is within the normal range. The level of serum estradiol was 42.89 ± 16.54 pg/ml (95% CI 29.66~56.12 pg/ml. Three patients had higher levels (43.79~71.21 pg/ml and the others' were within normal range of 27.00~34.91 pg/ml. Six patients without the development of gynecomastia had normal free testosterone. One patient died because of large tumor burden. The sex hormones had no significant changes before and after tamoxifene treatment.(P > 0.05 Conclusions Testosterone levels were not decreased in the six GIST patients with gynecomastia. Three patients had increased serum estradiol level which suggests that imbalance of sex hormones may be the cause of gynecomastia during treatment with imatinib mesylate.

  1. An Extremely Rare Case of Lower Urinary Tract Symptoms: Floating Benign Mesenchymal Mass in Abdomen

    Directory of Open Access Journals (Sweden)

    Bulent Kati

    2017-01-01

    Full Text Available A 48-year-old man admitted to the urology outpatient clinic with major symptoms of right-side pain and intermittent lower urinary tract symptoms (LUTSs such as low urine flow rate, dysuria, and frequency. Uroflowmetry showed low urine flow, and laboratory tests revealed no pathology. Ultrasound (US showed a 7 cm calcific mass above the bladder and a kidney cyst with a diameter of 5.3 cm in the upper pole of the right kidney. Enhanced computed tomography confirmed the US findings. Laparoscopic transperitoneal renal cyst decortication was performed. There was no sign of additional tumors. An independent mass in the abdomen was diagnosed, and the mass was removed. Based on the pathology, the diagnosis was a benign mesenchymal calcific mass. This is the first report of LUTSs due to a free benign mesenchymal mass in the published literature.

  2. A Label Free Disposable Device for Rapid Isolation of Rare Tumor Cells from Blood by Ultrasounds

    Directory of Open Access Journals (Sweden)

    Itziar González

    2018-03-01

    Full Text Available The use of blood samples as liquid biopsy is a label-free method for cancer diagnosis that offers benefits over traditional invasive biopsy techniques. Cell sorting by acoustic waves offers a means to separate rare cells from blood samples based on their physical properties in a label-free, contactless and biocompatible manner. Herein, we describe a flow-through separation approach that provides an efficient separation of tumor cells (TCs from white blood cells (WBCs in a microfluidic device, “THINUS-Chip” (Thin-Ultrasonic-Separator-Chip, actuated by ultrasounds. We introduce for the first time the concept of plate acoustic waves (PAW applied to acoustophoresis as a new strategy. It lies in the geometrical chip design: different to other microseparators based on either bulk acoustic waves (BAW or surface waves (SAW, SSAW and tSAW, it allows the use of polymeric materials without restrictions in the frequency of work. We demonstrate its ability to perform high-throughput isolation of TCs from WBCs, allowing a recovery rate of 84% ± 8% of TCs with a purity higher than 80% and combined viability of 85% at a flow rate of 80 μL/min (4.8 mL/h. The THINUS-Chip performs cell fractionation with low-cost manufacturing processes, opening the door to possible easy printing fabrication.

  3. MRI Findings of Causalgia of the Lower Extremity Following Transsphenoidal Resection of Pituitary Tumor

    Directory of Open Access Journals (Sweden)

    D. Ryan Ormond

    2012-01-01

    Full Text Available Background. Causalgia is continuing pain, allodynia, or hyperalgesia after nerve injury with edema, changes in skin blood flow, or abnormal sudomotor activity. Here we report a case of lower extremity causalgia following elective transsphenoidal resection of a pituitary tumor in a young man. Clinical Presentation. A 33-year-old man with acromegaly underwent elective sublabial transsphenoidal resection of his pituitary tumor. During the three-hour surgery, the lower limbs were kept in a supine, neutral position with a pillow under the knees. The right thigh was slightly internally rotated with a tape to expose fascia lata, which was harvested to repair the sella. Postoperatively, he developed causalgia in a distal sciatic and common peroneal nerve distribution. Pain was refractory to several interventions. Finally, phenoxybenzamine improved his pain significantly. Conclusions. Malpositioning in the operating room resulted in causalgia in this young man. Phenoxybenzamine improved, and ultimately resolved, his symptoms. Improvement in his pain symptoms correlated with resolution of imaging changes in the distal sciatic and peroneal nerves on the side of injury.

  4. Violence is Rare in Autism: When It Does Occur, Is It Sometimes Extreme?

    Science.gov (United States)

    Allely, C S; Wilson, P; Minnis, H; Thompson, L; Yaksic, E; Gillberg, C

    2017-01-02

    A small body of literature has suggested that, rather than being more likely to engage in offending or violent behavior, individuals with autism spectrum disorder (ASD) may actually have an increased risk of being the victim rather than the perpetrator of violence (Sobsey, Wells, Lucardie, & Mansell, 1995 ). There is no evidence that people with ASD are more violent than those without ASD (Im, 2016). There is nevertheless a small subgroup of individuals with ASD who exhibit violent offending behaviours and our previous work has suggested that other factors, such as adverse childhood experiences, might be important in this subgroup (Allely, Minnis, Thompson, Wilson, & Gillberg, 2014 ). Fitzgerald ( 2015 ) highlights that school shootings and mass killings are not uncommonly carried out by individuals with neurodevelopmental disorders, with frequent evidence of warning indicators. The aim of the present review is to investigate this in more detail using the 73 mass shooting events identified by Mother Jones (motherjones.com) in their database for potential ASD features. There are 73 mass shooting events but there are two events where there is a pair of shooters which meant that 75 mass shooter cases were investigated. This exercise tentatively suggests evidence of ASD in six of 75 included cases (8%) which is about eight times higher when compared to the prevalence of ASD found in the general population worldwide (motherjones.com). The 8% figure for individuals with ASD involved mass killings is a conservative estimate. In addition to the six cases which provide the 8% figure, there were 16 other cases with some indication of ASD. Crucially, ASD may influence, but does not cause, an individual to commit extreme violent acts such as a mass shooting episode.

  5. Cross-cultural adaptation and validation of the Japanese version of the Toronto Extremity Salvage Score (TESS) for patients with malignant musculoskeletal tumors in the upper extremities.

    Science.gov (United States)

    Akiyama, Toru; Uehara, Kosuke; Ogura, Koichi; Shinoda, Yusuke; Iwata, Shintaro; Saita, Kazuo; Tanzawa, Yoshikazu; Nakatani, Fumihiko; Yonemoto, Tsukasa; Kawano, Hirotaka; Davis, Aileen M; Kawai, Akira

    2017-01-01

    The Toronto Extremity Salvage Score (TESS) is a widely used disease-specific patient-completed questionnaire for the assessment of physical function in patients with musculoskeletal tumors; however, there had not been the validated Japanese version of the TESS. The aim of this study was to validate the Japanese version of the TESS in patients with musculoskeletal tumors in the upper extremity. After developing a Japanese version of the TESS, the questionnaire was administered to 53 patients to examine its reliability and validity in comparison with the Musculoskeletal Tumor Society (MSTS) scoring system and Short Form-36 (SF-36). Test-retest reliability with intraclass correlation coefficient (0.93) and internal consistency with Cronbach's alpha (0.90) were excellent. Factor analysis showed that the construct structure consisted of 3-item clusters, and the Akaike Information Criterion network also demonstrated that the items could be divided into 3 domains according to their content. The TESS strongly correlated with the MSTS rating scale (r = 0.750; P TESS had low correlations with the SF-36 mental health and role-emotional subscales and the MSTS scoring system manual dexterity domain. Our study suggests that the TESS is a reliable and valid instrument to measure patient-reported physical functioning in patients with upper extremity sarcoma. Copyright © 2016 The Japanese Orthopaedic Association. Published by Elsevier B.V. All rights reserved.

  6. Tree species traits but not diversity mitigate stem breakage in a subtropical forest following a rare and extreme ice storm.

    Directory of Open Access Journals (Sweden)

    Karin Nadrowski

    Full Text Available Future climates are likely to include extreme events, which in turn have great impacts on ecological systems. In this study, we investigated possible effects that could mitigate stem breakage caused by a rare and extreme ice storm in a Chinese subtropical forest across a gradient of forest diversity. We used Bayesian modeling to correct stem breakage for tree size and variance components analysis to quantify the influence of taxon, leaf and wood functional traits, and stand level properties on the probability of stem breakage. We show that the taxon explained four times more variance in individual stem breakage than did stand level properties; trees with higher specific leaf area (SLA were less susceptible to breakage. However, a large part of the variation at the taxon scale remained unexplained, implying that unmeasured or undefined traits could be used to predict damage caused by ice storms. When aggregated at the plot level, functional diversity and wood density increased after the ice storm. We suggest that for the adaption of forest management to climate change, much can still be learned from looking at functional traits at the taxon level.

  7. A rare case report of an adenomatoid odontogenic tumor associated with odontoma in the maxilla

    Directory of Open Access Journals (Sweden)

    Agnes Assao

    2017-01-01

    Conclusions: Therefore, it is necessary similar cases to be published to increase the knowledge about the clinical behavior and evolution of this tumor, to enable such lesions to be more clearly defined in the next classification of odontogenic tumors.

  8. Neurothekeoma of petrous apex: A rare entity

    Directory of Open Access Journals (Sweden)

    Zarina Abdul Assis

    2013-01-01

    Full Text Available Intraosseous nerve sheath tumors are very rare tumors accounting for lesser than 0.2% of primary bone tumors. We present an 18-year-old female who presented with left facial paresis for the last 1 year. Magnetic resonance imaging (MRI demonstrated expansile, multiseptated, enhancing bony lesion in the left petrous apex. There was also abnormal enhancement of the 7-8 th nerve complex within the internal auditory canal. Tumor was excised by subtemporal extradural approach. The lesion was diagnosed as intraosseous neurothekeoma on histopathology. This is an extremely rare tumor and its MRI appearance in this location is being described for the first time in literature.

  9. A Hard Ball for a Tennis Player: A Rare Case of Large Calcifying Sertoli Cell Testicular Tumor

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    Simone Albisinni

    2017-07-01

    Full Text Available A 46 year old tennis player was addressed to our clinic after incidental finding of right testicular calcification on plain x-ray of the spine. Urologic consultation revealed a hard non-tender testicular mass which required inguinal orchiectomy. Final histology revealed large cell calcifying Sertoli cell tumor: we herein present the case and review current physiopathology of such rare testicular disease.

  10. Exposure of tumor-bearing mice to extremely high-frequency electromagnetic radiation modifies the composition of fatty acids in thymocytes and tumor tissue.

    Science.gov (United States)

    Gapeyev, Andrew B; Kulagina, Tatiana P; Aripovsky, Alexander V

    2013-08-01

    To test the participation of fatty acids (FA) in antitumor effects of extremely high-frequency electromagnetic radiation (EHF EMR), the changes in the FA composition in the thymus, liver, blood plasma, muscle tissue, and tumor tissue in mice with Ehrlich solid carcinoma exposed to EHF EMR were studied. Normal and tumor-bearing mice were exposed to EHF EMR with effective parameters (42.2 GHz, 0.1 mW/cm2, 20 min daily during five consecutive days beginning the first day after the inoculation of tumor cells). Fatty acid composition of various organs and tissues of mice were determined using a gas chromatography. It was shown that the exposure of normal mice to EHF EMR or tumor growth significantly increased the content of monounsaturated FA (MUFA) and decreased the content of polyunsaturated FA (PUFA) in all tissues examined. Exposure of tumor-bearing mice to EHF EMR led to the recovery of FA composition in thymocytes to the state that is typical for normal animals. In other tissues of tumor-bearing mice, the exposure to EHF EMR did not induce considerable changes that would be significantly distinguished between disturbances caused by EHF EMR exposure or tumor growth separately. In tumor tissue which is characterized by elevated level of MUFA, the exposure to EHF EMR significantly decreased the summary content of MUFA and increased the summary content of PUFA. The recovery of the FA composition in thymocytes and the modification of the FA composition in the tumor under the influence of EHF EMR on tumor-bearing animals may have crucial importance for elucidating the mechanisms of antitumor effects of the electromagnetic radiation.

  11. Brown tumor in mandible as a first sign of vitamin D deficiency: A rare case report and review

    Directory of Open Access Journals (Sweden)

    K V Arunkumar

    2012-01-01

    Full Text Available Central giant cell granulomas (CGCGs are uncommon but the most aggressive benign intraosseous tumors of jaws, with an unpredictable outcome. They account for less than 7% of all benign jaw lesions, with a female to male ratio of about 2:1. The classical "brown tumor" is commonly seen in the long bones, pelvis, and ribs. Facial bone involvement is rare and usually appears as solitary or multilocular soap bubble like radiolucencies. CGCGs are traditionally treated by both surgical and intralesional injection, with a variable recurrence rate. Here, we report a 12-year-old female patient with mandibular brown tumor as a first sign of secondary hyperthyroidism induced due to vitamin D deficiency and hypocalcemia.

  12. Frontal bone hemangioma in an 8-year-old female: A common tumor in a rare location

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    Abhimanyu Sharma

    2016-01-01

    Full Text Available Intraosseous hemangioma is a rare bone tumor accounting for 0.7%–1.0% of all bone tumors. In the skull, frontal bone is the commonly involved bone. An 8-year-old female presented to our outpatient department with complaints of pain and swelling over forehead for 4 months. X-ray revealed a lytic expansile lesion involving frontal bone with sunburst pattern of bony spicules radiating to periphery of the lesion. Magnetic resonance imaging revealed the presence of a well-circumscribed lesion with both intra as well as extracranial components. Histopathology revealed a vascular tumor consisting of both small (capillary and large (cavernous sized vessels. A diagnosis of mixed type of hemangioma of the frontal bone was given. Recognition of hemangioma on radiology and confirmation by histopathology is essential for proper management as it might be confused clinically with other locally aggressive/malignant lesions.

  13. Primitive neuroectodermal tumor of the zygomaticoorbital complex: a rare location and ways of surgical repair of the area

    Directory of Open Access Journals (Sweden)

    Ch. R. Ragimov

    2015-01-01

    Full Text Available Primitive neuroectodermal tumor in the zygomaticoorbital region is a rare neoplasm of the head and neck. Due to the necessity for wide radical excision of a primary tumor, there may be serious functional and cosmetic disorders that substantially affect quality of life in patients. Restoration of this region is one of the challenges of reconstructive surgery because of the specific features of the relief of bone structures. The paper describes a clinical case of the site of primitive neuroectodermal tumor in the zygomaticoorbital complex and a method for repairing postresectional defect and completely recovering the function of the organ of vision and aesthetic parameters of the face.

  14. Probability estimation of rare extreme events in the case of small samples: Technique and examples of analysis of earthquake catalogs

    Science.gov (United States)

    Pisarenko, V. F.; Rodkin, M. V.; Rukavishnikova, T. A.

    2017-11-01

    The most general approach to studying the recurrence law in the area of the rare largest events is associated with the use of limit law theorems of the theory of extreme values. In this paper, we use the Generalized Pareto Distribution (GPD). The unknown GPD parameters are typically determined by the method of maximal likelihood (ML). However, the ML estimation is only optimal for the case of fairly large samples (>200-300), whereas in many practical important cases, there are only dozens of large events. It is shown that in the case of a small number of events, the highest accuracy in the case of using the GPD is provided by the method of quantiles (MQs). In order to illustrate the obtained methodical results, we have formed the compiled data sets characterizing the tails of the distributions for typical subduction zones, regions of intracontinental seismicity, and for the zones of midoceanic (MO) ridges. This approach paves the way for designing a new method for seismic risk assessment. Here, instead of the unstable characteristics—the uppermost possible magnitude M max—it is recommended to use the quantiles of the distribution of random maxima for a future time interval. The results of calculating such quantiles are presented.

  15. Czaszkogardlak – rzadki łagodny guz nowotworowy - opis przypadku = Craniopharyngioma – rare benign tumor – case report

    Directory of Open Access Journals (Sweden)

    Kamila Woźniak

    2016-08-01

    • 2.        Katedra i Klinika Rehabilitacji, Collegium Medicum im. Ludwika Rydygiera w Bydgoszczy, Uniwersytet Mikołaja Kopernika w Toruniu     Streszczenie Czaszkogardlak (guz Erdheima, łac. craniopharyngioma to stosunkowo rzadki, łagodny guz nowotworowy o lokalizacji wewnątrzczaszkowej, wywodzący się z pozostałości kieszonki Rathkego. Nazwę craniopharyngioma wprowadził Harvey Cushing w 1932. Jedne z pierwszych prób operacyjnego leczenia guza podjął Ludvig Puusepp w 1923 roku. Objawy kliniczne zależą od lokalizacji guza. Są to przede wszystkim objawy wynikające z ucisku masy guza na sąsiadujące struktury anatomiczne. Autorzy przedstawiają opis przypadku klinicznego, leczonego operacyjnie w Klinice Neurochirurgii, Neurotraumatologii i Neurochirurgii Dziecięcej Szpitala Uniwersyteckiego nr 1 im. dr A. Jurasza w Bydgoszczy z rozpoznanym w badaniu histopatologicznym craniopharyngioma.   Słowa kluczowe: czaszkogardlak, craniopharyngioma, guz Erdheima, nowotwór łagodny.     Abstract Craniopharyngioma (Erdheima tumor is a relatively rare benign tumor of intracranial location, derived from the remnants of Rathke's pouch. The name craniopharyngioma introduced Harvey Cushing in 1932. One of the first attempts at surgical treatment of the tumor took Ludvig Puusepp in 1923. Clinical symptoms depend on the site of a tumor. These are primarily the symptoms resulting from the comppression of the tumor mass on adjacent anatomical structures. The authors present a clinical case operating treated in the Department of Neurosurgery, Neurotraumatology and Pediatric Neurosurgery, Dr Antoni Jurasz University Hospital No.1 in Bydgoszcz histopathological diagnosed with craniopharyngoma.   Key words: craniopharyngioma, Erdheima tumor, benign tumor.

  16. Cystadenoma: a rare tumor originated in minor salivary gland Cistadenoma: um tumor raro em glândula salivar menor

    Directory of Open Access Journals (Sweden)

    Jean Nunes dos Santos

    2008-06-01

    Full Text Available Cystadenoma of salivary glands is an uncommon benign neoplasm that presents intraluminal papillary projections. The authors describe one case of cystadenoma located in the buccal mucosa and highlight its histomorphological features and differential diagnosis.O cistadenoma de glândula salivar é uma neoplasia benigna incomum, que exibe projeções papilíferas intraluminais. Os autores descrevem um caso de cistadenoma localizado na mucosa jugal, discutindo os aspectos histomorfológicos e o diagnóstico diferencial desse tumor.

  17. Renal tumor leading to acute respiratory distress syndrome – a rare ...

    African Journals Online (AJOL)

    Arun Kumar Agnihotri

    renal cell carcinoma (RCC). KEY WORDS: ARDS; Renal tumor; Adult respiratory distress syndrome. INTRODUCTIONᴪ. ARDS due to ... unable to maintain saturation in spite of high flow ... Blood investigations showed mild leukocytosis.

  18. Cross-cultural adaptation and validation of the Japanese version of the Toronto Extremity Salvage Score (TESS) for patients with malignant musculoskeletal tumors in the lower extremities.

    Science.gov (United States)

    Ogura, Koichi; Uehara, Kosuke; Akiyama, Toru; Iwata, Shintaro; Shinoda, Yusuke; Kobayashi, Eisuke; Saita, Kazuo; Yonemoto, Tsukasa; Kawano, Hirotaka; Chuman, Hirokazu; Davis, Aileen M; Kawai, Akira

    2015-11-01

    Before this work a Japanese version of the Toronto Extremity Salvage Score (TESS), a disease-specific patient-completed questionnaire widely used to assess the physical function of patients with musculoskeletal tumors, had not been developed. The purpose of this study was cross-cultural adaptation and validation of the English-language version of the TESS to facilitate international comparisons of treatment results. The TESS was translated into Japanese, back-translated into English, and reviewed by a committee to develop a consensus Japanese version of the TESS. One hundred and two patients were assessed by use of this Japanese version to examine its reliability and validity. Test-retest reliability and internal consistency determined by using the intraclass correlation coefficient (0.941) and Cronbach's alpha test (0.978), respectively, were excellent. Factor analysis showed that the structure consisted of a three-item cluster; the Akaike information criterion (AIC) network also demonstrated that the items could be divided into three domains in accordance with their content. The Japanese version of the TESS correlated with the Musculoskeletal Tumor Society rating scale (r = 0.811; P TESS is a reliable and valid instrument for measuring patient-reported functional outcome for patients with lower extremity sarcoma, and that it enables international comparisons of treatment results. The spatial association of each item demonstrated by using the AIC network also suggested that the underlying structure of the TESS reflected its coverage of a wide range of physical functions.

  19. Value of computerized tomography in the diagnosis of bone and musculoskeletal system tumors of the pelvis and extremities

    International Nuclear Information System (INIS)

    Langer, R.; Langer, M.

    1983-01-01

    49 cases of tumors of the musculoskeletal system of the pelvis and the extremities have been investigated by computerized tomography. The CT was performed only after conventional radiologic methods. The results are discussed and compared with those described in the literature. The advantages and disadvantages of CT of musculoskeletal disorders are discussed. We consider the CT to be of value in disorders of the musculoskeletal system, specially, if operative- or radiotherapy is planned. (orig.) [de

  20. A Patient with Advanced Gastric Cancer Presenting with Extremely Large Uterine Fibroid Tumor

    Directory of Open Access Journals (Sweden)

    Kwang-Kuk Park

    2014-01-01

    Full Text Available Introduction. Uterine fibroid tumors (uterine leiomyomas are the most common benign uterine tumors. The incidence of uterine fibroid tumors increases in older women and may occur in more than 30% of women aged 40 to 60. Many uterine fibroid tumors are asymptomatic and are diagnosed incidentally. Case Presentation. A 44-year-old woman was admitted to our hospital with general weakness, dyspepsia, abdominal distension, and a palpable abdominal mass. An abdominal computed tomography scan showed a huge tumor mass in the abdomen which was compressing the intestine and urinary bladder. Gastroduodenal endoscopic and biopsy results showed a Borrmann type IV gastric adenocarcinoma. The patient was diagnosed with gastric cancer with disseminated peritoneal carcinomatosis. She underwent a hysterectomy with both salphingo-oophorectomy and bypass gastrojejunostomy. Simultaneous uterine fibroid tumor with other malignancies is generally observed without resection. But in this case, a surgical resection was required to resolve an intestinal obstruction and to exclude the possibility of a metastatic tumor. Conclusion. When a large pelvic or ovarian mass is detected in gastrointestinal malignancy patients, physicians try to exclude the presence of a Krukenberg tumor. If the tumors cause certain symptoms, surgical resection is recommended to resolve symptoms and to exclude a metastatic tumor.

  1. Benign neuroendocrine and other rare benign tumors of the pancreas; Benigne neuroendokrine und andere seltene benigne Tumoren des Pankreas

    Energy Technology Data Exchange (ETDEWEB)

    Happel, B.; Ba-Ssalamah, A. [Medizinische Universitaet Wien, Universitaetsklinik fuer Radiodiagnostik, Wien (Austria); Niederle, B. [Medizinische Universitaet Wien, Universitaetsklinik fuer Chirurgie, Wien (Austria); Puespoek, A. [Medizinische Universitaet Wien, Klinische Abteilung fuer Gastroenterologie und Hepatologie, Universitaetsklinik fuer Innere Medizin 3, Wien (Austria); Schima, W. [KH Goettlicher Heiland, Abteilung fuer Radiologie und Bildgebende Diagnostik, Wien (Austria)

    2008-08-15

    Neuroendocrine tumors (NET) of the pancreas are rare neoplasms, which arise from cells of the islets of Langerhans. The most common NET are the insulinoma, gastrinoma and hormone inactive NET. Very rare entities are the schwannoma, leiomyoma, teratoma, intrapancreatic lipoma, hemangioma and the intrapancreatic accessory spleen. Essential for therapy, which in most cases is difficult, are an exact localization and various modalities of imaging diagnostics. (orig.) [German] Neuroendokrine Tumoren (NET) des Pankreas sind seltene Neoplasien, die aus Zellen der Langerhans-Inseln entstehen. Zu den haeufigsten NET zaehlen Insulinome, Gastrinome und hormoninaktive NET. Als sehr selten auftretende Entitaeten sind das Schwannom, Leiomyom, Teratom, intrapankreatische Lipom, Haemangiom sowie die intrapankreatische Nebenmilz zu nennen. Fuer die Therapie sind die exakte Lokalisation und verschiedene Modalitaeten der bildgebenden Diagnostik, die sich in aller Regel schwierig gestaltet, essenziell. (orig.)

  2. Hyalinizing trabecular tumor of the thyroid: Diagnosed of a rare tumor using ultrasonography, cytology, and intraoperative frozen sections

    Energy Technology Data Exchange (ETDEWEB)

    Jang, Hyun Sik; Kim, Eun Kyung; Kwak, Jin Young; Moon, Hee Jung; Yoon, Jung Hyun [Dept. of Radiology, Severance Hospital, Research Institute of Radiological Science, Yonsei University, College of Medicine, Seoul (Korea, Republic of); Park, Cheol Keun; Son, Eun Ju [Gangnam Severance Hospital, Yonsei University, College of Medicine, Seoul (Korea, Republic of)

    2016-03-15

    The goal of this study was to evaluate the clinicopathological and imaging features of thyroid nodules surgically diagnosed as hyaline trabecular tumor (HTT), and to assess the role of cytology and frozen sections (FS) in the diagnosis of HTT. This study included 21 thyroid nodules in 21 patients treated from August 2005 to March 2015 (mean age, 53.3 years) who were either diagnosed as HTT or had HTT suggested as a possible diagnosis based on cytology, FS, or the final pathology report. Patients' medical records were retrospectively reviewed for cytopathologic results and outcomes during the course of follow-up. Sonograms were reviewed and categorized. Twelve nodules from 12 patients were surgically confirmed as HTT. Ultrasonography (US)-guided fine needle aspiration (FNA) was performed on 11 nodules, of which six (54.5%) were papillary thyroid carcinoma (PTC) or suspicious for PTC and three (27.3%) were HTT or suspicious for HTT. Intraoperative FS suggested the possibility of HTT in seven nodules, of which four (57.1%) were confirmed as HTT. US-FNA suggested the diagnosis of HTT in 10 nodules, of which three (30.0%) were confirmed as HTT. Common US features of the 12 pathologically confirmed cases of HTT were hypoechogenicity or marked hypoechogenicity (83.4%), absence of calcifications (91.7%), parallel shape (100.0%), presence of vascularity (75.0%), and probable benignity (58.3%). HTT should be included in the differential diagnosis of solid tumors with hypoechogenicity or marked hypoechogenicity and otherwise benign US features that have been diagnosed as PTC through cytology.

  3. Hyalinizing trabecular tumor of the thyroid: Diagnosed of a rare tumor using ultrasonography, cytology, and intraoperative frozen sections

    International Nuclear Information System (INIS)

    Jang, Hyun Sik; Kim, Eun Kyung; Kwak, Jin Young; Moon, Hee Jung; Yoon, Jung Hyun; Park, Cheol Keun; Son, Eun Ju

    2016-01-01

    The goal of this study was to evaluate the clinicopathological and imaging features of thyroid nodules surgically diagnosed as hyaline trabecular tumor (HTT), and to assess the role of cytology and frozen sections (FS) in the diagnosis of HTT. This study included 21 thyroid nodules in 21 patients treated from August 2005 to March 2015 (mean age, 53.3 years) who were either diagnosed as HTT or had HTT suggested as a possible diagnosis based on cytology, FS, or the final pathology report. Patients' medical records were retrospectively reviewed for cytopathologic results and outcomes during the course of follow-up. Sonograms were reviewed and categorized. Twelve nodules from 12 patients were surgically confirmed as HTT. Ultrasonography (US)-guided fine needle aspiration (FNA) was performed on 11 nodules, of which six (54.5%) were papillary thyroid carcinoma (PTC) or suspicious for PTC and three (27.3%) were HTT or suspicious for HTT. Intraoperative FS suggested the possibility of HTT in seven nodules, of which four (57.1%) were confirmed as HTT. US-FNA suggested the diagnosis of HTT in 10 nodules, of which three (30.0%) were confirmed as HTT. Common US features of the 12 pathologically confirmed cases of HTT were hypoechogenicity or marked hypoechogenicity (83.4%), absence of calcifications (91.7%), parallel shape (100.0%), presence of vascularity (75.0%), and probable benignity (58.3%). HTT should be included in the differential diagnosis of solid tumors with hypoechogenicity or marked hypoechogenicity and otherwise benign US features that have been diagnosed as PTC through cytology

  4. Keratocystic odontogenic tumor of the right mandibular condyle: A rare case

    Directory of Open Access Journals (Sweden)

    Kamala Rawson

    2014-01-01

    Full Text Available Odontogenic keratocyst (OKC was first described by Hans Philipsen in 1956. The World Health Organization (WHO has designated OKC as a Keratocystic Odontogenic Tumor (KCOT. KCOT is defined as ′a benign uni- or multicystic, intraosseous tumor of odontogenic origin, with a characteristic lining of parakeratinized stratified squamous epithelium, with a potential for aggressive, infiltrative behavior′. Radiographically, most OKCs are unilocular, presenting a well-defined peripheral rim and a central cavity having satellite cysts. It is characterized by a thin fibrous capsule and a lining of keratinized stratified squamous epithelium, which is typically corrugated, usually about 6 to 10 cells in thickness, and generally without rete pegs. The present case report describes an unusual case of KCOT with minimal clinical presentation indicative of a tumor highlighting the potential difficulties in determining the diagnosis.

  5. A rare case of primary mesenteric gastrointestinal stromal tumor with metastasis to the cervix uteri

    Science.gov (United States)

    Gupta, Nupur; Mittal, Suneeta; Lal, Neena; Misra, Renu; Kumar, Lalit; Bhalla, Sunita

    2007-01-01

    Background Gastrointestinal stromal tumors are CD117 (C Kit) positive mesenchymal neoplasms, that may arise anywhere in the gastrointestinal tract. Their current therapy is imatinib mesylate before or after surgery. Case presentation We describe a case of 17-year-old female with metastasis to the cervix uteri of a primary mesenteric gastrointestinal tumor. Conclusion Surgery remains the mainstay of known curative treatment. The manifestations of GIST are not restricted to the typical locations within the bowel; may have very unusual metastatic sites or infiltrations per continuitatem. PMID:18045506

  6. Genetic Analysis of Multiple Endocrine Neoplasia Type 1 ( Leads to Misdiagnosis of an Extremely Rare Presentation of Intrasellar Cavernous Hemangioma as MEN1

    Directory of Open Access Journals (Sweden)

    Dong Min Lee

    2014-06-01

    Full Text Available BackgroundMultiple endocrine neoplasia type 1 (MEN1 is a rare inherited disorder characterized by the simultaneous occurrence of endocrine tumors in target tissues (mainly the pituitary, endocrine pancreas, and parathyroid glands. MEN1 is caused by mutations in the MEN1 gene, which functions as a tumor suppressor and consists of one untranslated exon and nine exons encoding the menin protein. This condition is usually suspected when we encounter patients diagnosed with tumors in multiple endocrine organs, as mentioned above.MethodsA 65-year-old woman who underwent surgery for a pancreatic tumor (serous cystadenoma 5 years previously was referred to our hospital due to neurologic symptoms of diplopia and left ptosis. Brain magnetic resonance imaging revealed a 3.4-cm lesion originating from the cavernous sinus wall and extending into the sellar region. It was thought to be a nonfunctioning tumor from the results of the combined pituitary function test. Incidentally, we found that she also had a pancreatic tumor, indicating the necessity of genetic analysis for MEN1.ResultsGenomic analysis using peripheral leukocytes revealed a heterozygous c.1621G>A mutation in the MEN1 gene that was previously reported to be either a pathogenic mutation or a simple polymorphism. We pursued a stereotactic approach to the pituitary lesion, and microscopic findings of the tumor revealed it to be an intrasellar cavernous hemangioma, a rare finding in the sellar region and even rarer in relation to oculomotor palsy. The patient recovered well from surgery, but refused further evaluation for the pancreatic lesion.ConclusionThere is great emphasis placed on genetic testing in the diagnosis of MEN1, but herein we report a case where it did not assist in diagnosis, hence, further discussion on the role of genetic testing in this disease is needed. Also, in cases of pituitary tumor with cranial nerve palsy, despite its low prevalence, intrasellar cavernous hemangioma

  7. A rare late complication after concomitant chemoradiation of an oropharyngeal tumor: cervical necrotizing fasciitis

    NARCIS (Netherlands)

    Karakullukçu, Barış; van der Vlies, Dalith; Hoebers, Frank J. P.

    2012-01-01

    Chemoradiation is increasingly being used to treat locally advanced head and neck carcinomas. Possible rare complications of this treatment modality have begun to appear, as the number of treated patients increase. In this report, we present a case who underwent chemoradiation due to T3N3M0 tonsil

  8. An autopsy report of a rare pediatric lung tumor: Pleuropulmonary blastoma

    Directory of Open Access Journals (Sweden)

    Gupta Kirti

    2008-04-01

    Full Text Available An autopsy report of pleuropulmonary blastoma (PPB is described in a two-and-a-half-year-old male child who died within a few days of starting chemotherapy. Autopsy revealed a large tumor almost occupying the whole of left hemithorax with widespread extension to pleura. The diagnosis was confirmed to be PPB, type III on autopsy.

  9. Small cell neuroendocrine carcinoma of the endometrium, a rare aggressive tumor

    International Nuclear Information System (INIS)

    Rajab, Khalil E.; Sandhu, Amarjit K.; Rajeswari, Mangla S.; Malik, A.

    2005-01-01

    This is a report of a young infertile woman with a history of 8 years amenorrhea, who presented with history of vaginal bleeding of 2 months duration. Investigations revealed a small cell neuroendocrine carcinoma of the endometrium, which penetrated half of the thickness of uterine wall. We have described the clinical progress and management of this rare and highly malignant cancer. A review of the pathological types and behavior of clear cell neuroendocrine carcinoma is presented. (author)

  10. Prevalence of Malignant Soft Tissue Tumors inExtremities: An Epidemiological Study in Syria

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    Habib Reshadi

    2014-06-01

    Full Text Available Background:   Although the majority of soft tissue masses are benign, it is important to consider malignancy in differential diagnoses. Because most soft tissue sarcomas present as a painless mass, clinicians must watch for signs suggestive of malignancy, including large size, rapid growth, and site deep into the deep fascia.The purpose of this study was to determine the relative prevalence according to sex and age, site of tumor, skeletal distribution, and treatment (surgery, chemotherapy and radiotherapy before and after surgery, and ascertain the relative frequency of these tumors in specific anatomic sites and age groups based on pathological studies. Methods: A total of 308 patients, with a musculoskeletal tumor were evaluated retrospectively. All of the patients enrolled into this study were referred to the Beirouni Hospital of Damascus University with a proven diagnosis of alignant soft tissue tumors from the beginning of January 2008 until the end of 2010. The prevalence of the malignant soft tissue tumors in these patients was analyzed. For purposes of analysis, all lesions were placed in 1 of 9 categories: hand and wrist, forearm, humorous (arm, proximal limb girdle (axilla and shoulder, foot and ankle, thigh, hip and buttocks region, trunk, and other lesions. Age and sex also were recorded. Results: Malignant tumors consisted of seven diagnostic categories: malignant fibrous histiocytoma (23%, liposarcoma (22%, rhabdomyosarcoma (9%, leiomyosarcoma (8%, malignant schwannoma (5%, dermatofibrosarcoma protuberans (5%, synovial sarcoma (10%, fibrosarcoma (13%, extraskeletal chondrosarcoma (1%, and extraskeletal Ewing sarcoma (4%. Conclusions: Despite the multitude of pathologic possibilities, most malignant soft-tissue tumors are classified into a small number of diagnoses. These may be further defined when the site of the lesion and the age of the patient are considered. Knowledge of tumor prevalence will assist radiologists in

  11. Prevalence of Malignant Soft Tissue Tumors inExtremities: An Epidemiological Study in Syria

    Directory of Open Access Journals (Sweden)

    Habib Reshadi

    2014-06-01

    Full Text Available Background:   Although the majority of soft tissue masses are benign, it is important to consider malignancy in differential diagnoses. Because most soft tissue sarcomas present as a painless mass, clinicians must watch for signs suggestive of malignancy, including large size, rapid growth, and site deep into the deep fascia.The purpose of this study was to determine the relative prevalence according to sex and age, site of tumor, skeletal distribution, and treatment (surgery, chemotherapy and radiotherapy before and after surgery, and ascertain the relative frequency of these tumors in specific anatomic sites and age groups based on pathological studies. Methods: A total of 308 patients, with a musculoskeletal tumor were evaluated retrospectively. All of the patients enrolled into this study were referred to the Beirouni Hospital of Damascus University with a proven diagnosis of alignant soft tissue tumors from the beginning of January 2008 until the end of 2010. The prevalence of the malignant soft tissue tumors in these patients was analyzed. For purposes of analysis, all lesions were placed in 1 of 9 categories: hand and wrist, forearm, humorous (arm, proximal limb girdle (axilla and shoulder, foot and ankle, thigh, hip and buttocks region, trunk, and other lesions. Age and sex also were recorded. Results: Malignant tumors consisted of seven diagnostic categories: malignant fibrous histiocytoma (23%, liposarcoma (22%, rhabdomyosarcoma (9%, leiomyosarcoma (8%, malignant schwannoma (5%, dermatofibrosarcoma protuberans (5%, synovial sarcoma (10%, fibrosarcoma (13%, extraskeletal chondrosarcoma (1%, and extraskeletal Ewing sarcoma (4%. Conclusions: Despite the multitude of pathologic possibilities, most malignant soft-tissue tumors are classified into a small number of diagnoses. These may be further defined when the site of the lesion and the age of the patient are considered. Knowledge of tumor prevalence will assist radiologists in

  12. Spre[ing amelanotic malignant melanoma: A rare differential diagnosis with tumors of the glandula submandibularis

    International Nuclear Information System (INIS)

    Ehrenberg, C.; Helmberger, H.

    1998-01-01

    The case reported emphasizes the importance of immediate performance of imaging scans in case of the slightest suspicion that clinical symptoms might indicate malignancy of a detected lesion. Despite the superficially only marginal macroscopic findings, MR imaging as well as the CT scans revealed an [vanced, malignant process that h[ been spre[ing. Particularly the soft tissue differential diagnosis obtained with MRI yields the information required for diagnostic characterization of the space occupying tumor mass. It will however be necessary in any case to verify the diagnosis by biopsy or extirpation and cytologic examination of tissue, as the imaging methods do not always unambigiously reveal the malignant dignity of the tumor. (orig./CB) [de

  13. A very rare spinal cord tumor primary spinal oligodendroglioma: A review of sixty cases in the literature

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    Askin Esen Hasturk

    2017-01-01

    Full Text Available Literature review. In this study, we evaluated a case of primary spinal oligodendroglioma (PSO with a rare localization between L3 and S2, and also examined sixty cases in the literature in terms of demographic characteristics, clinical, radiological, and histopathological characteristics, and treatment planning. A case of PSO has been presented, and the relevant literature between 1931 and 2016 was reviewed. A total of 57 papers regarding PSO were found and utilized in this review. The main treatment options include radical surgical excision with neuromonitoring, followed by radiotherapy. Despite these treatment protocols, the relapse rate is high, and treatment does not significantly prolong survival. Oligodendrogliomas are rare among the primary spinal cord tumors. Oligodendrogliomas are predominantly found in the cervical spinal cord, thoracic spinal cord, or junctions during childhood and adulthood. Extension to the sacral region, inferior to the Conus, is very rare. Furthermore, of the sixty cases in the literature, the case we present here is the first to be reported in this particular age group. These localizations usually occur in the pediatric age group and after relapses. While for a limited number of cases the oligodendroglioma initiates in the thoracic region and reaches as far as L2, we encountered a case of an oligodendroglioma within the range of L3 to S2. Clinical findings are observed in accordance with location, and magnetic resonance imaging is the gold standard for diagnosis.

  14. A rare case of an ACTH/CRH co-secreting midgut neuroendocrine tumor mimicking Cushing’s disease

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    Regina Streuli

    2017-06-01

    Full Text Available Ectopic ACTH/CRH co-secreting tumors are a very rare cause of Cushing’s syndrome and only a few cases have been reported in the literature. Differentiating between Cushing’s disease and ectopic Cushing’s syndrome may be particularly difficult if predominant ectopic CRH secretion leads to pituitary corticotroph hyperplasia that may mimic Cushing’s disease during dynamic testing with both dexamethasone and CRH as well as bilateral inferior petrosal sinus sampling (BIPSS. We present the case of a 24-year-old man diagnosed with ACTH-dependent Cushing’s syndrome caused by an ACTH/CRH co-secreting midgut NET. Both high-dose dexamethasone testing and BIPSS suggested Cushing’s disease. However, the clinical presentation with a rather rapid onset of cushingoid features, hyperpigmentation and hypokalemia led to the consideration of ectopic ACTH/CRH-secretion and prompted a further workup. Computed tomography (CT of the abdomen revealed a cecal mass which was identified as a predominantly CRH-secreting neuroendocrine tumor. To the best of our knowledge, this is the first reported case of an ACTH/CRH co-secreting tumor of the cecum presenting with biochemical features suggestive of Cushing’s disease.

  15. Inflammatory Myofibroblastic Tumor of the Bladder: 2 Rare Cases Managed with Laparoscopic Partial Cystectomy

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    Sofia Santos Lopes

    2016-01-01

    Full Text Available Two cases of inflammatory myofibroblastic tumor (IMT of the bladder are reported here. Both patients were male and presented with macroscopic hematuria; in the first case terminal hematuria was associated with irritative voiding symptoms. The second case was a smoker with hematuria unresponsive to medical treatment and anemia. Clinical presentation, pathological features, treatment, and prognosis are discussed. Due to rarity of this pathological condition, there are no guidelines concerning treatment and follow-up. We present our follow-up scheme and highlight the use of laparoscopic partial cystectomy as a successful treatment approach.

  16. Rectal malignant melanoma mistaken for thrombotic hemorrhoids - rare tumor with poor prognosis

    International Nuclear Information System (INIS)

    Kovacova, E.; Hvizdakova, A.; Vyskocil, M.; Kinova, S.; Sesovsky, V.; Kobzova, D.; Palkovic, M.

    2011-01-01

    Rectal malignant melanoma originates in the melanocytes of the anorectal area. Represent less than 1 % of all melanomas, and 4 % of all malignant tumors of the rectum and anus. The most common clinical manifestation is bleeding, the clinical examination may be mistaken for benign lesions or hemorrhoids. Given the rarity of the diagnosis are not well-defined therapeutic procedures. Prognosis for patient is poor. The authors present a case of 70-year old patient with rectal melanoma diagnosed at an advanced stage of disease, initially with diagnosis a thrombotic hemorrhoid. (author)

  17. Endometrioid adenocarcinoma associated with endometrial stromal sarcoma: A rare, often unrecognized collision tumor

    Directory of Open Access Journals (Sweden)

    Grace Kim

    2015-08-01

    Full Text Available We are reporting 3 cases of the uterine corpus with collision of endometrioid adenocarcinoma (EAC with endometrial stromal sarcoma (ESS. The patients' ages ranged from 36 to 59 years old. The major clinical presentation was abnormal uterine bleeding. Microscopically, all 3 cases presented with 2 separate components, EAC Grade 1 and ESS (one low grade and two high grades. The EAC component ranged from 10% to 70%, and the ESS component ranged from 30% to 70% of total tumor volume. The EAC component was stage 1A in two cases and stage II in one case. The ESS component was stages IA, IIB, and IIIB. Adjuvant hormonal therapy was administrated to one patient while a second patient was treated with chemo/radiation therapy. Two patients were still alive with no evidence of disease at 4 years post-therapy. One patient was lost for follow-up. Collision tumor should be distinguished from carcinosarcoma due to its different treatment modality, outcome and, prognosis.

  18. Bone and Gallium scintigraphy in primary malignant and benign bone tumors of the extremities

    International Nuclear Information System (INIS)

    Sepahdari, S.; Martin, W.B.; Ryan, J.; Simon, M.; Kirchner, P.

    1985-01-01

    A six yer prospective evaluation of 129 patients suspected of having a primary bone tumor included Tc-99m MDP bone scintigraphy followed by Ga-67 imaging at 48-72 hours. Blood pool images were part of bone scintigraphy in nearly half of the patients. Extent and intensity of tracer uptake in tumor and adjacent bone and joints were recorded for each tracer by two observers blind to the diagnosis. Tissue samples obtained in every patient by biopsy or tumor excision after scintigraphy, revealed 72 malignant and 57 benign bone tumors. The bone scan was positive in 95% (69/72) of malignancies. The scintigraphic intensity of benign and malignant lesions was comparable with both Tc-99m MDP and Ga-67. On the other hand, bone scintigraphy showed 72% (52/72) of bone malignancies to have abnormal proximal and distal bone/joint uptake whereas the Ga-67 images revealed this in only 6% (4/65) of malignancies. Benign lesions manifested this enhanced contiguous bone/joint uptake on only 8% (5/55) of bone and 0% of Ga-67 scans. This study concludes positive bone, blood pool, or Ga-67 images have less specificity for malignancy than the presence of increased Tc-99m MDP deposition in a contiguous bone/joint, but negative scintigraphic results strongly favor a benign lesion. Ga-67 was more accurate than Tc-99m MDP in portraying intraosseous extent of malignant tumors; however, this is now preferably done with C.T

  19. Exome sequencing in schizophrenic patients with high levels of homozygosity identifies novel and extremely rare mutations in the GABA/glutamatergic pathways.

    Directory of Open Access Journals (Sweden)

    Edoardo Giacopuzzi

    Full Text Available Inbreeding is a known risk factor for recessive Mendelian diseases and previous studies have suggested that it could also play a role in complex disorders, such as psychiatric diseases. Recent inbreeding results in the presence of long runs of homozygosity (ROHs along the genome, which are also defined as autozygosity regions. Genetic variants in these regions have two alleles that are identical by descent, thus increasing the odds of bearing rare recessive deleterious mutations due to a homozygous state. A recent study showed a suggestive enrichment of long ROHs in schizophrenic patients, suggesting that recent inbreeding could play a role in the disease. To better understand the impact of autozygosity on schizophrenia risk, we selected, from a cohort of 180 Italian patients, seven subjects with extremely high numbers of large ROHs that were likely due to recent inbreeding and characterized the mutational landscape within their ROHs using Whole Exome Sequencing and, gene set enrichment analysis. We identified a significant overlap (17%; empirical p-value = 0.0171 between genes inside ROHs affected by low frequency functional homozygous variants (107 genes and the group of most promising candidate genes mutated in schizophrenia. Moreover, in four patients, we identified novel and extremely rare damaging mutations in the genes involved in neurodevelopment (MEGF8 and in GABA/glutamatergic synaptic transmission (GAD1, FMN1, ANO2. These results provide insights into the contribution of rare recessive mutations and inbreeding as risk factors for schizophrenia. ROHs that are likely due to recent inbreeding harbor a combination of predisposing low-frequency variants and extremely rare variants that have a high impact on pivotal biological pathways implicated in the disease. In addition, this study confirms that focusing on patients with high levels of homozygosity could be a useful prioritization strategy for discovering new high-impact mutations in

  20. Exome sequencing in schizophrenic patients with high levels of homozygosity identifies novel and extremely rare mutations in the GABA/glutamatergic pathways.

    Science.gov (United States)

    Giacopuzzi, Edoardo; Gennarelli, Massimo; Minelli, Alessandra; Gardella, Rita; Valsecchi, Paolo; Traversa, Michele; Bonvicini, Cristian; Vita, Antonio; Sacchetti, Emilio; Magri, Chiara

    2017-01-01

    Inbreeding is a known risk factor for recessive Mendelian diseases and previous studies have suggested that it could also play a role in complex disorders, such as psychiatric diseases. Recent inbreeding results in the presence of long runs of homozygosity (ROHs) along the genome, which are also defined as autozygosity regions. Genetic variants in these regions have two alleles that are identical by descent, thus increasing the odds of bearing rare recessive deleterious mutations due to a homozygous state. A recent study showed a suggestive enrichment of long ROHs in schizophrenic patients, suggesting that recent inbreeding could play a role in the disease. To better understand the impact of autozygosity on schizophrenia risk, we selected, from a cohort of 180 Italian patients, seven subjects with extremely high numbers of large ROHs that were likely due to recent inbreeding and characterized the mutational landscape within their ROHs using Whole Exome Sequencing and, gene set enrichment analysis. We identified a significant overlap (17%; empirical p-value = 0.0171) between genes inside ROHs affected by low frequency functional homozygous variants (107 genes) and the group of most promising candidate genes mutated in schizophrenia. Moreover, in four patients, we identified novel and extremely rare damaging mutations in the genes involved in neurodevelopment (MEGF8) and in GABA/glutamatergic synaptic transmission (GAD1, FMN1, ANO2). These results provide insights into the contribution of rare recessive mutations and inbreeding as risk factors for schizophrenia. ROHs that are likely due to recent inbreeding harbor a combination of predisposing low-frequency variants and extremely rare variants that have a high impact on pivotal biological pathways implicated in the disease. In addition, this study confirms that focusing on patients with high levels of homozygosity could be a useful prioritization strategy for discovering new high-impact mutations in genetically

  1. Mandibular phosphaturic mesenchymal tumor-mixed connective tissue variant in a young girl.

    Science.gov (United States)

    Luo, Lisa; Low, Nelson; Vandervord, John

    2013-11-01

    Phosphaturic mesenchymal tumor-mixed connective tissue variant (PMTMCT) is an extremely rare tumor associated with tumor-induced osteomalacia. The majority occur in middle age and arise from the extremities. This report describes a young girl with PMTMCT arising in the mandible and with no evidence of paraneoplastic syndrome.

  2. Cystic dysplasia of the testis: a very rare paediatric tumor of the testis.

    Science.gov (United States)

    Eberli, Daniel; Gretener, Heini; Dommann-Scherrer, Corina; Pestalozzi, Dietegen; Fehr, Jean-Luc

    2002-01-01

    To describe a case of cystic dysplasia of the testis (CDT), an uncommon cause of scrotal swelling in the pediatric patient. Clinic, therapy, fertility, and radiographic and pathologic findings are discussed and the 30 previously reported cases are reviewed. A 9-year-old boy presented with asymptomatic scrotal swelling. A scrotal ultrasound showed a multicystic scrotal mass in the rete testis and an ipsilateral renal agenesis. The growth in size of the mass forced the authors to perform an operative exploration. Intraoperative findings included a multicystic mass in the rete testis of the right testicle. Testicle-sparing total removal of the multicystic mass was performed and the pathologic examination revealed a benign, multilobulated configuration of the cysts in the region of the rete testis. These findings were similar to those found in previously reported cases of CDT. Ipsilateral renal agenesis is the most common associated anomaly. As a pathogenetic factor, mal-junction of the Wolffian duct in the 5th week of gestation is most creditable. CDT is a rare cause of pediatric scrotal mass. When feasible, a testicle-sparing approach should be considered and all patients should undergo evaluation for associated urologic anomalies.

  3. Long-Term Clinical and Functional Outcomes After Treatment for Localized Ewing's Tumor of the Lower Extremity

    International Nuclear Information System (INIS)

    Indelicato, Daniel J.; Keole, Sameer R.; Shahlaee, Amir H.; Shi Wenyin; Morris, Christopher G.; Gibbs, Charles P.; Scarborough, Mark T.; Marcus, Robert B.

    2008-01-01

    Purpose: Retrospective review describing the 35-year University of Florida experience with Ewing's tumors of the lower extremity. Patients and Methods: Fifty-three patients were treated between 1971 and 2006. Thirty patients were treated with radiotherapy (RT) alone and 23 patients were treated with surgery ± RT. Larger tumors and tumors of the femur were treated more often with definitive RT. Median potential follow-up was 19.2 years. Functional outcome was assessed using the Toronto Extremity Salvage Score (TESS). Results: Before 1985, 24% of patients were treated with surgery; since then, the rate has increased to 61%. The 15-year actuarial overall survival (OS), cause-specific survival (CSS), freedom from relapse, and limb preservation rates were 68% vs. 47% (p = 0.21), 73% vs. 47% (p = 0.13), 73% vs. 40% (p = 0.03), and 43% vs. 40% (p = 0.52), respectively, for patients treated with surgery ± RT vs. RT alone. Excluding 8 patients who underwent amputation or rotationplasty, the 15-year actuarial local control rate was 100% for the surgery ± RT group and 68% for the definitive RT group (p = 0.03). The ranges of the TESS for surgery ± RT vs. RT alone were 70-100 (mean, 94) and 97-100 (mean, 99), respectively. Twenty-six percent (6/23) of patients had complications related to surgery requiring amputation or reoperation. Conclusions: Overall survival and CSS were not statistically compromised, but we observed an increased risk of relapse and local failure in patients treated with RT alone, thereby justifying a transition toward primary surgical management in suitable patients. However, despite an adverse risk profile, patients treated with RT alone had similar long-term amputation-free survival and demonstrated comparable functional outcomes. Poor results observed in Ewing's of the femur mandate innovative surgical and RT strategies

  4. Mandelbrot's Extremism

    NARCIS (Netherlands)

    Beirlant, J.; Schoutens, W.; Segers, J.J.J.

    2004-01-01

    In the sixties Mandelbrot already showed that extreme price swings are more likely than some of us think or incorporate in our models.A modern toolbox for analyzing such rare events can be found in the field of extreme value theory.At the core of extreme value theory lies the modelling of maxima

  5. Focal neuronal gigantism: a rare complication of therapeutic radiation.

    Science.gov (United States)

    Gaughen, J R; Bourne, T D; Aregawi, D; Shah, L M; Schiff, D

    2009-11-01

    Radiation therapy, a mainstay in the treatment of many brain tumors, results in a variety of well-documented acute and chronic complications. Isolated cortical damage following irradiation represents an extremely rare delayed therapeutic complication, described only twice in the medical literature. We report this rare delayed complication in a patient following treatment of a right frontal anaplastic oligodendroglioma.

  6. MRI findings associated with microscopic residual tumor following unplanned excision of soft tissue sarcomas in the extremities

    Energy Technology Data Exchange (ETDEWEB)

    Wang, Lee; Chelala, Lydia; Jose, Jean; Subhawong, Ty K. [University of Miami Miller School of Medicine/Jackson Memorial Hospital, Department of Radiology, Miami, FL (United States); Pretell-Mazzini, Juan [University of Miami Miller School of Medicine, Department of Orthopaedic Surgery, Division of Musculoskeletal Oncology, Miami, FL (United States); Kerr, Darcy A. [University of Miami Miller School of Medicine, Department of Pathology, Miami, FL (United States); Yang, Xuan [University of Miami Miller School of Medicine, Department of Public Health Sciences, Miami, FL (United States)

    2018-02-15

    MRI is often used to determine the presence of residual disease following unplanned excisions (UPE) of soft tissue sarcomas (STS). We sought to identify MRI features associated with histologic evidence of residual disease after TBE. This was an IRB-approved retrospective review of 27 patients with R1-type UPE of STS over a 32-month period, with subsequent MRI and TBE. MRI studies were retrospectively evaluated to determine depth of tissue involvement, presence of nodular enhancement, and maximum length of soft tissue edema normalized to extremity size. MRI findings were correlated with histology from unplanned excision and TBE. Among the 21 subjects, there were 13 males and 8 females, mean age 58. Eighteen of 21 STS were grade 2 or 3. Deep compartments were involved in 5/21 cases. Original margins were positive in 17/21 UPE, with inadequate margin assessment in the remaining 4 cases. Residual tumor was present at TBE in 11/21 cases; it was found in 4/6 cases with nodular enhancement and 7/15 cases without nodular enhancement (sensitivity = 0.36; specificity = 0.80; PPV = 0.67; NPV = 0.53). Increased extent of soft tissue edema increased the likelihood of residual tumor at TBE (OR = 35.0; 95% CI = 1.6 to 752.7; p = 0.023). Nodular enhancement is neither sensitive nor specific in predicting residual microscopic tumor in TBE following UPE. Extensive soft tissue edema on MRI after UPE increases the likelihood of finding a residual microscopic tumor, justifying ample margins at TBE and consideration of adjuvant therapy. (orig.)

  7. Gross tumor volume and clinical target volume: soft-tissue sarcoma of the extremities

    International Nuclear Information System (INIS)

    Lartigau, E.; Kantor, G.; Lagarde, P.; Taieb, S.; Ceugnart, L.; Vilain, M.O.; Penel, N.; Depadt, G.

    2001-01-01

    Soft tissue sarcomas of the extremities are currently treated with more conservative and functional approaches, combining surgery, radiotherapy and chemotherapy. The role of external beam radiotherapy and brachytherapy has been defined through randomized studies performed in the 80's and 90's. However, the ubiquity of tumour location for these tumours makes difficult a systematic definition of local treatments. Tumour volume definition is based on pre and post surgical imaging (MRI) and on described pathological report. The clinical target volume will take into account quality of the resection and anatomical barriers and will be based on an anatomy and not only on safety margins around the tumour bed. General rules for this irradiation (doses, volumes) and principal results will be presented. (authors)

  8. Multiple Primary Merkel Cell Carcinomas Presenting as Pruritic, Painful Lower Leg Tumors

    OpenAIRE

    Laura Blumenthal; Timothy VandenBoom; Edward Melian; Anthony Peterson; Kelli A. Hutchens

    2015-01-01

    Merkel cell carcinoma (MCC) is a rare and highly aggressive neuroendocrine tumor of the skin which almost exclusively presents as a solitary tumor. It is most often seen on sun-exposed regions, historically almost exclusively on the head and neck, with only rare case reports on the extremities. Although recent studies have shown increased incidence with up to 20% on the extremities, here we present one of these rare emerging presentations, with the addition of a unique treatment option. Our p...

  9. A rare case of anal carcinosarcoma with human papilloma virus infection in both biphasic tumor elements: An immunohistochemical, molecular and ultrastructural study.

    Science.gov (United States)

    Hickman, Richard A; Bradshaw, Azore-Dee; Cassai, Nicholas; Neto, Antonio Galvao; Zhou, David; Fu, Tinghao; Lee, Peng; Pei, Zhiheng; Wieczorek, Rosemary

    2016-12-01

    Carcinosarcoma of the anus is rare and has yet to be reportedly associated with the keratinocyte-specific Human Papilloma Virus (HPV). We describe a case of anal carcinosarcoma with HPV infection in both the epithelial and mesenchymal components of the tumor by immunohistochemistry, chromogenic in-situ hybridization (CISH) and further supported by electron microscopy (EM). Microscopic examination of the tumor showed nests of poorly-differentiated invasive squamous cell carcinoma with basaloid features intermixed with a hypercellular, atypical spindle cell proliferation. Immunohistochemistry demonstrated that the epithelial component was positive for AE1/AE3, p63, CK5/6 and p16, whilst the mesenchymal component was positive for smooth muscle actin, vimentin, and focally positive for desmin and p16, consistent with carcinosarcoma. The tumor was negative for GATA-3, CK7 and CK20. CISH demonstrated that the tumor was positive for high risk HPV (subtype 16/18) in both tumor components. EM further supported the presence of intracellular virus particles (~50 nm) that is compatible with HPV infection. Infection of both epithelial and mesenchymal tumor components by HPV has not been previously observed in the gastrointestinal tract. This finding may represent initial epithelial HPV infection with subsequent divergent tumoral differentiation and suggests the presence of viral replication in both biphasic tumor components.

  10. A rare case of anal carcinosarcoma with human papilloma virus infection in both biphasic tumor elements: An immunohistochemical, molecular and ultrastructural study

    Directory of Open Access Journals (Sweden)

    Richard A. Hickman

    2016-12-01

    Full Text Available Carcinosarcoma of the anus is rare and has yet to be reportedly associated with the keratinocyte-specific Human Papilloma Virus (HPV. We describe a case of anal carcinosarcoma with HPV infection in both the epithelial and mesenchymal components of the tumor by immunohistochemistry, chromogenic in-situ hybridization (CISH and further supported by electron microscopy (EM. Microscopic examination of the tumor showed nests of poorly-differentiated invasive squamous cell carcinoma with basaloid features intermixed with a hypercellular, atypical spindle cell proliferation. Immunohistochemistry demonstrated that the epithelial component was positive for AE1/AE3, p63, CK5/6 and p16, whilst the mesenchymal component was positive for smooth muscle actin, vimentin, and focally positive for desmin and p16, consistent with carcinosarcoma. The tumor was negative for GATA-3, CK7 and CK20. CISH demonstrated that the tumor was positive for high risk HPV (subtype 16/18 in both tumor components. EM further supported the presence of intracellular virus particles (~50 nm that is compatible with HPV infection. Infection of both epithelial and mesenchymal tumor components by HPV has not been previously observed in the gastrointestinal tract. This finding may represent initial epithelial HPV infection with subsequent divergent tumoral differentiation and suggests the presence of viral replication in both biphasic tumor components. Keywords: Carcinosarcoma, Human Papilloma Virus, Gastrointestinal

  11. Embolic Brain Infarcts: A Rare Fatal Complication of Preoperative Embolization of a Massive Solitary Fibrous Tumor of the Pleura

    Energy Technology Data Exchange (ETDEWEB)

    Patel, Shreyas R., E-mail: Shrey000@gmail.com; Vachhani, Prasann; Moeslein, Fred [University of Maryland Medical Center, Department of Diagnostic Radiology and Nuclear Medicine (United States)

    2017-02-15

    Solitary fibrous tumor of the pleura (SFTP) is a rare intrathoracic neoplasm, often giant in size and highly vascular, which can make surgical resection very challenging. Preoperative percutaneous embolization before surgical removal can significantly reduce the risk of uncontrollable intraoperative hemorrhage. However, a rare potential life threatening complication could result from embolization of SFTP and must be taken into consideration. This report describes a 69-year-old female with a large right thoracic SFTP, who underwent preoperative angiography and embolization and developed diffuse embolic brain infarcts immediately after the administration of polyvinyl alcohol particles.

  12. Triple composite tumor of stomach: A rare combination of alpha fetoprotein positive hepatoid adenocarcinoma, tubular adenocarcinoma and large cell neuroendocrine carcinoma

    Directory of Open Access Journals (Sweden)

    Lipika Lipi

    2014-01-01

    Full Text Available A 50-year-old male patient presented with pain abdomen of 6 months duration. Computed tomography scan revealed a large mass in the stomach occluding the lumen. Histopathology revealed a triple composite tumor comprising of tubular adenocarcinoma arising on a background of high-grade dysplasia, hepatoid adenocarcinoma (positive for Hep Par-1 and alpha fetoprotein and large cell neuroendocrine carcinoma (positive for synaptophysin and chromogranin with nodal metastasis.Triple composite tumors are distinctly rare with few reports in literature.

  13. Meningeous sarcoma: a rare tumor among the central nervous system neoplasia in childhood; Sarcoma meningeo: rara entidade dentre as neoplasias de sistema nervoso central na infancia

    Energy Technology Data Exchange (ETDEWEB)

    Rondinelli, Patricia Imperatriz Porto; Salvajoli, Joao Victor; Sredni, Simone Treiger; Araujo, Maria Betania Mahler [Hospital do Cancer de Sao Paulo, SP (Brazil). Dept. de Pediatria

    2003-07-01

    We describe a case of meningeous malignancy in childhood, diagnosed by the Pediatric Department of the Cancer Hospital in Sao Paulo, Brazil, and do revise the world literature as well. The meningeous sarcoma (M S) is an extremely aggressive tumor, which appears in the central nervous system, at any age, but mainly in children. They represent a tiny percentage of brain tumors in children and sporadic cases are related in the world literature. Consequently, there are not enough clinical experiences about this distinct entity to allow the conclusion about which is the best therapeutic approach. (author)

  14. Wilm's tumor in adulthood

    International Nuclear Information System (INIS)

    Matveev, B.P.; Bukharkin, B.V.; Gotsadze, D.T.

    1984-01-01

    Wilms' tumor occurs extremely rarely in adults. There is no consensus in the literature on the problems of clinical manifestations, diagnosis and treatment of the diseasa. Ten adult patients (aged 16-29) with Wilms' tumor formed the study group. They made up 0.9 per cent of the total number of kidney tumor patients. The peculiarities of the clinical course that distinguish adult nephroblastoma from renal cancer and Wilms' tumor of the infancy were analysed. The latent period appeared to be long. Problems of diagnosis are discussed. Angiography proved to be of the highest diagnostic value. Complex treatment including transperitoneal nephrectory, radiation and chemotherapy was carried out in 7 cases, palliative radiation treatmenchemotherapy andn 3. Unlike pediatric nephroblastomt - i Wilms' tumor in adults was resistant to radiation. Treatment results still remained unsatisfactory: 6 patients died 7-19 months after the beginning of treatment

  15. Primary solitary peritoneal tumor of the abdominal wall—report of a rare case and review of the literature

    Science.gov (United States)

    Efthimiadis, Christoforos; Ioannidis, Aristeidis; Grigoriou, Marios

    2017-01-01

    Abstract Abdominal wall tumors are sometimes diagnosed as metastases of ovarian cancer, however, primary peritoneal tumors should be taken into consideration in the final diagnosis. A 49-year-old female patient was admitted in our Department for the excision of a pulpable abdominal wall lump, with no other abnormalities shown on imaging investigation. On histology examination, the excised specimen revealed characteristics of metastatic high-grade serous ovarian carcinoma. Total hysterectomy, bilateral oophorectomy, omentectomy and appendectomy were performed. No signs of malignancy were proved on histology, leading to the final diagnosis of a primary serous peritoneal tumor. This is the third described case of solitary primary serous peritoneal tumor located in the abdominal wall. This condition should be included in the differential diagnosis of a probable metastatic ovarian carcinoma, as both present similar histologic characteristics. PMID:28616156

  16. Primary solitary peritoneal tumor of the abdominal wall-report of a rare case and review of the literature.

    Science.gov (United States)

    Efthimiadis, Christoforos; Ioannidis, Aristeidis; Kofina, Konstantinia; Grigoriou, Marios

    2017-06-01

    Abdominal wall tumors are sometimes diagnosed as metastases of ovarian cancer, however, primary peritoneal tumors should be taken into consideration in the final diagnosis. A 49-year-old female patient was admitted in our Department for the excision of a pulpable abdominal wall lump, with no other abnormalities shown on imaging investigation. On histology examination, the excised specimen revealed characteristics of metastatic high-grade serous ovarian carcinoma. Total hysterectomy, bilateral oophorectomy, omentectomy and appendectomy were performed. No signs of malignancy were proved on histology, leading to the final diagnosis of a primary serous peritoneal tumor. This is the third described case of solitary primary serous peritoneal tumor located in the abdominal wall. This condition should be included in the differential diagnosis of a probable metastatic ovarian carcinoma, as both present similar histologic characteristics.

  17. Primary solitary peritoneal tumor of the abdominal wall?report of a rare case and review of the literature

    OpenAIRE

    Efthimiadis, Christoforos; Ioannidis, Aristeidis; Kofina, Konstantinia; Grigoriou, Marios

    2017-01-01

    Abstract Abdominal wall tumors are sometimes diagnosed as metastases of ovarian cancer, however, primary peritoneal tumors should be taken into consideration in the final diagnosis. A 49-year-old female patient was admitted in our Department for the excision of a pulpable abdominal wall lump, with no other abnormalities shown on imaging investigation. On histology examination, the excised specimen revealed characteristics of metastatic high-grade serous ovarian carcinoma. Total hysterectomy, ...

  18. Glomus Tumor of the Larynx: A Rare Synchronous Paraganglioma in a Patient with Bilateral Carotid Body Tumor Detected on 68Ga-DOTANOC PET/CT.

    Science.gov (United States)

    Tripathy, Sarthak; Mukherjee, Anirban; Singh, Chirom A; Jat, Bhinyaram; Bal, Chandrasekhar; Shamim, Shamim Ahmed

    2017-01-01

    Paragangliomas are neoplasms arising from extra-adrenal chromaffin tissue. They frequently cause symptoms by overproduction of catecholamines with known predilection to multicentricity. We describe the case of a patient with bilateral carotid body tumor who underwent a baseline 68 Gallium labeled [1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acid]-1-NaI3-Octreotide.

  19. Impact of Pathologist Involvement in Sarcoma and Rare Tumor Patient Support Groups on Facebook: A Survey of 542 Patients and Family Members.

    Science.gov (United States)

    Haller, Jasmine; David, Marjorie Parker; Lee, Nathan E; Shalin, Sara C; Gardner, Jerad M

    2018-01-29

    - Patients with rare tumors have difficulty finding reliable information about their disease. Facebook patient support groups allow patients to educate one another. - To investigate how these patients perceive the value of pathologists, both in Facebook groups and real-world patient care. - Survey links were posted in 12 Facebook patient groups: 6 with an active pathologist member (angiosarcoma, epithelioid hemangioendothelioma, epithelioid sarcoma, dermatofibrosarcoma protuberans [×2], and desmoid fibromatosis), and 6 without "active" pathologist involvement (aggressive angiomyxoma, chondrosarcoma, Ewing sarcoma, leiomyosarcoma, liposarcoma, and osteosarcoma). - A total of 542 people responded (403 were patients): 264 from groups with a pathologist, and 278 from groups without active pathologist involvement. Of groups with an active pathologist, respondents agreed the pathologist's posts helped them better understand their disease (107 of 119; 90%) and relieved some of their disease-related anxiety (92 of 119; 77%). And for these groups 98% (117 of 119) of respondents agreed that having a pathologist in their group was a good thing; 83% (192 of 232) wanted more pathologists involved. More respondents from groups with an active pathologist (219 of 236; 93%) than without one (215 of 252; 85%) agreed: "pathologists are an important part of the patient care team for patients with cancer and other rare tumors" ( P = .008). - This study is the first to evaluate the impact of pathologist interaction with Facebook patient support groups and to assess perceptions about the specialty of pathology from a large group of patients with rare tumors. Pathologist involvement in Facebook patient groups appears to positively influence patient perception of the importance of pathologists. We hope these data will encourage more pathologists to participate in Facebook patient support groups.

  20. Dome-type carcinoma of the colon; a rare variant of adenocarcinoma resembling a submucosal tumor: a case report

    Directory of Open Access Journals (Sweden)

    Yamada Masayoshi

    2012-03-01

    Full Text Available Abstract Background Dome-type carcinoma (DC is a distinct variant of colorectal adenocarcinoma and less than 10 cases have been described in the literature. Most of the previously reported cases were early lesions and no endoscopic observations have been described so far. We herein report a case of a DC invading the subserosal layer, including endoscopic findings. Case presentation A highly elevated lesion in the transverse colon was diagnosed by colonoscopy in a 77-year-old man. The tumor appeared to be similar to a submucosal tumor (SMT, however, a demarcated area of reddish and irregular mucosa was observed at the top of the tumor. There were no erosions or ulcers. Laparoscopic-assisted right hemicolectomy was performed and pathological examination revealed a well-circumscribed tumor invading the subserosal layer. The tumor was a well-differentiated adenocarcinoma associated with a dense lymphocytic infiltration and showed expansive growth. The overlying mucosal layer showed high-grade dysplasia. Conclusion The present lesion was diagnosed as a DC of the colon invading the subserosal layer. Because the association of mucosal dysplasia is common in DCs, the detection of dysplastic epithelium would be important to discriminate DCs from SMTs.

  1. A Rare Case of Intra-Endometrial Leiomyoma of Uterus Simulating Degenerated Submucosal Leiomyoma Accompanied by a Large Sertoli-Leydig Cell Tumor.

    Science.gov (United States)

    Jeong, Kyungah; Lee, Sa Ra; Park, Sanghui

    2016-03-01

    A 50-year-old peri-menopausal woman presented with hard palpable mass on her lower abdomen and anemia from heavy menstrual bleeding. Ultrasonography showed a 13×12 cm sized hypoechoic solid mass in pelvis and a 2.5×2 cm hypoechoic cystic mass in uterine endometrium. Abdomino-pelvic computed tomography revealed a hypodense pelvic mass without enhancement, suggesting a leiomyoma of intraligamentary type or sex cord tumor of right ovary with submucosal myoma of uterus. Laparoscopy revealed a large Sertoli-Leydig cell tumor of right ovary with a very rare entity of intra-endometrial uterine leiomyoma accompanied by adenomyosis. The final diagnosis of ovarian sex-cord tumor (Sertoli-Leydig cell), stage Ia with intra-endometrial leiomyoma with adenomyosis, was made. Considering the large size of the tumor and poorly differentiated nature, 6 cycles of chemotherapy with Taxol and Carboplatin regimen were administered. There is neither evidence of major complications nor recurrence during 20 months' follow-up.

  2. A Rare Case: Struma Ovarii in a 14-Year-Old Girl.

    Science.gov (United States)

    Iltar, Elif; Ureyen, Isin; Toptas, Tayfun; Savas, Melike; Çekiç, Sema; Uysal, Aysel

    2018-02-01

    Ovarian tumors presented with ovarian mass in childhood and adolescence are uncommon but an important part of gynecological cases. Struma ovarii is one of the rare cystic benign ovarian tumors that is observed predominantly in women who are between the ages of 40 and 60 years old. It is extremely rare in adolescents. Herein, we present a 14-year-old adolescent girl with struma ovarii who presented to the emergency room with abdominal pain.

  3. The diagnostic value of time-resolved MR angiography with Gadobutrol at 3 T for preoperative evaluation of lower extremity tumors: Comparison with computed tomography angiography

    International Nuclear Information System (INIS)

    Wu, Gang; Jin, Teng; Li, Ting; Li, Xiaoming

    2016-01-01

    To evaluate the diagnostic value of time resolved magnetic resonance angiography with interleaved stochastic trajectory (TWIST) using Gadobutrol for preoperative evaluation of lower extremity tumors. This prospective study was approved by the local Institutional Review Board. 50 consecutive patients (31 men, 19 women, age range 18–80 years, average age 42.7 years) with lower extremity tumors underwent TWIST and computed tomography angiography (CTA). Image quality of TWIST and CTA were evaluated by two radiologists according to a 4-point scale. The degree of arterial stenosis caused by tumor was assessed using TWSIT and CTA separately, and the intra-modality agreement was determined using a kappa test. The number of feeding arteries identified by TWIST was compared with that by CTA using Wilcoxon signed rank test. The ability to identify arterio-venous fistulae (AVF) were compared using a chi-square test. Image quality of TWIST and CTA were rated as 3.88 ± 0.37 and 3.97 ± 0.16, without statistically significant difference (P = 0.135). Intra-modality agreement was excellent for the assessment of arterial stenosis (kappa = 0.806 ± 0.073 for Reader 1, kappa = 0.805 ± 0.073 for Reader 2). Readers identified AVF with TWIST in 27 of 50 cases, and identified AVF with CTA in 14 of 50 (P < 0.001). Mean feeding arteries identified with TWIST was significantly more than that with CTA (2.08 ± 1.72 vs 1.62 ± 1.52, P = 0.02). TWIST is a reliable imaging modality for the assessment of lower extremity tumors. TWIST is comparable to CTA for the identification of AVF and feeding arteries

  4. Bilateral granulosa cell tumors: a novel malignant manifestation of multiple endocrine neoplasia 1 syndrome found in a patient with a rare menin in-frame deletion

    Directory of Open Access Journals (Sweden)

    Hall MJ

    2015-02-01

    Full Text Available Michael J Hall,1 Julie Innocent,2 Christina Rybak,1 Colleen Veloski,3 Walter J Scott,4 Hong Wu,5 John A Ridge,4 John P Hoffman,4 Hossein Borghaei,2 Aruna Turaka,6 Mary B Daly1 1Department of Clinical Genetics, 2Department of Medical Oncology, 3Department of Internal Medicine, 4Department of Surgical Oncology, 5Department of Pathology, 6Department of Radiation Oncology, Fox Chase Cancer Center, Philadelphia, PA, USA Introduction: Multiple endocrine neoplasia 1 (MEN1 is a cancer syndrome resulting from mutations of the MEN1 gene. The syndrome is characterized by neoplasia of the parathyroid and pituitary glands, and malignant tumors of the endocrine pancreas. Other manifestations include benign lipomas, angiofibromas, and carcinoid tumors commonly originating in the colon, thymus, and lung. This is the first report of MEN1 syndrome manifesting as bilateral granulosa cell ovarian tumors, and which is associated with a rare intronic mutation of the MEN1 gene. Case report: A 41-year-old woman presented with abdominal pain, increasing abdominal girth, and dysmenorrhea. Ultrasound demonstrated enlarged ovaries and uterine fibroids. After an exploratory laparotomy, she subsequently underwent bilateral salpingo–oophorectomy with hysterectomy where the pathology revealed bilateral cystic granulosa cell tumors of the ovaries. Additional workup including computed tomography imaging discovered a thymic mass, which the pathology showed was malignant, along with a pancreatic mass suspicious for a neuroendocrine tumor. Hyperparathyroidism was also discovered and was found to be secondary to a parathyroid adenoma. Genetic testing revealed an exceedingly rare mutation in the MEN1 gene (c.654 + 1 G>A. Discussion: Mutations of the menin gene leading to MEN1 syndrome are classically nonsense or missense mutations producing a dysfunctional protein product. Recently, researchers described a novel mutation of MEN1 (c.654 + 1 G>A in a male proband meeting the criteria

  5. Report of two cases of fluorodeoxyglucose positron emission tomography/computed tomography appearance of hibernoma: A rare benign tumor

    International Nuclear Information System (INIS)

    Agrawal, Archi; Kembhavi, Seema; Purandare, Nilendu; Shah, Sneha; Rangarajan, Venkatesh

    2014-01-01

    False-positive findings are commonly seen in positron emission tomography computed tomography imaging. One of the most common false positive finding is uptake of fluorodeoxyglucose in brown adipose tissue. Herein, we report two cases with incidentally detected hibernomas-a brown fat containing tumor with metabolic activity

  6. Rare germline alterations in cancer-related genes associated with the risk of multiple primary tumor development

    DEFF Research Database (Denmark)

    Villacis, Rolando A. R.; Basso, Tatiane R; Canto, Luisa M

    2017-01-01

    Multiple primary tumors (MPT) have been described in carriers of inherited cancer predisposition genes. However, the genetic etiology of a large proportion of MPT cases remains unclear. We reviewed 267 patients with hereditary cancer predisposition syndromes (HCPS) that underwent genetic counseli...

  7. The Rare Benign Lesion That Mimics a Malignant Tumor in Breast Parenchyma: Nodular Fasciitis of the Breast

    Directory of Open Access Journals (Sweden)

    Hilal Erinanc

    2018-01-01

    Full Text Available We herein report the clinical and pathological findings of a rare case of nodular fasciitis in the breast parenchyma of a 48-year-old female. Because of potentially malignant findings on ultrasonography and during clinical examination, the patient underwent an excisional biopsy. Histologically, the lesion was composed of spindle to round shaped cells arranged in short bundles in a storiform pattern. Immunohistochemically, the cells were positive for vimentin and SMA and negative for desmin, S100, and CD34. Based on these morphological and immunohistochemical features, a diagnosis of nodular fasciitis was made. We emphasize that nodular fasciitis of the breast may show clinical features and imaging findings similar to those of breast cancer. The histopathologic diagnosis of nodular fasciitis can also be challenging. The purpose of this case report is to highlight the characteristics and the differential diagnosis of this rare neoplasm.

  8. Rare Events

    Science.gov (United States)

    2009-10-01

    Limited Operational Exercise 1. 1A Limited Operational Exercise is a multiplayer experiment designed to exploit and study information sharing and...1.4 Summary of the Study The “rare event” of interest is an extreme, deliberate act of violence , destruction or socioeconomic disruption, such as an...connection with terrorism inves- tigations. The programs then use some combination of doctrinal revision and rewards to induce the people to abandon violence

  9. Adult Type Granulosa Cell Tumor: A Very Rare Case of Sex-Cord Tumor of the Testis with Review of the Literature

    Directory of Open Access Journals (Sweden)

    Dimosthenis Miliaras

    2013-01-01

    Full Text Available Granulosa cell tumor (GST is a sex-cord/stromal neoplasm of the gonads, more commonly arising in the ovaries, while approximately 80 cases have been reported in the testes. Out of these, 30 cases were of the adult type, while the remainder 50 cases were of the juvenile type. The latter mostly concerned infants and followed a benign course. However, the adult type testicular GCTs may be potentially malignant as it also happens in female patients with such neoplasms. We present a case of an adult type GCT located at the left testis. The patient was subjected to total orchiectomy and received no further treatment. Histology showed typical GCT histomorphology with Call-Exner bodies in some places. The immunoprofile of the tumor was CD99 (+, calretinin (+, inhibin (+, alpha smooth muscle actin (+, vimentin (+, ER (−, PR (−, keratin AE1/AE3 (−, alpha fetoprotein (−, CD117 (−, and placental alkaline phosphatase (−. Two years after surgery, the patient is alive and well with no signs of recurrence.

  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 price of tumor control: an analysis of rare side effects of anti-CTLA-4 therapy in metastatic melanoma from the ipilimumab network.

    Directory of Open Access Journals (Sweden)

    Caroline J Voskens

    Full Text Available Ipilimumab, a cytotoxic T-lymphocyte antigen-4 (CTLA-4 blocking antibody, has been approved for the treatment of metastatic melanoma and induces adverse events (AE in up to 64% of patients. Treatment algorithms for the management of common ipilimumab-induced AEs have lead to a reduction of morbidity, e.g. due to bowel perforations. However, the spectrum of less common AEs is expanding as ipilimumab is increasingly applied. Stringent recognition and management of AEs will reduce drug-induced morbidity and costs, and thus, positively impact the cost-benefit ratio of the drug. To facilitate timely identification and adequate management data on rare AEs were analyzed at 19 skin cancer centers.Patient files (n = 752 were screened for rare ipilimumab-associated AEs. A total of 120 AEs, some of which were life-threatening or even fatal, were reported and summarized by organ system describing the most instructive cases in detail. Previously unreported AEs like drug rash with eosinophilia and systemic symptoms (DRESS, granulomatous inflammation of the central nervous system, and aseptic meningitis, were documented. Obstacles included patientś delay in reporting symptoms and the differentiation of steroid-induced from ipilimumab-induced AEs under steroid treatment. Importantly, response rate was high in this patient population with tumor regression in 30.9% and a tumor control rate of 61.8% in stage IV melanoma patients despite the fact that some patients received only two of four recommended ipilimumab infusions. This suggests that ipilimumab-induced antitumor responses can have an early onset and that severe autoimmune reactions may reflect overtreatment.The wide spectrum of ipilimumab-induced AEs demands doctor and patient awareness to reduce morbidity and treatment costs and true ipilimumab success is dictated by both objective tumor responses and controlling severe side effects.

  12. Large cell neuroendocrine carcinoma originating from the uterine endometrium: a report on magnetic resonance features of 2 cases with very rare and aggressive tumor

    Directory of Open Access Journals (Sweden)

    Natsuko Makihara

    2012-06-01

    Full Text Available Neuroendocrine carcinomas (NEC of the female genital tract are aggressive and uncommon tumors, which usually involve the uterine cervix and ovary, and are seen very rarely in the endometrium. Only less than 10 cases of large cell NEC (LCNEC of the endometrium have been reported in the literature and their radiological findings are not well described. We report here two cases of pathologically proven LCNEC of the uterine endometrium. In both cases, the uterine body was enlarged and the tumor occupied part of the uterine cavity. Endometrial mass exhibited heterogeneous high intensity on T2-weighted magnetic resonance (MR images, and diffusion-weighted MR images revealed high intensity throughout the tumor, consistent with malignancy. LCNEC is a highly malignant neoplasm without particular findings in terms of diagnostic imaging and pathology, so its preoperative definitive diagnosis is very difficult. However, when laboratory test, pathologic diagnosis and MR imaging suggest a poorly differentiated uterine malignancy, positron emission tomography-computed tomography scan should be performed as a general assessment to help with diagnosis.

  13. Xanthogranulomatous Prostatitis, a Rare Prostatic Entity

    Directory of Open Access Journals (Sweden)

    Alejandro Noyola

    2017-01-01

    Full Text Available There are several benign prostatic pathologies that can clinically mimic a prostate adenocarcinoma. Xanthogranulomatous prostatitis is a benign inflammatory condition of the prostate and a rare entity. A 47-year old male, with 3 years of lower urinary tract symptoms, with a palpable hypogastric tumor, digital rectal examination: solid prostate, of approximately 60 g. Initial PSA was 0.90 ng/mL. He underwent surgical excision of the lower abdominal nodule and prostatectomy. Histopathology showed xanthogranulomatous prostatitis, without malignancy. Xanthogranulomatous prostatitis is an extremely rare entity that can simulate prostate adenocarcinoma, therefore having a correct histopathological diagnosis is essential.

  14. Brown tumors of the anterior skull base as the initial manifestation of true normocalcemic primary hyperparathyroidism: report of three cases and review of the literature.

    Science.gov (United States)

    Khalatbari, Mahmoud Reza; Hamidi, Mehrdokht; Moharamzad, Yashar; Setayesh, Ali; Amirjamshidi, Abbas

    2013-01-01

    Brown tumor is a bone lesion secondary to hyperparathyroidism of various etiologies. Skeletal involvement in primary hyperparathyroidism secondary to parathyroid adenoma is very uncommon and brown tumor has become extremely a rare clinical entity. Hyperparathyroidism is usually associated with high levels of serum calcium. Brown tumor as the only and initial symptom of normocalcemic primary hyperparathyroidism is extremely rare. Moreover, involvement of the skull base and the orbit is exceedingly rare. The authors would report three cases of brown tumor of the anterior skull base that were associated with true normocalcemic primary hyperparathyroidism. Clinical manifestations, neuroimaging findings, pathological findings, diagnosis and treatment of the patients are discussed and the relevant literature is reviewed.

  15. Fatty rind of intramuscular soft-tissue tumors of the extremity: is it different from the split fat sign?

    Energy Technology Data Exchange (ETDEWEB)

    Sung, Jinkyeong; Kim, Jee-Young [The Catholic University of Korea, Departments of Radiology, St. Vincent' s Hospital, College of Medicine, Suwon, Gyeonggi-do (Korea, Republic of)

    2017-05-15

    To analyze intramuscular soft-tissue tumors with fatty rind, and to evaluate the difference between fatty rind and split fat sign on magnetic resonance imaging (MRI). We retrospectively analyzed 50 pathologically confirmed intramuscular masses on MRI. We evaluated the distribution and shape of fatty rind and muscle atrophy. Fatty rind was found more frequently in benign lesions (80% [36 out of 45]) compared with malignant lesions (25% [1 out of 5]; P = 0.013). Thirty-six benign lesions were peripheral nerve sheath tumors (PNSTs; n = 19), hemangiomas (n = 11), myxomas (n = 2), ganglion cysts (n = 2), giant cell tumor (n = 1), and leiomyoma (n = 1). One malignant lesion was a low-grade fibromyxoid sarcoma. In all masses with fatty rind, fat was confined to the proximal and the distal ends. In 12 cases, complete or partial circumferential fatty rind was also noted. Fatty rinds at both ends showed crescent, triangular, or combined shape. The prevalence of crescent-shaped fatty rind was significantly higher in benign PNST (17 out of 38) compared with the other tumors (1 out of 32; P < 0.001). Complete circumferential fat was noted only in hemangioma (n = 5). Triangular fatty rind was related to peripheral location of the mass or muscle atrophy. Most intramuscular tumors with fatty rinds were benign, and PNST was the most common tumor type. Fatty rind could be caused by displaced neurovascular bundle fat, fatty atrophy of the muscle involved, or intermuscular or perimysial fat. Crescent-shaped fatty rind was noted more frequently in benign PNSTs. (orig.)

  16. Fatty rind of intramuscular soft-tissue tumors of the extremity: is it different from the split fat sign?

    International Nuclear Information System (INIS)

    Sung, Jinkyeong; Kim, Jee-Young

    2017-01-01

    To analyze intramuscular soft-tissue tumors with fatty rind, and to evaluate the difference between fatty rind and split fat sign on magnetic resonance imaging (MRI). We retrospectively analyzed 50 pathologically confirmed intramuscular masses on MRI. We evaluated the distribution and shape of fatty rind and muscle atrophy. Fatty rind was found more frequently in benign lesions (80% [36 out of 45]) compared with malignant lesions (25% [1 out of 5]; P = 0.013). Thirty-six benign lesions were peripheral nerve sheath tumors (PNSTs; n = 19), hemangiomas (n = 11), myxomas (n = 2), ganglion cysts (n = 2), giant cell tumor (n = 1), and leiomyoma (n = 1). One malignant lesion was a low-grade fibromyxoid sarcoma. In all masses with fatty rind, fat was confined to the proximal and the distal ends. In 12 cases, complete or partial circumferential fatty rind was also noted. Fatty rinds at both ends showed crescent, triangular, or combined shape. The prevalence of crescent-shaped fatty rind was significantly higher in benign PNST (17 out of 38) compared with the other tumors (1 out of 32; P < 0.001). Complete circumferential fat was noted only in hemangioma (n = 5). Triangular fatty rind was related to peripheral location of the mass or muscle atrophy. Most intramuscular tumors with fatty rinds were benign, and PNST was the most common tumor type. Fatty rind could be caused by displaced neurovascular bundle fat, fatty atrophy of the muscle involved, or intermuscular or perimysial fat. Crescent-shaped fatty rind was noted more frequently in benign PNSTs. (orig.)

  17. Rare extragonadal teratomas in children: complete tumor excision as a reliable and essential procedure for significant survival. Clinical experience and review of the literature.

    Science.gov (United States)

    Paradies, Guglielmo; Zullino, Francesca; Orofino, Antonio; Leggio, Samuele

    2014-01-01

    Extragonadal teratomas are rare tumors in neonates and infants and can sometimes show unusual, distinctive feature such as an unusual location, a clinical sometimes acute, presentation and a "fetiform" histotype of the lesion. We have extrapolated, from our entire experience of teratomas, 4 unusual cases, mostly operated as emergencies; 2 of them were treated just after birth. Aim of this paper is to report the clinical and pathological findings, to evaluate the surgical approach and the long-term biological behaviour in these cases, in the light of survival and current insights reported in the literature. The Authors reviewed the most significant (Tables I and II) clinical, laboratory, radiologic, and pathologic findings, surgical procedures, early and long-term results in 4 children, 1 male and 3 females (M/F ratio: 1/3), suffering from extragonadal teratomas, located in the temporo-zygomatic region of the head (Case n. 1, Fig. 1), retroperitoneal space (Case n. 2, Fig. 2) ,liver (Case n. 3, Figg. 3-5), kidney (Case n. 4, Fig. 6, 7), respectively. Of the 4 patients, 2 were treated neonatally (1 T. of the head, 1 retroperitoneal T.) A prenatal diagnosis had already been made in 2 of the 4 patients, between the 2nd and 3rd trimester of pregnancy, All the infants were born by scheduled caesarean section in a tertiary care hospital and were the immediately referred to thew N.I.C.Us. Because of a mostly acute clinical presentation, the 4 patients were then referred to the surgical unit at different ages: 7 days, 28 days, 7 months, and 4 years respectively. The initial clinical presentation (Table II) was consistent with the site of the mass and/or its side effects. The 2 newborns (Case 1 and 2) both with a prenatally diagnosed mass located at the temporozygomatic region and in the abdominal cavite respectively, already displayed, at birth a mass with a tendency to further growth. The symptoms and signs described to the primary care physician by the parents of the 2

  18. Embryonal Rhabdomyosarcoma of the Adult Urinary Bladder: A Rare Case Report of Misclassification as Inflammatory Myofibroblastic Tumor

    Directory of Open Access Journals (Sweden)

    Kelven Weijing Chen

    2015-01-01

    Full Text Available Embryonal rhabdomyosarcoma (ERMS of the adult urinary bladder is a rare malignant tumour. Inflammatory myofibroblastic tumour (IMT of the bladder is a benign genitourinary tumour that may appear variable histologically but usually lacks unequivocal malignant traits. Techniques like flow cytometry and immunohistochemistry may be used to differentiate these two tumours. Our patient, a 46-year-old male, had rapidly recurring lower urinary tract symptoms after two transurethral resections of the prostate. He subsequently underwent a transvesical prostatectomy which showed IMT on histology. However, his symptoms did not resolve and an open resection done at our institution revealed a 6 cm tumour arising from the right bladder neck. This time, histology was ERMS with diffuse anaplasia of the bladder. Rapid recurrence of urinary symptoms with prostate regrowth after surgery is unusual. Differential diagnoses of uncommon bladder malignancies should be considered if there is an inconsistent clinical course as treatment approaches are different.

  19. Embryonal Rhabdomyosarcoma of the Adult Urinary Bladder: A Rare Case Report of Misclassification as Inflammatory Myofibroblastic Tumor

    Science.gov (United States)

    Chen, Kelven Weijing; Wu, Fiona Mei Wen; Lee, Victor Kwan Min; Esuvaranathan, Kesavan

    2015-01-01

    Embryonal rhabdomyosarcoma (ERMS) of the adult urinary bladder is a rare malignant tumour. Inflammatory myofibroblastic tumour (IMT) of the bladder is a benign genitourinary tumour that may appear variable histologically but usually lacks unequivocal malignant traits. Techniques like flow cytometry and immunohistochemistry may be used to differentiate these two tumours. Our patient, a 46-year-old male, had rapidly recurring lower urinary tract symptoms after two transurethral resections of the prostate. He subsequently underwent a transvesical prostatectomy which showed IMT on histology. However, his symptoms did not resolve and an open resection done at our institution revealed a 6 cm tumour arising from the right bladder neck. This time, histology was ERMS with diffuse anaplasia of the bladder. Rapid recurrence of urinary symptoms with prostate regrowth after surgery is unusual. Differential diagnoses of uncommon bladder malignancies should be considered if there is an inconsistent clinical course as treatment approaches are different. PMID:25737794

  20. Pseudoanaplastic tumors of bone

    Energy Technology Data Exchange (ETDEWEB)

    Bahk, Won-Jong [Uijongbu St. Mary Hospital, The Catholic University of Korea, Department of Orthopaedic Surgery, Gyunggido, 480-821 (Korea); Mirra, Joseph M. [Orthopaedic Hospital, Orthopedic Oncology, Los Angeles, California (United States)

    2004-11-01

    To discuss the concept of pseudoanaplastic tumors of bone, which pathologically show hyperchromatism and marked pleomorphism with quite enlarged, pleomorphic nuclei, but with no to extremely rare, typical mitoses, and to propose guidelines for their diagnosis. From a database of 4,262 bone tumors covering from 1971 to 2001, 15 cases of pseudoanaplastic bone tumors (0.35% of total) were retrieved for clinical, radiographic and pathologic review. Postoperative follow-up after surgical treatment was at least 3 years and a maximum of 7 years. There were eight male and seven female patients. Their ages ranged from 10 to 64 years with average of 29.7 years. Pathologic diagnoses of pseudoanaplastic variants of benign bone tumors included: osteoblastoma (4 cases), giant cell tumor (4 cases), chondromyxoid fibroma (3 cases), fibrous dysplasia (2 cases), fibrous cortical defect (1 case) and aneurysmal bone cyst (1 case). Radiography of all cases showed features of a benign bone lesion. Six cases, one case each of osteoblastoma, fibrous dysplasia, aneurysmal bone cyst, chondromyxoid fibroma, giant cell tumor and osteoblastoma, were initially misdiagnosed as osteosarcoma. The remaining cases were referred for a second opinion to rule out sarcoma. Despite the presence of significant cytologic aberrations, none of our cases showed malignant behavior following simple curettage or removal of bony lesions. Our observation justifies the concept of pseudoanaplasia in some benign bone tumors as in benign soft tissue tumors, especially in their late evolutionary stage when bizarre cytologic alterations strongly mimic a sarcoma. (orig.)

  1. Highly differentiated keratinizing squamous cell cancer of the cervix: a rare, locally aggressive tumor not associated with human papillomavirus or squamous intraepithelial lesions.

    Science.gov (United States)

    Morrison, C; Catania, F; Wakely, P; Nuovo, G J

    2001-10-01

    The purpose of this study is to report an unusual variant of cervical squamous cell carcinoma, not associated with either human papillomavirus infection or antecedent squamous intraepithelial lesions. Five women had a diagnosis of invasive cervical cancer discovered at hysterectomy performed for prolapse (two cases), leiomyoma (one case), or a vaginal fistula (two cases). The women ranged in age from 47 to 78 years (mean 59 years). Four of the five had a history of normal Papanicolaou (Pap) smears; the other had a Pap smear diagnosis of atypical squamous cells of undetermined significance (ASCUS). All had large cervical tumors (two with parametrial involvement and one with vaginal involvement) that showed extensive keratin formation, an inverted pattern of growth, and, except for one case, minimal cytologic atypia. There was extensive hyperkeratosis and parakeratosis adjacent to each tumor; none had evidence of squamous intraepithelial lesion. Human papillomavirus testing by polymerase chain reaction in situ hybridization and reverse-transcribed polymerase chain reaction in situ was negative in each case, compared with a detection rate of 107 of 108 (99%) for squamous intraepithelial lesion-associated cervical squamous cell and adenocarcinomas. Two of the women died of extensive local recurrence; two other women were recently diagnosed. We conclude that highly differentiated keratinizing squamous cell carcinoma of the cervix is a rare entity not associated with human papillomavirus infection or squamous intraepithelial lesion and thus difficult to detect on routine cervical cancer screening.

  2. Collision tumor: serous cystadenocarcinoma and dermoid cyst in the same ovary

    DEFF Research Database (Denmark)

    Bige, Ozgur; Demir, Ahmet; Koyuncuoglu, Meral

    2008-01-01

    INTRODUCTION: Collision tumor means the coexistence of two adjacent, but histologically distinct tumors without histologic admixture in the same tissue or organ. Collision tumors involving ovaries are extremely rare. CASE: We present a case of 45-year-old parous woman with a left dermoid cyst......, with unusual imaging findings, massive ascites and peritoneal carcinomatosis. The patient underwent cytoreductive surgery. The histopathology revealed a collision tumor consisting of an invasive serous cystadenocarcinoma and a dermoid cyst....

  3. An extremely rare clinical entity: congenitally corrected transposition with situs ınversus and single coronary artery presented with complete atrioventricular block in a young man.

    Science.gov (United States)

    Cirakoglu, Omer Faruk; Bayraktar, Ali; Sayin, Muhammet Rasit

    2018-05-01

    Congenitally corrected transposition of the great arteries is a rare form of CHD. Situs inversus is a much less common variant of a congenitally corrected transposition of the great arteries. In rare cases, transposition events may be accompanied by various cardiac anomalies. However, situs inversus patients with congenitally corrected transposition, single coronary artery anomaly, and atrioventricular block together have not been reported previously. This combination of abnormalities is presented as a first in the literature.

  4. A Rare Cause of Testicular Metastasis: Upper Tract Urothelial Carcinoma

    Directory of Open Access Journals (Sweden)

    Alper Nesip Manav

    2014-01-01

    Full Text Available Metastatic testicular cancers are rare. Primary tumor sources are prostate, lung, and gastrointestinal tract for metastatic testicular cancers. Metastasis of urothelial carcinoma (UC to the testis is extremely rare. Two-thirds of upper tract urothelial carcinoma (UTUC is of invasive stage at diagnosis and metastatic sites are the pelvic lymph nodes, liver, lung, and bone. We report a rare case of metastatic UTUC to the testis which has not been reported before, except one case in the literature. Testicular metastasis of UC should be considered in patients with hematuria and testicular swelling.

  5. Lynch syndrome-associated extracolonic tumors are rare in two extended families with the same EPCAM deletion.

    Science.gov (United States)

    Lynch, Henry T; Riegert-Johnson, Douglas L; Snyder, Carrie; Lynch, Jane F; Hagenkord, Jill; Boland, C Richard; Rhees, Jennifer; Thibodeau, Stephen N; Boardman, Lisa A; Davies, Janine; Kuiper, Roland P; Hoogerbrugge, Nicoline; Ligtenberg, Marjolijn J L

    2011-10-01

    The Lynch syndrome (LS) is an inherited cancer syndrome showing a preponderance of colorectal cancer (CRC) in context with endometrial cancer and several other extracolonic cancers, which is due to pathogenic mutations in the mismatch repair (MMR) genes, MLH1, MSH2, MSH6, and PMS2. Some families were found to show a LS phenotype without an identified MMR mutation, although there was microsatellite instability and absence of MSH2 expression by immunohistochemistry. Studies of a subset of these families found a deletion at the 3' end of the epithelial cell adhesion molecule (EPCAM) gene, causing transcription read-through resulting in silencing of MSH2 through hypermethylation of its promoter. The tumor spectrum of such families appears to differ from classical LS. Our study of two large families (USA Family R and Dutch Family A) with an EPCAM deletion was carried out using each institution's standard family study protocol. DNA was extracted from peripheral blood and EPCAM deletion analysis was performed. Both families were found to harbor the same deletion at the 3' end of EPCAM. Analysis showed that the deletion originated from a common ancestor. Family R and Family A members showed segregation of CRC with the presence of this EPCAM mutation. Compared with classic LS, there were almost no extracolonic cancers. Members of Family R and Family A, all with the same EPCAM deletion, predominantly presented with CRC but no LS-associated endometrial cancer, confirming findings seen in other, smaller, LS families with EPCAM mutations. In these EPCAM mutation carriers, cancer surveillance should be focused on CRC.

  6. Sclerosing Stromal Tumor of Ovary: A Case Report

    Directory of Open Access Journals (Sweden)

    Menka Khanna

    2012-01-01

    Full Text Available Sclerosing stromal tumor (SST is an extremely rare and distinctive sex cord stromal tumor which occurs predominantly in the second and third decades of life. We report a case of a 32-year-old woman who developed a sclerosing stromal tumor of ovary and presented with irregular menstruation and pelvic pain. Her hormonal status was normal but CA-125 was raised. She was suspected to have a malignant tumor on computed tomography and underwent bilateral salpingo-oopherectomy. It is therefore necessary to keep in mind the possibility of sclerosing stromal tumor in a young woman.

  7. Synovial Sarcoma of the Buccal Mucosa: A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Kumar T. S. Mahesh

    2013-01-01

    Full Text Available Synovial sarcoma (SS is a rare malignant neoplasm that arises most commonly in joint capsules and articular tendons, but its relationship to the synovium is not always obvious. Synovial sarcoma is a malignant soft tissue tumor representing 5.6% to 10% of all soft tissue sarcomas. They are termed SS because of their histologic resemblance to the synovium, but they rarely involve a synovial structure and are thought to arise from pluripotential mesenchymal cells. The tumor usually occurs in close association with tendon sheaths, bursae, and joint capsules, primarily in the para-articular regions of the extremities, with approximately 9% occurring in the head and neck region. Synovial sarcoma has been reported rarely in the oral cavity. We report a very rare case of Synovial sarcoma of the buccal mucosa in a 24-year-old male patient.

  8. Facial Localization of Malignant Chondroid Syringoma: A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Deniz Tural

    2013-01-01

    Full Text Available First described by Hirsch and Helwig in 1961, chondroid syringomas (CSs are rare, benign tumors of the skin arising from the eccrine sweat glands with tumor differentiation in the epithelial and mesenchymal tissues. They most commonly occur in the head and neck, although they may be also found in the axilla, trunk, limbs, and genitalia. The incidence of CS is <0.01% of all primary skin tumors. Malingnant chondroid syringomas (MCS, which are also called malignant mixed tumors of the skin, are extremely uncommon. MCSs commonly involve the limbs and rarely head and neck. In this article, we present a case of malignant chondroid syringoma localized in the face at the left nasolabial region in the light of literature review.

  9. Brown tumor of lumber spint in patient with chronic renal failure ...

    African Journals Online (AJOL)

    Brown tumors are erosive bone lesions caused by increased osteoclastic activity. They usually occur in the severe forms of secondary hyperparathyroidism, as in patients with hemodialysis-dependent chronic renal disease. Involvement of the lumbar spine with this tumor causing neural compression is extremely rare.

  10. Malignant tumors of the upper aerodigestive tract as seen in a ...

    African Journals Online (AJOL)

    and nasal cavity are more commonly affected than the oral cavity unlike in other populations. Nonepithelial tumors are extremely rare below the age of 20 years. Key words: Malignant tumors, Nigeria, upper aerodigestive tract. Date of Acceptance: 28‑May‑2014. Address for correspondence: Dr. Sabageh Donatus,.

  11. Germ Cell Tumor Located in the Midline of the Anterior Neck

    OpenAIRE

    Tatyana PIRDOPSKA; Ivan TERZIEV; Sv. HRISTOVA; W. MLADENOVSKY; R. PETKOV

    2011-01-01

    Primary germ cell tumors involving midline of the anterior neck are extremely rare. Here we report a 68-year-old male who was operated due to a mass lesion in the anterior neck with infiltration of the isthmus of the thyroid gland. Histopathological examination revealed a germ cell tumor with extragonadal localization in the anterior neck infiltrating the isthmus of the thyroid gland.

  12. Tumors and Pregnancy

    Science.gov (United States)

    Tumors during pregnancy are rare, but they can happen. Tumors can be either benign or malignant. Benign tumors aren't cancer. Malignant ones are. The most common cancers in pregnancy are breast cancer, cervical cancer, lymphoma, and melanoma. ...

  13. CMCTS stabilized Fe3O4 particles with extremely low toxicity as highly efficient near-infrared photothermal agents for in vivo tumor ablation

    Science.gov (United States)

    Shen, Song; Kong, Fenfen; Guo, Xiaomeng; Wu, Lin; Shen, Haijun; Xie, Meng; Wang, Xinshi; Jin, Yi; Ge, Yanru

    2013-08-01

    With the potential uses of photothermal therapy (PTT) in cancer treatment with excellent efficacy, and the growing concerns about the nanotoxicity of hyperthermia agents such as carbon nanotubes and gold-based nanomaterials, the importance of searching for a biocompatible hyperthermia agent cannot be emphasized too much. In this work, a novel promising hyperthermia agent employing magnetic Fe3O4 particles with fairly low toxicity was proposed. This hyperthermia agent showed rapid heat generation under NIR irradiation. After modification with carboxymethyl chitosan (CMCTS), the obtained Fe3O4@CMCTS particles could disperse stably in PBS and serum without any aggregation. The modification of CMCTS could decrease the adsorption of bovine serum albumin (BSA) and improve the cellular uptake. In a comparative study with hollow gold nanospheres (HAuNS), Fe3O4@CMCTS particles exhibited a comparable photothermal effect and fairly low cytotoxicity. The in vivo magnetic resonance (MR) images of mice revealed that by attaching a magnet to the tumor, Fe3O4@CMCTS particles accumulated in the tumor after intravenous injection and showed a low distribution in the liver. After being exposed to a 808 nm laser for 5 min at a low power density of 1.5 W cm-2, the tumors on Fe3O4@CMCTS-injected mice reached a temperature of ~52 °C and were completely destroyed. Thus, a kind of multifunctional magnetic nanoparticle with extremely low toxicity and a simple structure for simultaneous MR imaging, targeted drug delivery and photothermal therapy can be easily fabricated.With the potential uses of photothermal therapy (PTT) in cancer treatment with excellent efficacy, and the growing concerns about the nanotoxicity of hyperthermia agents such as carbon nanotubes and gold-based nanomaterials, the importance of searching for a biocompatible hyperthermia agent cannot be emphasized too much. In this work, a novel promising hyperthermia agent employing magnetic Fe3O4 particles with fairly low

  14. Malignant mixed mullerian tumor arising from the uterine cervix: A case report

    International Nuclear Information System (INIS)

    Shim, Jong Joon; Shim, Jae Chan; Lee, Kyoung Eun; Lee, Ghi Jai; Kim, Ho Kyun; Suh, Jung Ho; Lee, Hye Kyung

    2012-01-01

    Malignant mixed mullerian tumors (MMMTs) are a rare uterine tumor and contribute to approximately 1-3% of all corpus malignant tumors. MMMTs are usually in the uterine corpus, but can also arise from the uterine cervix, vagina, ovaries and fallofian tubes. MMMTs of the uterine cervix are extremely rare. MMMTs are highly malignant and tend to maintain a rapid growth and exhibit a high rate of recurrence. Therefore, the prognosis of patients diagnosed with these types of tumors is extremely poor. We report a rare case of a malignant mixed mullerian tumor arising from the uterine cervix and introduce CT and MRI findings. CT and magnetic resonance findings of the uterine cervical MMMT in our case show highly aggressive features, such as parametrial involvement, pelvic and paraaortic lymphadenopathy, and distant metastasis and high enhancement

  15. Calcitonin-producing well-differentiated neuroendocrine carcinoma (carcinoid tumor of the urinary bladder: case report

    Directory of Open Access Journals (Sweden)

    De Rosa Gaetano

    2005-07-01

    Full Text Available Abstract Background The occurrence of calcitonin-secreting primary carcinoid tumor of the urinary bladder is extremely rare. Case presentation The case of a 68-year-old male with carcinoid tumor arising in the urinary bladder is presented. Transurethral resection of a polypoid small tumor 0.4 cm in diameter was performed. Immunohistochemical study using neuroendocrine markers allowed a straightforward diagnosis of a low-grade neuroendocrine carcinoma (carcinoid tumor of the urinary bladder. Immunohistochemistry demonstrated calcitonin immunoreactivity in the most of the tumor cells. Conclusion This tumor shows specific clinical, macroscopical and histological features and must be considered in the differential diagnosis of bladder neoplasms.

  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. Solitary cysticercosis of the biceps brachii in a vegetarian: a rare and unusual pseudotumor

    Energy Technology Data Exchange (ETDEWEB)

    Abdelwahab, Ibrahim Fikry [Department of Radiology, New York Methodist Hospital Affiliated with Weill Medical College of Cornell University, 506 Sixth Street, Brooklyn, NY 11215 (United States); Klein, Michael J. [Department of Pathology, Mount Sinai Medical Center, 1 Gustave Levy Place, New York, NY 10029 (United States); Hermann, George [Department of Radiology, Mount Sinai Medical Center, 1 Gustave Levy Place, New York, NY 10029 (United States); Abdul-Quader, Mohammed [Department of Radiology, Columbia Presbyterian Medical Center, 177 Fort Washington Avenue, New York, NY 10032 (United States)

    2003-07-01

    We report a 40-year-old man with cysticercosis presenting as a solitary tumor in the biceps brachii muscle. Physical examination revealed an intramuscular mass and magnetic resonance imaging suggested a cyst. The histologic diagnosis was a cysticercus. Such solitary presentation of muscular cysticercosis is extremely rare with only a handful of sporadic reports in the literature. (orig.)

  18. Solitary cysticercosis of the biceps brachii in a vegetarian: a rare and unusual pseudotumor

    International Nuclear Information System (INIS)

    Abdelwahab, Ibrahim Fikry; Klein, Michael J.; Hermann, George; Abdul-Quader, Mohammed

    2003-01-01

    We report a 40-year-old man with cysticercosis presenting as a solitary tumor in the biceps brachii muscle. Physical examination revealed an intramuscular mass and magnetic resonance imaging suggested a cyst. The histologic diagnosis was a cysticercus. Such solitary presentation of muscular cysticercosis is extremely rare with only a handful of sporadic reports in the literature. (orig.)

  19. Spontaneous Rupture of Splenic Artery Aneurysm during the First Trimester of Pregnancy: Report of an Extremely Rare Case and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Theodoros Pavlis

    2012-01-01

    Full Text Available Splenic artery aneurysm (SAA occurs predominantly in women and the majority of them are asymptomatic until rupture. In cases of spontaneous rupture of an SAA, maternal and fetal mortality rates remain extremely high. Furthermore, the spontaneous ruptures of SAAs predominantly appear during the third trimester of pregnancy. We present the third known case of spontaneous SAA rupture during the first trimester of pregnancy, which manifested as sudden hypovolemic collapse and was successfully confronted with combined aggressive resuscitation and emergency surgical operation.

  20. PRIMARY HEMANGIOMA OF A SUBMENTAL LYMPH NODE –A RARE ENTITY

    Directory of Open Access Journals (Sweden)

    Shri LakshmiS, Durga PrasadD, Subba Rao D, PrasanthiC, Vandana Gangadharan, Kishore Kumar C

    2015-04-01

    Full Text Available ABSTRACT Primary vascular tumors occurring in lymph nodes are extremely rare. Nodal hemangiomas are benign vascular tumors that can occur at any age and seen mostly in females. It is usually asymptomatic, affects only one node, and does not recur. Four histologic types of hemangioma have been identified: capillary/cavernous, lobular capillary, cellular, and epithelioid. This case has been reported for its rarity

  1. Soft-Tissue Chondroma of Anterior Gingiva: A Rare Entity

    Directory of Open Access Journals (Sweden)

    Dhana Lakshmi Jeyasivanesan

    2018-01-01

    Full Text Available Soft-tissue chondroma is a rare, benign, slow-growing tumor made up of heterotopic cartilaginous tissue. It occurs most commonly in the third and fourth decades in the hands and feet. Oral soft-tissue chondromas are uncommon and soft-tissue chondroma of gingiva is extremely uncommon. Here, we report an unusual case of soft-tissue chondroma of gingiva in a 50-year-old woman.

  2. Bone Fractures Following External Beam Radiotherapy and Limb-Preservation Surgery for Lower Extremity Soft Tissue Sarcoma: Relationship to Irradiated Bone Length, Volume, Tumor Location and Dose

    International Nuclear Information System (INIS)

    Dickie, Colleen I.; Parent, Amy L.; Griffin, Anthony M.; Fung, Sharon; Chung, Peter W.M.; Catton, Charles N.; Ferguson, Peter C.; Wunder, Jay S.; Bell, Robert S.; Sharpe, Michael B.; O'Sullivan, Brian

    2009-01-01

    Purpose: To examine the relationship between tumor location, bone dose, and irradiated bone length on the development of radiation-induced fractures for lower extremity soft tissue sarcoma (LE-STS) patients treated with limb-sparing surgery and radiotherapy (RT). Methods and Materials: Of 691 LE-STS patients treated from 1989 to 2005, 31 patients developed radiation-induced fractures. Analysis was limited to 21 fracture patients (24 fractures) who were matched based on tumor size and location, age, beam arrangement, and mean total cumulative RT dose to a random sample of 53 nonfracture patients and compared for fracture risk factors. Mean dose to bone, RT field size (FS), maximum dose to a 2-cc volume of bone, and volume of bone irradiated to ≥40 Gy (V40) were compared. Fracture site dose was determined by comparing radiographic images and surgical reports to fracture location on the dose distribution. Results: For fracture patients, mean dose to bone was 45 ± 8 Gy (mean dose at fracture site 59 ± 7 Gy), mean FS was 37 ± 8 cm, maximum dose was 64 ± 7 Gy, and V40 was 76 ± 17%, compared with 37 ± 11 Gy, 32 ± 9 cm, 59 ± 8 Gy, and 64 ± 22% for nonfracture patients. Differences in mean, maximum dose, and V40 were statistically significant (p = 0.01, p = 0.02, p = 0.01). Leg fractures were more common above the knee joint. Conclusions: The risk of radiation-induced fracture appears to be reduced if V40 <64%. Fracture incidence was lower when the mean dose to bone was <37 Gy or maximum dose anywhere along the length of bone was <59 Gy. There was a trend toward lower mean FS for nonfracture patients.

  3. Bone fractures following external beam radiotherapy and limb-preservation surgery for lower extremity soft tissue sarcoma: relationship to irradiated bone length, volume, tumor location and dose.

    Science.gov (United States)

    Dickie, Colleen I; Parent, Amy L; Griffin, Anthony M; Fung, Sharon; Chung, Peter W M; Catton, Charles N; Ferguson, Peter C; Wunder, Jay S; Bell, Robert S; Sharpe, Michael B; O'Sullivan, Brian

    2009-11-15

    To examine the relationship between tumor location, bone dose, and irradiated bone length on the development of radiation-induced fractures for lower extremity soft tissue sarcoma (LE-STS) patients treated with limb-sparing surgery and radiotherapy (RT). Of 691 LE-STS patients treated from 1989 to 2005, 31 patients developed radiation-induced fractures. Analysis was limited to 21 fracture patients (24 fractures) who were matched based on tumor size and location, age, beam arrangement, and mean total cumulative RT dose to a random sample of 53 nonfracture patients and compared for fracture risk factors. Mean dose to bone, RT field size (FS), maximum dose to a 2-cc volume of bone, and volume of bone irradiated to >or=40 Gy (V40) were compared. Fracture site dose was determined by comparing radiographic images and surgical reports to fracture location on the dose distribution. For fracture patients, mean dose to bone was 45 +/- 8 Gy (mean dose at fracture site 59 +/- 7 Gy), mean FS was 37 +/- 8 cm, maximum dose was 64 +/- 7 Gy, and V40 was 76 +/- 17%, compared with 37 +/- 11 Gy, 32 +/- 9 cm, 59 +/- 8 Gy, and 64 +/- 22% for nonfracture patients. Differences in mean, maximum dose, and V40 were statistically significant (p = 0.01, p = 0.02, p = 0.01). Leg fractures were more common above the knee joint. The risk of radiation-induced fracture appears to be reduced if V40 Fracture incidence was lower when the mean dose to bone was lower mean FS for nonfracture patients.

  4. Disseminated Intracranial Ewing’s Sarcoma in an Adult:A Rare and Difficult Diagnosis

    Directory of Open Access Journals (Sweden)

    Emil Lou

    2012-06-01

    Full Text Available The Ewing sarcoma family of tumors comprises a rare class of cancers of mesenchymal origin. Cases of Ewing’s sarcoma in the central nervous system – specifically, intracranial Ewing’s – are extremely rare. Almost all reported cases have occurred in children. However, this rare presentation can also occur in the adult population. It is important to distinguish these tumors from primitive neuroectodermal tumors at the time of diagnosis. Testing for EWSR1(22q12 gene rearrangement using fluorescence in situ hybridization is a useful tool for making the distinction between these 2 similar but distinct entities. We present here the case of a middle-aged male patient with intracranial Ewing’s sarcoma, and discuss diagnostic challenges and potential new treatment approaches for this rare disease.

  5. Statistics of Extremes

    KAUST Repository

    Davison, Anthony C.; Huser, Raphaë l

    2015-01-01

    Statistics of extremes concerns inference for rare events. Often the events have never yet been observed, and their probabilities must therefore be estimated by extrapolation of tail models fitted to available data. Because data concerning the event

  6. Malignant Trigeminal Nerve Sheath Tumor and Anaplastic Astrocytoma Collision Tumor with High Proliferative Activity and Tumor Suppressor P53 Expression

    Directory of Open Access Journals (Sweden)

    Maher Kurdi

    2014-01-01

    Full Text Available Background. The synchronous development of two primary brain tumors of distinct cell of origin in close proximity or in contact with each other is extremely rare. We present the first case of collision tumor with two histological distinct tumors. Case Presentation. A 54-year-old woman presented with progressive atypical left facial pain and numbness for 8 months. MRI of the brain showed left middle cranial fossa heterogeneous mass extending into the infratemporal fossa. At surgery, a distinct but intermingled intra- and extradural tumor was demonstrated which was completely removed through left orbitozygomatic-temporal craniotomy. Histopathological examination showed that the tumor had two distinct components: malignant nerve sheath tumor of the trigeminal nerve and temporal lobe anaplastic astrocytoma. Proliferative activity and expressed tumor protein 53 (TP53 gene mutations were demonstrated in both tumors. Conclusions. We describe the first case of malignant trigeminal nerve sheath tumor (MTNST and anaplastic astrocytoma in collision and discuss the possible hypothesis of this rare occurrence. We propose that MTNST, with TP53 mutation, have participated in the formation of anaplastic astrocytoma, or vice versa.

  7. Primary malignant mixed müllerian tumor of the peritoneum a case report with review of the literature

    DEFF Research Database (Denmark)

    Kurshumliu, Fisnik; Rung-Hansen, Helle; Skovlund, Vibeke Ravn

    2011-01-01

    Malignant mixed Müllerian tumor is a rare malignancy of the genital tract and extremely uncommon in extragenital sites. This report describes a case of malignant mixed Müllerian tumor arising in the lower peritoneum of a 72-year-old female patient. The patient presented with ascites, lower...

  8. A Rare Cause of Abdominal Pain in Childhood: Cardiac Angiosarcoma

    Directory of Open Access Journals (Sweden)

    Elvan Caglar Citak

    Full Text Available Abstract Cardiac angiosarcomas are extremely rare in childhood, they are rapidly progressive tumours that often present themselves as diagnostic dilemmas, resulting in delayed diagnosis. Also, extracardiac manifestations, including abdominal pain, are extremely rare in patients with intracardiac tumors. We herein present the case of a 15-year-old girl who presented with abdominal pain. Echocardiography and thoracic computed tomography showed right atrial mass. The patient underwent surgery, chemotherapy, and radiotherapy. Eight months after treatment, abdominal recurrence was detected. The abdominal mass was resected, and radiotherapy and new chemotherapy protocol were given. The present case illustrates a rare case of primary cardiac angiosarcoma posing a diagnostic dilemma in an adolescent girl.

  9. Multi-focal lobular carcinoma in situ arising in benign phylodes tumor: A case report

    International Nuclear Information System (INIS)

    Lee, Taeg Ki; Choi, Chang Hwan; Kim, Youn Jeong; Kim, Mi Young; Lee, Kyung Hee; Cho, Soon Gu

    2015-01-01

    Coexistent breast malignancy arising in phyllodes tumor is extremely rare, and most of them are incidental reports after surgical excision. Coexistent malignancy in phyllodes tumor can vary from in-situ to invasive carcinoma. Lobular neoplasia is separated into atypical lobular hyperplasia and lobular carcinoma in situ (LCIS). LCIS is known to have a higher risk of developing invasive cancer. We reported imaging findings of multifocal LCIS arising in benign phyllodes tumor

  10. Multi-focal lobular carcinoma in situ arising in benign phylodes tumor: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Taeg Ki; Choi, Chang Hwan; Kim, Youn Jeong; Kim, Mi Young; Lee, Kyung Hee; Cho, Soon Gu [Inha University Hospital, Incheon (Korea, Republic of)

    2015-08-15

    Coexistent breast malignancy arising in phyllodes tumor is extremely rare, and most of them are incidental reports after surgical excision. Coexistent malignancy in phyllodes tumor can vary from in-situ to invasive carcinoma. Lobular neoplasia is separated into atypical lobular hyperplasia and lobular carcinoma in situ (LCIS). LCIS is known to have a higher risk of developing invasive cancer. We reported imaging findings of multifocal LCIS arising in benign phyllodes tumor.

  11. Chondroma of Falx: Case Report of a Rare Condition

    Directory of Open Access Journals (Sweden)

    Shahryar Shahriarian

    2012-03-01

    Full Text Available Chondroma is a benign tumor which mostly occurs in extremities but also sometimes in brain. Most intracranial chondromas arise from skull base, but chondroma of falx origin is a rare circumstance. Indeed, the intracranial chondromas rise from falx is mostly in relation with syndromic disorders such as Mafuccis syndrome or Olliers syndrome. Here, we reported a rare case of falxian intracranial chondroma in a young man who has normal physical examination and no signs of any syndromic disorder. The goal of this paper was to raise awareness about chondromas and suggest that chondroma be ruled out in any patient with masses arising from falx.

  12. C-Window Peaks on CE-HPLC are Extremely Rare in Northern India, and Only Infrequently Represent HbC.

    Science.gov (United States)

    Dass, Jasmita; Mittal, Suchi; Saraf, Amrita; Kotwal, Jyoti

    2018-01-01

    Hemoglobin C (HbC, HBB:c.19G > A) is a structural variant that has been reported rarely from India. This was a retrospective review of all high performance liquid chromatography (HPLCs) submitted over a 14 year period to a tertiary care center in North India with an aim of finding hemoglobins that elute in the C-window. Of the 32,364 HPLCs screened, 6 cases showed peaks in the C-window. Of these 6 cases, only two cases contained hemoglobin C. These was one case each of HbC/β thalassemia and compound heterozygosity for HbC and HbD. There were 4 cases which showed very similar red cell indices and chromatograms with multiple peaks eluting in D-window, C-window and an additional peak with a retention time of 4.74 min. These four cases were compound heterozygous for an α chain variant HbQ-India and a β-chain variant HbD.

  13. An integrated wave modelling framework for extreme and rare events for climate change in coastal areas – the case of Rethymno, Crete

    Directory of Open Access Journals (Sweden)

    Vasiliki K. Tsoukala

    2016-04-01

    Full Text Available Coastal floods are regarded as among the most dangerous and harmful of all natural disasters affecting urban areas adjacent to the shorelines. Rapid urbanization combined with climate change and poor governance often results in significant increase of flood risk, especially for coastal communities. Wave overtopping and wave run-up are the key mechanisms for monitoring the results of coastal flooding and as such, significant efforts are currently focusing on their predicting. In this paper, an integrated methodology is proposed, accounting for wave overtopping and wave run-up under extreme wave scenarios caused by storm surges. By taking advantage of past and future climatic projections of wind data, a downscaling approach is proposed, utilizing a number of appropriate numerical models than can simulate the wave propagation from offshore up to the swash zone. The coastal zone of Rethymno in Greece is selected as a case study area and simulations of wave characteristics with the model SWAN for the period 1960–2100 in the offshore region are presented. These data are given as boundary conditions to further numerical models (MIKE21 PMS and HD in order to investigate the spatial evolution of the wave and the hydrodynamic field in intermediate and shallow waters. Finally, the calculated wave height serves as input to empirical formulas and time dependent wave propagation models (MIKE21 BW to estimate the wave run-up and wave overtopping (EurOtop. It is suggested that the proposed procedure is generic enough to be applicable to any similar region.

  14. Inflammatory Myofibroblastic Tumor of the Nasal Septum

    Directory of Open Access Journals (Sweden)

    Yuri Okumura

    2013-01-01

    Full Text Available We report an extremely rare case of inflammatory myofibroblastic tumor of the posterior edge of the nasal septum. An 11-year-old boy presented with frequent epistaxis and nasal obstruction persisting for one year. Based on the clinical presentation and imaging studies, juvenile angiofibroma was suspected, but angiography suggested the possibility of another type of tumor. Transnasal endoscopic surgery found that the tumor protruded into the nasopharynx from the posterior end of the nasal septum. Histological examination identified spindle cells with immunoreaction for vimentin, smooth muscle actin, and anaplastic lymphoma kinase (ALK, but not for desmin and cytokeratin. This is a report of inflammatory myofibroblastic tumor mimicking juvenile angiofibroma. This case suggests that angiography is helpful in the differential diagnosis of epipharyngeal tumor in adolescence.

  15. Bilateral metachronous periosteal tibial amyloid tumors

    International Nuclear Information System (INIS)

    Murata, H.; Kusuzaki, Katsuyuki; Hashiguchi, S.; Ueda, Hidetaka; Hirasawa, Yasusuke

    2000-01-01

    Localized primary periosteal amyloid tumors are extremely rare. A case of bilateral tibial amyloid tumor is presented. A 62-year-old woman initially presented with a painful mass in the anterior aspect of the right leg. There was no evidence of underlying systemic disease, including chronic infection or malignancy. Based on the results of resistance with Congo red staining to treatment with potassium permanganate and positivity for kappa light chain, we classified this particular case as AL-type amyloidosis. The patient noticed a swelling in the opposite leg 2 years later. The second tumor was also an AL-type amyloidoma. Amyloid tumors are generally solitary. This is the first case of bilateral periosteal amyloid tumors of the AL-type occurring in the tibiae. (orig.)

  16. Rare Infratentorial and Supratentorial Localization of Juvenile Angiofibroma: A Case Report.

    Science.gov (United States)

    Pašalić, Ivan; Trninić, Ines; Nemir, Jakob; Jednačak, Hrvoje; Žarković, Kamelija; Mrak, Goran

    2016-01-01

    Angiofibromas are rare tumors of the head and neck that mostly occur in the sphenopalatine region. We present a case of angiofibroma in a young male patient with an unusual and extremely rare localization, which to our knowledge has not been described before. It was situated in the tentorium and spread to the supratentorial and infratentorial regions. The patient initially presented with symptoms of increased intracranial pressure. After a diagnostic evaluation was done, the whole tumor was successfully removed using the supratentorial and infratentorial approach and the microsurgical technique. © 2016 S. Karger AG, Basel.

  17. Oral melanotic neuroectodermal tumor of infancy

    Directory of Open Access Journals (Sweden)

    Seema Chaudhary

    2014-01-01

    Full Text Available Melanotic neuroectodermal tumor of infancy (MNTI is an extremely rare, benign neoplasm of neural crest origin composed of relatively primitive pigment-producing cells. It usually affects new born or infants of <1 year of age, generally involving the face or cranium. This case report describes a 4-month-old child with MNTI involving the anterior region of maxilla. A partial maxillectomy was performed on the left side of maxilla and the patient is now under follow-up.

  18. Strategy to find molecular signatures in a small series of rare cancers: validation for radiation-induced breast and thyroid tumors.

    Directory of Open Access Journals (Sweden)

    Nicolas Ugolin

    Full Text Available Methods of classification using transcriptome analysis for case-by-case tumor diagnosis could be limited by tumor heterogeneity and masked information in the gene expression profiles, especially as the number of tumors is small. We propose a new strategy, EMts_2PCA, based on: 1 The identification of a gene expression signature with a great potential for discriminating subgroups of tumors (EMts stage, which includes: a a learning step, based on an expectation-maximization (EM algorithm, to select sets of candidate genes whose expressions discriminate two subgroups, b a training step to select from the sets of candidate genes those with the highest potential to classify training tumors, c the compilation of genes selected during the training step, and standardization of their levels of expression to finalize the signature. 2 The predictive classification of independent prospective tumors, according to the two subgroups of interest, by the definition of a validation space based on a two-step principal component analysis (2PCA. The present method was evaluated by classifying three series of tumors and its robustness, in terms of tumor clustering and prediction, was further compared with that of three classification methods (Gene expression bar code, Top-scoring pair(s and a PCA-based method. Results showed that EMts_2PCA was very efficient in tumor classification and prediction, with scores always better that those obtained by the most common methods of tumor clustering. Specifically, EMts_2PCA permitted identification of highly discriminating molecular signatures to differentiate post-Chernobyl thyroid or post-radiotherapy breast tumors from their sporadic counterparts that were previously unsuccessfully classified or classified with errors.

  19. Dedifferentiated liposarcoma of the anterior mediastinum. A rare case

    International Nuclear Information System (INIS)

    Harth, S.; Litzlbauer, H.D.; Behrens, C.B.; Roller, F.C.; Gamerdinger, U.; Burchert, D.; Krombach, G.A.

    2016-01-01

    Liposarcoma accounts for approximately 14 % of all malignant soft-tissue tumors, regardless of anatomical location (Kransdorf MJ et al. Imaging of soft tissue tumors. Philadelphia: Lippincott Williams and Wilkins, 2014). Primary mediastinal liposarcomas are rare. Liposarcoma is classified into four histologic subtypes: Myxoid/round cell, pleomorphic, atypical lipomatous tumor/well-differentiated liposarcoma and dedifferentiated liposarcoma. Dedifferentiated liposarcoma occurs most commonly in the retroperitoneum and in the soft tissues of the extremities. Like atypical lipomatous tumor/well-differentiated liposarcoma, it is characterized by amplification of MDM2 and CDK4 genes on chromosome 12 (Crago AM et al. Curr Opin Oncol 2011; 23: 373 - 378). Possible symptoms of mediastinal liposarcoma are dyspnea, wheezing, chest pain, cough, superior vena cava syndrome, and weight loss (Macchiarini P et al. Lancet Oncol 2004; 5: 107 - 118).

  20. Dedifferentiated liposarcoma of the anterior mediastinum. A rare case

    Energy Technology Data Exchange (ETDEWEB)

    Harth, S.; Litzlbauer, H.D.; Behrens, C.B.; Roller, F.C.; Gamerdinger, U.; Burchert, D.; Krombach, G.A.

    2016-01-15

    Liposarcoma accounts for approximately 14 % of all malignant soft-tissue tumors, regardless of anatomical location (Kransdorf MJ et al. Imaging of soft tissue tumors. Philadelphia: Lippincott Williams and Wilkins, 2014). Primary mediastinal liposarcomas are rare. Liposarcoma is classified into four histologic subtypes: Myxoid/round cell, pleomorphic, atypical lipomatous tumor/well-differentiated liposarcoma and dedifferentiated liposarcoma. Dedifferentiated liposarcoma occurs most commonly in the retroperitoneum and in the soft tissues of the extremities. Like atypical lipomatous tumor/well-differentiated liposarcoma, it is characterized by amplification of MDM2 and CDK4 genes on chromosome 12 (Crago AM et al. Curr Opin Oncol 2011; 23: 373 - 378). Possible symptoms of mediastinal liposarcoma are dyspnea, wheezing, chest pain, cough, superior vena cava syndrome, and weight loss (Macchiarini P et al. Lancet Oncol 2004; 5: 107 - 118).

  1. Spinal tumors

    International Nuclear Information System (INIS)

    Goethem, J.W.M. van; Hauwe, L. van den; Oezsarlak, Oe.; Schepper, A.M.A. de; Parizel, P.M.

    2004-01-01

    Spinal tumors are uncommon lesions but may cause significant morbidity in terms of limb dysfunction. In establishing the differential diagnosis for a spinal lesion, location is the most important feature, but the clinical presentation and the patient's age and gender are also important. Magnetic resonance (MR) imaging plays a central role in the imaging of spinal tumors, easily allowing tumors to be classified as extradural, intradural-extramedullary or intramedullary, which is very useful in tumor characterization. In the evaluation of lesions of the osseous spine both computed tomography (CT) and MR are important. We describe the most common spinal tumors in detail. In general, extradural lesions are the most common with metastasis being the most frequent. Intradural tumors are rare, and the majority is extramedullary, with meningiomas and nerve sheath tumors being the most frequent. Intramedullary tumors are uncommon spinal tumors. Astrocytomas and ependymomas comprise the majority of the intramedullary tumors. The most important tumors are documented with appropriate high quality CT or MR images and the characteristics of these tumors are also summarized in a comprehensive table. Finally we illustrate the use of the new World Health Organization (WHO) classification of neoplasms affecting the central nervous system

  2. Metaphyseal giant cell tumor

    International Nuclear Information System (INIS)

    Pereira, L.F.; Hemais, P.M.P.G.; Aymore, I.L.; Carmo, M.C.R. do; Cunha, M.E.P.R. da; Resende, C.M.C.

    1986-01-01

    Three cases of metaphyseal giant cell tumor are presented. A review of the literature is done, demostrating the lesion is rare and that there are few articles about it. Age incidence and characteristics of the tumor are discussed. (Author) [pt

  3. Oncocytic carcinoma of lip: A rare neoplasm of minor salivary gland

    Directory of Open Access Journals (Sweden)

    Sonia Chhabra

    2012-01-01

    Full Text Available Oncocytic carcinoma is an extremely rare neoplasm of the salivary gland, with only a few cases reported in literature till date. We report the occurrence of this rare lesion in lip in a 43-year-old female presenting with a progressively increasing swelling for which excision was done. Fine needle aspiration was done and the smears revealed tumor cells with well-defined cell borders, round to oval, central to eccentrically located moderately pleomorphic nuclei with fine chromatin, prominent nucleoli and abundant eosinophilic granular cytoplasm. Microscopic examination of the resected tumor showed solid sheets, nests, islands and cords of oncocytic cells diffusely infiltrating the surrounding tissues. After 5 months, the patient again presented with bilateral submandibular and right axillary lymphadenopathy revealing metastatic deposits from oncocytic carcinoma. We report this case of oncocytic carcinoma because of its unusual location, the minor salivary gland of lip being a rare site for the tumor.

  4. Rare case of pancreatic cancer with leptomeningeal carcinomatosis

    Science.gov (United States)

    Yoo, In Kyung; Lee, Hong Sik; Kim, Chang Duk; Chun, Hoon Jai; Jeen, Yoon Tae; Keum, Bora; Kim, Eun Sun; Choi, Hyuk Soon; Lee, Jae Min; Kim, Seung Han; Nam, Seung Joo; Hyun, Jong Jin

    2015-01-01

    Leptomeningeal carcinomatosis occurs very rarely in patients with pancreatic cancer. Leptomeningeal carcinomatosis is characterized by multifocal seeding of the leptomeninges by malignant cells that originate from a solid tumor. To the best of our knowledge, brain metastasis from pancreatic cancer is extremely rare. Leptomeningeal carcinomatosis is estimated to occur in 3% to 8% of cases of solid tumors. The clinical manifestation usually involves neurological symptoms, including dizziness, headache, vomiting, nausea, and hemiparesis, symptoms similar to those of meningitis or brain tumors. Diagnostic methods for leptomeningeal carcinomatosis include brain magnetic resonance imaging and cerebrospinal fluid examination. Here, we describe a case of leptomeningeal carcinomatosis in which the primary tumor was later determined to be pancreatic cancer. Brain magnetic resonance imaging findings showed mild enhancement of the leptomeninges, and cerebrospinal fluid cytology was negative at first. However, after repeated spinal taps, atypical cells were observed on cerebrospinal fluid analysis and levels of tumor markers such as carbohydrate antigen 19-9 in cerebrospinal fluid were elevated. Abdominal computed tomography, performed to determine the presence of extracerebral tumors, revealed pancreatic cancer. Pancreatic cancer was confirmed histopathologically on examination of an endoscopic ultrasound-guided fine needle aspiration specimen. PMID:25624740

  5. A Rare Complication of the Thyroid Malignancies: Jugular Vein Invasion

    International Nuclear Information System (INIS)

    Dikici, Atilla Süleyman; Yıldırım, Onur; Er, Mehmet Emin; Kılıç, Fahrettin; Tutar, Onur; Kantarcı, Fatih; Mihmanlı, Ismail

    2015-01-01

    Unilateral invasion of the internal jugular vein (IJV) after subtotal thyroidectomy caused by local recurrence of papillary thyroid carcinoma is extremely rare. We report a case of papillary thyroid carcinoma which invades IJV with hypervascular tumor thrombus. We report a case of a 52-year-old woman with a history of previous thyroid operation who presented with a 2-month history of a painless, growing, hard, solitary mass on the left side of the neck. Clinical examination revealed also ipsilateral cervical lymphadenopathy. Radiological examination showed a necrotic and cystic mass arising from the operated area extending and invading the left jugular vein wall with hypervascular tumor thrombus. Cytological examination of the mass confirmed a papillary thyroid carcinoma (PTC) and enlarged metastatic lymph nodes. Therefore, total thyroidectomy with left neck dissection and segmental resection of the left internal jugular vein were performed, and the tumor thrombus was cleared successfully. Invasion of IJV with hypervascular tumor thrombosis is an extremely rare condition in papillary thyroid carcinoma. Thrombosis of IJV is probably underdiagnosed. Early-stage diagnosis is important for long-term survival rates

  6. Primary intraosseous squamous cell carcinoma in odontogenic keratocyst: A rare entity

    Science.gov (United States)

    Saxena, Chitrapriya; Aggarwal, Pooja; Wadhwan, Vijay; Bansal, Vishal

    2015-01-01

    Squamous cell carcinoma (SCC) arising from the wall of an odontogenic cyst (also known as primary intraosseous carcinoma) is a rare tumor which occurs only in jaw bones. This tumor was first described by Loos in 1913 as a central epidermoid carcinoma of the jaw. Primary intraosseous carcinomas (PIOC) may theoretically arise from the lining of an odontogenic cyst or de novo from presumed odontogenic cell rests. According to the new histological classification of tumors of the World Health Organization, odontogenic keratocyst is nowadays considered a specific odontogenic tumor and the PIOC derived from it is considered as a specific entity which is different from other PIOCs derived from the odontogenic cysts. The following report describes a case of such extremely rare entity that is primary intraosseous SCC of the mandible derived from an OKC in a 60-year-old male patient with brief review of literature. PMID:26980976

  7. Myxofibrosarcoma of maxilla: A case report of rare entity

    Directory of Open Access Journals (Sweden)

    Venkateswarlu Nallapu

    2015-01-01

    Full Text Available Myxofibrosarcoma is one of the most common sarcomas in the extremities of elderly patients that rarely affect the oral cavity. In this article we describe a case of primary myxofibrosarcoma of maxilla in a 19-year-old boy. Microscopically the tumor showed loosely arranged spindle-shaped tumor cells with mild pleomorphic spindle-shaped nuclei, some with minute nucleoli in a fibromyxoid stroma. The cells were strongly immunoreactive for vimentin and negative for CD-68, S-100 protein and desmin. Based on clinical, histological and immunohistochemical findings, a final diagnosis of myxofibrosarcoma was established. It is an unusual aggressive variant that frequently recurs and metastasizes, reinforcing the importance of its correct diagnosis. We report a rare case of myxofibrosarcoma in maxilla, with emphasis on immunohistochemical findings.

  8. Tumor carcinoide apendicular Appendiceal carcinoid tumor

    Directory of Open Access Journals (Sweden)

    Julio Vázquez Palanco

    2008-12-01

    Full Text Available El objetivo de este trabajo fue dar a conocer un interesante caso de tumor carcinoide que se presentó con cuadro clínico de apendicitis aguda. El paciente fue un varón de 8 años de edad, al cual se realizó apendicectomía a causa de una apendicitis aguda. El resultado anatomopatológico confirmó un tumor de células endocrinas (argentafinoma, tumor carcinoide en el tercio distal del órgano, que infiltraba hasta la serosa, y apendicitis aguda supurada. El paciente fue enviado a un servicio de oncohematología para tratamiento oncoespecífico. Por lo inusual de estos tumores en edades tempranas y por lo que puede representar para el niño una conducta no consecuente, decidimos presentar este caso a la comunidad científica nacional e internacional. Es extremadamente importante el seguimiento de los pacientes con apendicitis aguda y de las conclusiones del examen histológico, por lo que puede representar para el niño una conducta inadecuada en una situación como esta.The objective of this paper was to make known an interesting case of carcinoid tumor that presented a clinical picture of acute appendicitis.The patient was an eight-year-old boy that underwent appendectomy due to an acute appendicitis. The anatomopathological report confirmed an endocrine cell tumor (argentaffinoma, carcinoid tumor in the distal third of the organ that infiltrated up to the serosa, and acute suppurative appendicitis. The patient was referred to an oncohematology service for oncospecific treatment. As it is a rare tumor at early ages, and taking into account what a inconsequent behavior may represent for the child, it was decided to present this case to the national and international scientific community. The follow-up of the patients with acute appendicitis and of the conclusions of the histological examination is extremely important considering what an inadequate conduct may represent for the child in a situation like this.

  9. An Intra-Abdominal Desmoid Tumor, Embedded in the Pancreas, Preoperatively Diagnosed as an Extragastric Growing Gastrointestinal Stromal Tumor

    Directory of Open Access Journals (Sweden)

    Mari Mizuno

    2017-04-01

    Full Text Available A 45-year-old woman was found to have a pancreatic tumor by abdominal ultrasound performed for a medical check-up. Abdominal contrast-enhanced computed tomography showed a hypovascular tumor measuring 30 mm in diameter in the pancreatic tail. Endoscopic ultrasound-guided fine needle aspiration was performed. An extragastric growing gastrointestinal stromal tumor was thereby diagnosed preoperatively, and surgical resection was planned. Laparoscopic surgery was attempted but conversion to open surgery was necessitated by extensive adhesions, and distal pancreatectomy, splenectomy, and partial gastrectomy were performed. The histological diagnosis was an intra-abdominal desmoid tumor. A desmoid tumor is a fibrous soft tissue tumor arising in the fascia and musculoaponeurotic tissues. It usually occurs in the extremities and abdominal wall, and only rarely in the abdominal cavity. We experienced a case with an intra-abdominal desmoid tumor that was histologically diagnosed after laparotomy, which had been preoperatively diagnosed as an extragastric growing gastrointestinal stromal tumor. Although rare, desmoid tumors should be considered in the differential diagnosis of intra-abdominal tumors. Herein, we report this case with a literature review.

  10. Pediatric gliosarcoma with fibrosarcomatous differentiation: Report of a rare case

    Directory of Open Access Journals (Sweden)

    Shantha Ravisankar

    2012-01-01

    Full Text Available Gliosarcoma is a rare variant of glioblastoma with a biphasic pattern showing glial and mesenchymal differentiation. It is seen in adults during their fifth to sixth decades of life and is extremely rare in children. We report a case of primary gliosarcoma with fibrosarcomatous differentiation in an 11-year-old boy presenting with headache and vomiting. Imaging showed a contrast-enhancing isodense space-occupying lesion with areas of calcification in the right temporoparietal cortex. A total excision was done and, on histopathologic examination, a differential diagnostic consideration of gliosarcoma and teratoma with malignant transformation was made. After immunohistochemical analysis, a final diagnosis of gliosarcoma with fibrosarcomatous differentiation was then made. Primary gliosarcoma is a very rare tumor in children with a poor prognosis.

  11. Intracerebellar malignant nerve sheath tumor in a child: case report and review of literature.

    Science.gov (United States)

    Joshi, Krishna Chaitanya; Chakravarthy, Hariprakash; Subramanian, Nirmala

    2015-05-01

    Intracerebellar malignant nerve sheath tumor (ICMNST) is an extremely rare entity, only two cases have been reported previously, and this is the first case to be reported in a child. The histogenesis, diagnosis, and management of this entity are very ambiguous, and natural history in a child is unknown. The authors report a 7-year-old girl who presented with ataxia and signs of raised intracranial pressure and discuss the challenges in diagnosis, surgical strategy, and treatment. Following gross total resection and radiation to tumor bed, the patient had unremarkable recovery and is recurrence free at 1-year follow-up. ICMNSTs are extremely rare tumors of the cerebellum. Preoperative radiological diagnosis is not possible due to its close radiological resemblance to other common posterior fossa tumors. Immunohistochemistry plays a pivotal role in clinching the diagnosis. Though the reported adult counterparts have shown dismal prognosis, the pediatric counterparts may fare better with good surgical resection followed by radiotherapy.

  12. Bone tumors

    International Nuclear Information System (INIS)

    Moylan, D.J.; Yelovich, R.M.

    1991-01-01

    Primary bone malignancies are relatively rare with less than 4,000 new cases per year. Multiple myeloma (more correctly a hematologic malignancy) accounts for 40%; osteosarcomas, 28%; chondrosarcomas, 13%; fibrosarcomas arising in bone, 4%; and Ewing's sarcoma, 7%. The authors discuss various treatments for bone tumors, including radiotherapy, chemotherapy and surgery

  13. Neuroendocrine tumor of the appendix inside an incarcerated Amyand’s hernia

    Directory of Open Access Journals (Sweden)

    Khaled Y. Elbanna

    2015-01-01

    An incidental finding of neuroendocrine tumor of the appendix in a patient with s hernia is extremely rare. A high index of suspicion is the key to diagnose such a coincidence in order to safely and optimally treat such a condition.

  14. Retroperitoneal "triton" tumor. Report of a case and review of literature

    Directory of Open Access Journals (Sweden)

    Palacios Acosta José Martín

    2014-07-01

    Full Text Available The triton tumor was described in 1932 by Masson, as a peripheral nerve sheath malignancy with rabdomioblástica differentiation. The retroperitoneal location is extremely rare, only nine cases have been reported in children. The clinical picture depends on the size of the tumor and the organs involved, their retroperitoneal location is usually asymptomatic. The mainstay of treatment is the surgical excision of the tumor. We report the case of a child with retroperitoneal location of the tumor. A complete resection of it was performed. The patient had an uneventful postoperative course. He is currently under control. There is no evidence of relapse.

  15. Inactivation of the Tumor Suppressor Genes Causing the Hereditary Syndromes Predisposing to Head and Neck Cancer via Promoter Hypermethylation in Sporadic Head and Neck Cancers

    OpenAIRE

    Smith, Ian M.; Mithani, Suhail K.; Mydlarz, Wojciech K.; Chang, Steven S.; Califano, Joseph A.

    2010-01-01

    Fanconi anemia (FA) and dyskeratosis congenita (DC) are rare inherited syndromes that cause head and neck squamous cell cancer (HNSCC). Prior studies of inherited forms of cancer have been extremely important in elucidating tumor suppressor genes inactivated in sporadic tumors. Here, we studied whether sporadic tumors have epigenetic silencing of the genes causing the inherited forms of HNSCC. Using bisulfite sequencing, we investigated the incidence of promoter hypermethylation of the 17 Fan...

  16. The novel RAF1 mutation p.(Gly361Ala) located outside the kinase domain of the CR3 region in two patients with Noonan syndrome, including one with a rare brain tumor.

    Science.gov (United States)

    Harms, Frederike L; Alawi, Malik; Amor, David J; Tan, Tiong Y; Cuturilo, Goran; Lissewski, Christina; Brinkmann, Julia; Schanze, Denny; Kutsche, Kerstin; Zenker, Martin

    2018-02-01

    Noonan syndrome is characterized by typical craniofacial dysmorphism, postnatal growth retardation, congenital heart defect, and learning difficulties and belongs to the RASopathies, a group of neurodevelopmental disorders caused by germline mutations in genes encoding components of the RAS-MAPK pathway. Mutations in the RAF1 gene are associated with Noonan syndrome, with a high prevalence of hypertrophic cardiomyopathy (HCM). RAF1 mutations cluster in exons encoding the conserved region 2 (CR2), the kinase activation segment of the CR3 domain, and the C-terminus. We present two boys with Noonan syndrome and the identical de novo RAF1 missense variant c.1082G>C/p.(Gly361Ala) affecting the CR3, but located outside the kinase activation segment. The p.(Gly361Ala) mutation has been identified as a RAF1 allele conferring resistance to RAF inhibitors. This amino acid change favors a RAF1 conformation that allows for enhanced RAF dimerization and increased intrinsic kinase activity. Both patients with Noonan syndrome showed typical craniofacial dysmorphism, macrocephaly, and short stature. One individual developed HCM and was diagnosed with a disseminated oligodendroglial-like leptomeningeal tumor (DOLT) of childhood at the age of 9 years. While there is a well-established association of NS with malignant tumors, especially childhood hemato-oncological diseases, brain tumors have rarely been reported in Noonan syndrome. Our data demonstrate that mutation scanning of the entire coding region of genes associated with Noonan syndrome is mandatory not to miss rare variants located outside the known mutational hotspots. © 2017 Wiley Periodicals, Inc.

  17. Spreading amelanotic malignant melanoma: A rare differential diagnosis with tumors of the glandula submandibularis; Metastasiertes amelanotisches Melanom: Eine seltene Differentialdiagnose bei Tumoren der Glandula submandibularis

    Energy Technology Data Exchange (ETDEWEB)

    Ehrenberg, C.; Helmberger, H. [Technische Univ. Muenchen (Germany). Inst. fuer Roentgendiagnostik; Werner, M. [Institut fuer Allgemeine Pathologie und Pathologische Anatomie, Klinikum rechts der Isar, Technische Univ. Muenchen (Germany)

    1998-09-01

    The case reported emphasizes the importance of immediate performance of imaging scans in case of the slightest suspicion that clinical symptoms might indicate malignancy of a detected lesion. Despite the superficially only marginal macroscopic findings, MR imaging as well as the CT scans revealed an advanced, malignant process that had been spreading. Particularly the soft tissue differential diagnosis obtained with MRI yields the information required for diagnostic characterization of the space occupying tumor mass. It will however be necessary in any case to verify the diagnosis by biopsy or extirpation and cytologic examination of tissue, as the imaging methods do not always unambigiously reveal the malignant dignity of the tumor. (orig./CB) [Deutsch] Der vorliegende Fall unterstreicht die Bedeutung einer umgehend durchgefuehrten Bildgebung beim geringsten Zweifel an der Benignitaet eines klinischen Symptoms. Trotz des aeusserlich nur marginalen makroskopischen Befundes zeigt die Kernspintomographie in Uebereinstimmung mit der Computertomographie einen fortgeschrittenen, bereits abgesiedelten Prozess. Insbesondere die MRT laesst aufgrund ihrer guten Weichteildifferenzierung einen Rueckschluss auf den Gewebstyp der Raumforderung zu. Einschraenkend bleibt jedoch festzuhalten, dass die maligne Dignitaet des Tumors in beiden bildgebenden Verfahren nicht ohne weiteres erkennbar ist, so dass die Biopsie bzw. Extirpation zur Histologiegewinnung unverzichtbar ist. (orig./MG)

  18. Primary chondrosarcoma of breast - cytology with histopathological correlation: A rare case report with review of literature

    Directory of Open Access Journals (Sweden)

    Sankappa P. Sinhasan

    2014-01-01

    Full Text Available Malignant mesenchymal tumors of the breast other than angiosarcoma are extremely rare and comprise <0.5% of breast tumors. Primary chondrosarcoma of the breast is an extremely rare entity and only 10 cases are reported as single case reports in literature until date. A diagnosis of primary mammary sarcoma can be established only after excluding metaplastic carcinomas and malignant phyllodes by extensive sampling for evidence of in situ or invasive carcinoma. Here, we report a primary chondrosarcoma of breast in a 55-year-old lady diagnosed precisely on fine-needle aspiration cytology and confirmed by histopatholigcal examination after total mastectomy. We emphasize on diagnostic difficulties encountered in cytology smears and discuss differential diagnoses.

  19. Rare earths

    Energy Technology Data Exchange (ETDEWEB)

    Cranstone, D A

    1979-01-01

    Rare earth elements are commonly extracted from the minerals monazite, bastnaesite, and xenotine. New uses for these elements are constantly developing; they have found applications in glass polishing, television tube phosphors, high-strength low-alloy steels, magnets, catalysts, refractory ceramics, and hydrogen sponge alloys. In Canada, rare earths have been produced as byproducts of the uranium mining industry, but there was no production of rare earths in 1978 or 1979. The world sources of and markets for the rare earth elements are discussed.

  20. A very rare case report of long-term survival: A patient operated on in 1994 of glioblastoma multiforme and currently in perfect health

    Directory of Open Access Journals (Sweden)

    Riccardo Caruso

    2017-01-01

    Conclusion: The fact that there are extremely rare cases of long-term survival and even zero recurrence of the glioblastoma should serve as a stimulus to continue the research effort and not give up the fight against this tumor on a day-to-day basis.

  1. Rare (Earth Elements [score

    Directory of Open Access Journals (Sweden)

    Camilo Méndez

    2014-12-01

    Full Text Available Rare (Earth Elements is a cycle of works for solo piano. The cycle was inspired by James Dillon’s Book of Elements (Vol. I-V. The complete cycle will consist of 14 pieces; one for each selected rare (earth element. The chosen elements are Neodymium, Erbium, Tellurium, Hafnium, Tantalum, Technetium, Indium, Dysprosium, Lanthanium, Cerium, Europium, Terbium, Yttrium and Darmstadtium. These elements were selected due to their special atomic properties that in many cases make them extremely valuable for the development of new technologies, and also because of their scarcity. To date, only 4 works have been completed Yttrium, Technetium, Indium and Tellurium.

  2. Rare muon processes: Experiment

    International Nuclear Information System (INIS)

    Walter, H.K.

    1998-01-01

    The decay properties of muons, especially their rare decays, can be used to study very accurately deviations from the Standard Model. Muons with extremely low energies and good spatial definition are preferred for the majority of such studies. With the upgrade of the 590-MeV ring accelerator, PSI possesses the most powerful cyclotron in the world. This makes it possible to operate high-intensity beams of secondary pions and muons. A short review on rare muon processes is presented, concerning μ-e conversion and muonium-antimuonium oscillations. A possible new search for μ→eγ is also mentioned

  3. Stewart-Treves Syndrome on the Lower Extremity Associated to Idiopathic Chronic Lymphedema Visualized on FDG PET/CT

    DEFF Research Database (Denmark)

    Brittain, Jane Maestri; Nymark, Tine; Hildebrandt, Malene Grubbe

    2017-01-01

    Angiosarcomas are highly malignant and rare tumors of vascular or lymphatic endothelial cell origin with a poor prognosis. Lymphangiosarcoma associated with chronic lymphedema is known as Stewart-Treves syndrome. Stewart-Treves syndrome is primarily described in patients with lymphedema of an upper...... extremity occurring after breast cancer surgery including radical axillary lymph node dissection and subsequent radiotherapy. It is rarely described in the presence of idiopathic chronic lymphedema of the lower extremities. We present a case of lymphangiosarcoma visualized on F-FDG PET/CT, where Stewart...

  4. Malignant Phyllodes Tumor Presenting in Bone, Brain, Lungs, and Lymph Nodes

    Directory of Open Access Journals (Sweden)

    Eric D. Johnson

    2016-12-01

    Full Text Available Introduction: Phyllodes tumors (PTs are rare fibroepithelial tumors of the breast which are classified as benign, borderline, or malignant. Malignant PTs account for <1% of malignant breast tumors, and borderline tumors have potential to progress to malignant tumors. Metastatic recurrences are most commonly documented in bone and lungs. We report an extremely rare presentation of recurrent malignant PTs involving the brain, lung, lymph nodes, and bone. Case: A 66-year-old female presented with a large breast mass. Biopsy identified malignant PT, treated by mastectomy. One year later she presented with acute back pain; imaging showed pathological L4 spinal compression fracture. Core biopsy confirmed PT. Staging identified additional metastases in the lymph nodes, brain, and lung. Discussion: PTs are rare and fast-growing tumors that originate from periductal stromal tissues and are composed of both epithelial and stromal components. Histologically, they are classified as benign, borderline, or malignant. The prognosis of the malignant type is poorly defined, with local recurrence occurring in 10–40% and metastases in 10%. Chemotherapy and radiotherapy are generally ineffective in this tumor type. The most common metastatic sites for malignant cases are the lung and bones, but in rare instances, PTs may metastasize elsewhere. Conclusion: We report a rare presentation of recurrent malignant PT presenting as pathological fracture of the lumbar spine with impingement on the spinal column, along with cerebellar, nodal, and pulmonary metastases. Only 1 similar case has been previously reported.

  5. Extremity perfusion for sarcoma

    NARCIS (Netherlands)

    Hoekstra, Harald Joan

    2008-01-01

    For more than 50 years, the technique of extremity perfusion has been explored in the limb salvage treatment of local, recurrent, and multifocal sarcomas. The "discovery" of tumor necrosis factor-or. in combination with melphalan was a real breakthrough in the treatment of primarily irresectable

  6. Vaginal Approach to Excise a Rare Paraurethral Leiomyoma

    Directory of Open Access Journals (Sweden)

    Jagan K. Kansal

    2016-11-01

    Full Text Available Leiomyomas are benign tumors of smooth muscle origin occurring throughout the genitourinary system. While leiomyomas in the uterus are frequently seen, urethral and paraurethral leiomyomas are extremely rare with a hand full of cases in the literature. Typically, periurethral leiomyomas can present with a mass protruding from the urethra originating from the proximal and posterior portion of the urethra. Herein, we present a new case of a paraurethral leiomyoma causing mass effect on the bladder leading to lower urinary tract symptoms (LUTS with no gross involvement of the urethra.

  7. The Bt-DUX: Development of a subjective measure of health-related quality of life in patients who underwent surgery for lower extremity malignant bone tumor

    NARCIS (Netherlands)

    W.P. Bekkering (Peter); T.P.M. Vliet Vlieland (Theodora); H.M. Koopman (Hendrik); G.R. Schaap (Gerard); H.W.B. Schreuder; A. Beishuizen (Auke); W.J.E. Tissing (Wim); P.M. Hoogerbrugge (Peter); J.K. Anninga (Jacob); A.H.M. Taminiau (Antonie)

    2009-01-01

    textabstractBackground. To examine the practical applicability, internal consistency, and validity of the Bt-DUX, a disease-specific Health Related Quality of Life (HRQoL) instrument. The Bt-Dux was developed to examine patients' individual values of their life after a malignant bone tumor of the

  8. Primitive neuroectodermal tumor of the cervix: a case report

    Directory of Open Access Journals (Sweden)

    Boroujeni Parisa

    2011-09-01

    Full Text Available Abstract Introduction Peripheral primitive neuroectodermal tumor of the cervix uteri is extremely rare. Between 1987 and 2010, there were only nine cases reported in the English literature, with considerably different management policies. Case presentation A 45-year-old Iranian woman presented to our facility with a primitive neuroectodermal tumor of the cervix uteri. Her clinical stage IB2 tumor was treated successfully with chemotherapy. Our patient underwent radical hysterectomy. There was no trace of the tumor after four years of follow-up. Conclusions According to current knowledge, primitive neuroectodermal tumors belong to the Ewing's sarcoma family, and the improvement of treatment outcome in our patient was due to dose-intensive neoadjuvant chemotherapy, surgery and consolidation chemotherapy in accordance with the protocol for bony Ewing's sarcoma.

  9. Primary Neuroendocrine Tumor of the Left Hepatic Duct: A Case Report with Review of the Literature

    Directory of Open Access Journals (Sweden)

    Ajay H. Bhandarwar

    2012-01-01

    Full Text Available Primary Biliary Tract Neuroendocrine tumors (NET are extremely rare tumors with only 77 cases been reported in the literature till now. We describe a case of a left hepatic duct NET and review the literature for this rare malignancy. To the best of our knowledge the present case is the first reported case of a left hepatic duct NET in the literature. In spite of availability of advanced diagnostic tools like Computerized Tomography (CT Scan and Endoscopic Retrograde Cholangio Pancreaticography (ERCP a definitive diagnosis of these tumors is possible only after an accurate histopathologic diagnosis of operative specimens with immunohistochemistry and electron microscopy. Though surgical excision remains the gold standard treatment for such tumors, patients with unresectable tumors have good survival with newer biologic agents like Octreotride.

  10. Laparoscopic Management of Sclerosing Stromal Tumors of the Ovary Combined with Ectopic Pregnancy.

    Science.gov (United States)

    Liu, Hua-Qian; Liu, Qiang; Sun, Xue-Bing; Chang, Wen-Min

    2015-01-01

    Like other stromal-derived gynecological tumors, a sclerosing stromal tumor of the ovary (SSTO) is a rare benign tumor that is difficult to distinguish from a malignant ovarian tumor in clinical practice. An SSTO is routinely treated with laparotomy. Here, we present two extremely rare cases of SSTO with contralateral and ipsilateral tubal pregnancies, in which laparoscopic surgery was performed to remove the tumors. After surgery, one patient (case 1) became pregnant twice within 29 months, and the other patient (case 2) did not become pregnant within 6 months postoperatively. These two cases suggest that laparoscopic management is not only useful in treating SSTO and complicating diseases, but it may also help to reduce unnecessary surgical injury to the ovary. © 2015 S. Karger AG, Basel.

  11. Pott's Puffy Tumor Arising from Frontal Sinusitis

    Energy Technology Data Exchange (ETDEWEB)

    Lim, Ji Yeon; Kang, Hyun Koo [Seoul Veterans Hospital, Seoul (Korea, Republic of)

    2010-02-15

    Pott's puffy tumor is an extremely rare and potentially life-threatening complication of frontal sinusitis. We report a case of a 64-year-old man who presented at our emergency department with mild tenderness on the glabellar area and diplopia. Computed Tomography (CT) revealed frontal sinusitis and osteomyelitis of the frontal bone. Following sinus trephination and long-term antibiotic therapy, the patient achieved a complete recovery.

  12. Rare earths

    International Nuclear Information System (INIS)

    1984-01-01

    The conference was held from September 12 to 13, 1984 in Jetrichovice, Czechoslovakia. The participants heard 16 papers of which 4 were inputted in INIS. These papers dealt with industrial separation processes of rare earths, the use of chemical methods of separation from the concentrate of apatite and bastnesite, the effect of the relative permittivity of solvents in the elution of rare earth elements from a cation exchanger, and the determination of the content of different rare earth elements using X-ray fluorescence analysis and atomic absorption spectroscopy. (E.S.)

  13. MR imaging appearances of soft tissue flaps following reconstructive surgery of the lower extremity

    Energy Technology Data Exchange (ETDEWEB)

    Magerkurth, Olaf [Dept. of Radiology, Hospital Baden, Baden (Switzerland); Girish, Gandikota; Jacobson, Jon A.; Kim, Sung Moon; Brigido, Monica; Dong, Qian; Jamadar, David A. [Dept. of Radiology, University of Michigan Hospitals, Ann Arbor (United States)

    2015-02-15

    MR imaging appearances of different types of reconstructive muscle flaps following reconstructive surgery of the lower extremity with associated post-surgical changes due to altered anatomy, radiation, and potential complications, can be challenging. A multidisciplinary therapeutic approach to tumors allows for limb salvage therapy in a majority of the patients. Decision-making for specific types of soft tissue reconstruction is based on the body region affected, as well as the size and complexity of the defect. Hematomas and infections are early complications that can jeopardize flap viability. The local recurrence of a tumor within six months after a complete resection with confirmed tumor-free margins and adjuvant radiation therapy is rare. Identification of a new lesion similar to the initial tumor favors a finding of tumor recurrence.

  14. Merkel cell carcinoma with axillary metastasis; a case report of a rare disease

    Directory of Open Access Journals (Sweden)

    Serdar Culcu

    2018-04-01

    Full Text Available Merkel cell carcinoma is a rare primer neuroendocrine carcinoma of the skin. It is an extremely aggressive tumor. This rare carcinoma is seen with high local and regional recurrence ratios and distant metastasis. We report that a 64 years old female patient who had undergo an excision in another center because of a mass on 4 cm proximal of her right elbow had been diagnosed with Merkel cell carcinoma with positive surgical margins. She was treated with wide re-excision and axillary dissection at our clinic. Keywords: Merkel cell carcinoma, Skin, Axillary metastasis

  15. Rosette forming glioneuronal tumor of the fourth ventricle in squash cytology smear

    Directory of Open Access Journals (Sweden)

    Amita Radhakrishnan Nair

    2014-01-01

    Full Text Available Rosette forming glioneuronal tumor (RGNT is a recently recognized and extremely rare glioneuronal tumor occurring in the fourth ventricle. It is crucial for the cytopathologist to be aware of this entity as it can be easily mistaken for more common neoplasms occurring at this site. We present here the cytology of such a rare case of RGNT that was misdiagnosed as ependymoma. The varying cytological features of this entity, as well as the common diagnostic difficulties encountered in cytology, are highlighted in this report.

  16. Rare particles

    International Nuclear Information System (INIS)

    Kutschera, W.

    1984-01-01

    The use of Accelerator Mass Spectrometry (AMS) to search for hypothetical particles and known particles of rare processes is discussed. The hypothetical particles considered include fractionally charged particles, anomalously heavy isotopes, and superheavy elements. The known particles produced in rare processes discussed include doubly-charged negative ions, counting neutrino-produced atoms in detectors for solar neutrino detection, and the spontaneous emission of 14 C from 223 Ra. 35 references

  17. Mouse Models Recapitulating Human Adrenocortical Tumors: What is lacking?

    Directory of Open Access Journals (Sweden)

    Felicia Leccia

    2016-07-01

    Full Text Available Adrenal cortex tumors are divided into benign forms such as primary hyperplasias and adrenocortical adenomas (ACAs, and malignant forms or adrenocortical carcinomas (ACCs. Primary hyperplasias are rare causes of ACTH-independent hypercortisolism. ACAs are the most common type of adrenal gland tumors and they are rarely functional, i.e producing steroids. When functional, adenomas result in endocrine disorders such as Cushing’s syndrome (hypercortisolism or Conn’s syndrome (hyperaldosteronism. In contrast, ACCs are extremely rare but highly aggressive tumors that may also lead to hypersecreting syndromes. Genetic analyses of patients with sporadic or familial forms of adrenocortical tumors led to the identification of potentially causative genes, most of them being involved in PKA, Wnt/β-catenin and P53 signaling pathways. Development of mouse models is a crucial step to firmly establish the functional significance of candidate genes, to dissect mechanisms leading to tumors and endocrine disorders and in fine to provide in vivo tools for therapeutic screens. In this article we will provide an overview on the existing mouse models (xenografted and genetically engineered of adrenocortical tumors by focusing on the role of PKA and Wnt/β-catenin pathways in this context. We will discuss the advantages and limitations of models that have been developed heretofore and we will point out necessary improvements in the development of next generation mouse models of adrenal diseases.

  18. Primary Vaginal Extraosseous Ewing Sarcoma/Primitive Neuroectodermal Tumor with Cranial Metastasis

    Directory of Open Access Journals (Sweden)

    Chi-Man Yip

    2009-06-01

    Full Text Available Extraosseous Ewing sarcoma is now regarded as a member of the Ewing sarcoma/primitive neuroectodermal tumor (PNET family. It typically involves the soft tissues of the chest wall, pelvis, paravertebral region, abdominal wall, retroperitoneal region and extremities of children, adolescents and young adults, but it seldom occurs in the female genital tract. We report an extremely rare case of retrospective diagnosis of vaginal extraosseous Ewing sarcoma/PNET which metastasized to the right frontoparietal scalp, skull, and dura. Surgical resection, followed by adjuvant radiotherapy and chemotherapy resulted in a favourable clinical outcome. Both the vaginal and head tumors had similar light microscopic features supporting the diagnosis.

  19. Cytomorphology and immunohistochemistry of extrarenal rhabdoid tumor: A case report with review of literature

    Directory of Open Access Journals (Sweden)

    Manjula Jain

    2011-01-01

    Full Text Available Extrarenal rhabdoid tumor (ERRT is a rare, aggressive tumor with extremely poor prognosis. We report a case of ERRT with intraspinal extension in a 1.5-year-old child diagnosed by fine needle aspiration cytology (FNAC and immunohistochemistry. The child presented with a right lumbar region lump of two months duration. Ultrasound guided FNAC was performed and cell block was prepared. Smears were highly cellular and showed a dispersed population of large round cells having abundant pale eosinophillic cytoplasm, centrally to eccentrically placed nucleus with large prominent nucleoli. Immunohistochemistry was carried out on cell block which was positive for epithelial membrane antigen EMA and Vimentin. It was negative for leucocyte common antigen [LCA], wilms tumor 1, WT1, desmin and neuron specific enolaseNSE, thus ruling out other tumors like lymphoma, Wilms tumor, rhabdomyosarcoma, and neuroblastoma. A final diagnosis of ERRT was given. ERRT is an extremely rare tumor of retroperitoneal area; it should be included in the differential diagnosis of malignant round cell tumor in children. Cell block in this case is mandatory for putting up the panel of immunohistochemistry which can clinch the diagnosis of rhabdoid tumor and treatment can be started as early as possible.

  20. Small cell extraskeletal osteosarcoma: a rare case report

    Directory of Open Access Journals (Sweden)

    Neelam Sood

    2014-01-01

    Full Text Available Extraskeletal osteosarcoma is a rare malignant mesenchymal neoplasm and its small cell variant is one among the rarest variant. This article describes a 60-year-old woman presenting with a large, lobulated, painful mass in left thigh with associated history of trauma since 18 months. Her magnetic resonance imaging showed a variegated mixed intensity lesion with associated cystic degeneration, necrosis and matrix arborizing nearby muscles. Fine needle aspiration cytology showed a small cell lesion with very scant osteoid. Tumor was excised and histopathological diagnosis was small cell osteosarcoma involving adjacent muscles and fat with sparing of lymph nodes. The aim of this article is to present the clinical, radiological, cyto-histological and immunohistochemical features of this extremely rare lesion.

  1. Intraosseous adenoid cystic carcinoma of maxilla: A rare case report

    Directory of Open Access Journals (Sweden)

    Prasannasrinivas Suresh Deshpande

    2013-01-01

    Full Text Available Adenoid cystic carcinoma (ACC accounts for approximately 6-10% of all salivary gland tumors. Palatal minor salivary glands, parotid, and sub-mandibular glands are usually affected. Rarely, these lesions arising intraosseously have been reported. Mandible is commonly involved than maxilla. The present case is a giant ACC involving the right maxilla. A thorough clinical and radiographic evaluation was performed to assess the involvement of surrounding vital structures along with a meticulous metastatic work-up. Computed tomography showed a giant lesion in maxilla encroaching the left nasal fossa, antrum, buccal space, and oral cavity. No metastasis was noted. Histological evaluation from multiple sites showed both cribriform and solid patterns. Radiotherapy was given as patient did not comply for surgery. Though central ACC is extremely rare, especially in maxilla, it should be included in the differentials for lesions in maxilla. A prompt diagnosis with treatment and long-term follow-up is advised in such cases.

  2. A Rare Case of Metastatic Choriocarcinoma of Lung Origin

    Directory of Open Access Journals (Sweden)

    Parth Rali

    2017-01-01

    Full Text Available Choriocarcinoma is part of the spectrum of gestational trophoblastic disease that occurs in women of reproductive age. Although the most common metastatic site of choriocarcinoma is the lung, primary pulmonary choriocarcinoma is rare. To diagnose primary pulmonary choriocarcinoma, the patient should have no previous gynecologic malignancy, have elevated human chorionic gonadotropin, and have pathological confirmation of the disease excluding gonadal primary site of the tumor. Due to the paucity of data, there are no guidelines for treatment. Prognosis of this malignancy is extremely poor. We report a rare case of metastatic primary lung choriocarcinoma in a 69-year-old postmenopausal woman who was treated with combination of surgery, chemotherapy, and radiation. The patient had a good outcome and is doing well after 1-year follow-up.

  3. Hybrid Odontogenic Lesion: A Rare Entity

    Directory of Open Access Journals (Sweden)

    Reza Imani

    2017-03-01

    Full Text Available Hybrid tumors are very rare tumors composed of two different tumor entities, each of which conforms to an exactly defined tumor category. A 14-year-old boy was referred for an intraosseous painless lesion with a histopathological feature of multiple odontogenic lesions including calcifying odontogenic cyst, complex odontoma and ameloblastic fibro-odontoma. The final diagnosis considered to be a hybrid odontogenic lesion.

  4. Four Different Tumors Arising in a Nevus Sebaceous

    Directory of Open Access Journals (Sweden)

    Takeshi Namiki

    2016-04-01

    Full Text Available Nevus sebaceous is known by its association with one or more secondary tumors, but more than three multiple tumors arising from a nevus sebaceous is extremely rare. A 67-year-old female presented with a light brown plaque on the back of her head that contained a dome-shaped black node and an erosive lesion. Histopathological examination showed atypical basaloid cells in the black node. At the periphery of that node, structures resembling follicular germs extruded from interlacing cords in the upper portion and tumor nests with sebocytes were in the lower portion. In the erosive lesion, papillated structures with an apocrine epithelium were observed. In the light brown plaque, enlargement of sebaceous lobules was noted. From those histopathological features, a diagnosis of syringocystadenoma papilliferum, sebaceoma, trichoblastoma and basal cell carcinoma arising from a nevus sebaceous was made. We discuss the rarity of multiple tumors arising from a nevus sebaceous.

  5. Intracranial schwannoma presenting as a subfrontal tumor: case report.

    Science.gov (United States)

    Huang, P P; Zagzag, D; Benjamin, V

    1997-01-01

    Intracerebral schwannomas not associated with cranial nerves account for less than 1% of surgically treated schwannomas of the central and peripheral nervous system. Subfrontal schwannomas are extremely rare, with only 15 cases reported to date. A 33-year-old man presented with a 4-month history of progressive headaches and lethargy. Radiographic studies revealed a large subfrontal tumor thought to be a meningioma preoperatively. The patient underwent a craniotomy for resection of his tumor. Intraoperatively, a large extra-axial tumor arising from the floor of the left frontal fossa was encountered. Microscopic examination of the tumor revealed a schwannoma. Several theories on the possible origin of intracerebral schwannomas have been considered. Because of the age of the patient at presentation, many authors have postulated a developmental origin for these lesions. However, extra-axial schwannomas not associated with cranial nerves often present later in life, suggesting a different pathogenesis for this subgroup.

  6. Neuroendocrine tumor of the skin of head and neck

    Directory of Open Access Journals (Sweden)

    Stošić Srboljub

    2005-01-01

    Full Text Available Background. Merkel cell carcinom is a rare neuroendrocine tumor of skin which manifests it self through aggressive growth and early regional metastasis. It develops mainly in older population. Locally, the tumor spreads intracutaneously. Case report. We showed two cases (females of 89 and 70 years old hospitalized within the last two years. The first patient was treated surgically three times. After the surgery, the patient was treated with radio therapy, and died 3 years from the beginning of the treatment. The second patient with this neuroendocrine tumor with the high malignancy potential and huge regional metastasis, was treated surgically, and died a month and a half after the operation. Conclusion. These two cases confirmed the aggressive and recidivant growth of this tumor with the difficult pathologic investigation, and the extremely bad prognosis inspite of the treatment.

  7. Endocrine tumor of the digestive tract - clinical case study

    International Nuclear Information System (INIS)

    Szwedziak, K.; Olejniczak, W.; Brichkovkiy, V.

    2008-01-01

    Introduction: Endocrine tumors of the digestive tract (ETDT) are neoplasms which stem from the APUD (amine precursors uptake and decarboxylation) cells. There are neuroendocrine pancreatic and gastroenteral carcinoid tumors which stand for 2% of digestive tract tumors, 0,5% of all human malignant neoplasms. All of them have secretion granulations in the cytoplasm. That is why a number of immune histochemic techniques is used in search for biogenic amines and hormones such as gastrin, CCK, GIP, VIP, motilin, glucagon, GRP, PP, GHRH and the others. In the majority of cases neuroendocrine tumors of the rectum are described as dysfunctional, which means that specific clinical symptoms are not connected with their hormonal overproduction. Material and methods: We describe a case of fifty seven years old male patient admitted to the Department of General and Transplant Surgery for the diagnosis and treatment of the rectal tumor. Per rectum examination revealed hard tumor. The pathologic examination of the biopsy taken from the lesion and CT scanning confirmed the presence of endocrine tumor of the digestive tract. Results: Anterior resection of the rectum was performed, the postoperative course was uneventful. At present patient is subjected to complementary treatment with the use of somatostatin analogue of the prolonged action. Conclusion: The endocrine tumors of the rectum are extremely rare, they occur in this localization in 0,26-0,52 out of 100.000 all rectal tumors. Diagnosis is usually made upon the microscopic examination and the immune histochemic reactions. (author)

  8. An inguinal hernia sac tumor of extrahepatic cholangiocarcinoma origin

    Directory of Open Access Journals (Sweden)

    Yamazaki Hidehiro

    2006-03-01

    Full Text Available Abstract Background Metastatic hernia sac tumor from biliary malignancy is extremely rare with only one such case previously reported. We herein report an additional case of extrahepatic cholangiocarcinoma presenting as a hernia sac tumor. Case presentation A 78-year-old man presented with an irreducible right inguinal hernia associated with a firm tumor, 2.0 cm in diameter. A computed tomography scan demonstrated a soft tissue density mass with heterogeneous enhancement within the right inguinal canal. The patient underwent a hernia repair and the hernia sac tumor was resected. Histological examination of the tumor revealed a metastatic adenocarcinoma suggesting the tumor was of pancreato-biliary origin. Further investigation using imaging studies disclosed a primary tumor in the upper bile duct. The patient died of the disease nine months after the resection. Conclusion Hernia sac tumors should be considered when an irreducible, growing mass appears within an inguinal hernia. Computed tomography may be useful for the early detection of hernia sac tumors from undiagnosed intra-abdominal malignancies.

  9. A Case Report of Primary Cardiac Tumor in A Neonate

    Directory of Open Access Journals (Sweden)

    Sh. Rejaei

    2008-04-01

    Full Text Available Introduction: Primary cardiac tumors are extremely rare in infants and children . Most primary cardiac tumors in pediatric age group are benign, and less than 10% of such tumors are malignant. Many of these tumors are asymptomatic and incidentally diagnosed. The clinical manifestations are very different and includes direct cardiac effect, systemic effect , and embolic phenomena. Every infant or child with an unusual cardiac murmur, unexplained congestive heart failure, or arrhythmia should be evaluated for cardiac tumors. Echocardiography has contributed significantly to the evaluation of these patients. Surgery is the only treatment for primary cardiac tumors that require intervention with a relatively good prognosis. Case Report: The patient was a 20 days old neonate presented with severe congestive heart failure. Evaluation of the patient showed primary cardiac tumor in the left atrium and ventricle. We recommended surgical removal of the tumor but her parents denied surgical intervention at all. Conclusion: After about one year follow up, congestive heart failure symptoms were controlled and the tumor size was decreased.

  10. Long-Term Tumor Control despite Late Pseudoprogression on 18F-FDG-PET following Extremely Hypofractionated Stereotactic Radiotherapy for Retropharyngeal Lymph Node Metastasis from Esthesioneuroblastoma

    Directory of Open Access Journals (Sweden)

    Kazuhiro Ohtakara

    2014-08-01

    Full Text Available 18F-FDG-PET is a valuable adjunct to conventional imaging for evaluating treatment response following stereotactic body radiotherapy (SBRT for head and neck malignancies (HNM. The effect of treatment-related inflammation is generally deemed negligible after 12 weeks following conventionally fractionated radiotherapy. Herein, we describe an unusual case showing pseudoprogression on 18F-FDG-PET 2 years after SBRT for retropharyngeal lymph node metastasis (RPLNm from esthesioneuroblastoma. A 36-year-old man presented with right RPLNm 32 months after the diagnosis of esthesioneuroblastoma associated with ectopic adrenocorticotropic hormone production. The RPLNm was treated with SBRT in 2 fractions over 8 days using dynamic conformal arcs with concomitant chemotherapy with cisplatin and etoposide. Although follow-up MRI showed sustained lesion regression, the early/delayed maximum standardized uptake (SUVmax values on dual-time-point 18F-FDG-PET obtained 1 and 2 years after SBRT were 7.7/8.3 and 8.5/10.1, respectively, suggesting local progression. Despite no subsequent focal or systemic treatment, the SUVmax values gradually decreased thereafter over a period of 4 years (3.3/3.4 at 76 months. MRI obtained 7 years after SBRT revealed sustained tumor regression. No obvious relevant toxicities have occurred. Thus, caution should be exercised in the interpretation of the SUVmax change following ablative irradiation for HNM.

  11. The greenhouse effect and extreme weather

    International Nuclear Information System (INIS)

    Groenaas, Sigbjoern; Kvamstoe, Nils Gunnar

    2002-01-01

    The article asserts that an anthropogenic global warming is occurring. This greenhouse effect is expected to cause more occurrences of extreme weather. It is extremely difficult, however, to relate specific weather catastrophes to global warming with certainty, since such extreme weather conditions are rare historically. The subject is controversial. The article also discusses the public debate and the risk of floods

  12. Statistics of Extremes

    KAUST Repository

    Davison, Anthony C.

    2015-04-10

    Statistics of extremes concerns inference for rare events. Often the events have never yet been observed, and their probabilities must therefore be estimated by extrapolation of tail models fitted to available data. Because data concerning the event of interest may be very limited, efficient methods of inference play an important role. This article reviews this domain, emphasizing current research topics. We first sketch the classical theory of extremes for maxima and threshold exceedances of stationary series. We then review multivariate theory, distinguishing asymptotic independence and dependence models, followed by a description of models for spatial and spatiotemporal extreme events. Finally, we discuss inference and describe two applications. Animations illustrate some of the main ideas. © 2015 by Annual Reviews. All rights reserved.

  13. A rare adrenal incidentaloma: adrenal schwannoma.

    Science.gov (United States)

    Adas, Mine; Ozulker, Filiz; Adas, Gokhan; Koc, Bora; Ozulker, Tamer; Sahin, Ilknur Mansuroglu

    2013-01-01

    Adrenal schwannoma is an extremely uncommon cause of incidentaloma. It originates from neural sheath Schwann cells of the adrenal gland. We report the case of a left adrenal schwannoma incidentally discovered in a 32-year-old woman during examination of bloated feeling and stomach ache. The patient was incidentally found to have a left adrenal mass of 9 cm on abdominal ultrasonography. Computed tomography (CT) of the abdomen and [(18)F] fluorodeoxyglucose positron emission tomography (PET) were also performed. Metabolic evaluation was unremarkable. Due to the large size of the tumor, left adrenalectomy was performed. The postoperative course was uneventful. Histological examination established the diagnosis of schwannoma. This diagnosis was supported by immunohistochemistry of S-100 and vimentin positivity. In conclusion, adrenal schwannoma is an extremely rare entity and can grow considerably in size. The present case report emphasizes that clinicians should be aware of the possibility of retroperitoneal schwannoma. Total excision of benign schwannoma is associated with a favorable outcome. To our knowledge, there are case reports of schwannoma with CT and magnetic resonance imaging findings in the literature, although this is the first schwannoma case with PET-CT imaging.

  14. Rapidly Growing Chondroid Syringoma of the External Auditory Canal: Report of a Rare Case

    Science.gov (United States)

    Vasileiadis, Ioannis; Kapetanakis, Stylianos; Petousis, Aristotelis; Karakostas, Euthimios; Simantirakis, Christos

    2011-01-01

    Introduction. Chondroid syrinoma of the external auditory canal is an extremely rare benign neoplasm representing the cutaneous counterpart of pleomorphic adenoma of salivary glands. Less than 35 cases have been reported in the international literature. Case Presentation. We report a case of a 34-year-old male in whom a rapidly growing, well-circumscribed tumor arising from the external auditory canal was presented. Otoscopy revealed a smooth, nontender lesion covered by normal skin that almost obstructs the external auditory meatus. MRI was performed to define the extension of the lesion. It confirmed the presence of a 1.5 × 0.8 cm T2 high-signal intensity lesion in the superior and posterior wall of EAC without signs of bone erosion. The patient underwent complete resection of the tumor. The diagnosis was confirmed by histopathologic examination. Conclusion. Although chondroid syringoma is extremely rare, it should always be considered in the differential diagnosis of an aural polyp. Chondroid syringomas are usually asymptomatic, slow-growing, single benign tumors in subcutaneous or intradermal location. In our case, the new information is that this benign tumor could present also as a rapidly growing lesion, arising the suspicion for malignancy. PMID:21941560

  15. Rapidly Growing Chondroid Syringoma of the External Auditory Canal: Report of a Rare Case

    Directory of Open Access Journals (Sweden)

    Ioannis Vasileiadis

    2011-01-01

    Full Text Available Introduction. Chondroid syrinoma of the external auditory canal is an extremely rare benign neoplasm representing the cutaneous counterpart of pleomorphic adenoma of salivary glands. Less than 35 cases have been reported in the international literature. Case Presentation. We report a case of a 34-year-old male in whom a rapidly growing, well-circumscribed tumor arising from the external auditory canal was presented. Otoscopy revealed a smooth, nontender lesion covered by normal skin that almost obstructs the external auditory meatus. MRI was performed to define the extension of the lesion. It confirmed the presence of a 1.5×0.8 cm T2 high-signal intensity lesion in the superior and posterior wall of EAC without signs of bone erosion. The patient underwent complete resection of the tumor. The diagnosis was confirmed by histopathologic examination. Conclusion. Although chondroid syringoma is extremely rare, it should always be considered in the differential diagnosis of an aural polyp. Chondroid syringomas are usually asymptomatic, slow-growing, single benign tumors in subcutaneous or intradermal location. In our case, the new information is that this benign tumor could present also as a rapidly growing lesion, arising the suspicion for malignancy.

  16. Renal inflammatory myofibroblastic tumor

    DEFF Research Database (Denmark)

    Heerwagen, S T; Jensen, C; Bagi, P

    2007-01-01

    Renal inflammatory myofibroblastic tumor (IMT) is a rare soft-tissue tumor of controversial etiology with a potential for local recurrence after incomplete surgical resection. The radiological findings in renal IMT are not well described. We report two cases in adults with a renal mass treated...

  17. Chondroblastoma of the thoracic spine: a rare location. Case report with radiologic-pathologic correlation

    Energy Technology Data Exchange (ETDEWEB)

    Venkatasamy, A. [University Hospital of Strasbourg, Department of Radiology, Strasbourg (France); Chenard, M.P. [University Hospital of Strasbourg, Department of Pathology, Strasbourg (France); Massard, G. [University Hospital of Strasbourg, Department of Thoracic Surgery, Strasbourg (France); Steib, J.P. [University Hospital of Strasbourg, Department of Spine Surgery, Strasbourg (France); Bierry, G. [University Hospital of Strasbourg, Department of Radiology, Strasbourg (France); University Hospital of Strasbourg, Department of Pathology, Strasbourg (France); University Hospital of Strasbourg, Department of Thoracic Surgery, Strasbourg (France); University Hospital of Strasbourg, Department of Spine Surgery, Strasbourg (France)

    2017-03-15

    Chondroblastoma is a rare benign cartilage neoplasm that arises from the appendicular skeleton in the vast majority of the cases (80%). Chondroblastoma of the spine is an even more rare condition (30 cases reported), and vertebral chondroblastomas, unlike chondroblastomas of the extremities, present with the appearance of an aggressive tumor on CT and MR imaging and occur at least a decade later. Even though vertebral chondroblastomas are very uncommon tumors, they should nonetheless be included in the differential diagnosis when encountered with an aggressive vertebral mass, and a histological confirmation should be performed. We present a case of chondroblastoma of the thoracic spine of a 27-year-old female for which detailed radiologic-pathologic correlation was obtained. (orig.)

  18. Extreme cosmos

    CERN Document Server

    Gaensler, Bryan

    2011-01-01

    The universe is all about extremes. Space has a temperature 270°C below freezing. Stars die in catastrophic supernova explosions a billion times brighter than the Sun. A black hole can generate 10 million trillion volts of electricity. And hypergiants are stars 2 billion kilometres across, larger than the orbit of Jupiter. Extreme Cosmos provides a stunning new view of the way the Universe works, seen through the lens of extremes: the fastest, hottest, heaviest, brightest, oldest, densest and even the loudest. This is an astronomy book that not only offers amazing facts and figures but also re

  19. Macrodystrophia Lipomatosa: A rare presentation | Kachewar ...

    African Journals Online (AJOL)

    Macrodystrophia lipomatosa is a rare congenital nonhereditary mesenchymal hamartomatous malformation resulting in localized gigantism of parts of extremities that manifests clinically as macrodactyly or megalodactyly. Radiological and Pathological hallmark is the disproportionate fibroadipose tissue proliferation in ...

  20. LEFT RENAL TUMOR: A RARE CADAVERIC FINDINGS

    Directory of Open Access Journals (Sweden)

    Siva Prasad

    2015-08-01

    Full Text Available A benign tumour is a non - cancerous growth that does not spread (metastasize to other parts of the body and is not usually life - threatening. Many researchers believe that most of the following types of kidney tumours are benign. However, others feel that some of these tumours have the potential to develop into renal cell carcinoma. There are no diagnostic tests that can confirm if a benign tumour has the potential to become cancerous. Benign tumours are sometimes found along with renal cell carcinoma when a removed kidney is examined by a pathologist. For these reasons, benign kidney tumours are treated like malignant tumours. In this case we have observed a male cadaver with a large tumour in the left kidney during our routine dissections of undergraduates .

  1. Intracranial phosphaturic mesenchymal tumor, mixed connective tissue variant presenting without oncogenic osteomalacia.

    Science.gov (United States)

    Bower, Regina S; Daugherty, Wilson P; Giannini, Caterina; Parney, Ian F

    2012-01-01

    Phosphaturic mesenchymal tumor, mixed connective tissue variant (PMTMCT) is a rare tumor typically occurring in soft tissues and bone, causing oncogenic (tumor-induced) osteomalacia (TIO) through secretion of the phosphaturic hormone, fibroblast growth factor-23 (FGF-23). Rare tumors identical to PMTMCT occur without known TIO. Intracranial localization of PMTMCT is extremely rare, with only two cases reported in the literature. We present a very unusual case of a patient with an intracranial PMTMCT that presented with neurologic changes without osteomalacia. A 67-year-old woman presented with progressive incontinence, apathy, and abulia after having undergone a total knee replacement 1 month earlier. Imaging disclosed a large left frontal anterior fossa mass. She underwent uncomplicated surgical resection of this tumor. Surprisingly, histopathology suggested PMTMCT. Reverse transcription polymerase chain reaction (RT-PCR) assay demonstrating FGF-23 expression in the tumor confirmed the diagnosis. Serum FGF-23 levels postoperatively were normal and she had no clinical or laboratory evidence of osteomalacia or phosphaturia. This report should serve to alert clinicians to the possibility that PMTMCT can be included in the differential diagnosis of intracranial masses even in the absence of tumor-induced osteomalacia.

  2. Primary malignant peripheral nerve sheath tumor at unusual location

    Directory of Open Access Journals (Sweden)

    Souvagya Panigrahi

    2013-01-01

    Full Text Available Malignant peripheral nerve sheath tumor (MPNST is a rare soft tissue sarcoma. Most arise in association with major nerve trunks. Their most common anatomical sites are the proximal portions of the upper and lower extremities and the trunk. MPNSTs have rarely been reported in literature to occur in other unusual body parts. We review all such cases reported till now in terms of site of origin, surgical treatment, adjuvant therapy and outcome and shortly describe our experience with two of these cases. Both of our case presented with lump at unusual sites resembling neurofibroma, one at orbitotemporal area and other in the paraspinal region with characteristic feature of neurofibroma with the exception that both had very short history of progression. They underwent gross total removal of the tumor with adjuvant radiotherapy postoperatively. At 6-month follow-up both are doing well with no evidence of recurrence.

  3. Granular cell tumors of the urinary bladder

    Directory of Open Access Journals (Sweden)

    Kayani Naila

    2007-03-01

    Full Text Available Abstract Background Granular cell tumors (GCTs are extremely rare lesions of the urinary bladder with only nine cases being reported in world literature of which one was malignant. Generally believed to be of neural origin based on histochemical, immunohistochemical, and ultrastructural studies; they mostly follow a clinically benign course but are commonly mistaken for malignant tumors since they are solid looking, ulcerated tumors with ill-defined margins. Materials and methods We herein report two cases of GCTs, one benign and one malignant, presenting with gross hematuria in a 14- and a 47-year-old female, respectively. Results Histopathology revealed characteristic GCTs with positive immunostaining for neural marker (S-100 and negative immunostaining for epithelial (cytokeratin, Cam 5.2, AE/A13, neuroendocrine (neuron specific enolase, chromogranin A, and synaptophysin and sarcoma (desmin, vimentin markers. The benign tumor was successfully managed conservatively with transurethral resection alone while for the malignant tumor, radical cystectomy, hysterectomy with bilateral salpingo-oophorectomy, anterior vaginectomy, plus lymph node dissection was done. Both cases show long-term disease free survival. Conclusion We recommend careful pathologic assessment for establishing the appropriate diagnosis and either a conservative or aggressive surgical treatment for benign or localized malignant GCT of the urinary bladder, respectively.

  4. Tumorous Conditions of the Hand: A Retrospective Review of 402 Cases

    Directory of Open Access Journals (Sweden)

    Ali CAVİT

    2018-01-01

    Full Text Available Objective: Knowledge concerning treatment and care of hand lesions is often based on small case series, case reports and a few large general case series. The aim of this study is to present our experience with hand tumors’ and tumor-like lesions’ incidence, age range and localizations. Material and Method: Between 2006-2016, 402 patients operated and histopathologically diagnosed with bone and soft tissue tumorous conditions of the hand were evaluated retrospectively. Results: Three hundred sixty one out of 402 cases (89.8% were soft tissue tumors and 41 cases (10.2% were osseous tumors of the hand. A total of 10 malignant tumors (2.5% were encountered in the hand. The average age of the patients was 41.9 years (ranged from 1 to 83 years. Among 361 soft tissue tumors, only 6 cases (1.6% were malignant and they were squamous cell tumors (n=5 and synovial sarcoma (n=1. The most common soft tissue pathology was ganglion cyst (n=125. The most common bone tumor was enchondroma, diagnosed in 26 patients (6.4% of all patients. Primary malignant bone tumors were extremely rare in the hand; one osteosarcoma and one chondrosarcoma were reported. Metastatic tumors to the hand were seen in two patients; and they were lung carcinoma and chondrosarcoma metastasis. Conclusion: Up-date knowledge and a thorough understanding of the nature and demographic characteristics of the tumorous conditions of the hand are crucial for accurate diagnosis and appropriate treatment.

  5. Giant osteoma of the skull vault: A rare case of mixed variety

    Directory of Open Access Journals (Sweden)

    Harisha P.N.

    2016-12-01

    Full Text Available Osteoma is the most common primary bone tumor in the craniofacial skeleton. However, most of these are small, asymptomatic and arise from the facial bones or in relation to the paranasal sinuses. Cranial vault osteomas, that too giant and symptomatic are much rarer. We report a case of sixty year-old gentleman presented with a very slowly increasing, painless, hard swelling on the left side of his head. Computerized tomography scan showed the left parietal calvarial tumor to be having large exostotic and enostotic components. He underwent an en-bloc excision of the tumor and cranioplasty. Giant, symptomatic cranial vault osteoma with concurrent exostotic and enostotic components is extremely rare. These lesions can be safely and completely excised with careful planning and attention to detail.

  6. Rhabdomyosarcoma of the extremity

    International Nuclear Information System (INIS)

    Rao, Bhaskar N

    1997-01-01

    Rhabdomyosarcoma is the most common soft tissue sarcoma accounting for almost 55%. These tumors arise from unsegmented mesoderm or primitive mesenchyma, which have the capacity to differentiate into muscle. Less than 5% occur in the first year of life. Extremity rhabdomyosarcoma are mainly seen in the adolescent years. The most common histologic subtype is the alveolar variant. Other characteristics of extremity rhabdomyosarcoma include a predilection for lymph node metastasis, a high local failure, and a relatively low survival rate. They often present as slow painless masses; however, lesions in the hand and foot often present as painful masses and imaging studies may show invasion of the bone. Initial diagnostic approaches include needle biopsy or incisional biopsy for larger lesions. Excisional biopsy is indicated preferably for lesions less than 2.5 cm. following this in most instances therapy is initiated with multi agent chemotherapy depending upon response, the next modality may be either surgery with intent to cure or radiation therapy. Amputation of an extremity for local control is not considered in most instances. Prognostic factors that have been determined over the years to be of significance by multi variant analysis have included age, tumor size, invasiveness, presence of either nodal or distant metastasis, and complete excision whenever feasible, with supplemental radiation therapy for local control

  7. Treatment of neuroendocrine tumors (NETs) expressing SMT 90Y and 177Lu

    International Nuclear Information System (INIS)

    Oliva González, Juan P.; Baum, Richard

    2016-01-01

    Neuroendocrine tumors (NETs) are a relatively rare and extremely heterogeneous group, essentially characterized by a different metabolism and endocrine histologically pattern. NETs are a challenge for physicians not only for diagnosis but also for early treatment. In addition to this, QT or RT treatments that require a high rate of cell proliferation to be effective, they are not in these tumors as slow growth. The primary treatment of NETs is surgery, either with a curative intent or tumor shrinkage. Peptide Receptors Radiotherapy (RTPR) consists of the administration for therapeutic purposes of Radiolabeled Synthetic Peptides that bind specifically and with high affinity to receptors of tumor cells. The RTPR of TNE with SMT analogues is effective for handling or metastizados inoperable patients. The Conference gives an accurate picture of the treatment of these tumors both 90 Y as 177 Lu. (author)

  8. Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres

    Directory of Open Access Journals (Sweden)

    Yan Tan

    2014-01-01

    Full Text Available Clear cell myomelanocytic tumors (CCMTs of the falciform ligament/ligamentum teres are extremely rare. CCMTs are a variant of perivascular epithelioid cell tumors. We present a case of hepatic CCMT in a 54-year-old woman with abdominal pain. The patient had an 8.8 cm well-demarcated tumor in the right lobe of the liver. Contrast-enhanced computed tomography showed a heterogeneous mass that enhanced significantly in the arterial and portal venous phases, and was less enhanced in the delayed phase. The patient underwent a right hemihepatectomy and cholecystectomy. The tumor cells had clear to slightly eosinophilic cytoplasm, vesicular nuclei, and were positive for HMB-45 and smooth muscle actin. The patient had no recurrence after 36 months follow-up. A review of the literature identified 10 hepatic CCMTs. Hepatic CCMTs are usually benign tumors of young women that present as large masses located in the right lobe of the liver.

  9. Late diagnosis of testicular germ cell tumors and its impact on prognosis

    International Nuclear Information System (INIS)

    Puskacova, J.; Kolenova, A.; Mocna, A.; Cechvalova, A.; Kaiserova, E.; Molcan, J.

    2015-01-01

    Introduction: Testicular tumors in children and adolescents are rare diseases with very good prognosis. Biological characteristics of germ cell tumors depends on the type of histology, stage and age at the time of diagnosis. Case report: 14 years old boy was urgently admitted to the hospital because of hemoptysis. Chest X ray showed round shaped lesions bilaterally. Surprisingly, extremely enlarged left testicle was found. Ultrasound confirmed tumor in left testicle, tumor markers were elevated and dissemination in lungs, retroperitoneal lymph nodes and CNS as well, was present. Despite three chemotherapeutic regimens the patient died 8 months from the diagnosis. Conclusions: Testicular tumors in adolescent boys are usually diagnosed in advanced stage after several months history of continuous enlargement. Whole body examination of patients and self examination of testicles in pubertal boys could lead to earlier diagnosis and improve the chance to cure. (author)

  10. Adult type granulosa cell tumor in adult testis: report of a case and review of the literature

    Directory of Open Access Journals (Sweden)

    Zhanyong Bing

    2011-10-01

    Full Text Available Granulosa cell tumors can be classified into juvenile and adult types and more commonly occur in ovaries. Adult testicular granulosa cell tumors are extremely rare and only 29 cases of adult type have previously been reported. We report here a 28-year-old Caucasian man with a left testicular adult type granulosa cell tumor. The tumor measured 2.6 x 2.6 x 2.5 cm and was mitotically active (10/10 HPF. Immunohistochemical stains showed the tumor diffusely positive for inhibin and vimentin, and negative for epithelial membrane antigen, cytokeratins, synaptophysin, HMB-45, OCT-4, placental-like alkaline phosphatase and lymphoid markers . The reported granulosa cell tumors in adult testis were briefly reviewed.

  11. Tumor-induced osteomalacia with elevated fibroblast growth factor 23: a case of phosphaturic mesenchymal tumor mixed with connective tissue variants and review of the literature.

    Science.gov (United States)

    Hu, Fang-Ke; Yuan, Fang; Jiang, Cheng-Ying; Lv, Da-Wei; Mao, Bei-Bei; Zhang, Qiang; Yuan, Zeng-Qiang; Wang, Yan

    2011-11-01

    Tumor-induced osteomalacia (TIO), or oncogenic osteomalacia (OOM), is a rare acquired paraneoplastic disease characterized by renal phosphate wasting and hypophosphatemia. Recent evidence shows that tumor-overexpressed fibroblast growth factor 23 (FGF23) is responsible for the hypophosphatemia and osteomalacia. The tumors associated with TIO are usually phosphaturic mesenchymal tumor mixed connective tissue variants (PMTMCT). Surgical removal of the responsible tumors is clinically essential for the treatment of TIO. However, identifying the responsible tumors is often difficult. Here, we report a case of a TIO patient with elevated serum FGF23 levels suffering from bone pain and hypophosphatemia for more than three years. A tumor was finally located in first metacarpal bone by octreotide scintigraphy and she was cured by surgery. After complete excision of the tumor, serum FGF23 levels rapidly decreased, dropping to 54.7% of the preoperative level one hour after surgery and eventually to a little below normal. The patient's serum phosphate level rapidly improved and returned to normal level in four days. Accordingly, her clinical symptoms were greatly improved within one month after surgery. There was no sign of tumor recurrence during an 18-month period of follow-up. According to pathology, the tumor was originally diagnosed as "lomangioma" based upon a biopsy sample, "proliferative giant cell tumor of tendon sheath" based upon sections of tumor, and finally diagnosed as PMTMCT by consultation one year after surgery. In conclusion, although an extremely rare disease, clinicians and pathologists should be aware of the existence of TIO and PMTMCT, respectively.

  12. Cardiac Calcified Amorphous Tumor of the Mitral Valve Presenting as Transient Ischemic Attack.

    Science.gov (United States)

    Abbasi Teshnizi, Mohammad; Ghorbanzadeh, Atefeh; Zirak, Nahid; Manafi, Babak; Moeinipour, Aliasghar

    2017-01-01

    Cardiac calcified amorphous tumors (CATs) are an extremely rare nonneoplastic intracardiac masses. They have been reported in the literature in only a few cases. Thus, the incidence, pathogenesis, and best approach to the treatment are not certain. We report a case of CATs on the atrial surface of the anterior mitral valve leaflet in a 37-year-old female who was diagnosed by histopathological examination after surgical removal.

  13. Cardiac Calcified Amorphous Tumor of the Mitral Valve Presenting as Transient Ischemic Attack

    Directory of Open Access Journals (Sweden)

    Mohammad Abbasi Teshnizi

    2017-01-01

    Full Text Available Cardiac calcified amorphous tumors (CATs are an extremely rare nonneoplastic intracardiac masses. They have been reported in the literature in only a few cases. Thus, the incidence, pathogenesis, and best approach to the treatment are not certain. We report a case of CATs on the atrial surface of the anterior mitral valve leaflet in a 37-year-old female who was diagnosed by histopathological examination after surgical removal.

  14. Description of a neural sheath tumor of the trigeminal nerve: immunohistochemical and electron microscopy study

    OpenAIRE

    Khademi, Bijan; Owji, Seied Mohammad; Khosh, Khadije Jamshidi; Mohammadianpanah, Mohammad; Gandomi, Behrooz

    2006-01-01

    CONTEXT: Malignant neural sheath tumors of the trigeminal nerve affecting the nasal cavity and the paranasal sinuses are extremely rare. With conventional optical microscopy, their identification is difficult, and it is necessary to confirm them by means of electron microscopy and immunohistochemical techniques. CASE REPORT: The patient was a 41-year-old woman with a ten-month progressive history of pain followed by painful edema in the left facial region, and with symptoms of bleeding, secre...

  15. Malignant granular cell tumor of the abdominal wall mimicking desmoid tumor: A case report with CT imaging findings and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Yoon, Je Hong; Ahn, Sung Eun; Lee, Dong Ho; Park, Seong Jin; Moon, Sung Kyoung; Lim, Joo Won [Dept. Radiology, Kyung Hee University Hospital, Kyung Hee University School of Medicine, Seoul (Korea, Republic of)

    2016-08-15

    Granular cell tumors (GCTs) are extremely rare mesenchymal neoplasms of Schwann cell origin. Malignant GCTs (MGCTs) comprise 0.5-2% of all GCTs. In the present report, we describe a case of a 66-year-old man with MGCT of the abdominal wall. The patient visited our hospital due to a recently growing palpable soft tissue mass in the abdominal wall. Computed tomography scan revealed a 4.3 × 4.1 × 2.9 cm sized mass arising from the left abdominal wall, which was contemplated as a desmoid tumor before surgical excision. Histopathological examination confirmed MGCT.

  16. Evolution caused by extreme events.

    Science.gov (United States)

    Grant, Peter R; Grant, B Rosemary; Huey, Raymond B; Johnson, Marc T J; Knoll, Andrew H; Schmitt, Johanna

    2017-06-19

    Extreme events can be a major driver of evolutionary change over geological and contemporary timescales. Outstanding examples are evolutionary diversification following mass extinctions caused by extreme volcanism or asteroid impact. The evolution of organisms in contemporary time is typically viewed as a gradual and incremental process that results from genetic change, environmental perturbation or both. However, contemporary environments occasionally experience strong perturbations such as heat waves, floods, hurricanes, droughts and pest outbreaks. These extreme events set up strong selection pressures on organisms, and are small-scale analogues of the dramatic changes documented in the fossil record. Because extreme events are rare, almost by definition, they are difficult to study. So far most attention has been given to their ecological rather than to their evolutionary consequences. We review several case studies of contemporary evolution in response to two types of extreme environmental perturbations, episodic (pulse) or prolonged (press). Evolution is most likely to occur when extreme events alter community composition. We encourage investigators to be prepared for evolutionary change in response to rare events during long-term field studies.This article is part of the themed issue 'Behavioural, ecological and evolutionary responses to extreme climatic events'. © 2017 The Author(s).

  17. Central nervous system tumors

    International Nuclear Information System (INIS)

    Curran, W.J. Jr.

    1991-01-01

    Intrinsic tumors of the central nervous system (CNS) pose a particularly challenging problem to practicing oncologists. These tumors rarely metastasize outside the CNS, yet even histologically benign tumors can be life-threatening due to their local invasiveness and strategic location. The surrounding normal tissues of the nervous system is often incapable of full functional regeneration, therefore prohibiting aggressive attempts to use either complete surgical resection or high doses of irradiation. Despite these limitations, notable achievements have recently been recorded in the management of these tumors

  18. Spontaneous Tumor Lysis Syndrome

    Directory of Open Access Journals (Sweden)

    Alicia C. Weeks MD

    2015-08-01

    Full Text Available Tumor lysis syndrome (TLS is a known complication of malignancy and its treatment. The incidence varies on malignancy type, but is most common with hematologic neoplasms during cytotoxic treatment. Spontaneous TLS is thought to be rare. This case study is of a 62-year-old female admitted with multisystem organ failure, with subsequent diagnosis of aggressive B cell lymphoma. On admission, laboratory abnormalities included renal failure, elevated uric acid (20.7 mg/dL, and 3+ amorphous urates on urinalysis. Oliguric renal failure persisted despite aggressive hydration and diuretic use, requiring initiation of hemodialysis prior to chemotherapy. Antihyperuricemic therapy and hemodialysis were used to resolve hyperuricemia. However, due to multisystem organ dysfunction syndrome with extremely poor prognosis, the patient ultimately expired in the setting of a terminal ventilator wean. Although our patient did not meet current TLS criteria, she required hemodialysis due to uric acid nephropathy, a complication of TLS. This poses the clinical question of whether adequate diagnostic criteria exist for spontaneous TLS and if the lack of currently accepted guidelines has resulted in the underestimation of its incidence. Allopurinol and rasburicase are commonly used for prevention and treatment of TLS. Although both drugs decrease uric acid levels, allopurinol mechanistically prevents formation of the substrate rasburicase acts to solubilize. These drugs were administered together in our patient, although no established guidelines recommend combined use. This raises the clinical question of whether combined therapy is truly beneficial or, conversely, detrimental to patient outcomes.

  19. Extremely Elevated CA 125 and CA 19-9 in Endometrioma; A Case Series

    Directory of Open Access Journals (Sweden)

    Vugar Bayramov

    2010-03-01

    Full Text Available Although endometriosis is a benign condition, increased levels of the tumor markers CA 125 and CA 19-9 may be seen. However, these tumor markers reach to very high levels, rarely. In this report, 4 patients between 20 and 43 year-old with extremely elevated CA 125, CA 19-9 and CA 15-3 levels are discussed. In endometriosis extremely increased tumor markers are determined in the case of ruptured endometrioma cyst. There are two mechanisms to clarify extremely elevated levels of CA 125 in endometriosis. First, the peritoneal irritation of CA 125 molecule after the rupture of endometioma cyst and CA 125 secretion from the periton. And the second is penetration of the CA 125 moecule easily to the circulation through the peritoneal endothelial surface after the cyst rupture. In conclusion, the diagnosis of ruptured endometrioma cyst should be kept in mind especially in young patients with extremely elevated serum CA 125 levels with regard to the history and ultrasonographical signs and invasive procedures should be avoided.

  20. Suprasellar ganglioglioma presenting with diabetes insipidus in a young boy: a rare clinical presentation.

    Science.gov (United States)

    Gupta, Ruchika; Suri, Vaishali; Arora, Raman; Sharma, Mehar C; Mishra, Shashwat; Singh, Manmohan; Sarkar, Chitra

    2010-02-01

    Gangliogliomas are rare tumors composed of an admixture of glial and neuronal components. These usually occur in young patients, who present with therapy-resistant seizures. Clinical presentation of ganglioglioma with diabetes insipidus is extremely rare with only one case reported earlier in the available literature. Due to this rarity, ganglioglioma is not considered in the differential diagnosis in a patient with diabetes insipidus. A 7-year boy presented with polyuria, polydipsia, and progressive visual loss for 18 months. Investigations revealed diabetes insipidus. Radiographic studies of the brain showed a solid and cystic mass in the suprasellar region effacing the third ventricle. Intraoperatively, diffuse thickening of bilateral optic nerves and optic chiasma was noted and a diagnosis of optic glioma was considered. A biopsy of the mass was taken, which on histopathological examination showed features of ganglioglioma. The patient was referred for further radiotherapy but was lost to follow-up. Diabetes insipidus as a presenting symptom of ganglioglioma is extremely rare. This benign tumor should be kept in mind in patients with central diabetes insipidus and a suprasellar mass lesion. This report describes the second such case in the literature.

  1. Rare earth metals, rare earth hydrides, and rare earth oxides as thin films

    International Nuclear Information System (INIS)

    Gasgnier, M.

    1980-01-01

    The review deals with pure rare earth materials such as rare earth metals, rare earth hydrides, and rare earth oxides as thin films. Several preparation techniques, control methods, and nature of possible contaminations of thin films are described. These films can now be produced in an extremely well-known state concerning chemical composition, structure and texture. Structural, electric, magnetic, and optical properties of thin films are studied and discussed in comparison with the bulk state. The greatest contamination of metallic rare earth thin films is caused by reaction with hydrogen or with water vapour. The compound with an f.c.c. structure is the dihydride LnH 2 (Ln = lanthanides). The oxygen contamination takes place after annealing at higher temperatures. Then there appears a compound with a b.c.c. structure which is the C-type sesquioxide C-Ln 2 O 3 . At room atmosphere dihydride light rare earth thin films are converted to hydroxide Ln(OH) 3 . For heavy rare earth thin films the oxinitride LnNsub(x)Osub(y) is observed. The LnO-type compound was never seen. The present review tries to set the stage anew for the investigations to be undertaken in the future especially through the new generations of electron microscopes

  2. Bone tumor

    Science.gov (United States)

    Tumor - bone; Bone cancer; Primary bone tumor; Secondary bone tumor; Bone tumor - benign ... The cause of bone tumors is unknown. They often occur in areas of the bone that grow rapidly. Possible causes include: Genetic defects ...

  3. Bednar Tumor: An Uncommon Entity.

    Science.gov (United States)

    Amonkar, Gayathri P; Rupani, Asha; Shah, Ajay; Deshpande, Ramesh

    2016-01-01

    Bednar tumor is an uncommon variant of dermatofibrosarcoma protuberans. Also known as pigmented dermatofibrosarcoma protuberans, this tumor is of intermediate grade. It is seen in adults and has a predisposition to affect the shoulder region. We report a rare case of Bednar tumor in a 40-year-old female patient. The diagnosis of Bednar tumor must be considered while reporting pigmented subcutaneous spindle cell lesions.

  4. Low-grade Myxofibrosarcoma in the Mandible: A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Neda Kargahi

    2017-04-01

    Full Text Available Myxofibrosarcoma is a malignant neoplasm of connective tissue origin commonly found in the extremities. It is very rare in the head and neck regions. Only 25 cases of myxofibrosarcoma have been reported in the head and neck regions until 2014. Here we report a 61-year-old male with this neoplasm in the lower border of his mandible. During a two-year follow-up, this case recurred four times despite a complete resection. This study suggested combined surgical and adjuvant radiotherapy for unresectable lesions and tumors with positive margins to prevent recurrence and risk of progression.

  5. Risk factors for oligodendroglial tumors: a pooled international study

    DEFF Research Database (Denmark)

    McCarthy, Bridget J; Rankin, Kristin M; Aldape, Ken

    2011-01-01

    Oligodendroglial tumors are rare subtypes of brain tumors and are often combined with other glial tumors in epidemiological analyses. However, different demographic associations and clinical characteristics suggest potentially different risk factors. The purpose of this study was to investigate p...

  6. Giant lumbar paraspinal atypical teratoid/rhabdoid tumor in a child

    Directory of Open Access Journals (Sweden)

    Agrawal Amit

    2009-01-01

    Full Text Available Atypical teratoid/rhabdoid tumor (AT/RT is a highly aggressive and uncommon tumor of the central nervous system, primarily affecting young children. AT/RT of the paraspinal region with involvement of the spine and spinal cord is extremely rare, with only few case reports in the literature. We report an unusual case of giant lumbar paraspinal AT/RT with intraspinal extension in a previously healthy 18-month-old female child. To the best of our knowledge, this kind of presentation has not been reported previously in the English literature.

  7. Adenomatoid odontogenic tumor of the mandible with unusual radiographic features: A case report

    International Nuclear Information System (INIS)

    Narayanan, Veena S.; Naidu, Giridhar; Haldar, Maya; Ragavendra, Raju; Mhaske-Jedhe, Shubang

    2013-01-01

    Adenomatoid odontogenic tumor (AOT) usually presents as a unilocular, pericoronal radiolucency in the maxillary anterior region in adolescent females. Very few conditions occur in such a narrow age range and at such a restrictive site. Rarely, these tumors present with varied clinical features. A case of AOT of the mandible is reported with unusual features such as large size, multilocular appearance, and aggressive behavior. The role of radiology in diagnosis of atypical AOT is extremely important. The unique radiological manifestations of the lesion helped in the diagnosis, and it was managed conservatively with no evidence of recurrence.

  8. Adenomatoid odontogenic tumor of the mandible with unusual radiographic features: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Narayanan, Veena S. [Dept. of Oral Medicine and Radiology, Coorg Institute of Dental Sciences, Virajpe (India); Naidu, Giridhar; Haldar, Maya [Dept. of Oral Medicine and Radiology, Peoples' Dental Academy, Bhopal (India); Ragavendra, Raju; Mhaske-Jedhe, Shubang [Dept. of Oral Pathology and Microbiology, Peoples' Dental Academy, Bhopal (India)

    2013-06-15

    Adenomatoid odontogenic tumor (AOT) usually presents as a unilocular, pericoronal radiolucency in the maxillary anterior region in adolescent females. Very few conditions occur in such a narrow age range and at such a restrictive site. Rarely, these tumors present with varied clinical features. A case of AOT of the mandible is reported with unusual features such as large size, multilocular appearance, and aggressive behavior. The role of radiology in diagnosis of atypical AOT is extremely important. The unique radiological manifestations of the lesion helped in the diagnosis, and it was managed conservatively with no evidence of recurrence.

  9. Os Odontoideum: Rare Cervical Lesion

    Directory of Open Access Journals (Sweden)

    Kristie A Robson

    2011-05-01

    Full Text Available We report the case of a 22-year-old Marine who presented to the emergency department, after a martial arts exercise, with transient weakness and numbness in all extremities. Computed tomography cervical spine radiographs revealed os odontoideum. Lateral flexion–extension radiographs identified atlanto-axillary instability. This abnormality is rare and can be career ending for military members who do not undergo surgical fusion. [West J Emerg Med. 2011;12(4:520–522.

  10. Macrodystrophia lipomatosa: a rare presentation

    Directory of Open Access Journals (Sweden)

    Sushil G. Kachewar

    2011-07-01

    Full Text Available Macrodystrophia lipomatosa is a rare congenital nonhereditary mesenchymal hamartomatous malformation resulting in localized gigantism of parts of extremities that manifests clinically as macrodactyly or megalodactyly. Radiological and Pathological hallmark is the disproportionate fibroadipose tissue proliferation in subcutaneous tissue, nerve sheaths, and periosteum that leads to soft tissue and bony enlargement. As imaging is the key to early diagnosis, findings on various modalities like plain radiographs, CT scan and MRI are highlighted here.

  11. Pleomorphic Hyalinizing Angiectatic Tumor Arising in the Hand

    Science.gov (United States)

    Kane, Patrick M.; Gaspar, Michael P.; Whiting, Benjamin B.; Culp, Randall W.

    2016-01-01

    Background: Background: Pleomorphic hyalinizing angiectatic tumors (PHATs) are extremely rare, non-metastasizing tumors of uncertain origin that are typically seen in the lower extremities. To date, it is estimated that less than 100 cases have been reported worldwide since first described in 1996. Methods: The case of a 35-year-old male with a several-year history of a dorsal hand mass is presented. Although the patient was initially asymptomatic, in the months prior to presentation, the patient complained of pain with power grasp and direct pressure over the mass. The patient underwent uncomplicated surgical excision, during which the mass was noted to be adherent to the underlying extensor tendons. Results: Immunopathology confirmed the mass to be PHAT. We believe this is the first documented case of this rare tumor occurring in the hand. Conclusions: History and epidemiology of PHAT are reviewed. Then, in the context of the presented case, pre-operative evaluation, surgical management, pathologic findings and post-operative follow-up are all discussed. PMID:27698646

  12. A Versatile Microarray Platform for Capturing Rare Cells

    Science.gov (United States)

    Brinkmann, Falko; Hirtz, Michael; Haller, Anna; Gorges, Tobias M.; Vellekoop, Michael J.; Riethdorf, Sabine; Müller, Volkmar; Pantel, Klaus; Fuchs, Harald

    2015-10-01

    Analyses of rare events occurring at extremely low frequencies in body fluids are still challenging. We established a versatile microarray-based platform able to capture single target cells from large background populations. As use case we chose the challenging application of detecting circulating tumor cells (CTCs) - about one cell in a billion normal blood cells. After incubation with an antibody cocktail, targeted cells are extracted on a microarray in a microfluidic chip. The accessibility of our platform allows for subsequent recovery of targets for further analysis. The microarray facilitates exclusion of false positive capture events by co-localization allowing for detection without fluorescent labelling. Analyzing blood samples from cancer patients with our platform reached and partly outreached gold standard performance, demonstrating feasibility for clinical application. Clinical researchers free choice of antibody cocktail without need for altered chip manufacturing or incubation protocol, allows virtual arbitrary targeting of capture species and therefore wide spread applications in biomedical sciences.

  13. Xeroderma pigmentosum: A rare case report with review of literature

    Directory of Open Access Journals (Sweden)

    B Anand

    2012-01-01

    Full Text Available Xeroderma pigmentosum, or XP, is an autosomal recessive genetic disorder in which the ability to repair DNA damage caused by ultraviolet (UV light is deficient. In extreme cases, all exposure to sunlight must be forbidden, no matter how small. As such, individuals with the disease are often colloquially referred to as ′Children of the Night′. Mutations in XP genes that regulate nucleotide excision repair, not only predispose persons with xeroderma pigmentosum to multiple malignancies, but also promote premature cutaneous and ocular ageing, and in some cases promote progressive neurodegenerative changes. There is a great involvement of many parts of the body, especially head and neck. The oral manifestations are mainly related to the occurrence of malignant tumors in the lips, tongue and buccal mucosa. This paper reports a rare case of XP in a 40-year-old female presenting with dermatological, oral and ophthalmological involvement.

  14. Peripheral soft tissue ewing's sarcoma: a rare case report

    Directory of Open Access Journals (Sweden)

    Farzana Shegufta

    2013-07-01

    Full Text Available A 22 years male patient presented with gradual left forearm swelling for 6 months. X ray forearm revealed large soft tissue swelling with tiny calcification and mild scalloping at inner aspect of ulna and ultrasonogram (USG revealed soft tissue mass having calcification and necrotic areas within and spectral Doppler showed arterial type of blood flow with no augmentation. Later computerized tomography (CT scan showed soft tissue mass with necrotic area and calcification with no bony involvement. Magnetic resonance imaging (MRI with contrast revealed a large heterogeneously enhancing lobulated mixed intensity lesion in antero-medial compartment of the left forearm involving flexor group of muscles causing displacement of fat plane. MRI and subsequent histopathology of the lesion revealed it as a rare soft tissue Ewing’s sarcoma / primitive neuroectodermal tumor (PNET in extremity. Ibrahim Med. Coll. J. 2013; 7(2: 43-46

  15. Multicentric malignant gastrointestinal stromal tumor

    International Nuclear Information System (INIS)

    Shukla, Shailaja; Singh, Sanjeet K; Pujani, Mukta

    2009-01-01

    Malignant gastrointestinal stromal tumor (GIST) is a rare type of sarcoma that is found in the digestive system, most often in the wall of the stomach. Multiple GISTs are extremely rare and usually associated with type 1 neurofibromatosis and familial GIST. We report here a case of a 70-year-old woman who reported pain in the abdomen, loss of appetite, and weight loss for six months. Ultrasound examination showed a small bowel mass along with multiple peritoneal deposits and a mass within the liver. Barium studies were suggestive of a neoplastic pathology of the distal ileum. A differential diagnosis of adenocarcinoma/lymphoma with metastases was entertained. Perioperative findings showed two large growths arising from the jejunum and the distal ileum, along with multiple smaller nodules on the serosal surface and adjoining mesentery of the involved bowel segments. Segmental resection of the involved portions of the intestine was performed. Histopathological features were consistent with those of multicentric malignant GIST-not otherwise specified (GIST-NOS). Follow-up examination three months after surgery showed no evidence of recurrence. (author)

  16. A rare cause of hemoptysis

    Directory of Open Access Journals (Sweden)

    Meghan Aversa

    2014-01-01

    Full Text Available Angiosarcomas are rare, malignant, endothelial-cell tumors of vascular origin that can arise at any body site. They frequently metastasize to the lung, heralded by dyspnea, hemoptysis, chest pain, pneumothoraces, and diffuse pulmonary hemorrhage. However, in most cases lung metastases are discovered after the diagnosis of a primary angiosarcoma has already been established. Very rarely will an undiagnosed metastatic angiosarcoma present as diffuse pulmonary hemorrhage. We describe the case of a 59-year-old male who presented to hospital with dyspnea and hemoptysis. CT chest revealed rapidly progressing nodular changes and broncho-alveolar lavage returns were progressively bloody. Open lung wedge biopsy ultimately revealed metastatic angiosarcoma and extensive pulmonary hemorrhage. Our case highlights the key clinical, radiological, and pathological features of this rare malignancy that frequently metastasizes to the lung and reminds clinicians to consider it as a cause of hemoptysis and pulmonary hemorrhage.

  17. Rare Cause of Dysphagy: Giant Polypoid Esophageal Well-Differentiated Liposarcoma

    Directory of Open Access Journals (Sweden)

    Ladislav Mica

    2007-06-01

    Full Text Available Liposarcoma represents one of the most frequent (10–20% malignant mesenchymal tumors in the adult, affecting mostly the soft tissue of extremities, the trunk or the retroperitoneum. This tumor type occurs exceptionally rarely in the gastrointestinal tract with only few cases described in the literature. In this case we present a 73-year-old male patient who was admitted due to loss of weight, anorexia and postprandial emesis with dysphagy. Gastrographin esophagography failed to make precise diagnostics. CT scan of the upper gastrointestinal tract revealed a large esophageal tumor filling out the whole length of the esophagus. The tumor was removed by parasternocleidomastoidal approach with a stapler. Histopathological examination revealed a well-differentiated liposarcoma (grade I. Well-differentiated liposarcomas are characterised by amplified material of the 12q13–15 chromosomal region, present in the form of giant or ring chromosomes and leading to the overexpression of MDM2 and CDK4 genes. MDM2 and CDK4 proteins can be detected immunhistochemically, which was the case in the reported tumor. Overexpression of these proteins leads to suppression of tumor suppressor genes, leading to increased cell survival.

  18. Extremely 54Cr- and 50Ti-rich Presolar Oxide Grains in a Primitive Meteorite: Formation in Rare Types of Supernovae and Implications for the Astrophysical Context of Solar System Birth

    Science.gov (United States)

    Nittler, Larry R.; O’D. Alexander, Conel M.; Liu, Nan; Wang, Jianhua

    2018-04-01

    We report the identification of 19 presolar oxide grains from the Orgueil CI meteorite with substantial enrichments in 54Cr, with 54Cr/52Cr ratios ranging from 1.2 to 56 times the solar value. The most enriched grains also exhibit enrichments at mass-50, most likely due in part to 50Ti, but close-to-normal or depleted 53Cr/52Cr ratios. There is a strong inverse relationship between 54Cr enrichment and grain size; the most extreme grains are all attractive, as these likely occur much more frequently than high-density SN Ia, and their evolutionary timescales (∼20 Myr) are comparable to those of molecular clouds. Self-pollution of the Sun’s parent cloud from an ECSN may explain the heterogeneous distribution of n-rich isotopic anomalies in planetary materials, including a recently reported dichotomy in Mo isotopes in the solar system. The stellar origins of three grains with solar 54Cr/52Cr, but anomalies in 50Cr or 53Cr, as well as of a grain enriched in 57Fe, are unclear.

  19. Virilization caused by an ectopic adrenal tumor located behind the iliopsoas muscle.

    Science.gov (United States)

    Mavroudis, Konstantinos; Aloumanis, Kyriakos; Papapetrou, Peter D; Voros, Dionisios; Spanos, Iraklis

    2007-06-01

    Virilization due to androgen-secreting neoplasms in women is a result of androgen overproduction from benign or malignant tumors that are found in the ovaries or rarely in the adrenal glands. Virilizing tumors that arise from ectopic adrenal tissue are extremely rare. We describe a very rare case of an ectopic androgen-producing adrenal tumor. Case report study. Endocrinology outpatient department of university-affiliated teaching hospital. A 45-year-old woman with symptoms of virilization of abrupt onset and rapid progression, with high serum androgen hormone levels and normal glucocorticoid secretion. Basal hormonal levels, stimulation and suppression tests, imaging techniques, and selective venous sampling. Localization and surgical removal of the source of androgen production. An ectopic mass was detected behind the left iliopsoas muscle. The patient was operated on and an oblong-shaped lesion, weighing 6 g, was removed. Histologically, the tissue was identified to be of adrenal origin. Postoperatively the androgen levels decreased to normal levels. This case illustrates difficulties in detecting and localizing the rare contingence of an ectopic adrenocortical androgen-secreting tumor.

  20. Collision tumor of the thyroid: follicular variant of papillary carcinoma and squamous carcinoma

    Directory of Open Access Journals (Sweden)

    Kane Subhadra V

    2006-09-01

    epidemiology, biology and establish standardized protocols for treatment of these extremely rare tumors.

  1. Schwannoma of the Recurrent Laryngeal Nerve : A Rare Entity

    NARCIS (Netherlands)

    de Heer, Linda M; Teding van Berkhout, F; Priesterbach, Loudy; Buijsrogge, Marc P

    Neurogenic tumors are the most common posterior mediastinal tumors in adults. Schwannomas originating from the recurrent laryngeal nerve are rare. The present study describes a 46-year-old man with a tumor in the left superior mediastinum. Because of the narrow relationship with the aorta and the

  2. Primary malignancy of seminal vesicle: A rare entity

    Directory of Open Access Journals (Sweden)

    Rajaraman Ramamurthy

    2011-01-01

    Full Text Available We report a rare case of seminal vesicle malignancy (primitive neuro ectodermal tumor in a 40-year-old male patient. He was treated with enbloc resection of the tumor and ureteric reimplantation. In view of the rarity of this entity, management of these tumors should be individualized.

  3. Current Perspectives on Desmoid Tumors: The Mayo Clinic Approach

    Directory of Open Access Journals (Sweden)

    Scott Okuno

    2011-08-01

    Full Text Available Desmoid tumors are a rare group of locally aggressive, non malignant tumors of fibroblastic origin that can lead to significant morbidity due to local invasion. Despite advances in the understanding of these tumors, their natural history is incompletely understood and the optimal treatment is still a matter of debate. Local control is the main goal of treatment and there has been a change in philosophy regarding the management of these tumors from aggressive surgical resection to function preservation. A multidisciplinary approach is essential to plan local control with acceptable morbidity. The current Mayo Clinic algorithm for the treatment of these tumors is based on institutional experience and the available evidence in the literature: asymptomatic/non progressive lesions away from vital structures are managed with observation and regular imaging; primary or recurrent desmoid tumors which are symptomatic or progressive or near vital structures are managed with wide surgical resection when wide surgical margins are possible with minimal functional and cosmetic loss. When positive or close surgical margins are likely, surgical resection with adjuvant radiotherapy or definitive radiotherapy is preferred. If likely functional or cosmetic deficit is unacceptable, radiotherapy is the treatment of choice. Unresectable lesions are considered for radiotherapy, chemotherapy or newer modalities however an unresectable lesion associated with a painful, functionless, infected extremity is managed with an amputation.

  4. Current Perspectives on Desmoid Tumors: The Mayo Clinic Approach

    International Nuclear Information System (INIS)

    Joglekar, Siddharth B.; Rose, Peter S.; Sim, Franklin; Okuno, Scott; Petersen, Ivy

    2011-01-01

    Desmoid tumors are a rare group of locally aggressive, non malignant tumors of fibroblastic origin that can lead to significant morbidity due to local invasion. Despite advances in the understanding of these tumors, their natural history is incompletely understood and the optimal treatment is still a matter of debate. Local control is the main goal of treatment and there has been a change in philosophy regarding the management of these tumors from aggressive surgical resection to function preservation. A multidisciplinary approach is essential to plan local control with acceptable morbidity. The current Mayo Clinic algorithm for the treatment of these tumors is based on institutional experience and the available evidence in the literature: asymptomatic/non progressive lesions away from vital structures are managed with observation and regular imaging; primary or recurrent desmoid tumors which are symptomatic or progressive or near vital structures are managed with wide surgical resection when wide surgical margins are possible with minimal functional and cosmetic loss. When positive or close surgical margins are likely, surgical resection with adjuvant radiotherapy or definitive radiotherapy is preferred. If likely functional or cosmetic deficit is unacceptable, radiotherapy is the treatment of choice. Unresectable lesions are considered for radiotherapy, chemotherapy or newer modalities however an unresectable lesion associated with a painful, functionless, infected extremity is managed with an amputation

  5. Bone tumors

    International Nuclear Information System (INIS)

    Unni, K.K.

    1988-01-01

    This book contains the proceedings on bone tumors. Topics covered include: Bone tumor imaging: Contribution of CT and MRI, staging of bone tumors, perind cell tumors of bone, and metastatic bone disease

  6. Dermatofibrosarcoma Protuberans: A Rare Malignancy of the Breast

    Directory of Open Access Journals (Sweden)

    Salim Al-Rahbi

    2015-09-01

    Full Text Available Dermatofibrosarcoma protuberans (DFSP of the breast is a rare skin cancer with only a few cases reported in the literature. It corresponds to approximately 1% of all soft tissue sarcomas and to less than 0.1% of all malignancies with annual incidence of 0.8–4.5 cases per million. DFSP usually affects young and middle-aged adults and it can affect any site, but most commonly the trunk and extremities. This tumor arises from the deep dermis and subcutaneous tissues and is usually locally aggressive, but rarely metastasizes. Surgery including wide local excision with free margins remains the main modality of treatment. We report a case in a 28-year-old Omani woman who presented with a right breast lump to a local secondary hospital. She was offered excision biopsy of the lump and the histology came as DFSP with involved margins. The patient was then referred to the Breast Unit at the Royal Hospital where she was reviewed by multidisciplinary team including breast surgeons, oncologists, radiation oncologists, pathologists, and breast radiologists. Her laboratory and radiological investigations did not show any signs of distal metastasis and breast ultrasound did not reveal any residual breast lesions. We performed a wide re-excision of the previous surgical scar with the underlying breast tissues down to the level of the pectoral fascia followed by treatment with radiotherapy. The final histology report showed the presence of a 4×5mm residual tumor, and all resection margins were tumor-free. At six months follow-up, she had no signs of local recurrence. We publish this case report because of its rarity.

  7. Neuroblastoma in early childhood: A rare case report and review of literature

    Directory of Open Access Journals (Sweden)

    Ritesh R Kalaskar

    2016-01-01

    Full Text Available Neuroblastoma is an extremely rare pediatric neoplasm whose prognosis becomes poor and poor as the age advances. It can be sporadic or nonfamilial in origin. It is primarily a tumor of abdominal origin from where it metastasis to lymph nodes, liver, intracranial and orbital sites, and central nervous system. There is no standard dental treatment protocol for the management of neuroblastoma due to its poor survival rate and rarity. However, dental treatment may follow the protocol of preventive and restorative. Surgicals should be performed under supervision as it may trigger metastasis. We report a rare case of neuroblastoma in a 3-year-old child presenting classical oral manifestations such as bilateral palatal swelling, rolled border ulcer on the posterior part of hard palate adjacent to primary molars, and bilateral proptosis.

  8. Intrapontine malignant nerve sheath tumor

    DEFF Research Database (Denmark)

    Kozić, Dusko; Nagulić, Mirjana; Samardzić, Miroslav

    2008-01-01

    . On pathological examination, the neoplasm appeared to be an intrapontine nerve sheath tumor originating most likely from the intrapontine segment of one of the cranial nerve fibres. The tumor showed exophytic growth, with consequent spread to adjacent subaracnoid space. MR spectroscopy revealed the presence......The primary source of malignant intracerebral nerve sheath tumors is still unclear We report the imaging and MR spectroscopic findings in a 39-year-old man with a very rare brain stem tumor MR examination revealed the presence of intraaxial brain stem tumor with a partial exophytic growth...

  9. Large Complex Odontoma of Mandible in a Young Boy: A Rare and Unusual Case Report

    Directory of Open Access Journals (Sweden)

    G. Siva Prasad Reddy

    2014-01-01

    Full Text Available Odontomas are the most common odontogenic tumors. They are broadly classified in to Compound Odontoma and Complex Odontoma. Among them complex odontoma is a rare tumor. Occasionally this tumor becomes large, causing expansion of bone followed by facial asymmetry. Otherwise these tumors are asymptomatic and are generally diagnosed on radiographic examination. We report a rare case of complex odontoma of mandible in a young boy. The tumor was treated by surgical excision under general anesthesia.

  10. Osteosarcoma of the Maxilla: A Rare Case with Unusual Clinical Presentation

    Directory of Open Access Journals (Sweden)

    Pouyan Amini Shakib

    2013-08-01

    Full Text Available Osteosarcoma (OS is a malignant mesenchymal tumor, which rarely occurs in the maxilla. Although variable histologic and radiographic features of OS have been reported previously, in the majority of the cases painful swelling of the jaw is mentioned as the first clinical presentation. Furthermore, early diagnosis and wide surgical resection of the tumor are the most important determinant factors of prognosis. Therefore, the unusual clinical presentations of OS should be considered meticulously to expedite the diagnosis process. We describe a case of OS of the maxilla with extremely unusual presentation in a 42-year-old female, that was initially designated as “epulis fissuratum”. Here, we highlight the importance of combining the clinical, radiographic and histopathologic examination to obtain a definitive diagnosis and also the significance of early effective surgical intervention in evaluation of pathologic lesions.

  11. Isolated Juvenile Xanthogranuloma in Thoracic Spine: Intraoperative Cytological Diagnosis of a Rare Presentation

    Directory of Open Access Journals (Sweden)

    Shashi Singhvi

    2014-06-01

    Full Text Available Juvenile Xanthogranulomas (JXG are benign proliferative disorders of non-Langerhans histiocytes, which present in children as multiple, self-limited, cutaneous lesions. The extracutaneous manifestations of JXG are uncommon, and isolated JXG involving the spinal column is extremely rare. We report here a case of isolated juvenile xanthogranuloma in thoracic spine correctly diagnosed intraoperatively on crush smear cytology and later confirmed by histopathological and immunohistochemical studies. This case report draws attention to the fact that isolated xanthogranuloma should be considered among possible diagnoses of spinal tumor in children. Also, since the long term survival depends on complete surgical resection, a correct intraoperative diagnosis is extremely important for optimal management and prognosis of the patient. [J Interdiscipl Histopathol 2014; 2(3.000: 158-162

  12. Multiple Primary Merkel Cell Carcinomas Presenting as Pruritic, Painful Lower Leg Tumors

    Directory of Open Access Journals (Sweden)

    Laura Blumenthal

    2015-10-01

    Full Text Available Merkel cell carcinoma (MCC is a rare and highly aggressive neuroendocrine tumor of the skin which almost exclusively presents as a solitary tumor. It is most often seen on sun-exposed regions, historically almost exclusively on the head and neck, with only rare case reports on the extremities. Although recent studies have shown increased incidence with up to 20% on the extremities, here we present one of these rare emerging presentations, with the addition of a unique treatment option. Our patient is an 80-year-old male with a 3-month history of multiple raised, rapidly enlarging tumors on the right ankle. Two separate biopsies were performed and demonstrated sheets and clusters of small blue cells filling the dermis with scant cytoplasm, dusty chromatin, and nuclear molding. Subsequent immunohistochemical stains confirmed the diagnosis of multiple primary MCC. Despite the characteristic immunohistochemical profile of primary MCC, the possibility of a metastatic neuroendocrine carcinoma from an alternate primary site was entertained, given his unusual clinical presentation. A complete clinical workup including CT scans of the chest, abdomen, and pelvis showed no evidence of disease elsewhere. Instead of amputation, the patient opted for nonsurgical treatment with radiation therapy alone, resulting in a rapid and complete response. This case represents an unusual presentation of primary MCC and demonstrates further evidence that radiation as monotherapy is an effective local treatment option for inoperable MCC.

  13. Multiple Primary Merkel Cell Carcinomas Presenting as Pruritic, Painful Lower Leg Tumors

    Science.gov (United States)

    Blumenthal, Laura; VandenBoom, Timothy; Melian, Edward; Peterson, Anthony; Hutchens, Kelli A.

    2015-01-01

    Merkel cell carcinoma (MCC) is a rare and highly aggressive neuroendocrine tumor of the skin which almost exclusively presents as a solitary tumor. It is most often seen on sun-exposed regions, historically almost exclusively on the head and neck, with only rare case reports on the extremities. Although recent studies have shown increased incidence with up to 20% on the extremities, here we present one of these rare emerging presentations, with the addition of a unique treatment option. Our patient is an 80-year-old male with a 3-month history of multiple raised, rapidly enlarging tumors on the right ankle. Two separate biopsies were performed and demonstrated sheets and clusters of small blue cells filling the dermis with scant cytoplasm, dusty chromatin, and nuclear molding. Subsequent immunohistochemical stains confirmed the diagnosis of multiple primary MCC. Despite the characteristic immunohistochemical profile of primary MCC, the possibility of a metastatic neuroendocrine carcinoma from an alternate primary site was entertained, given his unusual clinical presentation. A complete clinical workup including CT scans of the chest, abdomen, and pelvis showed no evidence of disease elsewhere. Instead of amputation, the patient opted for nonsurgical treatment with radiation therapy alone, resulting in a rapid and complete response. This case represents an unusual presentation of primary MCC and demonstrates further evidence that radiation as monotherapy is an effective local treatment option for inoperable MCC. PMID:26594171

  14. An adolescent with a phyllodes tumor: A case report and review

    Directory of Open Access Journals (Sweden)

    Deepa Makhija

    2016-12-01

    Full Text Available Phyllodes tumors are rare fibroepithelial tumors that account for less than 0.5% of all breast tumors. Presentation in children is even rarer. In this paper, we describe a case of an adolescent with a phyllodes tumor. The rare presentation at this age, its distinguishing features, the preoperative diagnostic difficulties, and the management protocols of this uncommon tumor are highlighted.

  15. Conservative multimodal management of a primitive neuroectodermal tumor of the thyroid

    Directory of Open Access Journals (Sweden)

    Juliette Haudebourg

    2013-04-01

    Full Text Available Primitive neuroectodermal tumors (PNET represent 1% of sarcomas. Head and neck peripheral PNETs have an intermediate prognosis between abdominopelvic disease and extremities. We here report the case of a 40-year old male who presented with primitive neuroectodermal tumor of the thyroid and was treated by multimodal treatment, including surgery, chemotherapy and intermediate dose radiotherapy. The patient is alive and fit with a functional larynx at 27 months. Multimodal treatments yield five-year survival rates of about 60%. Major drug regimens use vincristine, doxorubicin, ifosfamide or cyclophosphamide, dactinomycin and/or etoposide. Complete surgical excision is undertaken whenever possible to improve long-term survival. However, the relative radiosensitivity of tumors of the Ewing family, suggest multimodal treatment including adjuvant conformal radiotherapy in case of positive margins or poor response to chemotherapy rather than resection with 2-3 cm margins, which would imply laryngeal sacrifice for thyroid tumors. The role of expert rare tumor networks is crucial for optimal decision-making and management of such rare tumors on a case by case basis.

  16. Neuroendocrine tumors of the pancreas.

    LENUS (Irish Health Repository)

    Davies, Karen

    2009-04-01

    Pancreatic endocrine tumors are rare neoplasms accounting for less than 5% of pancreatic malignancies. They are broadly classified into either functioning tumors (insulinomas, gastrinomas, glucagonomas, VIPomas, and somatostatinomas) or nonfunctioning tumors. The diagnosis of these tumors is difficult and requires a careful history and examination combined with laboratory tests and radiologic imaging. Signs and symptoms are usually related to hormone hypersecretion in the case of functioning tumors and to tumor size or metastases with nonfunctioning tumors. Surgical resection remains the treatment of choice even in the face of metastatic disease. Further development of novel diagnostic and treatment modalities offers potential to greatly improve quality of life and prolong disease-free survival for patients with pancreatic endocrine tumors.

  17. Neuroendocrine tumors of the pancreas.

    LENUS (Irish Health Repository)

    Davies, Karen

    2012-02-01

    Pancreatic endocrine tumors are rare neoplasms accounting for less than 5% of pancreatic malignancies. They are broadly classified into either functioning tumors (insulinomas, gastrinomas, glucagonomas, VIPomas, and somatostatinomas) or nonfunctioning tumors. The diagnosis of these tumors is difficult and requires a careful history and examination combined with laboratory tests and radiologic imaging. Signs and symptoms are usually related to hormone hypersecretion in the case of functioning tumors and to tumor size or metastases with nonfunctioning tumors. Surgical resection remains the treatment of choice even in the face of metastatic disease. Further development of novel diagnostic and treatment modalities offers potential to greatly improve quality of life and prolong disease-free survival for patients with pancreatic endocrine tumors.

  18. Hidradenocarcinoma eccrinale syringomatodes of the eyelid--case of a rare malignancy.

    Science.gov (United States)

    Karolina, Ziaja; Arkadiusz, Pogrzebielski; Jolanta, Orłowska-Heitzman; Bozena, Romanowska-Dixon

    2010-01-01

    Presentation of a case of very rare malignant tumor of eccrine sweat glands in the eyelid. A 44 years old man with a tumor of the upper left eyelid is presented. Sweat gland carcinomas are rare malignant tumors of the skin adnexa. Diagnosis of these carcinomas is difficult due to their infrequency.

  19. Granular cell tumor: An uncommon benign neoplasm

    Directory of Open Access Journals (Sweden)

    Tirthankar Gayen

    2015-01-01

    Full Text Available Granular cell tumor is a distinctly rare neoplasm of neural sheath origin. It mainly presents as a solitary asymptomatic swelling in the oral cavity, skin, and rarely internal organs in the middle age. Histopathology is characteristic, showing polyhedral cells containing numerous fine eosinophilic granules with indistinct cell margins. We present a case of granular cell tumor on the back of a 48-year-old woman which was painful, mimicking an adnexal tumor.

  20. Testicular calculus: A rare case.

    Science.gov (United States)

    Sen, Volkan; Bozkurt, Ozan; Demır, Omer; Tuna, Burcin; Yorukoglu, Kutsal; Esen, Adil

    2015-01-01

    Testicular calculus is an extremely rare case with unknown etiology and pathogenesis. To our knowledge, here we report the third case of testicular calculus. A 31-year-old man was admitted to our clinic with painful solid mass in left testis. After diagnostic work-up for a possible testicular tumour, he underwent inguinal orchiectomy and histopathologic examination showed a testicular calculus. Case hypothesis: Solid testicular lesions in young adults generally correspond to testicular cancer. Differential diagnosis should be done carefully. Future implications: In young adults with painful and solid testicular mass with hyperechogenic appearance on scrotal ultrasonography, testicular calculus must be kept in mind in differential diagnosis. Further reports on this topic may let us do more clear recommendations about the etiology and treatment of this rare disease.

  1. Pancreatic metastasis from invasive pleomorphic lobular carcinoma of the breast: a rare case report.

    Science.gov (United States)

    Sun, Xiangjie; Zuo, Ke; Huang, Dan; Yu, Baohua; Cheng, Yufan; Yang, Wentao

    2017-07-11

    Invasive pleomorphic lobular carcinoma (PLC) is an aggressive subtype of invasive lobular carcinoma of the breast, which has its own histopathological and biological features. The metastatic patterns for PLC are distinct from those of invasive ductal carcinoma. In addition, pancreatic metastasis from PLC is extremely rare. We report a rare case of a 48-year-old woman presenting with clinical gastrointestinal symptoms and pancreatic metastasis of PLC. The pancreatic tumor was composed of pleomorphic tumor cells arranged in the form of solid sheets and nests and as single files, with frequent mitotic figures, nucleolar prominence, high nuclear to cytoplasmic ratio and loss of cohesion. The malignant cells were positive for p120 (cytoplasmic) and GATA3 and negative for estrogen receptor, progesterone receptor, human epidermal growth factor receptor 2, E-cadherin, gross cystic disease fluid protein 15 and mammaglobin, which indicated a lobular carcinoma phenotype of the breast. To the best of our knowledge, this is one of the few reported cases in the literature of pancreatic metastasis of invasive lobular carcinoma of the breast, of which the definitive diagnosis was obtained only after surgery. Rare metastasis sites should be considered, particularly, when a patient has a medical history of PLC.

  2. Carotid Body Tumor Presenting as Parotid Swelling Misdiagnosed ...

    African Journals Online (AJOL)

    Carotid body tumor (CBT) also known as chemodectoma is a rare tumor of neuroendocrine tissue of carotid body and is the most commonly seen jugular paraganglioma. In most cases, it is benign but it can be malignant. Extra adrenal paraganglioma is rare. We present such a rare case where unusual presentation of ...

  3. Retroperitoneal Schwannoma: A Rare Case

    Directory of Open Access Journals (Sweden)

    Murat Kalaycı

    2011-01-01

    Case Presentation. A 38-year-old woman was admitted to the emergency service with the complaints of progressive abdominal pain and nausea for the last 24 hours. Abdominal examination was compatible with acute abdomen. Acute appendicitis was diagnosed by CT. During CT evaluation, a round shaped soft-tissue mass at the retroperitoneal area inferior to the right kidney was detected, The mass was resected and histology revealed schwannoma. Conclusion. Rare tumoral lesions with benign course such as schwannoma can be detected incidentally.

  4. Imaging of primary bone tumors in veterinary medicine: Which differences?

    Energy Technology Data Exchange (ETDEWEB)

    Vanel, Maïa, E-mail: maiavanel@yahoo.fr [Diagnostic Imaging Department, Faculty of Veterinary Medicine, University of Montreal, 3200 Rue Sicotte, PO Box 5000, Saint-Hyacinthe, QC (Canada); Blond, Laurent [Diagnostic Imaging Department, Faculty of Veterinary Medicine, University of Montreal, 3200 Rue Sicotte, PO Box 5000, Saint-Hyacinthe, QC (Canada); Vanel, Daniel [The Rizzoli Institute, Via del Barbiano 1-10, 40136, Bologna (Italy)

    2013-12-01

    Veterinary medicine is most often a mysterious world for the human doctors. However, animals are important for human medicine thanks to the numerous biological similarities. Primary bone tumors are not uncommon in veterinary medicine and especially in small domestic animals as dogs and cats. As in human medicine, osteosarcoma is the most common one and especially in the long bones extremities. In the malignant bone tumor family, chondrosarcoma, fibrosarcoma and hemangiosarcoma are following. Benign bone tumors as osteoma, osteochondroma and bone cysts do exist but are rare and of little clinical significance. Diagnostic modalities used depend widely on the owner willing to treat his animal. Radiographs and bone biopsy are the standard to make a diagnosis but CT, nuclear medicine and MRI are more an more used. As amputation is treatment number one in appendicular bone tumor in veterinary medicine, this explains on the one hand why more recent imaging modalities are not always necessary and on the other hand, that pronostic on large animals is so poor that it is not much studied. Chemotherapy is sometimes associated with the surgery procedure, depending on the agressivity of the tumor. Although, the strakes differs a lot between veterinary and human medicine, biological behavior are almost the same and should led to a beneficial team work between all.

  5. Imaging of primary bone tumors in veterinary medicine: Which differences?

    International Nuclear Information System (INIS)

    Vanel, Maïa; Blond, Laurent; Vanel, Daniel

    2013-01-01

    Veterinary medicine is most often a mysterious world for the human doctors. However, animals are important for human medicine thanks to the numerous biological similarities. Primary bone tumors are not uncommon in veterinary medicine and especially in small domestic animals as dogs and cats. As in human medicine, osteosarcoma is the most common one and especially in the long bones extremities. In the malignant bone tumor family, chondrosarcoma, fibrosarcoma and hemangiosarcoma are following. Benign bone tumors as osteoma, osteochondroma and bone cysts do exist but are rare and of little clinical significance. Diagnostic modalities used depend widely on the owner willing to treat his animal. Radiographs and bone biopsy are the standard to make a diagnosis but CT, nuclear medicine and MRI are more an more used. As amputation is treatment number one in appendicular bone tumor in veterinary medicine, this explains on the one hand why more recent imaging modalities are not always necessary and on the other hand, that pronostic on large animals is so poor that it is not much studied. Chemotherapy is sometimes associated with the surgery procedure, depending on the agressivity of the tumor. Although, the strakes differs a lot between veterinary and human medicine, biological behavior are almost the same and should led to a beneficial team work between all

  6. Vulvar Villoglandular Adenocarcinoma of Colonic Type: A Rare Finding

    Directory of Open Access Journals (Sweden)

    Heidarali Esmaeili

    2018-01-01

    Full Text Available Colonic type villoglandular adenocarcinoma of the lower genital tract is an extremely rare condition. Its origin is not clearly understood; however, the cloacal remnants are the more accepted source for this carcinoma.We report the case of a 67-year-old female patient who presented with a 1.2 cm polypoidal nodule at the right side of the fourchette. Morphologic studies revealed a colonic type mucinous adenocarcinoma that arose from within a villous adenoma. Immunohistochemical staining showed positive cytokeratin 7, cytokeratin 20, carcinoembryonic antigen, P53, and progesterone receptor, but negative for estrogen receptor and caudal type homeobox transcription factor 2. Extensive work-up failed to reveal other primary cancers in this patient. Ultimately, she underwent a radical vulvectomy. No recurrence was seen in eight months follow up of this patient after surgery. Careful, thorough histological evaluation and clinical clues enable correct diagnosis of the rare colonic type vulvar villoglandular adenocarcinoma. Due to rarity of this tumor, its management is questionable. Therefore, additional investigation is necessary for its management.

  7. Intrathoracic desmoid tumor in a child

    International Nuclear Information System (INIS)

    Krause, L.M.; Schey, W.L.; Bassuk, A.; Shrock, P.

    1985-01-01

    The case history of a child with an intrathoracic desmoid tumor is presented. The insidious nature of the tumor's development and its possible relationship to previous surgery are noted. Desmoid tumors are rare in the childhood population and an intrathoracic site as the origin of one has not been reported previously. (orig.)

  8. Solid-pseudo papillary tumor of the pancreas: Frantz's tumor

    International Nuclear Information System (INIS)

    Oliveira, Bruno Righi Rodrigues de; Moreira, Reni Cecilia Lopes; Campos, Marcelo Esteves Chaves

    2010-01-01

    The pseudo papillary solid tumor of the pancreas, also known as Frantz's tumor, is a rare disease, taking place in approximately 0.17% to 2.7% of non-endocrine tumors of the pancreas. Recently, the increase of its incidence has been noted with more than two-thirds of the total cases described in the last 10 years. A possible explanation is a greater knowledge of the disease and a greater uniformity of conceptualization in the last years. Generally, it affects young adult females. In most of the series, the tumor principally attacks the body and tail of the pancreas. The objective of the present report is to present the diagnostic and therapeutic option used in this rare pancreatic tumor of low-grade malignancy. (author)

  9. Fetus in fetu: A very rare anomaly

    Directory of Open Access Journals (Sweden)

    Sanjay Choudhuri

    2014-11-01

    Full Text Available “Fetus in fetu” is a condition seen rarely in the literature, less than 200 cases reported till now. It is a calcified mass or an encapsulated pendunculated tumor thought to be due to unequal division of totipotent cells of a blastocyst, resulting in a small cellular mass in a more mature embryo thus forming a monozygotic, diamniotic twin pregnancy.

  10. A rare indication for liver resection.

    Science.gov (United States)

    Popescu, I; Zamfir, R; Braşoveanu, V; Boroş, M; Herlea, V

    2005-01-01

    We present the rare case of a young female with a right upper abdomen tumoral mass and suffering abdominal discomfort. A combination of ultrasonography, computed tomography, magnetic resonance imaging and laparotomy was utilized to conclude a diagnosis of Riedel's lobe. Laparotomy and a resection of Riedel's lobe were selected as the correct therapeutic solutions.

  11. Rare endocrine cancers have novel genetic alterations

    Science.gov (United States)

    A molecular characterization of adrenocortical carcinoma, a rare cancer of the adrenal cortex, analyzed 91 cases for alterations in the tumor genomes and identified several novel genetic mutations as likely mechanisms driving the disease as well as whole genome doubling as a probable driver of the disease.

  12. Intrahepatic peribiliary perivascular epithelioid cell tumor (PEComa) associated with heterotopic pancreas: A case report.

    Science.gov (United States)

    Kiriyama, Yuka; Tsukamoto, Tetsuya; Mizoguchi, Yoshikazu; Ishihara, Shin; Horiguchi, Akihiko; Tokoro, Takamasa; Kato, Yutaro; Sugioka, Atsushi; Kuroda, Makoto

    2016-08-20

    Perivascular epithelioid-cell tumor (PEComa) is a group of rare mesenchymal neoplasms that express myomelanocytic-cell markers and exhibit a wide variety of histopathological features. Although heterotopic pancreas has been reported to occur in the gastrointestinal tract, intrahepatic heterotopic pancreas has been reported only rarely. We present a case of intrahepatic PEComa that showed a strong regional correlation with the presence of heterotopic pancreas. An intrahepatic tumor and biliary dilatation was incidentally discovered during a diagnostic evaluation to investigate low-back pain in a 47-year-old Japanese male. Cholangiocarcinoma was suspected and a left hemihepatectomy performed. Histological examination revealed a 3 × 3.8-mm tumor in the neighboring B2 bile duct. Histological and immunohistochemical investigations revealed the presence of a PEComa and pancreatic acini within the tumor mass. PEComa in the hepatobiliary and pancreatic regions are extremely rare. The presence of heterotopic pancreas is also relatively uncommon. The strong regional association of these 2 lesions raises the possibility of a PEComa originating from heterotopic pancreas or from an irritable response caused by heterotopic pancreas.

  13. Tooth-marked small theropod bone: an extremely rare trace

    DEFF Research Database (Denmark)

    Jacobsen, Aase Roland

    2001-01-01

    Tooth-marked dinosaur bones provide insight into feeding behaviours and biting strategies of theropod dinosaurs. The majority of theropod tooth marks reported to date have been found on herbivorous dinosaur bones, although some tyrannosaurid bones with tooth marks have also been reported. In 1988...

  14. A rare case of Charlin's syndrome

    Directory of Open Access Journals (Sweden)

    Mohit P Singh

    2017-01-01

    Full Text Available Charlin's syndrome is an extremely rare condition characterized by pain in the nasal and paranasal areas, which is precipitated by touching the lateral aspect of the ipsilateral nostril. We are presenting one such case of a 42-year-old man who was admitted to Dr. Chaudhary Hospital and Medical Research Centre.

  15. A Rare Seen Case Report: Sirenomelia

    Directory of Open Access Journals (Sweden)

    Nilay Piskinpasa

    2016-01-01

    Full Text Available Sirenomelia is a rare congenital anomaly characterized by abnormal development of the caudal body structures.The disease can be diagnosed antenatally by the careful ultrasound examination. In this paper we aimed to discuss a case which had one lower extremity,spinal angulations and anhydramnios image detected by ultrasound examination.We diagnosed sirenomelia after termination.

  16. Rare earth germanates

    International Nuclear Information System (INIS)

    Bondar', I.A.; Vinogradova, N.V.; Dem'yanets, L.N.

    1983-01-01

    Rare earth germanates attract close attention both as an independent class of compounds and analogues of a widely spread class of natural and synthetic minerals. The methods of rare earth germanate synthesis (solid-phase, hydrothermal) are considered. Systems on the basis of germanium and rare earth oxides, phase diagrams, phase transformations are studied. Using different chemical analysese the processes of rare earth germanate formation are investigated. IR spectra of alkali and rare earth metal germanates are presented, their comparative analysis being carried out. Crystal structures of the compounds, lattice parameters are studied. Fields of possible application of rare earth germanates are shown

  17. Pituitary gland tumors

    International Nuclear Information System (INIS)

    Jesser, J.; Schlamp, K.; Bendszus, M.

    2014-01-01

    This article gives an overview of the most common tumors of the pituitary gland and the differential diagnostics with special emphasis on radiological diagnostic criteria. A selective search of the literature in PubMed was carried out. Pituitary adenomas constitute 10-15 % of all intracranial tumors and are the most common tumors of the sellar region. Tumors smaller than 1 cm in diameter are called microadenomas while those larger than 1 cm in diameter are called macroadenomas. Approximately 65 % of pituitary gland adenomas secrete hormones whereby approximately 50 % secrete prolactin, 10 % secrete growth hormone (somatotropin) and 6 % secrete corticotropin. Other tumors located in the sella turcica can also cause endocrinological symptoms, such as an oversecretion of pituitary hormone or pituitary insufficiency by impinging on the pituitary gland or its stalk. When tumors spread into the space cranial to the sella turcica, they can impinge on the optic chiasm and cause visual disorders. A common differential diagnosis of a sellar tumor is a craniopharyngeoma. In children up to 10 % of all intracranial tumors are craniopharyngeomas. Other differential diagnoses for sellar tumors are metastases, meningiomas, epidermoids and in rare cases astrocytomas, germinomas or Rathke cleft cysts As these tumors are located in an anatomically complex region of the skull base and are often very small, a highly focused imaging protocol is required. The currently favored modality is magnetic resonance imaging (MRI) with the administration of a contrast agent. The sellar region should be mapped in thin slices. In cases of suspected microadenoma the imaging protocol should also contain a sequence with dynamic contrast administration in order to assess the specific enhancement characteristics of the tumor and the pituitary gland. (orig.) [de

  18. [Pituitary gland tumors].

    Science.gov (United States)

    Jesser, J; Schlamp, K; Bendszus, M

    2014-10-01

    This article gives an overview of the most common tumors of the pituitary gland and the differential diagnostics with special emphasis on radiological diagnostic criteria. A selective search of the literature in PubMed was carried out. Pituitary adenomas constitute 10-15% of all intracranial tumors and are the most common tumors of the sellar region. Tumors smaller than 1 cm in diameter are called microadenomas while those larger than 1 cm in diameter are called macroadenomas. Approximately 65% of pituitary gland adenomas secrete hormones whereby approximately 50% secrete prolactin, 10% secrete growth hormone (somatotropin) and 6% secrete corticotropin. Other tumors located in the sella turcica can also cause endocrinological symptoms, such as an oversecretion of pituitary hormone or pituitary insufficiency by impinging on the pituitary gland or its stalk. When tumors spread into the space cranial to the sella turcica, they can impinge on the optic chiasm and cause visual disorders. A common differential diagnosis of a sellar tumor is a craniopharyngeoma. In children up to 10% of all intracranial tumors are craniopharyngeomas. Other differential diagnoses for sellar tumors are metastases, meningiomas, epidermoids and in rare cases astrocytomas, germinomas or Rathke cleft cysts As these tumors are located in an anatomically complex region of the skull base and are often very small, a highly focused imaging protocol is required. The currently favored modality is magnetic resonance imaging (MRI) with the administration of a contrast agent. The sellar region should be mapped in thin slices. In cases of suspected microadenoma the imaging protocol should also contain a sequence with dynamic contrast administration in order to assess the specific enhancement characteristics of the tumor and the pituitary gland.

  19. Extrarenal retroperitoneal Wilms' tumor with subsequent pulmonary and peritoneal metastasis in a 4 year-old girl: A case report and review of literature

    Directory of Open Access Journals (Sweden)

    Jinyoung Park

    2016-05-01

    Full Text Available This report describes an extremely rare case of extrarenal Wilms' tumor in a 4 year-old girl who presented with abdominal mass and pain. Computed tomography (CT scanning revealed a well-defined mass lesion measuring 10 cm on the right side of her lower abdomen and pelvic cavity. The mass was surgically removed. Histologically, the tumor showed a triphasic pattern, consisting of stromal, epithelial and blastemal components. Immunohistochemistry showed that the tumor was positive for cytokeratin, vimentin and CD99. The histopathological diagnosis was extrarenal Wilms' tumor arising in the retroperitoneum and inguinal canal. The patient was administered adjuvant chemoradiotherapy due to subsequent pulmonary and peritoneal metastases. Follow-up 4 years later showed that she was doing well, with no recurrence of the tumor.

  20. Imaging tumors of the patella

    Energy Technology Data Exchange (ETDEWEB)

    Casadei, R., E-mail: roberto.casadei@ior.it [Department of Orthopaedic Oncology, Istituto Ortopedico Rizzoli, Bologna (Italy); Kreshak, J., E-mail: j.kreshak@yahoo.com [Department of Orthopaedic Oncology, Istituto Ortopedico Rizzoli, Bologna (Italy); Department of Pathology, Istituto Ortopedico Rizzoli, Bologna (Italy); Rinaldi, R. [Department of Radiology, Istituto Ortopedico Rizzoli, Bologna (Italy); Rimondi, E., E-mail: eugenio.rimondi@ior.it [Department of Radiology, Istituto Ortopedico Rizzoli, Bologna (Italy); Bianchi, G., E-mail: giuseppe.bianchi@ior.it [Department of Orthopaedic Oncology, Istituto Ortopedico Rizzoli, Bologna (Italy); Alberghini, M., E-mail: marco.alberghini@ior.it [Department of Pathology, Istituto Ortopedico Rizzoli, Bologna (Italy); Ruggieri, P. [Department of Orthopaedic Oncology, Istituto Ortopedico Rizzoli, Bologna (Italy); Vanel, D., E-mail: daniel.vanel@ior.it [Department of Radiology, Istituto Ortopedico Rizzoli, Bologna (Italy); Department of Pathology, Istituto Ortopedico Rizzoli, Bologna (Italy)

    2013-12-01

    Background: Patellar tumors are rare; only a few series have been described in the literature and radiographic diagnosis can be challenging. We reviewed all patellar tumors at one institution and reviewed the literature. Materials and methods: In an evaluation of the database at one institution from 1916 to 2009, 23,000 bone tumors were found. Of these, 41 involved the patella. All had imaging studies and microscopic diagnostic confirmation. All medical records, imaging studies, and pathology were reviewed. Results: There were 15 females and 26 males, ranging from 8 to 68 years old (average 30). There were 30 benign tumors; eight giant cell tumors, eight chondroblastomas, seven osteoid osteomas, two aneurysmal bone cysts, two ganglions, one each of chondroma, exostosis, and hemangioma. There were 11 malignant tumors: five hemangioendotheliomas, three metastases, one lymphoma, one plasmacytoma, and one angiosarcoma. Conclusion: Patellar tumors are rare and usually benign. As the patella is an apophysis, the most frequent lesions are giant cell tumor in the adult and chondroblastoma in children. Osteoid osteomas were frequent in our series and easily diagnosed. Metastases are the most frequent malignant diagnoses in the literature; in our series malignant vascular tumors were more common. These lesions are often easily analyzed on radiographs. CT and MR define better the cortex, soft tissue extension, and fluid levels. This study presents the imaging patterns of the more common patellar tumors in order to help the radiologist when confronted with a lesion in this location.

  1. Primitive neuroectodermal tumor of the maxillary sinus in an elderly male: A case report and literature review

    International Nuclear Information System (INIS)

    Shah, Saiquat; Huh, Kyung Hoe; Yi, Won Jin; Heo, Min Suk; Lee, Sam Sun; Choi, Soon Chul

    2014-01-01

    Primitive neuroectodermal tumor (PNET), which belongs to the Ewing's sarcoma (ES) family of tumors, is mainly seen in children and young adults. PNETs are extremely rare in the maxilla. Here, we report a case of PNET of the left maxillary sinus in an elderly male. Magnetic resonance imaging (MRI) revealed a slightly enhanced solid mass occupying the left maxillary sinus and infiltrating into the retroantral space. A partial maxillectomy was performed. Despite postoperative chemotherapy, follow-up computed tomography (CT) and MRI revealed a nodal metastasis in the submandibular space. Neck dissection was performed. However, the patient died 10 months after the second surgery because of distant metastasis to the liver. MRI and CT were particularly useful in detecting the extent of the tumor, recurrence, and metastasis. Further, a literature review of the previously reported PNET cases of the maxilla was carried out. In this paper, we also discuss the current approach for the diagnosis and management of these tumors.

  2. Primary malignant mixed müllerian tumor of the peritoneum a case report with review of the literature

    DEFF Research Database (Denmark)

    Kurshumliu, Fisnik; Rung-Hansen, Helle; Skovlund, Vibeke Ravn

    2011-01-01

    Malignant mixed Müllerian tumor is a rare malignancy of the genital tract and extremely uncommon in extragenital sites. This report describes a case of malignant mixed Müllerian tumor arising in the lower peritoneum of a 72-year-old female patient. The patient presented with ascites, lower...... abdominal mass and pleural effusion. The serum level of CA125 was elevated. At operation a diffuse carcinosis associated with tumor mass measuring 20 × 15 × 10 cm in the vesicouterine and Duglas' pouch were found. The uterus and the adnexa were unremarkable. Histopathology revealed a typical malignant mixed...... and the immunohistochemical analysis of our case, we believe that this is a monoclonal tumor with carcinoma being the "precursor" element. Nevertheless, further molecular and genetic evidence is needed to support such a conclusion....

  3. Primitive neuroectodermal tumor of the maxillary sinus in an elderly male: A case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Shah, Saiquat [Dept. of Dental Public Health, Bangladesh Dental College, Dhaka (Bangladesh); Huh, Kyung Hoe; Yi, Won Jin; Heo, Min Suk; Lee, Sam Sun; Choi, Soon Chul [Dept. of Oral and Maxillofacial Radiology and Dental Research Institute, School of Dentistry, Seoul National University, Seoul (Korea, Republic of)

    2014-12-15

    Primitive neuroectodermal tumor (PNET), which belongs to the Ewing's sarcoma (ES) family of tumors, is mainly seen in children and young adults. PNETs are extremely rare in the maxilla. Here, we report a case of PNET of the left maxillary sinus in an elderly male. Magnetic resonance imaging (MRI) revealed a slightly enhanced solid mass occupying the left maxillary sinus and infiltrating into the retroantral space. A partial maxillectomy was performed. Despite postoperative chemotherapy, follow-up computed tomography (CT) and MRI revealed a nodal metastasis in the submandibular space. Neck dissection was performed. However, the patient died 10 months after the second surgery because of distant metastasis to the liver. MRI and CT were particularly useful in detecting the extent of the tumor, recurrence, and metastasis. Further, a literature review of the previously reported PNET cases of the maxilla was carried out. In this paper, we also discuss the current approach for the diagnosis and management of these tumors.

  4. Multimodality Treatment in Ewing's Sarcoma Family Tumors of the Maxilla and Maxillary Sinus: Review of the Literature

    Science.gov (United States)

    Mamot, Christoph; Krasniqi, Fatime; Metternich, Frank

    2016-01-01

    The Ewing sarcoma family of tumors (ESFT) encompasses a group of highly aggressive, morphologically similar, malignant neoplasms sharing a common spontaneous genetic translocation that affect mostly children and young adults. These predominantly characteristic, small round-cell tumors include Ewing's sarcoma of the bone and soft tissue, as well as primitive neuroectodermal tumors (PNETs) involving the bone, soft tissue, and thoracopulmonary region (Askin's tumor). Extraosseous ESFTs are extremely rare, especially in the head and neck region, where literature to date consists of sporadic case reports and very small series. We hereby present a review of the literature published on ESFTs reported in the maxilla and maxillary sinus region from 1968 to 2016. PMID:27413360

  5. Adult Wilms tumor with inferior vena cava thrombus and distal deep vein thrombosis - a case report and literature review.

    Science.gov (United States)

    Ratajczyk, Krzysztof; Czekaj, Adrian; Rogala, Joanna; Kowal, Pawel

    2018-02-23

    Adult Wilms tumor (WT, nephroblastoma) is a rare, but well-described renal neoplasm. Although inferior vena cava tumor thrombosis is present in up to 10% of Wilms tumors in childhood, only few cases of this clinical manifestation in adults have been reported. To the best of our knowledge, this is the first case of adult WT infiltrating into inferior vena cava (IVC) with concomitant distal deep vein thrombosis. A 28-year-old male patient with gross hematuria and right flank pain was diagnosed with right kidney tumor penetrating to IVC. Preoperatively, acute distal thrombosis in inferior vena cava and lower extremities veins occurred. Right radical nephrectomy with tumor thrombectomy via cavotomy was performed. In order to prevent pulmonary embolism, IVC was ligated below left renal vein level. Histopathological examination revealed a triphasic nephroblastoma without anaplastic features. Postoperatively, patient was diagnosed with metastatic liver disease, which was treated with two lines of chemotherapy followed by radiotherapy with achievement of complete response. Adult WT occurs usually in young patients, under 40 years of age. Neoadjuvant chemotherapy proved to be effective in children, resulting with tumor shrinkage and venous tumor thrombus regression. Therefore, percutaneous biopsy should be always considered in young patients presenting with renal tumor invading venous system. IVC ligation is a safe treatment option in the event of complete inferior vena cava occlusion due to distal thrombosis concomitant to tumor thrombus, provided collateral venous pathways are well-developed.

  6. Tumors of the optic nerve

    DEFF Research Database (Denmark)

    Lindegaard, Jens; Heegaard, Steffen

    2009-01-01

    A variety of lesions may involve the optic nerve. Mainly, these lesions are inflammatory or vascular lesions that rarely necessitate surgery but may induce significant visual morbidity. Orbital tumors may induce proptosis, visual loss, relative afferent pupillary defect, disc edema and optic...... atrophy, but less than one-tenth of these tumors are confined to the optic nerve or its sheaths. No signs or symptoms are pathognomonic for tumors of the optic nerve. The tumors of the optic nerve may originate from the optic nerve itself (primary tumors) as a proliferation of cells normally present...... in the nerve (e.g., astrocytes and meningothelial cells). The optic nerve may also be invaded from tumors originating elsewhere (secondary tumors), invading the nerve from adjacent structures (e.g., choroidal melanoma and retinoblastoma) or from distant sites (e.g., lymphocytic infiltration and distant...

  7. An Erupted Dilated Odontoma: A Rare Presentation

    Directory of Open Access Journals (Sweden)

    Gaurav Sharma

    2016-01-01

    Full Text Available A dilated odontoma is an extremely rare developmental anomaly represented as a dilatation of the crown and root as a consequence of a deep, enamel-lined invagination and is considered a severe variant of dens invaginatus. An oval shape of the tooth lacking morphological characteristics of a crown or root implies that the invagination happened in the initial stages of morphodifferentiation. Spontaneous eruption of an odontoma is a rare occurrence and the occurrence of a dilated odontoma in a supernumerary tooth is even rarer with only a few case reports documented in the English literature. We present an extremely rare case of erupted dilated odontoma occurring in the supernumerary tooth in anterior maxillary region in an 18-year-old male, which, to the best of our knowledge, is the first ever case reported in English literature.

  8. Rare case of acute dengue encephalitis with correlated MRI findings

    International Nuclear Information System (INIS)

    Mathew, Rishi Philip; Basti, Ram Shenoy; Hegde, Pavan; Devdas, Jaidev M.; Khan, Habeeb Ullah; Bukelo, Mario Joseph

    2014-01-01

    Dengue encephalitis is extremely rare, with most patients showing no significant abnormality on neuroimaging (CT/MRI). We report one of the very few documented cases of dengue encephalitis, with abnormal signal intensities on all major sequences on brain MRI.

  9. Parathyroid adenoma with concurrent toxic thyroid adenoma: A rare ...

    African Journals Online (AJOL)

    recognized phenomenon. Primary hyperparathyroidism due to parathyroid adenoma in association with thyroid adenoma is extremely rare. These cases can present a diagnostic and therapeutic challenge to the treating physician as the patient may ...

  10. Mining with Rare Cases

    Science.gov (United States)

    Weiss, Gary M.

    Rare cases are often the most interesting cases. For example, in medical diagnosis one is typically interested in identifying relatively rare diseases, such as cancer, rather than more frequently occurring ones, such as the common cold. In this chapter we discuss the role of rare cases in Data Mining. Specific problems associated with mining rare cases are discussed, followed by a description of methods for addressing these problems.

  11. Possible neuro-Sweet disease mimicking brain tumor in the medulla oblongata--case report.

    Science.gov (United States)

    Akiba, Chihiro; Esaki, Takanori; Ando, Maya; Furuya, Tsuyoshi; Noda, Kazuyuki; Nakao, Yasuaki; Yamamoto, Takuji; Okuma, Yasuyuki; Mori, Kentaro

    2011-01-01

    A 62-year-old male presented with a rare case of possible neuro-Sweet Disease (NSD) mimicking brain tumor in the medulla oblongata, manifesting as numbness in the bilateral upper and lower extremities, gait disturbance, dysarthria, and swallowing disturbance which gradually deteriorated over 3 months. Magnetic resonance imaging showed a mass lesion in the medulla oblongata, extending to the upper cervical cord with rim enhancement by gadolinium. The preoperative diagnosis was brain tumor, such as glioma, or inflammatory disease. His neurological symptoms gradually deteriorated, so biopsy was performed through the midline suboccipital approach. Histological examination showed infiltration of inflammatory cells, mainly lymphocytes and macrophages. Human leukocyte antigen typing showed Cw1 and B54 which strongly suggested possible NSD. Steroid pulse therapy was started after surgery and the clinical symptoms improved. Neurosurgeons should be aware of inflammatory disorders such as NSD mimicking brain tumor.

  12. [A Case of an Abdominal Desmoplastic Small Round Cell Tumor with Metastasis in the Medulla Oblongata].

    Science.gov (United States)

    Azami, Ayaka; Takano, Yoshinao; Honda, Michitaka; Todate, Yukitoshi; Tada, Takeshi; Waragai, Mitsuru; Fukushima, Daizo; Suzuki, Nobuyasu; Sato, Atai; Abe, Tsuyoshi; Teranishi, Yasushi; Sakuma, Hideo

    2016-11-01

    A desmoplastic small round cell tumor(DSRCT)is a very rare malignant tumor that mainly occurs in the intra-abdominal cavity in young adults.This neoplasm has an extremely poor prognosis, with a clinical course characterized by rapid progression and metastasis.We present a 31-year-old man who presented with chief complaints of dysphagia, ataxic gait, and hoarseness.He first underwent surgical resection of a tumor in the medulla oblongata; however, the lesion was suspected to be a metastatic neoplasm.Following a thorough medical examination, the patient was diagnosed with retroperitoneal DSRCT with multiple metastatic lesions.He received multidisciplinary treatment including debulking surgery for the primary lesion; radiotherapy for metastatic lesions in the brain, abdomen, and cervical lymph nodes; hepatic artery embolization for liver metastasis; and systemic chemotherapy.The patient died of progressive disease 17 months after the initial diagnosis.

  13. Malignant peripheral nerve sheath tumor of the tongue with an unusual pattern of recurrence

    Directory of Open Access Journals (Sweden)

    Soumyajit Roy, MD

    2017-06-01

    Full Text Available Malignant peripheral nerve sheath tumor (MPNST of oral cavity is an extremely uncommon malignancy. Less than 15 cases have been reported since 1973 though none of them describes a distant metastasis. We present a rare case of MPNST of the tongue who presented with features of hypoglossal nerve palsy. Incisional biopsy showed a malignant spindle cell tumor in the sub-epithelial connective tissue. The tumor cells were immune-positive for S-100. He underwent surgery followed by adjuvant chemo-radiation. Later the disease recurred in the form of isolated pelvic bone metastasis. Palliative chemotherapy was offered to him. With this case report we intend to refer to such unusual presentation and pattern of recurrence in a MPNST of tongue.

  14. Current diagnostic approach of bone tumors in childhood; Abordagem diagnostica atual dos tumores osseos na infancia

    Energy Technology Data Exchange (ETDEWEB)

    Torre, Marcia Barbosa; Scatigno Neto, Andre [Sao Paulo Univ., SP (Brazil). Faculdade de Medicina. Hospital das Clinicas

    1995-09-01

    The authors analyze the magnetic resonance imaging (MRI) as the imaging modality of choice for evaluation of patients with bone tumors or soft tissue tumors. The advent of such a sensitive imaging modality is fortuitous and coincides with a recent change in the therapeutic approach to primary bone tumors. MRI is extremely valuable in monitoring the tumor response to the initial chemotherapy and is accurate defining the margins of tumor, facilitating planning of limb salvage surgical procedures. (author). 5 refs., 8 figs.

  15. Extremely Preterm Birth

    Science.gov (United States)

    ... Events Advocacy For Patients About ACOG Extremely Preterm Birth Home For Patients Search FAQs Extremely Preterm Birth ... Spanish FAQ173, June 2016 PDF Format Extremely Preterm Birth Pregnancy When is a baby considered “preterm” or “ ...

  16. Benign multicystic peritoneal mesothelioma mimicking recurrence of an ovarian borderline tumor: a case report

    Directory of Open Access Journals (Sweden)

    Takemoto Shuji

    2012-05-01

    Full Text Available Abstract Introduction Benign multicystic peritoneal mesothelioma is an extremely rare tumor that occurs mainly in women in their reproductive age. Its preoperative diagnosis and adequate treatment are quite difficult to attain. Case presentation Our patient was a 23-year-old Japanese woman who had a history of right oophorectomy and left ovarian cystectomy for an ovarian tumor at 20 years of age. The left ovarian tumor had been diagnosed on histology as a mucinous borderline tumor. Two years and nine months after the initial operation, multiple cysts were found in our patient. A laparotomy was performed and her uterus, left ovary, omentum and pelvic lymph nodes were removed due to suspicion of recurrence of the borderline tumor. A histological examination, however, revealed that the cysts were not a recurrence of the borderline tumor but rather benign multicystic peritoneal mesothelioma. There were no residual lesions and our patient was followed up with ultrasonography. She remains free from recurrence nine months after treatment. Conclusion We report a case of benign multicystic peritoneal mesothelioma mimicking recurrence of an ovarian borderline tumor. Benign multicystic peritoneal mesothelioma should be suspected when a multicystic lesion is present in the pelvis as in the case presented here, especially in patients with previous abdominal surgery.

  17. CT and MR imaging features in phosphaturic mesenchymal tumor-mixed connective tissue: A case report.

    Science.gov (United States)

    Shi, Zhenshan; Deng, Yiqiong; Li, Xiumei; Li, Yueming; Cao, Dairong; Coossa, Vikash Sahadeo

    2018-04-01

    Phosphaturic mesenchymal tumor-mixed connective tissue (PMT-MCT) is rare and usually benign and slow-growing. The majority of these tumors is associated with sporadic tumor-induced osteomalacia (TIO) or rickets, affect middle-aged individuals and are located in the extremities. Previous imaging studies often focused on seeking the causative tumors of TIO, not on the radiological features of these tumors, especially magnetic resonance imaging (MRI) features. PMT-MCT remains a largely misdiagnosed, ignored or unknown entity by most radiologists and clinicians. In the present case report, a review of the known literature of PMT-MCT was conducted and the CT and MRI findings from three patient cases were described for diagnosing the small subcutaneous tumor. Typical MRI appearances of PMT-MCT were isointense relative to the muscles on T1-weighted imaging, and markedly hyperintense on T2-weighted imaging containing variably flow voids, with markedly heterogeneous/homogenous enhancement on post contrast T1-weighted fat-suppression imaging. Short time inversion recovery was demonstrated to be the optimal sequence in localizing the tumor.

  18. Rare earth sulfates

    International Nuclear Information System (INIS)

    Komissarova, L.N.; Shatskij, V.M.; Pokrovskij, A.N.; Chizhov, S.M.; Bal'kina, T.I.; Suponitskij, Yu.L.

    1986-01-01

    Results of experimental works on the study of synthesis conditions, structure and physico-chemical properties of rare earth, scandium and yttrium sulfates, have been generalized. Phase diagrams of solubility and fusibility, thermodynamic and crystallochemical characteristics, thermal stability of hydrates and anhydrous sulfates of rare earths, including normal, double (with cations of alkali and alkaline-earth metals), ternary and anion-mixed sulfates of rare earths, as well as their adducts, are considered. The state of ions of rare earths, scandium and yttrium in aqueous sulfuric acid solutions is discussed. Data on the use of rare earth sulfates are given

  19. FIBROMATOSIS (DESMOID TUMOR OF THE BREAST. Fibromatosis (tumor desmoide de mama

    Directory of Open Access Journals (Sweden)

    Zhaneta P Boceska

    2016-03-01

    Full Text Available El tumor desmoide (fibromatosis es una entidad patológica extremadamente rara que se desarrolla de la fascia muscular y la aponeusorsis. Aunque sin potencial metastático, estos tumores son localmente muy agresivos y tienden a infiltrarse en los tejidos circundantes. Nosotros presentamos un caso de tumour desmoide de mama, que tuvo apariencias clínicas sugestivas a carcinoma. La paciente, de 56 años presentó una masa palpable de mama derecho. La citología por aspiracion con aguja fina (AGF no detectó ninguna célula maligna, por lo que se hizo una escisión local conservadora. La paciente no recibió ningun tratamiento postoperatorio adicional, y continúa viva y sana en los siguientes 18 meses. Desmoid tumor (fibromatosis is extremely rare benign pathological entity that develops from muscular fasciae and aponeuroses. Although without metastatic potential, these tumors are locally very aggressive and tend to infiltrate the surrounding tissues. We present a case of a desmoid tumor of the breast that had clinical appearance suggestive of carcinoma. The patient was 56 years old female with a previous history of surgical trauma who presented with a palpable mass in the right breast. A fine needle aspiration (FNA cytology did not reveal any malignant cells, thus conservative local excision was performed. The patient did not receive any additional postoperative treatment and was alive and free of disease after 18 months of follow-up. 

  20. A case report of extrarenal Wilms' tumor

    International Nuclear Information System (INIS)

    Kim, Jong Chul; Suh, Kwang Sun

    1997-01-01

    Extrarenal Wilms' tumor is a very rare disease, and usually occurs in pediatric patients. We present a case of extrarenal retroperitoneal Wilms' tumor in a six-year old girl with a six-month history of a palpable left abdominal mass. The ultrasonographic and CT features of this tumor showed a well-defined large, inhomogeneous predominantly solid mass which was separate from the left kidney. Surgical pathology confirmed this to be an extrarenal Wilms' tumor

  1. Gastrointestinal Stromal Tumors: A Case Report

    OpenAIRE

    Sashidharan, Palankezhe; Matele, Apoorva; Matele, Usha; Al Felahi, Nowfel; Kassem, Khalid F.

    2014-01-01

    Advances in the identification of gastrointestinal stromal tumors, its molecular and immunohiostochemical basis, and its management have been a watershed in the treatment of gastrointestinal tumors. This paradigm shift occurred over the last two decades and gastrointestinal stromal tumors have now come to be understood as rare gastrointestinal tract tumors with predictable behavior and outcome, replacing the older terminologies like leiomyoma, schwannoma or leiomyosarcoma. This report present...

  2. Primary primitive neuroectodermal tumor of the orbit

    Directory of Open Access Journals (Sweden)

    Das Dipankar

    2009-01-01

    Full Text Available Primitive neuroectodermal tumor (PNET is a small round cell malignant tumor of neuroectodermal origin. Most of the PNETs occur in the central nervous system (CNS. PNETs recognized outside of CNS are diagnosed as peripheral PNET (pPNET. This tumor which expresses MIC-2 gene (CD99 seems to be least aggressive after complete tumor resection. We describe a rare case of PNET in a young girl.

  3. Are Breast Tumor Stem Cells Responsible for Metastasis and Angiogenesis?

    National Research Council Canada - National Science Library

    Pan, Quintin

    2005-01-01

    .... The current dogma of metastasis is that most primary tumor cells have low metastatic potential, but rare cells, less than one in ten million, within large primary tumors acquire metastatic capacity...

  4. Orbital lymphomatoid granulomatosis - a rare cause of proptosis

    Energy Technology Data Exchange (ETDEWEB)

    Du Toit, Jacqueline; Kilborn, Tracy [Department of Radiology, Red Cross Children' s Hospital, Rondebosch (South Africa); Eyssen, Ann van [Department of Oncology, Red Cross Children' s Hospital, Rondebosch (South Africa); Pillay, Komala [Department of Pathology, Red Cross Children' s Hospital, Rondebosch (South Africa)

    2015-07-15

    A 1-year-old girl with unilateral proptosis was found to have primary orbital lymphomatoid granulomatosis - a condition rarely occurring in children. This multisystem angiocentric, angiodestructive, lymphoproliferative disease typically involves the lungs, with ocular involvement being extremely uncommon. Our case serves to illustrate the imaging findings of this unusual condition and highlight a rare cause of proptosis. (orig.)

  5. Nodules on the Hair: A Rare Case of Mixed Piedra

    OpenAIRE

    Khatu, Swapna S; Poojary, Shital Amin; Nagpur, Niranjan G

    2013-01-01

    Piedra is a superficial fungal infection of the hair shaft characterized by nodules along the hair shaft. Black piedra affects the scalp hair more frequently than white piedra. Occurrence of both types of piedra simultaneously in a patient is extremely rare. We describe here a rare case of mixed piedra of scalp hair.

  6. Nodules on the hair: a rare case of mixed piedra.

    Science.gov (United States)

    Khatu, Swapna S; Poojary, Shital Amin; Nagpur, Niranjan G

    2013-10-01

    Piedra is a superficial fungal infection of the hair shaft characterized by nodules along the hair shaft. Black piedra affects the scalp hair more frequently than white piedra. Occurrence of both types of piedra simultaneously in a patient is extremely rare. We describe here a rare case of mixed piedra of scalp hair.

  7. Acquired ventricular septal defect: A rare sequel of blunt chest ...

    African Journals Online (AJOL)

    Ventricular septal defect (VSD) is the most common congenital cardiac lesion encountered worldwide. Only very rarely is it acquired, and causation through blunt injury in a child is extremely rare. A previously healthy 7‑year‑old boy suffered blunt chest trauma while at play. He presented 11 days later with features of acute ...

  8. Basal Cell Ameloblastoma: A Rare Histological Variant of an ...

    African Journals Online (AJOL)

    Ameloblastomas are an inscrutable group of oral tumors. Basal cell ameloblastoma is a rare variant of ameloblastoma with very few cases reported until date. The tumor is composed of more primitive cells and has less conspicuous peripheral palisading. It shows remarkable similarity to basal cell carcinoma, basal cell ...

  9. Reinfection And Bilateral Infection : Two Rare Manifestations Of Sporotrichosis

    Directory of Open Access Journals (Sweden)

    Maiti P. K

    1999-01-01

    Full Text Available The localization of sporotrichosis is determined by the site of dermal injury by means of which the infective spores are introduced into subcutanceous tissue, but the reports of reinfection or bilateral infection are extremely rare. Although the probability of such occurrence is high in endemic areas, the rare incidence may be due to acquired host defense, a subject of much speculation. Two such rare cases are presented and some reports of cross infections of the disease are reviewed.

  10. Percutaneous radiofrequency ablation for a recurrent metastasis after resection of liver metastases from an ileal clear-cell sarcoma: Long-term local tumor control.

    Science.gov (United States)

    Seo, Jung Wook

    2017-12-01

    Clear-cell sarcomas (CCSs) in the gastrointestinal tract are extremely rare and aggressive tumors. We present the first case of a CCS arising in the ileum and metastasizing to the liver; our patient was a 60-year-old man. After the resection of the CCS and the liver metastases, a new liver metastasis developed, which was treated via percutaneous radiofrequency ablation only. At the 5-year follow-up, the ablated region was stable without local tumor progression. Percutaneous radiofrequency ablation is a viable local treatment option for recurrent metastases from an ileal CCS if they are detected when small and at an early stage in follow-up studies.

  11. Congenital epulides: A rare case report

    Directory of Open Access Journals (Sweden)

    Saurabh Kumar

    2014-01-01

    Full Text Available Congenital epulis (CE or "Granular cell epulis" also previously termed as "Neumann′s tumor" is a benign growth arising from the mucosa of the gingiva, typically seen as a mass protruding from the infant′s mouth, often interfering with respiration and feeding. These tumors generally present as a single mass arising from the upper alveolus. We report a rare case of two congenital epulides arising from the maxillary and mandibular alveolus in a day old female infant, which was surgically excised, allowing for early initiation of breast feeding. The tumor cells stained negative for S100 protein hence differentiating from other granular cell tumors. The clinical presentation, differential diagnosis in regard to the various neonatal oral swellings and the management of CE has been discussed.

  12. Extremely elevated alpha-fetoprotein due to acute exacerbation of chronic hepatitis B without malignancy: a case report.

    Science.gov (United States)

    Yoon, Young-Min; Kang, Da-Yeong; Kim, Da-Young; Seo, Jun-Won; Lim, Hyun-Jong; Lee, Hee-Jeong; Park, Sang-Gon

    2016-06-01

    Alpha-fetoprotein is produced by a variety of tumors such as hepatocellular carcinoma, hepatoblastoma, and germ cell tumors of the ovary and testes. However, we present a case of significantly elevated serum alpha-fetoprotein without evidence of malignant disease in a patient who is a carrier of chronic hepatitis B. A 60-year-old Korean man presented with markedly increased alpha-fetoprotein (2350 ng/mL; normal 7 × 105 IU/mL). Our patient was diagnosed with acute exacerbation of chronic hepatitis B, and we presumed that this condition might be related to extremely elevated alpha-fetoprotein. When our patient was treated with entecavir, the serum alpha-fetoprotein level immediately decreased, in parallel with the hepatitis B virus deoxyribonucleic acid copy number. We report a rare case of extremely elevated alpha-fetoprotein due to acute exacerbation of chronic hepatitis B without any malignancy, and a decrease in this tumor marker simultaneous with a decrease in hepatitis B virus deoxyribonucleic acid copy number on entecavir treatment. This case report is important due to the rarity of the case; furthermore, it provides details of a diagnostic process for a variety of benign diseases and malignant tumors that should be considered in patients with elevated alpha-fetoprotein. Thus, we present a case report, along with a review, that will be helpful for diagnosis and treatment of patients with elevated alpha-fetoprotein.

  13. Intimal aortic sarcoma mimicking ruptured thoracoabdominal type IV aneurysm. a rare case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Dedeilias Panagiotis

    2011-12-01

    Full Text Available Abstract Primary intimal aortic sarcoma represents a very rare and highly lethal medical entity. Diagnosis is made either by embolic events caused by the tumor or by surrounding tissue symptoms such as pain. Herein we report an extremely rare case of a 51-year-old man previously operated for ascending aortic aneurysm, who presented with clinical and radiological findings suggestive of a ruptured thoracoabdominal type IV aneurysm. The patient underwent radical resection of the aorta and surrounding tissue with placement of a composite 4-branched graft. The diagnosis was made by frozen section and regular histopathologic examination of the specimen and the patient received adjuvant chemotherapy. Nine months after surgery the patient is still alive and has no signs of recurrence. We review the literature and discuss the option of postoperative chemotherapy.

  14. Rare lung cancers

    International Nuclear Information System (INIS)

    Berzinec, P.

    2013-01-01

    The RARECARE Project (Rare Cancers in the Europe) supported by the European Union defined the rare cancers by the incidence rate of less than 6/100 000. There are several variants of lung cancer which are rare according to this definition. From the clinical point of view the most interesting are the rare adenocarcinomas and large cell neuroendocrine carcinoma. There are important differences in the diagnostic probability of EGFR and ALK mutations in the mutinous and non-mucin ous adenocarcinomas, in the signet ring cell adenocarcinomas, and large cell carcinomas. The optimal chemotherapy for neuroendocrine large cell carcinomas remains undefined. There is only very limited number of clinical trials aimed on the rare lung cancers and actually none phase III trial. Rare lung cancers continue to be a challenge both for the laboratory and the clinical research. (author)

  15. Insertion sequences enrichment in extreme Red sea brine pool vent

    KAUST Repository

    Elbehery, Ali H. A.; Aziz, Ramy K.; Siam, Rania

    2016-01-01

    Mobile genetic elements are major agents of genome diversification and evolution. Limited studies addressed their characteristics, including abundance, and role in extreme habitats. One of the rare natural habitats exposed to multiple

  16. Fine needle aspiration cytology of parapharyngeal tumors

    Directory of Open Access Journals (Sweden)

    Mondal Palash

    2009-01-01

    Full Text Available Background: Parapharyngeal tumors are rare and often pose diagnostic difficulties due to their location and plethora of presentations. Objectives: The study was undertaken to study the occurrence in the population and to evaluate the exact nature by fine needle aspiration cytology (FNAC. Materials and Methods: A total of five hundred and six cases of lateral neck lesions were studied over three and half years. Of these 56 suspected parapharyngeal masses were selected by clinical and radiological methods. Cytopathology evaluation was done by fine needle aspiration cytology with computed tomography and ultrasonography guidance wherever necessary. Histopathology confirmation was available in all the cases. Results: On FNAC diagnosis could be established in 54 cases while in two cases the material was insufficient to establish a diagnosis. The tumors encountered were, pleomorphic adenoma (33, schwannoma (3, neurofibroma (11, paraganglioma (5, angiofibroma (1 and adenoid cystic carcinoma (1. Four false positives and two false negative cases were encountered. Overall sensitivity was 96%, with specificity of 99% and accuracy being 98.8%. Conclusions: With proper clinical and radiological assessment, FNAC can be extremely useful in diagnosing most of these lesions except a few which need histopathological and even immunohistochemical confirmation.

  17. Towards Rare Itemset Mining

    OpenAIRE

    Szathmary , Laszlo; Napoli , Amedeo; Valtchev , Petko

    2007-01-01

    site de la conférence : http://ictai07.ceid.upatras.gr/; International audience; We describe here a general approach for rare itemset mining. While mining literature has been almost exclusively focused on frequent itemsets, in many practical situations rare ones are of higher interest (e.g., in medical databases, rare combinations of symptoms might provide useful insights for the physicians). Based on an examination of the relevant substructures of the mining space, our approach splits the ra...

  18. Rare earth elements and permanent magnets (invited)

    Science.gov (United States)

    Dent, Peter C.

    2012-04-01

    Rare earth (RE) magnets have become virtually indispensible in a wide variety of industries such as aerospace, automotive, electronics, medical, and military. RE elements are essential ingredients in these high performance magnets based on intermetallic compounds RECo5, RE2TM17 (TM: transition metal), and RE2TM14B. Rare earth magnets are known for their superior magnetic properties—high induction, and coercive force. These properties arise due to the extremely high magnetocrystalline anisotropy made possible by unique 3d-4f interactions between transition metals and rare earths. For more than 40 years, these magnets remain the number one choice in applications that require high magnetic fields in extreme operating conditions—high demagnetization forces and high temperature. EEC produces and specializes in RECo5 and RE2TM17 type sintered magnets. Samarium and gadolinium are key RE ingredients in the powder metallurgical magnet production processes which include melting, crushing, jet milling, pressing, sintering, and heat treating. The magnetic properties and applications of these magnets will be discussed. We will also briefly discuss the past, current, and future of the permanent magnet business. Currently, over 95% of all pure rare earth oxides are sourced from China, which currently controls the market. We will provide insights regarding current and potential new magnet technologies and designer choices, which may mitigate rare earth supply chain issues now and into the future.

  19. Rare earth octacyanomolybdates(4)

    International Nuclear Information System (INIS)

    Zubritskaya, D.I.; Sergeeva, A.N.; Pisak, Yu.V.

    1980-01-01

    Optimal conditions for synthesis of rare-earth octacyanomolybdates(4) of the Ln 4 [Mo(CN) 8 ] 3 xnH 2 O composition (where Ln is a rare-earth element, other than Pr, Pm, Lu, Tb) have been worked out. The synthesis has been accomplished by neutralization with octacianomolybdic acid with rare-earth carbonates. The composition and structure of the compounds synthesized have been studied by infrared-spectroscopy. It has been established that rare-earth octacyanomolybdates(4) form three isostructural groups

  20. Surgical Management of Malignant Tumors of the Trachea: Report of Two Cases and Review of Literature

    Directory of Open Access Journals (Sweden)

    Ryuji Yasumatsu

    2012-06-01

    Full Text Available Malignant neoplasms occurring from the trachea are extremely rare. Therefore, their clinical characteristics and surgical results have not been thoroughly discussed. These tumors are often misdiagnosed and treated as bronchial asthma or chronic obstructive pulmonary disease. It is critically important to probe the cause-effect relationship between the medical presentations and the clinical diagnosis. In this report, two cases of tracheal malignancy suffering from dyspnea due to obstruction of the proximal trachea are described, and a review of the literature is presented.

  1. Sinus Tumors

    Science.gov (United States)

    ... RESOURCES Medical Societies Patient Education About this Website Font Size + - Home > CONDITIONS > Sinus Tumors Adult Sinusitis Pediatric ... and they vary greatly in location, size and type. Care for these tumors is individualized to each ...

  2. Tumors markers

    International Nuclear Information System (INIS)

    Yamaguchi-Mizumoto, N.H.

    1989-01-01

    In order to study blood and cell components alterations (named tumor markers) that may indicate the presence of a tumor, several methods are presented. Aspects as diagnostic, prognostic, therapeutic value and clinical evaluation are discussed. (M.A.C.)

  3. Wilms tumor

    Science.gov (United States)

    ... suggested. Alternative Names Nephroblastoma; Kidney tumor - Wilms Images Kidney anatomy Wilms tumor References Babaian KN, Delacroix SE, Wood CG, Jonasch E. Kidney cancer. In: Skorecki K, Chertow GM, Marsden PA, ...

  4. Odontoameloblastoma: A rare case with unusual presentation

    Directory of Open Access Journals (Sweden)

    Supreet Jain

    2016-01-01

    Full Text Available The odontoameloblastoma (OA, also known as ameloblastic odontoma, is a very rare odontogenic tumor that is similar to ameloblastoma in its locally aggressive behavior. OA includes odontogenic ectomesenchyme in addition to odontogenic epithelium that resembles an ameloblastoma both in structure and in behavior. Its clinical presentation, however, often mimics the more innocuous odontoma, and hence, the recognition of its aggressive nature is commonly only ascertained after its histopathologic diagnosis following enucleation. This paper presents a rare case of OA with unusual clinical and radiological features.

  5. Extragastrointestinal Stromal Tumor during Pregnacy

    Directory of Open Access Journals (Sweden)

    Ilay Gözükara

    2012-01-01

    Full Text Available Extragastrointestinal stromal tumors (EGISTs are mesenchymal neoplasms without connection to the gastrointestinal tract. Gastrointestinal stromal tumors (GISTs and EGIST are similar according to their clinicopathologic and histomorphologic features. Both of them most often express immunoreactivity for CD-117, a c-kit proto-oncogene protein. The coexistence of GIST and pregnancy is very rare, with only two cases reported in the literature. In this paper, we presented the first EGIST case during pregnancy in the literature.

  6. 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...

  7. A rare presentation of hydatid cyst

    International Nuclear Information System (INIS)

    Sabir, N.; Yildirim, B.; Alatas, E.; Cetin, B.

    2005-01-01

    Hydatid disease, although known to occur in most body areas, is extremely rare in the female reproductive system. There are different modes of presentation for the disease; however, we report and discuss a case presented with cystic vesicles passing through the vagina, which is considered as a rare presentation for secondary involvement of the uterus and both ovaries. We confirmed diagnosis with radiological examinations and serological tests. We operated on the patient, and studied the excised cysts microscopically. The gynecologist should be aware of hydatid cyst when vaginally passing a grape like vesicle is presented by the patient. (author)

  8. Brain Tumors

    Science.gov (United States)

    A brain tumor is a growth of abnormal cells in the tissues of the brain. Brain tumors can be benign, with no cancer cells, ... cancer cells that grow quickly. Some are primary brain tumors, which start in the brain. Others are ...

  9. Urogenital tumors

    Energy Technology Data Exchange (ETDEWEB)

    Weller, R.E.

    1994-03-01

    An overview is provided for veterinary care of urogenital tumors in companion animals, especially the dog. Neoplasms discussed include tumors of the kidney, urinary bladder, prostate, testis, ovary, vagina, vulva and the canine transmissible venereal tumor. Topics addressed include description, diagnosis and treatment.

  10. Cowden Syndrome and Concomitant Pulmonary Neuroendocrine Tumor

    DEFF Research Database (Denmark)

    Langer, Seppo W; Ringholm, Lene; Dali, Christine I

    2015-01-01

    Cowden Syndrome is a rare autosomal dominantly inherited disorder. Patients with Cowden Syndrome are at increased risk of various benign and malignant neoplasms in breast, endometrium, thyroid, gastrointestinal tract, and genitourinary system. Neuroendocrine tumors are ubiquitous neoplasms that may...

  11. Adrenocortical tumors in children

    Directory of Open Access Journals (Sweden)

    R.C. Ribeiro

    2000-10-01

    Full Text Available Childhood adrenocortical tumors (ACT are rare. In the USA, only about 25 new cases occur each year. In Southern Brazil, however, approximately 10 times that many cases are diagnosed each year. Most cases occur in the contiguous states of São Paulo and Paraná. The cause of this higher rate has not been identified. Familial genetic predisposition to cancer (p53 mutations and selected genetic syndromes (Beckwith-Wiedemann syndrome have been associated with childhood ACT in general but not with the Brazilian counterpart. Most of the affected children are young girls with classic endocrine syndromes (virilizing and/or Cushing. Levels of urinary 17-ketosteroids and plasma dehydroepiandrosterone sulfate (DHEA-S, which are abnormal in approximately 90% of the cases, provide the pivotal clue to a diagnosis of ACT. Typical imaging findings of pediatric ACT consist of a large, well-defined suprarenal tumor containing calcifications with a thin capsule and central necrosis or hemorrhage. The pathologic classification of pediatric ACT is troublesome. Even an experienced pathologist can find it difficult to differentiate carcinoma from adenoma. Surgery is the single most important procedure in the successful treatment of ACT. The role of chemotherapy in the management of childhood ACT has not been established although occasional tumors are responsive to mitotane or cisplatin-containing regimens. Because of the heterogeneity and rarity of the disease, prognostic factors have been difficult to establish in pediatric ACT. Patients with incomplete tumor resection or with metastatic disease at diagnosis have a dismal prognosis. In patients with localized and completely resected tumors, the size of the tumor has predictive value. Patients with large tumors have a much higher relapse rate than those with small tumors.

  12. Primary malignant melanoma of the female urethra: Report of a rare neoplasm of the urinary tract

    Directory of Open Access Journals (Sweden)

    Namita Bhutani

    Full Text Available Introduction: Melanoma is a malignant tumor that can affect any area of the anatomical economy. Its occurance in the female urethra is extremely rare. We report a case of primary malignant urethral melanoma developed in an elderly female patient. Presentation of case: A 70 years old female presented with dysuria, poor stream, gross haematuria, intermittent blood spots, and a painful mass. On physical examination, there were no suspicious lesions on the skin. On external genital examination, a lesion at the level of the urethral meatus was observed. The mass was removed by wide local excision under spinal anaesthesia. The pathological diagnosis was malignant melanoma of the urethra. Discussion: The common presentations include bleeding and/or discharge per urethra, voiding dysfunction and the presence of tumor mass. Survival depends on the stage, location and size of the neoplasm at the time of diagnosis. Despite major surgery, radiotherapy or immunotherapy; malignant melanoma usually has a poor prognosis. Conclusion: Melanoma of the female urethra is an extremely uncommon pathology leading to paucity of literature and any definite recommendations regarding management. The histological and immunohistochemical findings can be helpful in making an early and accurate diagnosis of malignant melanoma in the urogenital region. Keywords: Case report, Female urethral cancer, Immunohistochemistry, Malignant melanoma, Urethral neoplasm

  13. WHY ARE PULSAR PLANETS RARE?

    Energy Technology Data Exchange (ETDEWEB)

    Martin, Rebecca G.; Livio, Mario; Palaniswamy, Divya [Department of Physics and Astronomy, University of Nevada, Las Vegas, 4505 South Maryland Parkway, Las Vegas, NV 89154 (United States)

    2016-12-01

    Pulsar timing observations have revealed planets around only a few pulsars. We suggest that the rarity of these planets is due mainly to two effects. First, we show that the most likely formation mechanism requires the destruction of a companion star. Only pulsars with a suitable companion (with an extreme mass ratio) are able to form planets. Second, while a dead zone (a region of low turbulence) in the disk is generally thought to be essential for planet formation, it is most probably rare in disks around pulsars, because of the irradiation from the pulsar. The irradiation strongly heats the inner parts of the disk, thus pushing the inner boundary of the dead zone out. We suggest that the rarity of pulsar planets can be explained by the low probability for these two requirements to be satisfied: a very low-mass companion and a dead zone.

  14. Intracerebral hemorrhage in brain tumors

    International Nuclear Information System (INIS)

    Fujita, Katsuzo; Matsumoto, Satoshi

    1980-01-01

    A series of 16 cases of intracerebral hemorrhage associated with brain tumors are described. The literature is reviewed and the incidence of these cases is reported to be low, but we had clinically encountered these cases more commonly than reported, since CT was introduced to the neurosurgical field as a diagnostic aid. The presenting symptoms were those of spontaneous intracerebral hemorrhage or brain tumor. The intracerebral hemorrhage associated with brain tumor may mask the cause of bleeding and confuse the diagnosis. The majority of the tumor causing the intracerebral hemorrhage are highly malignant as glioblastoma or metastatic brain tumor, but there are some benign tumors such as pituitary adenoma, hemangioblastoma, benign astrocytoma and meningioma, which would have good survival rates if discovered early. The mechanisms of massive hemorrhage with brain tumor are not clear. From pathological findings of our cases and other reports, the mechanism seems to be due to the vascular endothelial proliferation with subsequent obliteration of the lumen of the vessel. Thin walled, poorly formed vessels in tumor may also become distorted with growth of the tumor and these may easily rupture and bleed. Necrosis with subsequent loss of vessel support may be a factor in production of hemorrhage. Radiation therapy may be a predisposing factor. Children are rarely involved in these cases. The prognosis in the majority of cases would seen to be poor, since the majority of the tumor are highly malignant and most such patients are seen by the neurosurgeon some time after the hemorrhage has accomplished its fatal mischief. (author)

  15. Intracerebral hemorrhage in brain tumors

    Energy Technology Data Exchange (ETDEWEB)

    Fujita, K; Matsumoto, S [Kobe Univ. (Japan). School of Medicine

    1980-10-01

    A series of 16 cases of intracerebral hemorrhage associated with brain tumors are described. The literature is reviewed and the incidence of these cases is reported to be low, but we had clinically encountered these cases more commonly than reported, since CT was introduced to the neurosurgical field as a diagnostic aid. The presenting symptoms were those of spontaneous intracerebral hemorrhage or brain tumor. The intracerebral hemorrhage associated with brain tumor may mask the cause of bleeding and confuse the diagnosis. The majority of the tumor causing the intracerebral hemorrhage are highly malignant as glioblastoma or metastatic brain tumor, but there are some benign tumors such as pituitary adenoma, hemangioblastoma, benign astrocytoma and meningioma, which would have good survival rates if discovered early. The mechanisms of massive hemorrhage with brain tumor are not clear. From pathological findings of our cases and other reports, the mechanism seems to be due to the vascular endothelial proliferation with subsequent obliteration of the lumen of the vessel. Thin walled, poorly formed vessels in tumor may also become distorted with growth of the tumor and these may easily rupture and bleed. Necrosis with subsequent loss of vessel support may be a factor in production of hemorrhage. Radiation therapy may be a predisposing factor. Children are rarely involved in these cases. The prognosis in the majority of cases would seen to be poor, since the majority of the tumor are highly malignant and most such patients are seen by the neurosurgeon some time after the hemorrhage has accomplished its fatal mischief.

  16. Breast abscess as the initial manifestation of primary pure squamous cell carcinoma: a rare presentation and literature review.

    Science.gov (United States)

    Salemis, Nikolaos S

    2011-01-01

    Primary squamous cell carcinoma of the breast is a very rare tumor accounting for less than 0.4% of all breast cancers. Fewer than 100 cases have been reported in the literature so far. The diagnosis requires strict pathologic criteria to be fulfilled. Due to the rarity of this tumor the optimal treatment and prognosis are both unclear. Breast abscess as the initial presentation of a primary squamous cell breast carcinoma is an extremely rare clinical entity. In this study, we describe a case of a 61-year-old postmenopausal woman who presented with typical manifestations of a breast abscess and was diagnosed with a pure primary squamous cell breast carcinoma. Diagnostic evaluation and management of the patient are discussed along with a review of the literature. Despite its rarity, the possibility of a primary pure squamous cell breast carcinoma should always be considered in the differential diagnosis in postmenopausal patients presenting with manifestations of a breast abscess, especially in those who respond poorly to the initial treatment. Physicians should be aware of this rare malignancy in order to avoid delays in diagnosis and treatment.

  17. Paraneoplastic Cushing Syndrome Due To Wilm's Tumor.

    Science.gov (United States)

    Faizan, Mahwish; Manzoor, Jaida; Saleem, Muhammad; Anwar, Saadia; Mehmood, Qaiser; Hameed, Ambreen; Ali, Agha Shabbir

    2017-05-01

    Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to neoplasm. Paraneoplastic syndromes may be the first or the most prominent manifestations of cancer. Wilm's tumor is the most frequent pediatric renal malignancy and usually presents with abdominal mass. Unusual presentations like acquired von Willebrand disease, sudden death due to pulmonary embolism and Cushing syndrome have been described in the literature. Cushing syndrome, as the presenting symptom of a malignant renal tumor in children, is a very rare entity. Few case reports are available in the literature exploring the option of preoperative chemotherapy as well as upfront nephrectomy. We report a rare case of paraneoplastic Cushing syndrome due to a Wilm's tumor. Based on gradual decrease of postoperative weight, blood pressure, serum adrenocorticotropic hormone, and plasma cortisol levels, along with histological confirmation of Wilm's tumor, paraneoplastic Cushing syndrome due to Wilm's tumor was confirmed.

  18. Paraneoplastic cushing syndrome due to wilm's tumor

    International Nuclear Information System (INIS)

    Faizan, M.; Anwar, S.; Hameed, A.; Manzoor, J.; Saleem, M.; Mehmood, Q.; Ali, A. S.

    2017-01-01

    Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to neoplasm. Paraneoplastic syndromes may be the first or the most prominent manifestations of cancer. Wilm's tumor is the most frequent pediatric renal malignancy and usually presents with abdominal mass. Unusual presentations like acquired von Willebrand disease, sudden death due to pulmonary embolism and Cushing syndrome have been described in the literature. Cushing syndrome, as the presenting symptom of a malignant renal tumor in children, is a very rare entity. Few case reports are available in the literature exploring the option of preoperative chemotherapy as well as upfront nephrectomy. We report a rare case of paraneoplastic Cushing syndrome due to a Wilm's tumor. Based on gradual decrease of postoperative weight, blood pressure, serum adrenocorticotropic hormone, and plasma cortisol levels, alongwith histological confirmation of Wilm's tumor, paraneoplastic Cushing syndrome due to Wilm's tumor was confirmed. (author)

  19. Solitary Fibrous Tumor Arising from Stomach: CT Findings

    Science.gov (United States)

    Park, Sung Hee; Kwon, Jieun; Park, Jong-pil; Park, Mi-Suk; Lim, Joon Seok; Kim, Joo Hee; Kim, Ki Whang

    2007-01-01

    Solitary fibrous tumors are spindle-cell neoplasms that usually develop in the pleura and peritoneum, and rarely arise in the stomach. To our knowledge, there is only one case reporting a solitary fibrous tumor arising from stomach in the English literature. Here we report the case of a 26-year-old man with a large solitary fibrous tumor arising from the stomach which involved the submucosa and muscular layer and resembled a gastrointestinal stromal tumor in the stomach, based on what was seen during abdominal computed tomography. A solitary fibrous tumor arising from the stomach, although rare, could be considered as a diagnostic possibility for gastric submucosal tumors. PMID:18159603

  20. Tumor immunology

    International Nuclear Information System (INIS)

    Otter, W. den

    1987-01-01

    Tumor immunology, the use of immunological techniques for tumor diagnosis and approaches to immunotherapy of cancer are topics covered in this multi-author volume. Part A, 'Tumor Immunology', deals with present views on tumor-associated antigens, the initiation of immune reactions of tumor cells, effector cell killing, tumor cells and suppression of antitumor immunity, and one chapter dealing with the application of mathematical models in tumor immunology. Part B, 'Tumor Diagnosis and Imaging', concerns the use of markers to locate the tumor in vivo, for the histological diagnosis, and for the monitoring of tumor growth. In Part C, 'Immunotherapy', various experimental approaches to immunotherapy are described, such as the use of monoclonal antibodies to target drugs, the use of interleukin-2 and the use of drugs inhibiting suppression. In the final section, the evaluation, a pathologist and a clinician evaluate the possibilities and limitations of tumor immunology and the extent to which it is useful for diagnosis and therapy. refs.; figs.; tabs

  1. Primary Ewing's sarcoma-primitive neuroectodermal tumor of the uterus: a case report and literature review.

    Science.gov (United States)

    Park, Jeong-Yeol; Lee, Sun; Kang, Hyoung Jin; Kim, Hy-Sook; Park, Sang-Yoon

    2007-08-01

    Primary Ewing's sarcoma-primitive neuroectodermal tumor (ES-PNET) of the uterus is an extremely rare malignancy. A 30-year-old Korean woman presented with abnormal uterine bleeding with uterine enlargement. A computed tomography (CT) scan and magnetic resonance imaging (MRI) of the abdomen and pelvis showed a huge uterine mass measuring 18 x 20 x 21 cm, metastasis to both pelvic and para-aortic lymph nodes, and omental infiltration. The pathology report of the uterine mass described a uniformly hypercellular tumor, which was arranged in diffuse solid sheets of uniform, small, rounded, and sometimes spindle-shaped cells, with scanty cytoplasm. Immunohistochemically, the mass tested positive for vimentin, CD99, and chromogranin. The patient received several courses of combination chemotherapy and radiotherapy but died from tumor progression 16 months after the initial diagnosis. This is a rare case of primary uterine ES-PNET in a woman of reproductive age. A review of the literature indicates that primary uterine ES-PNET requires early diagnosis and multimodality treatment including surgery, chemotherapy, and radiotherapy. The behavior of this tumor is potentially aggressive.

  2. Adult primary hypoparathyroidism: A rare presentation

    OpenAIRE

    Chakrabarty, Ashima Datey

    2013-01-01

    The common causes of stridor in adults are abscesses or swelling of upper airway, tumors, paralysis or malfunction of vocal cords. Laryngospasm due to hypocalcemia is a rare cause of stridor in adults, although occasionally reported in the neonates. We report an elderly lady having stridor and laryngospasm, secondary to acquired hypoparathyroidism and secondary hypocalcemia, without risk factors for hypoparathyroidism such as recent neck surgery or irradiation. We did an extensive review of l...

  3. Primary periosteal lymphoma - rare and unusual

    Energy Technology Data Exchange (ETDEWEB)

    Abdelwahab, Ibrahim F. [Coney Island Hospital, Department of Radiology, New York, NY (United States); Hoch, Benjamin [Mount Sinai School of Medicine, CUNY, Department of Pathology, New York, NY (United States); Hermann, George [Mount Sinai School of Medicine, CUNY, Department of Radiology, New York, NY (United States); Bianchi, Stefano [Clinique et Fondation des Grangettes, Geneva (Switzerland); Klein, Michael J. [UAB School of Medicine, Department of Pathology, Birmingham, AL (United States); Springfield, Dempsey S. [Mount Sinai School of Medicine, CUNY, Department of Orthopedics, New York, NY (United States)

    2007-04-15

    We describe a primary periosteal lymphoma that involved only the periosteum without affecting the adjacent medulla or the regional lymph nodes. No other lymphomatous foci were found in either the distant lymph nodes or viscera. This unusual presentation simulates the imaging appearance of surface lesions of bone, namely benign and malignant tumors, and departs from the typical appearance of primary lymphoma of bone. Therefore, this rare type of lymphoma should be considered in the differential diagnosis of surface bone lesions. (orig.)

  4. Variable ultrasonography findings of extremity lymphangioma: Pathologic correlation

    International Nuclear Information System (INIS)

    Oh, Jong Young; Nam, Kyung Jin; Lee, Ki Nam; Kim, Chan Sung; Lee, Jin Hwa; Kim, Dae Chul

    2002-01-01

    The great majority of lymphangiomas occur in the neck (75%) and axilla (20%), but extremity lymphangioma is rare. We correlate variable sonographic features of extremity lymphangioma with pathologic findings. We reviewed the sonographic findings of extremity lymphangioma in 14 patients (M:F=8:6). The all cases were histologically confirmed by operation. The variable sonographic features of extremity lymphangioma were compared to pathologic findings. The multilocular cystic mass with ill defined boundaries was distinctive sonographic appearance of extremity lymphangioma. But there were variable sonographic findings such as heterogeneous echogenic mass or homogeneous echogenic portion. The histologic section of echogenic lesion reveals clusters of abnormal

  5. Overview of rare B-decays

    CERN Document Server

    Prisciandaro, Jessica

    2017-01-01

    Being extremely suppressed in the Standard Model, rare decays of heavy-flavoured particles are a powerful probe of New Physics, and allow to reach energies beyond those accessible through direct searches. Several new results have been obtained by the LHC experiments. In particular, $b \\to sl^+l^-$ transitions give access to a large spectrum of observables, which provide complementary information on possible New Physics contributions. In this sector, tensions with Standard Model predictions have been observed.

  6. Attribution of extreme rainfall from Hurricane Harvey, August 2017

    NARCIS (Netherlands)

    Van Oldenborgh, Geert Jan; Van Der Wiel, Karin; Sebastian, A.G.; Singh, Roop; Arrighi, Julie; Otto, Friederike; Haustein, Karsten; Li, Sihan; Vecchi, Gabriel; Cullen, Heidi

    2017-01-01

    During August 25-30, 2017, Hurricane Harvey stalled over Texas and caused extreme precipitation, particularly over Houston and the surrounding area on August 26-28. This resulted in extensive flooding with over 80 fatalities and large economic costs. It was an extremely rare event: the return

  7. Ergotamine-induced upper extremity ischemia: a case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Man Deuk; Lee, Gun [Bundang CHA General Hospital, Pochon (China); Shin, Sung Wook [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2005-06-15

    Ergotamine-induced limb ischemia is an extremely rare case. We present a case of a 64-year-old man, who developed ischemia on the right upper extremity due to long-term use of Ergot for migraine headache. Angiography revealed diffused, smooth, and tapered narrowing of the brachial artery. The patient was successfully treated with intravenous nitroprusside.

  8. Seasonal Climate Extremes : Mechanism, Predictability and Responses to Global Warming

    NARCIS (Netherlands)

    Shongwe, M.E.

    2010-01-01

    Climate extremes are rarely occurring natural phenomena in the climate system. They often pose one of the greatest environmental threats to human and natural systems. Statistical methods are commonly used to investigate characteristics of climate extremes. The fitted statistical properties are often

  9. Karcinoide tumorer og gobletcelle-karcinoide tumorer i appendix

    DEFF Research Database (Denmark)

    Knigge, Ulrich; Hansen, Carsten Palnaes

    2010-01-01

    Appendiceial carcinoid tumors (CAT) and goblet cell carcinoids (GCCAT) are rare. Most are asymptomatic and found after appendectomy. Metastases to regional nodes are seen in 10% of CATs larger than two cm. Ovarian or peritoneal metastases are seen in 20% of all GCCATs. Further surgical treatments...

  10. A Rare Location of Angiofibroma in the Inferior Turbinate in Young Woman

    Directory of Open Access Journals (Sweden)

    Salimov, Asif

    2015-01-01

    Full Text Available Introduction Juvenile nasopharyngeal angiofibroma is a rare benign neoplasm in the nasopharynx. The tumor tends to be locally aggressive and is typically seen in adolescent boys. Extranasopharyngeal angiofibromas have been reported sporadically in the literature. They most commonly originate from the maxillary sinus. Objectives A 26-year-old woman was referred to our clinic with intermittent epistaxis from the right nasal passage for the previous 2 months. Maxillofacial magnetic resonance imaging showed a lobular, contoured mass originating from the right inferior turbinate and hanging in the right nasal cavity, with dense contrast enhancement denoting hypervascularity. Resumed Report Vascular feeding of the mass was seen from the right internal maxillary artery with angiography, and this branch was embolized. On the following day, the patient underwent transnasal endoscopic excision of the mass. An approximately 3-cm-diameter mass was excised by partial turbinectomy, and the posterior edge of the remaining turbinate was cauterized. Conclusion Extranasopharyngeal angiofibromas are rarely seen, and the inferior turbinate is an extremely rare location for them. This young woman is the first case reported in the English literature of angiofibroma originating from the inferior turbinate. We should consider these neoplasms can be found in female, nonadolescent patients with extranasopharyngeal localization, and we should not perform biopsy because of its massive bleeding.

  11. A rare complication of ESWL: Focal metastatic multiple organ abscesses in a horseshoe kidney

    Directory of Open Access Journals (Sweden)

    Ugur Kuyumcuoglu

    2014-06-01

    Full Text Available Extracorporeal shock wave lithotripsy (ESWL is an effective treatment modality in the minimal invasive management of urinary system stone disease. Although the majority of the complications occuring after ESWL are minor (most common ones are gross haematuria, pain, perinephritic hematoma; bacteriuria may also occur in some cases which sometimes can lead to sepsis and even metastatic abscess formation in a very rare part of the cases treated. In this rare situation infection agent spreads quickly via hematogenous route and causes abscess formation in different parts of the body. Majority of such cases usually have an underlying systemic disease like diabetes mellitus (DM, malignancy, HIV or steroid use which lead to disruption of immune system functions. Abscess formation following ESWL is extremely rare and usually limited with some case reports published in the literature. Herein, we present a diabetic case with formation of multiple abscess foci in kidney, as well as in lungs and liver following ESWL. The patient was first admitted to our emergency department with high fever and respiratory distress and misdiagnosed as metastatic tumor foci based on radiologic findings. To the best of our knowledge, our case is the first one in the literature in whom simultanous abscess formation in multiple organ systems has been documented following an otherwise uneventful ESWL.

  12. A rare complication of ESWL: focal metastatic multiple organ abscesses in a horseshoe kidney.

    Science.gov (United States)

    Kuyumcuoglu, Ugur; Eryildirim, Bilal; Tuncer, Murat; Faydaci, Gokhan; Aktoz, Tevfik; Akdere, Hakan; Sarıca, Kemal

    2014-06-30

    Extracorporeal shock wave lithotripsy (ESWL) is an effective treatment modality in the minimal invasive management of urinary system stone disease. Although the majority of the complications occuring after ESWL are minor (most common ones are gross haematuria, pain, perinephritic hematoma); bacteriuria may also occur in some cases which sometimes can lead to sepsis and even metastatic abscess formation in a very rare part of the cases treated. In this rare situation infection agent spreads quickly via hematogenous route and causes abscess formation in different parts of the body. Majority of such cases usually have an underlying systemic disease like diabetes mellitus (DM), malignancy, HIV or steroid use which lead to disruption of immune system functions. Abscess formation following ESWL is extremely rare and usually limited with some case reports published in the literature. Herein, we present a diabetic case with formation of multiple abscess foci in kidney, as well as in lungs and liver following ESWL. The patient was first admitted to our emergency department with high fever and respiratory distress and misdiagnosed as metastatic tumor foci based on radiologic findings. To the best of our knowledge, our case is the first one in the literature in whom simultanous abscess formation in multiple organ systems has been documented following an otherwise uneventful ESWL.

  13. Perforating pilomatrixoma showing atypical presentation: A rare clinical variant

    Directory of Open Access Journals (Sweden)

    Nevra Seyhan

    2018-03-01

    Full Text Available Pilomatrixoma, also known as calcifying epithelioma of Malherbe, is a rare benign skin tumor arising from hair follicle stem cells. The most common localization is the head and neck region. Female/male ratio is 3/2. It shows deep subcutaneous placement and occurs in the first two decades of life. Its diameter ranges from 0.5 cm to 3 cm. Multiple lesions are rarely seen. Histopathologically it is characterized by basoloid and ghost cells. Perforating type is a rare clinical variant. Treatment is surgical excision. Our case is presented to draw attention to a rare clinical variant of pilomatrixioma.

  14. Endoscopic-modified medial maxillectomy and its limitation for a solitary fibrous tumor of the lacrimal sac and nasolacrimal duct.

    Science.gov (United States)

    Moriyama, Munehito; Kodama, Satoru; Hirano, Takashi; Suzuki, Masashi

    2017-06-01

    Solitary fibrous tumor (SFT) is an uncommon neoplasm that usually arises from the pleura. Recently, SFTs have been reported in the head and neck region located in subsites such as the orbit. SFTs of the lacrimal sac are extremely rare, with only six cases reported in the English literature. We describe a SFT arising from the right lacrimal sac and extending along the nasolacrimal duct into the nasal cavity. Although, the tumor could not be removed by endoscopic-modified medial maxillectomy (EMMM) alone, combined approach with EMMM and a small external incision achieved the complete removal of the tumor. The patient has remained disease-free 24 months after surgery. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  15. Pleural localized malignant mesothelioma mimicking a benign solitary fibrous tumor of the pleura on chest computed tomography: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Hwi Ryong; Chong, Se Min; Kim, Mi Kyung [Dept. of Radiology, (Korea, Republic of)

    2017-06-15

    Pleural malignant mesotheliomas arise from mesothelial cells in the pleura. They are characterized as diffuse or localized malignant mesotheliomas (LMM). Diffuse malignant mesotheliomas spread diffusely along pleural surfaces, while LMM are well-circumscribed nodular lesions with no gross or microscopic diffuse pleural spreading. Therefore, LMM can be radiologically confused with solitary fibrous tumors of the pleura (SFTP), which commonly presents as a solitary, well-demarcated peripheral mass abutting the pleural surface upon the completion of a computed tomography (CT). Therefore, this study reports on a 63-year-old female patient with a pathologically-proven LMM of the pleura, mimicking a benign SFTP upon having a chest CT. Although LMM is extremely rare, FDG PET/CT should be recommended for adequate tumor management in order to avoid misdiagnosing the tumor as a benign SFTP when an interfissural or pleural-based mass is seen on the chest CT.

  16. Pleural localized malignant mesothelioma mimicking a benign solitary fibrous tumor of the pleura on chest computed tomography: A case report

    International Nuclear Information System (INIS)

    Park, Hwi Ryong; Chong, Se Min; Kim, Mi Kyung

    2017-01-01

    Pleural malignant mesotheliomas arise from mesothelial cells in the pleura. They are characterized as diffuse or localized malignant mesotheliomas (LMM). Diffuse malignant mesotheliomas spread diffusely along pleural surfaces, while LMM are well-circumscribed nodular lesions with no gross or microscopic diffuse pleural spreading. Therefore, LMM can be radiologically confused with solitary fibrous tumors of the pleura (SFTP), which commonly presents as a solitary, well-demarcated peripheral mass abutting the pleural surface upon the completion of a computed tomography (CT). Therefore, this study reports on a 63-year-old female patient with a pathologically-proven LMM of the pleura, mimicking a benign SFTP upon having a chest CT. Although LMM is extremely rare, FDG PET/CT should be recommended for adequate tumor management in order to avoid misdiagnosing the tumor as a benign SFTP when an interfissural or pleural-based mass is seen on the chest CT

  17. [Primitive neuroectodermal tumor/Ewing's sarcoma of the penis in children: a case report and review of the literature].

    Science.gov (United States)

    Zhang, Da-Wei; Jin, Mei; Zhou, Chun-Ju; Song, Hong-Cheng; Ma, Xiao-Li

    2012-12-01

    To investigate the clinical manifestations, pathological characteristics and treatment of primitive neuroectodermal tumor/Ewing's sarcoma (PNET/EWS) of the penis in children. We analyzed the clinical data of a case of PNET/EWS and reviewed relevant literature. The patient was a 5-year-old boy, admitted for penis swelling with pain for 11 months. Biopsy showed a small round cell tumor, CD99 positive by immunohistochemical staining, with EWS translocation by fluorescence in situ hybridization on molecular biological examination. The tumor was confirmed to be PNET/EWS of the penis, and disappeared after 45 weeks of chemotherapy and local radiotherapy. PNET/EWS of the penis is an extremely rare disease, with no specific clinical symptoms except penis enlargement with pain. Immunohistochemistry and molecular biological examination contribute to its diagnosis.

  18. Rare Disease Video Portal

    OpenAIRE

    Sánchez Bocanegra, Carlos Luis

    2011-01-01

    Rare Disease Video Portal (RD Video) is a portal web where contains videos from Youtube including all details from 12 channels of Youtube. Rare Disease Video Portal (RD Video) es un portal web que contiene los vídeos de Youtube incluyendo todos los detalles de 12 canales de Youtube. Rare Disease Video Portal (RD Video) és un portal web que conté els vídeos de Youtube i que inclou tots els detalls de 12 Canals de Youtube.

  19. Rare earth germanates

    International Nuclear Information System (INIS)

    Bondar', I.A.; Vinogradova, N.V.; Dem'yanets, L.N.

    1983-01-01

    From the viewpoint of structural chemistry and general regularities controlling formation reactions of compounds and phases in melts, solid and gaseous states, recent achievements in the chemistry of rare earth germanates are generalized. Methods of synthesizing germanates, systems on the base of germanium oxides and rare earths are considered. The data on crystallochemical characteristics are tabulated. Individual compounds of scandium germanate are also characterized. Processes of germanate formation using the data of IR-spectroscopy, X-ray phase analysis are studied. The structure and morphotropic series of rare earth germanates and silicates are determined. Fields of their present and possible future application are considered

  20. Extreme environment electronics

    CERN Document Server

    Cressler, John D

    2012-01-01

    Unfriendly to conventional electronic devices, circuits, and systems, extreme environments represent a serious challenge to designers and mission architects. The first truly comprehensive guide to this specialized field, Extreme Environment Electronics explains the essential aspects of designing and using devices, circuits, and electronic systems intended to operate in extreme environments, including across wide temperature ranges and in radiation-intense scenarios such as space. The Definitive Guide to Extreme Environment Electronics Featuring contributions by some of the world's foremost exp