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Sample records for extremely rare tumor

  1. Extremely rare borderline phyllodes tumor in the male breast: a case report.

    Science.gov (United States)

    Kim, Jung Gyu; Kim, Shin Young; Jung, Hae Yoen; Lee, Deuk Young; Lee, Jong Eun

    2015-01-01

    Phyllodes tumor of the male breast is an extremely rare disease, and far fewer cases of borderline phyllodes tumors than benign or malignant tumors in the male breast have been reported. We report a case of borderline phyllodes tumor in the male breast with imaging findings of the tumor and pathologic correlation.

  2. 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.

  3. 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.

  4. Metastases of pancreatic neuroendocrine tumor to the liver as extremely rare indication for liver transplantation in children. Case report and review of the literature.

    Science.gov (United States)

    Ismail, Hor; Broniszczak, Dorota; Markiewicz-Kijewska, Małgorzata; Ciopiński, Mateusz; Teisseyre, Joanna; Kluge, Przemysław; Dembowska-Bagińska, Bożenna; Kościesza, Andrzej; Socha, Piotr; Kaliciński, Piotr

    2016-09-01

    Neuroendocrine tumors (NET) are extremely rare in children (0.75 cases per 100,000 children and adolescents a year) and the majority of these tumors are benign or present low grade of malignancy. According to the American registry Surveillance, Epidemiology, and End Results (SEER) Program of the National Cancer Institute, less than 2% of all neuroendocrine tumors in children occur in the pancreas, making it a rare site for these tumors. The majority of them are found in children over 10years of age, especially those with malignant potential. Treatment of NET consists of different methods: surgery, somatostatin analogues and chemotherapy. Radical surgical resection remains the standard of treatment; however, it is not always feasible because of distant metastases. The authors present a case report of pancreatic NET with multiple metastases to the liver. The patient was treated with pancreatic resection and liver transplantation for liver metastases. Prior to liver transplantation, the patient was treated with somatostatin analogues, sunitinib and chemotherapy. Management of liver metastases with liver transplantation is discussed.

  5. Bronchial carcinoid tumors: A rare malignant tumor

    African Journals Online (AJOL)

    2015-02-03

    Feb 3, 2015 ... Key words: Bronchial carcinoid tumor, diagnosis, outcome, treatment, West .... They can present with carcinoid syndrome, Cushing's ... 2012 reported BCT as a rare differential of pulmonary ... Management of carcinoid tumors.

  6. Endolymphatic sac tumor : a rare cerebellopontine angle tumor.

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    Joseph B

    2002-10-01

    Full Text Available Endolymphatic sac tumors (ELST are rare papillary tumors of the temporal bone. Previously named as aggressive papillary middle ear tumors, they have recently been shown to arise from the endolymphatic sac. They are a rare in cerebello-pontine angle (CPA. We present a case of an ELST who presented as a CPA tumor with hydrocephalus. He underwent a ventriculo-peritoneal shunt initially. On exploration of the CP angle, the tumor was found to be extremely vascular. He was re-explored following embolization, and a subtotal excision of the tumor was done. Extensive petrous bone infiltration and vascularity of the tumor makes total excision almost impossible with high risk of cranial nerve deficits, excessive blood loss and CSF leak. This tumor should be considered in the differential diagnosis of vascular CPA tumors which erode the petrous temporal bone. The relevant literature is reviewed.

  7. A Rare Tumor of the Orbit: Angiomyxoma

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    Adem Türk

    2014-12-01

    Full Text Available Angiomyxoma, which is a rare mesenchymal tumor, has a propensity for occurring in the trunk, head and neck, extremities, and the genital region. Development of the tumor in the orbit is extremely rare, and only a few studies have been reported to date. In this study, we present a 26-year-old female patient who was admitted with a complaint of growing mass at her left upper eyelid during the previous 9 months. Magnetic resonance imaging revealed a well-demarcated lesion appearing as hypointense on T1- and hyperintense on T2- weighted images in the left orbit; the mass was totally excised. Histopathological examination revealed a tumor comprising spindle shaped cells in myxoid stroma sprinkled with small vascular structure. The tumor tissue was positive for alcian blue and vimentin, and not for S100 on staining. Histopathological examination led to the diagnosis of orbital angiomyxoma. The case was followed-up for 18 months without any evidence of recurrence. (Turk J Ophthalmol 2014; 44: 496-8

  8. 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.

  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. Paravaginal peripheral primitive neuroectodermal tumor: A rare tumor

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    Samrat Dutta

    2011-01-01

    Full Text Available Peripheral primitive neuroectodermal tumor (PNET of the female genital tract, particularly those in the vaginal and paravaginal region, are extremely rare. A 36-year-old woman presented with clinical features similar to that of a case of cervical fibroid. It was only after surgery that the histopathology suggested it to be a malignant round cell tumour and was CD99 positive. She underwent adjuvant chemotherapy with the Ifosfamide and Etoposide alternating with Vincristine, Doxorubicin, and Cyclophosphamide regime and radical radiotherapy. She is disease free at 12 months of follow-up. The importance of immunostaining and adequate histopathology report lies in the fact that the correct diagnosis thus achieved enabled us to manage a rare case of paravaginal PNET with a multimodality approach.

  11. Sweat gland tumor (Eccrine Porocarcinoma of scalp: A rare tumor

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    Rana Roshani

    2005-01-01

    Full Text Available Eccrine Porocarcinoma is a rare neoplasm arising from sweat glands. It was first described by Pinkus and Mehregan as ′Epidermotropic eccrine carcinoma′. It may occur de novo or as a malignant transformation of an eccrine poroma. It is commonly found in older age group and in the lower extremities. Clinically, it may present as a verrucous plaque, polypoid growth or an ulcerative lesion of long duration. Local recurrence and metastasis to skin, lymphnodes, viscera, and bone may occur. Treatment is wide local excision. Metastatic lesions can be treated with chemotherapy. We report a case of eccrine porocarcinoma of the scalp in a 50 years old female who presented to us with a bosselated, firm, painless, non-tender, freely mobile swelling over left fronto-parietal region of 12 years duration. It was excised and histopathological diagnosis was Eccrine Porocarcinoma. In literature, scalp porocarcinoma is a very rare tumor.

  12. Horseshoe Appendix: An Extremely Rare Appendiceal Anomaly.

    Science.gov (United States)

    Singh, Ch Gyan; Nyuwi, Kuotho T; Rangaswamy, Raju; Ezung, Yibenthung S; Singh, H Manihar

    2016-03-01

    Appendiceal anomalies are extremely rare malformations that are usually found incidentally. Agenesis and duplication of the appendix has been well documented however, the cases of horseshoe appendix reported is very limited, only four cases reported so far. Here, we report a four and half-year-old who underwent interval appendectomy. Intraoperatively both the ends of the appendix were found to be communicating with the cecum with two separate base or stump located at a sagital disposal- the so called "horseshoe appendix".

  13. Rare and Challenging Tumor Entity: Phyllodes Tumor of the Prostate

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    Andreas Bannowsky

    2009-01-01

    Full Text Available Cystic epithelial-stromal tumors of the prostate are rare, with 82 cases reported in literature. These cases have been published under a variety of diagnoses, including phyllodes tumor and prostatic stromal proliferation of uncertain malignant potential as well as a malignant tumor called “prostatic stromal sarcoma”. We report a case of a 60-year-old man with the histological diagnosis of phyllodes tumor of the prostate in transurethral resection specimen.

  14. Rare and Challenging Tumor Entity: Phyllodes Tumor of the Prostate

    OpenAIRE

    Andreas Bannowsky; Andreas Probst; Helmut Dunker; Tillmann Loch

    2009-01-01

    Cystic epithelial-stromal tumors of the prostate are rare, with 82 cases reported in literature. These cases have been published under a variety of diagnoses, including phyllodes tumor and prostatic stromal proliferation of uncertain malignant potential as well as a malignant tumor called “prostatic stromal sarcoma”. We report a case of a 60-year-old man with the histological diagnosis of phyllodes tumor of the prostate in transurethral resection specimen.

  15. 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.

  16. 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.

  17. Intraosseous myoepithelioma: A rare, distinct tumor entity

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    Bharat Rekhi

    2014-01-01

    Full Text Available Primary musculoskeletal myoepithelial tumors (METs are distinctly rare tumors and are being increasingly recognized as a result of improved diagnostic criteria and objective confirmation with immunohistochemical markers, including epithelial markers. Recent studies have unraveled distinct molecular mechanisms underlying these tumors. Herein, we present our second diagnosed case of an intraosseous MET that occurred in the tibia of a 37-year-old lady. The case is discussed with regards to current clinicopathological perspectives on these rather uncommon tumors, including our personal experience.

  18. An extremely rare case: osteosclerotic metaphyseal dysplasia.

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    Kasapkara, C S; Küçükçongar, A; Boyunağa, O; Bedir, T; Oncü, F; Hasanoğlu, A; Tümer, L

    2013-01-01

    OMD (osteosclerotic metaphyseal dysplasia) is a very rare sclerosing bone disorder, first described by G. Nishimura in two Japanese siblings in 1993 (6). We report the case of a 12-month-old male with hypotonia, developmental delay and sclerosis of the metaphyses and epiphyses of specific bones. This 36-week gestation boy was born to a 26 year old gravida 5 para 1 Turkish mother and a 27 year old nonconsanguineous father. Radiographic findings obtained during the hospital stay included bilateral symmetrical osteosclerosis of the metaphyseal portions of the long bones in the upper and lower extremities with osteopenic shafts. Narrow bands of metaphyseal osteosclerosis were detected in the short tubular bones of the hands and feet. Growing parts of bilateral scapula, iliac, pubic and ischial bones show sclerotic bands. In addition superior and inferior plates of vertebras, transverse processes of sacral vertebras, all visible epiphyses, carpal and tarsal bones also show sclerotic changes. The scalp was unaffected. Based on the clinical, radiographic, and laboratory findings, a diagnosis of OMD was made. We do not know any of the osteosclerotic bone disorder with changes including hypotonia, mental and motor developmental delay and metaphyseal sclerosis of the bones with a unique distribution except OMD. The syndrome is characterized by developmental delay of a progressive nature, hypotonia, elevated alkaline phosphatase, and late-onset spastic paraplegia 18 years ago. Our patient is the 4th case of OMD described in the literature share some clinical and radiological similarities with other three reported cases of osteosclerotic metaphyseal dysplasias.

  19. Clear cell variant of calcifying epithelial odontogenic tumor of maxilla: Report of a rare case.

    Science.gov (United States)

    Badrashetty, Dinesh; Rangaswamy, Shruthi; Belgode, Niranjan

    2013-09-01

    The calcifying epithelial odontogenic tumor (CEOT) is a rare benign tumor of the jaws. Pindborg's tumor having clear cells is extremely rare. Twelve central lesions have been reported of which only three cases have occurred in maxilla. Clear cell variant is a distinct entity, has more aggressive biological behavior and higher chances of recurrence. Hence it is important that presence of clear cells be included in histopathological diagnosis. Here we present a rare case of clear cell CEOT having aggressive behavior.

  20. 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.

  1. Rare giant cell tumor involvement of the olecranon bone.

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    Yang, Chen; Gong, Yubao; Liu, Jianguo; Qi, Xin

    2014-06-01

    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.

  2. 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.

  3. A rare tumor of the lung: inflammatory myofibroblastic tumor

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    Hammas Nawal

    2012-07-01

    Full Text Available Abstract Inflammatory myofibroblastic tumor is a rare benign lesion whose tumor origin is now proven. It represents 0.7% of all lung tumors. We report the case of a three-year-old child who suffered from a chronic cough with recurrent respiratory infections. Chest X-ray and computed tomography revealed the presence of a left lower lobe lung mass. After pneumonectomy, histological examination combined with immunohistochemical study discovered an inflammatory myofibroblastic tumor. The virtual slide(s for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/8722069326962972.

  4. "MUCOEPIDERMOID CARCINOMA OF THE LARYNX: REPORT OF A RARE LARYNGEAL TUMOR"

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    S. Z. Madani-Kermani

    2004-06-01

    Full Text Available Mucoepidermoid carcinoma is a neoplasm of salivary gland origin, and its laryngeal occurrence is extremely rare. This malignant tumor is composed of two distinct cell types, the epidermoid and mucus cells. Prognosis is largely dependent on the histologic pattern. In this report, an extremely rare laryngeal cancer and its clinicopathologic features are described.

  5. Colopleural fistula caused by aspergillus: an extremely rare complication after lung resection—case report

    OpenAIRE

    Hayashi, Akio; Susaki, Yoshiyuki; Ose, Naoko; Takeuchi, Yukiyasu; Maeda, Hajime

    2016-01-01

    A colopleural fistula is a rare condition reported to be caused by Crohn’s disease, a malignant tumor of the gastrointestinal tract, and other clinical conditions. Some studies have noted that a sub-diaphragmatic abscess, usually organized following abdominal surgery, may play some role in the formation of this type of fistula. Therefore, a colopleural fistula is a complication very rarely encountered by thoracic surgeons after lung resection. We experienced an extremely rare case of colopleu...

  6. Intramuscular Myxoma – A Rare Tumor

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    Vishwanath Yaligod

    2013-10-01

    Full Text Available Introduction: Intramuscular myxoma is a rare benign soft tissue tumor involving the musculoskeletal system. The incidence is reported as varying from 0.1 to 0.13 per 100,000 population. Most patients present between the fifth and sixth decade of life. The swelling commonly occurs in the large muscles of the thigh, shoulder, buttocks and arms. The tumor can be diagnosed with certainty only with histopathological examination. Local recurrence is rare after excision. Case Report: A 73 year old male patient presented to us with a swelling of the anterior aspect of the middle third of the right thigh measuring about 8 x 4 centimeters. He was thoroughly investigated and the swelling excised in toto. The soft cystic swelling excised was found on histopathology to be an intramuscular myxoma. Conclusion: This case report is being presented since the tumor is rare and also an important consideration in the differential diagnosis of sarcomas, intramuscular lipoma, hemangioma, hematoma, and desmoid tumor. Another important feature is that it can be diagnosed with certainty only after excision. Keywords: Intramuscular myxoma, soft tissue swelling, benign.

  7. A RARE CARTILAGINOUS TUMOR OF THE TALUS

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    Alok Sobhan

    2014-06-01

    Full Text Available The present report describes an unusual and rare tumor originating from the talus. A 21 years old female presented with a swelling over the right ankle 4x3.5cms in size for one year. The lesion was osteolytic with surrounded peripheral rim of bone sclerosis. Subsequent pathological study confirmed the case as chondroblastoma. The lesion was curettaged and the resultant defect was filled by autogenous bone graft. Such tumors are as chondroblastoma usually originate from epiphyseal and apophyeal regions of long bones. Thorough clinical, radiological and histological assessment is required for appropriate management.

  8. Giant lipoblastoma of the thigh: A rare soft tissue tumor in an infant

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    Tanveer Akhtar

    2012-01-01

    Full Text Available Lipoblastoma is a rare lipomatous tumor encountered almost exclusively in infants and young children. It arises from embryonic white fat. The common site of involvement is the extremities. In spite of their potential for local invasion, they are benign tumors. We report a case of a lipoblastoma in an infant and review the literature pertaining to clinical management of these tumors.

  9. Hepatobiliary cystadenoma: a rare pediatric tumor.

    Science.gov (United States)

    Tran, Sifrance; Berman, Loren; Wadhwani, Nitin R; Browne, Marybeth

    2013-08-01

    Hepatobiliary cystadenoma is a rare hepatic neoplasm that has been reported only 10 times in the pediatric population. Although considered a benign cystic tumor of the liver, hepatobiliary cystadenoma has a high risk of recurrence with incomplete excision and a potential risk for malignant degeneration. Complete tumor excision with negative margins is the mainstay in treatment. Unfortunately, due to the paucity of cases and its vague presentation, hepatobiliary cystadenoma is rarely diagnosed preoperatively. Therefore, in patients with hepatic cystic masses without a clear diagnosis, total resection of the lesion with negative margins is indicated to adequately evaluate for malignant potential and limit the risk of recurrence. We describe a 2-year-old girl with an asymptomatic abdominal mass that was found to be hepatobiliary cystadenoma. In addition, the pathogenic, histopathologic and clinical features of hepatobiliary cystadenoma are reviewed.

  10. Mast cell leukemia: an extremely rare disease.

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    Lu, Dai-Yin; Gau, Jyh-Pyng; Hong, Ying-Chung; Liu, Chun-Yu; Yu, Yuan-Bin; Hsiao, Liang-Tsai; Liu, Jin-Hwang; Chen, Po-Min; Chiou, Tzeon-Jye; Tzeng, Cheng-Hwai

    2014-08-01

    Systemic mastocytosis is characterized by pathologic proliferation and accumulation of mast cells in at least one extracutaneous organ such as liver, spleen, bone marrow, or lymph nodes. The clinical features are highly variable depending on impairment of the involved organ systems. It often raises diagnostic challenges. Here we report a case of a 78-year-old patient with mast cell leukemia. The literature is reviewed regarding the diagnosis and updated management of this rare disease.

  11. 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.

  12. Neuroendocrine gastric carcinoma expressing somatostatin: A highly malignant, rare tumor

    Institute of Scientific and Technical Information of China (English)

    Jaques Waisberg; Leandro Luongo de Matos; Ana Maria do Amaral Antonio Mader; Sérgio Pezzolo; Esmeralda Miristene Eher; Vera Luiza Capelozzi; Manlio Basilio Speranzini

    2006-01-01

    Poorly differentiated gastric neuroendocrine carcinomas,although rare, deserve particular attention, as they are aggressive and have an extremely poor prognosis. In this report we describe a gastric neuroendocrine carcinoma with rapidly fatal outcome. Immunohistological staining of the resected specimens revealed that the tumor was an endocrine carcinoma. The tumor disclosed intense immunoreactivity to pan-neuroendocrine markers and diffuse somatostatin immunoreactivity. There were no psammoma bodies and no demonstrable association with von Recklinghausen's neurofibromatosis. In the gastrointestinal tract, neuroendocrine tumors producing predominantly somatostatin have been described only in the duodenum. To the best of our knowledge, the present report is the second case report of a neuroendocrine gastric carcinoma expressing diffusely somatostatin as the only neuroendocrine regulatory peptide.

  13. Triad of Intraspinal Meningioma, Schwannoma, and Ependymoma: Report of an Extremely Rare Case.

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    Rasheed, Faiza; Fatima, Saira; Ahmad, Zubair

    2016-02-01

    Mixed tumors composed of schwannoma and meningioma are extremely rare and are usually associated with neurofibromatosis type 2. So far, all the cases reported have involved the cerebellopontine angle. Only 3 reported cases did not have a clear association with neurofibromatosis type 2. We report a mixed tumor comprising schwannoma admixed with meningioma and ependymoma in the cervical spinal cord of a 22-year-old male. © The Author(s) 2015.

  14. 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

  15. Yolk sac tumor of the seminal vesicles: A rare malignant cause of hematospermia

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    Jonathan D Gill

    2015-01-01

    Full Text Available Extra-gonadal yolk sac tumors (YSTs are rare and generally associated with poor outcomes. Involvement of the seminal vesicles is extremely rare with only one previously described case. We report a case of a primary YST of the seminal vesicles and discuss the management strategy.

  16. Yolk sac tumor of the seminal vesicles: A rare malignant cause of hematospermia.

    Science.gov (United States)

    Gill, Jonathan D; Bhattarai, Selina; Patel, Chirag N; Paul, Alan B

    2015-01-01

    Extra-gonadal yolk sac tumors (YSTs) are rare and generally associated with poor outcomes. Involvement of the seminal vesicles is extremely rare with only one previously described case. We report a case of a primary YST of the seminal vesicles and discuss the management strategy.

  17. 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...

  18. Rare Renal Incidentaloma in Pregnancy: An Unusual Primitive Neuroectodermal Tumor Presentation

    OpenAIRE

    2015-01-01

    Peripheral Primitive Neuroectodermal Tumors (PNETs) are rare lesions that arise from outside the central nervous system and normally do not affect the genitourinary system. Primary renal presentations are extremely rare but given their aggressive behavior and characteristic cytomorphologic and genetic features should be considered well-defined distinct clinical entities in order to distinguish them from other primary tumors featuring round cells in the kidney. We report one case of PNET invol...

  19. Clear cell variant of calcifying epithelial odontogenic tumor of maxilla: Report of a rare case

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    Dinesh Badrashetty

    2013-01-01

    Full Text Available The calcifying epithelial odontogenic tumor (CEOT is a rare benign tumor of the jaws. Pindborg′s tumor having clear cells is extremely rare. Twelve central lesions have been reported of which only three cases have occurred in maxilla. Clear cell variant is a distinct entity, has more aggressive biological behavior and higher chances of recurrence. Hence it is important that presence of clear cells be included in histopathological diagnosis. Here we present a rare case of clear cell CEOT having aggressive behavior.

  20. Ameloblastic fibrosarcoma: a rare malignant odontogenic tumor.

    Science.gov (United States)

    Gilani, S M; Raza, A; Al-Khafaji, B M

    2014-02-01

    Ameloblastic fibrosarcoma (AFS) is a rare malignant odontogenic tumor. It can arise de novo, however one-third of cases may arise from a recurrent ameloblastic fibroma, in which case they appear to present at an older age. A 16-year-old female presented with one month history of right mandibular mass. Computerized tomography (CT) scan showed a large destructive mass. A biopsy of the mass was performed. Histologically, it consisted of a mixed epithelial-mesenchymal odontogenic neoplasm composed of benign islands of well-differentiated ameloblastic epithelium within a malignant fibrous stroma consisting of spindle cells or fibroblasts with a brisk mitotic activity. The malignant spindle cell proliferation showed positive staining with p-53 and a high proliferation index with ki-67. A diagnosis of AFS was rendered. The differential diagnosis includes other odontogenic sarcomas, ameloblastic carcinosarcoma and spindle cell carcinoma. Treatment of choice is wide surgical excision, with long-term follow-up. Postoperative chemotherapy and radiotherapy has been used successfully in a few reported cases. AFS is a locally aggressive malignant tumor, with regional and distant metastases being uncommon. Copyright © 2013 Elsevier Masson SAS. All rights reserved.

  1. Nasal chondromesenchymal hamartoma: radiographic and histopathologic analysis of a rare pediatric tumor

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    Johnson, Craig; Esguerra, Jorge; Wurzbach, Douglas [Northeastern Ohio Universities College of Medicine, Canton Affiliated Hospitals, Department of Diagnostic Radiology, Canton, OH (United States); Nagaraj, Usha [Northeastern Ohio Universities College of Medicine, Rootstown, OH (United States); Wasdahl, Daniel [Northeastern Ohio Universities College of Medicine, Canton Affiliated Hospitals, Department of Pathology, Canton, OH (United States)

    2007-01-15

    Nasal chondromesenchymal hamartoma (NCMH) is an extremely rare benign pediatric tumor that was described in 1998. Only 19 cases are reported in the literature. We present a 15-year-old girl with nasal obstruction and recurrent sinusitis. Her medical history was significant for a rare ovarian tumor and pleuropulmonary blastoma. CT demonstrated a partially calcified soft-tissue mass obstructing the nasal cavity. The patient underwent endoscopic surgical excision. Histologic and immunohistochemical analyses of the tumor were consistent with NCMH. The imaging characteristics of the tumor are reviewed. NCMH may be part of a syndrome associated with other pediatric neoplastic and dysplastic disease. (orig.)

  2. Away from the milky way: An extremely rare case of pulmonary ectopic breast

    Directory of Open Access Journals (Sweden)

    Rahim Mahmodlou

    2015-01-01

    Full Text Available Ectopic breast is defined as the presence of breast tissue outside the pectoral region. In this article, we are reporting an extremely rare case of pulmonary aberrant breast tissue. A 79-years-old Caucasian woman with the history of hypertension, diabetes mellitus and ischemic heart disease, presented to the emergency department for worsening dyspnea on exertion and pleuritic chest pain over the last few days. Both thoracic CT scan and the gross evaluation of tumor after its removal by thoracotomy were in favor of a soft tissue tumor such as pulmonary lipoma, whilst surprisingly the histological examinations revealed the mass to be ectopic breast tissue. Although it is extremely rare, this diagnosis should be considered in the evaluation of pulmonary masses.

  3. An Extremely Rare, Remote Intracerebral Metastasis of Oral Cavity Cancer: A Case Report

    OpenAIRE

    Mario Leimert; Juratli, Tareq A.; Claudia Lindner; Kathrin D Geiger; Johannes Gerber; Gabriele Schackert; Matthias Kirsch

    2013-01-01

    Distant brain metastases from oral squamous cell carcinomas (OSCC) are extremely rare. Here we describe a case of a 53-year-old man with a primary OSCC who referred to the neurosurgical department because of epileptic seizures. MR imaging revealed an enhancing lesion in the right parietal lobe. A craniotomy with tumor removing was performed. Histopathological examination verified an invasive, minimally differentiated metastasis of the primary OSCC. The patient refused whole brain radiation t...

  4. Pancreatic paraganglioma: An extremely rare entity and crucial role of immunohistochemistry for diagnosis

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    Mondita Borgohain

    2013-01-01

    Full Text Available Paragangliomas are rare neuroendocrine neoplasms arising in extra-adrenal chromaffin cells of autonomic nervous system and histologically akin to chemodectomas. They are rare, affecting about 1 in 2,000,000 population. It is a generic term applied to tumors of paraganglia regardless of the location. In rare instances, paragangliomas present around and involve the pancreas, thereby mimicking any one of the more common primary pancreatic lesions. Pancreatic paraganglioma is an extremely rare tumor. It grows slowly, so radical resection is recommended to achieve curability with good prognosis. These neoplasms present considerable diagnostic difficulty not only for the clinician and radiologist but also for the pathologist. Here, we report a case of a 55-year-old woman who presented with a left-sided abdominal swelling for 3 months duration, initially having clinical suspicion of an ovarian tumor. The radiological imaging revealed a lesion in the tail of pancreas with a differential diagnosis of pancreatic carcinoma and metastatic tumor. Only after exploratory laparotomy, the diagnosis was made as a rare case of pancreatic paraganglioma on the basis of histological examination and immunohistochemistry.

  5. Pancreatic paraganglioma: An extremely rare entity and crucial role of immunohistochemistry for diagnosis

    Science.gov (United States)

    Borgohain, Mondita; Gogoi, Gayatri; Das, Dipak; Biswas, Manjusha

    2013-01-01

    Paragangliomas are rare neuroendocrine neoplasms arising in extra-adrenal chromaffin cells of autonomic nervous system and histologically akin to chemodectomas. They are rare, affecting about 1 in 2,000,000 population. It is a generic term applied to tumors of paraganglia regardless of the location. In rare instances, paragangliomas present around and involve the pancreas, thereby mimicking any one of the more common primary pancreatic lesions. Pancreatic paraganglioma is an extremely rare tumor. It grows slowly, so radical resection is recommended to achieve curability with good prognosis. These neoplasms present considerable diagnostic difficulty not only for the clinician and radiologist but also for the pathologist. Here, we report a case of a 55-year-old woman who presented with a left-sided abdominal swelling for 3 months duration, initially having clinical suspicion of an ovarian tumor. The radiological imaging revealed a lesion in the tail of pancreas with a differential diagnosis of pancreatic carcinoma and metastatic tumor. Only after exploratory laparotomy, the diagnosis was made as a rare case of pancreatic paraganglioma on the basis of histological examination and immunohistochemistry. PMID:24083178

  6. Leiomyoma of the deferens: a curious and extremely rare disease.

    Science.gov (United States)

    De Rose, Aldo F; Mantica, Guglielmo; Piol, Nataniele; Toncini, Carlo; Spina, Bruno; Terrone, Carlo

    2017-08-01

    Leiomyomas are rare benign tumors that can occur in the male urinary tract. We present a unique case of leiomyoma of the vas deferens. We present the clinical case of a 69-year-old patient with a suspected bulk close to the right epididymis, which turned out to be a leiomyoma of the vas deferens. To our knowledge, it is the fourth case in literature. A proper identification and the knowledge of this pathology, even if it is a very unusual event, is necessary to avoid a surgical over treatment and preserve the testicle, by removing only the tumor.

  7. Meningeal carcinomatosis: an extremely rare involvement of urinary bladder carcinoma.

    Science.gov (United States)

    Uncu, Dogan; Arpaci, Fikret; Beyzadeoglu, Murat; Gunal, Armagan; Surenkok, Serdar; Ozturk, Mustafa; Ozet, Ahmet

    2010-01-01

    Meningeal carcinomatosis (MC) is a rare presentation of solid tumors, particularly breast cancer, lung cancer, and malignant melanoma. Recently, the incidence of MC has been reported to be increasing. It has a bad prognosis despite aggressive therapy. The usual clinical presentation is multifocal involvement of the neuraxis, with headache and radicular pain being the most common initial symptoms. The most frequent signs are motor deficits, altered mental status, and cranial nerve involvement. The treatment of MC remains controversial and no straightforward guidelines exist in the literature. MC from urinary bladder tumors is rare. In this case report, we present a 52-year-old male patient with meningeal metastasis from a primary urinary bladder carcinoma along with a review of the related literature. Free full text available at www.tumorionline.it

  8. Desmoplastic fibroma in the thoracic spine: an unusual localization of a rare primary bone tumor

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    Juergens, K.U.; Link, T.M.; Heindel, W. [Muenster Univ. (Germany). Inst. fuer Klinische Radiologie; Bullmann, V. [Dept. of Orthopedic Surgery, Univ. of Muenster (Germany); Brinkschmidt, C. [Gerhard Domagk Inst. of Pathology, Univ. of Muenster (Germany)

    2001-02-01

    A case of an intraspinal growing desmoplastic fibroma of the thoracic spine (T9-T11) is reported. Desmoplastic fibroma is a rare tumor of connective tissue that shows a locally infiltrative and destructive growth. An affection of the thoracic spine is an extremely rare manifestation. Preoperative CT documents the extent of osseous destruction and tumor associated cortical erosion. In central tumor areas an inhomogeneous, intermediate to low signal is demonstrated by MRI using T1- and T2-weighted spin-echo and turbo-spin-echo sequences. Contrast-enhanced MRI shows marked enhancement in peripheral areas depicting the extraosseous and intramedullary extension. (orig.)

  9. Nasal angiomyolipoma: Report of two cases of an extremely rare entity.

    Science.gov (United States)

    Aleem, Mohammed Abdul; Fatima, Ather; Kumudachalam, P; Priyadarshini, Ramya

    2017-01-01

    Nasal angiomyolipoma (AML) are extremely rare tumors and so far <15 cases have been reported in the literature, and this is the first instance that Nasal AML is reported from India. We report two cases of AML arising in the nasal cavity described in 60-year-old male and 50-year-old female patient. Grossly, they were well circumscribed, lobulated masses, and microscopically, they were composed of an intimate mixture of mature fat, smooth muscle cells, and thick-walled varying sized blood vessels. Immunoexpression pattern and histopathology were characteristic. Both the patients had complete resolution of symptoms after endoscopic excision of the tumor.

  10. Rare Malignant Tumors of Clitoris—a Case Report

    OpenAIRE

    Venugopal, P. R.; Ramachandran, Padma

    2013-01-01

    Tumors of the clitoris are very rare. Isolated case reports are available in literature. This case report highlights the importance of this disease, due to its high malignant potential and treatment options. We had come across a rare tumor of the clitoris in a young girl, reported to be malignant extrarenal rhabdoid tumor and patient had a short life span of 6 months.

  11. Angiomyxoma: a rare tumor of the umbilical cord

    OpenAIRE

    Göksever, Hale; Celiloğlu, Murat; Küpelioğlu, Ali

    2010-01-01

    Tumors of the umbilical cord are rare anomalies and should be considered when using prenatal ultrasound for detection of cystic lesions. Differential diagnosis of umbilical cord tumors should comprise umbilical cord teratoma, hemangioma and angiomyxoma. It can also be an umbilical cord polyp, umbilical cord cyst, hernia into the cord and omphalocele, which are mostly isolated findings, except omphalocele. Angiomyxoma is a rare tumor of the umbilical cord and is associated with incresaed perin...

  12. Rare Malignant Tumors of the Breast

    OpenAIRE

    Trevor Miller; Constance Albarracin; Selin Carkaci; Whitman, Gary J.; Adrada, Beatriz E.

    2015-01-01

    While the more common forms of breast cancer are well understood and recognized, there are many important rare malignancies that are less appreciated. Many of these cancers have imaging findings that, when understood, help to formulate a more educated differential diagnosis. In this article, the clinical features, imaging, and pathologic findings of rare breast malignancies will be discussed.

  13. Rare Malignant Tumors of the Breast

    Directory of Open Access Journals (Sweden)

    Trevor Miller

    2015-01-01

    Full Text Available While the more common forms of breast cancer are well understood and recognized, there are many important rare malignancies that are less appreciated. Many of these cancers have imaging findings that, when understood, help to formulate a more educated differential diagnosis. In this article, the clinical features, imaging, and pathologic findings of rare breast malignancies will be discussed.

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

    Directory of Open Access Journals (Sweden)

    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.

  15. A Rare Tumor in the Cervical Sympathetic Trunk: Ganglioneuroblastoma

    Directory of Open Access Journals (Sweden)

    Ozan Erol

    2016-01-01

    Full Text Available Ganglioneuroblastoma is a rare tumor with moderate malignancy, which is composed of mature ganglion cells and seen in sympathetic ganglia and adrenal medulla. The diagnosis is possible after cytological and immunohistochemical studies following a needle biopsy or surgical excision. There is no consensus regarding the need for chemo- or radiotherapy after surgery. In this case report, clinical behavior and diagnosis and treatment of the rare tumor cervical ganglioneuroblastoma were discussed.

  16. Giant benign nodular hidradenoma of the shoulder: A rare tumor in orthopedic practice

    Directory of Open Access Journals (Sweden)

    Singhal Vibhore

    2010-01-01

    Full Text Available A clear cell hidradenoma is a rare dermal tumor, which is believed to originate from the apical portion of the sweat glands. The usual size reported is 5-30 mm. It is generally found in the head, face, and upper extremity regions. This lesion has not been reported to be large enough to impinge a joint range of motion. Hence, its description in the orthopedic literature is extremely rare. We present a giant benign nodular hidradenoma presenting as painful restriction of the right shoulder joint in a 35-year-old male.

  17. Myoepithelial carcinoma of buccal mucosa: A rare tumor.

    Science.gov (United States)

    Lata, Jeevan; Ahmad, Fahad; Chand, Vimal

    2014-04-01

    Myoepithelial carcinoma is a rare neoplasm of salivary glands that account for Myoepithelial tumors were first described in 1943. Their malignant variant, myoepithelial carcinoma, was first reported by Stromeyer et al., in 1975, characterized by distinct morphologic heterogeneity and an infiltrative growth pattern into adjacent tissues. Here, we report a rare case of a 55-year-old female with myoepithelial carcinoma of buccal mucosa. It was also rare because of unusual location of tumor. Our patient was treated with wide local resection and remained free of disease for 15 months.

  18. Exploring the causes of rare extreme precipitation events

    Science.gov (United States)

    Schroeer, Katharina; Kirchengast, Gottfried

    2015-04-01

    Whereas trends of precipitation changes in general are disparate, an increase of extreme intensities of short precipitation events (daily to sub-hourly scale) with increasing temperatures seems unambiguous (e.g. Trenberth et al., Clim. Res. 47, 123-138, 2011; Berg et al., Nat. Clim. Change 13, 181-185, 2013; Kendon et al., Nat. Clim. Change 4, 570-576, 2014). In probability density functions (PDFs) of observed precipitation intensities that are frequently used in science and practice, high magnitude ("extreme") low frequency ("rare") precipitation events naturally appear at the tails of PDFs. Due to the factual data scarcity, rare extreme events ("REEs") are difficult to come by with statistical analyses. Amongst studies of extreme precipitation, statistical work nevertheless makes a major contribution to the research field. Usually as a first step, a threshold is defined to classify extreme events out of a sample (statistical extreme events, "SEEs"), where methods are affected by the sample size. Such thresholds can be described user-defined or constructed. Subsequently, a PDF is sought, fit and applied (e.g. Yilmaz et al., Hydrol. Earth Syst. Sci. 18, 4065-4076, 2014;, Papalexiou et al., Hydrol. Earth Syst. Sci. 17, 851-862, 2013). While these studies respond to the needs of engeneering practice in e.g. infrastructure design, or trend analysis of precipitation in climate studies, they a) have to ignore REEs because of practical or statistical/data limitations (i.e. left out as "residual risk") and b) tell us little about the underlying processes of the climate and weather system causing REEs. We define REEs in contrast to SEEs as to be of such occurrence that they cannot be sufficiently described nor predicted by means of a regular or fat-tailed PDF. We introduce a working hypothesis assuming that REEs are conditioned and caused by a conjunction of specific circumstances on different scales. We differentiate spatio-temporal circumstances of large

  19. 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.

  20. A rare renal pelvis tumor: Mucinous cystadenocarcinoma

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    Gül Türkcü

    2015-03-01

    Full Text Available Urothelial carcinomas are the most common neoplasms in the renal pelvis. However mucinous cystadenocarcinomas (MCA are very rare in this localization. Although some theories are attributed on the patogenesis of MCA, its exact etiology is not known. Herein, we present histopathological characteristic of a case with MCA. Multiple cystic lesions and millimetric calculi with ectasia of the left kidney were detected by abdominal ultrasound and magnetic resonance imaging. Left simple nephrectomy was performed because of a pre-diagnosis of atrophic pyonephrotic kidney. The sections of the nephrectomized kidney revealed, multilocular mucinous cysts and histopathological appearance of MCA. We aimed to present this rare case mimicking atrophic cystic kidney with clinical, radiological findings, and histopathological characteristics in the lights of literature.J Clin Exp Invest 2015; 6(1: 78-80

  1. Krukenberg Tumor: A Rare Cause of Ovarian Torsion

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    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.

  2. Pilocytic astrocytoma: A rare presentation as intraventricular tumor

    Science.gov (United States)

    Sattar, Sidra; Akhunzada, Naveed Z.; Javed, Gohar; Uddin, Zeeshan; Khan, Yasir A.

    2017-01-01

    Background: Pilocytic astrocytoma (PA) is the most prevalent central nervous system (CNS) tumor in pediatric population and accounts for an approximate of 5–6% of all gliomas. This neoplasm can occur at all levels of the neuraxis, with majority (67%) arising in the cerebellum and optic pathway. PAs are World Health Organization Grade I tumors and are the most benign of all astrocytomas characterized by an excellent prognosis. Other differentials include subependymal giant cell astrocytoma (SEGA), ependymoma, meningioma, and low-grade gliomas such as pilocytic or diffuse astrocytoma; calcification is more commonly regarded as a feature of benign or slow-growing tumors. Case Description: We present a case of a 17-year-old female presenting with an unusual cause of hydrocephalus, a rare case of a calcified pilocytic astrocytoma as an intraventricular tumor. Conclusion: PA rarely presents as an intraventricular tumor and should be included in the differential diagnosis of a large mass with massive intratumoral calcification. PMID:28680735

  3. Giant cystosarcoma phyllodes tumor of prostate: Case report of a rare entity

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    Bhat Dharitri

    2004-01-01

    Full Text Available Although glandular and stromal proliferations of prostate are very common in adult men, neoplastic proliferations of prostatic stroma are distinctly uncommon. These tumors are now grouped as Prostatic Stromal Proliferations of Uncertain Malignant Potential (PSPUMP. Phyllodes tumor of the prostate is a rare neoplasm in this group with cellular, sarcomatoid stroma and benign hyperplastic glands. It is a locally expansile tumor with clinical course varying from benign to aggressive. We report a case of a 45-year-old man presented with retention of urine and abdominal lump. On laparotomy it was a huge tumor of 4 kg and was histologicaly characterized by cellular pleomorphic stroma and hyperplastic epithelium. Immunohistochemistry demonstrated prostate specific antigen in the glands. It was diagnosed as cystosarcoma phyllodes tumor of prostate. This is extremely uncommon tumor similar in histology to that of breast and it′s clinical course varies with the grade. The patient was without recurrence one year after surgery.

  4. Myoepithelial carcinoma of buccal mucosa: A rare tumor

    Directory of Open Access Journals (Sweden)

    Jeevan Lata

    2014-01-01

    Full Text Available Myoepithelial carcinoma is a rare neoplasm of salivary glands that account for < 1% of all salivary gland tumors. The most common sites of involvement are major salivary glands mainly parotid gland. Intraorally, it can arise from minor salivary glands; palate is the most common site of occurrence. It also occurs in nasopharynx, paranasal sinuses, nasal cavity and larynx in head and neck region. Myoepithelial tumors were first described in 1943. Their malignant variant, myoepithelial carcinoma, was first reported by Stromeyer et al., in 1975, characterized by distinct morphologic heterogeneity and an infiltrative growth pattern into adjacent tissues. Here, we report a rare case of a 55-year-old female with myoepithelial carcinoma of buccal mucosa. It was also rare because of unusual location of tumor. Our patient was treated with wide local resection and remained free of disease for 15 months.

  5. Primary renal primitive neuroectodermal tumor: A rare presentation

    Directory of Open Access Journals (Sweden)

    V Goel

    2015-01-01

    Full Text Available Primitive Neuroectodermal Tumor of the kidney is a rare entity. Very few cases of primary renal PNET have been reported to date. Most literature about rPNET is isolated case reports. We report a case of rPNET in a 39-year-old male with a pre-operative diagnosis of renal cell carcinoma with renal vein thrombosis. The patient underwent radical nephrectomy with thrombolectomy, and histopathological examination revealed a highly aggressive tumor composed of monotonous sheets of round cells. Tumor cells were positive for CD 99 and FLI-1, hence confirming the diagnosis of Primitive Neuroectodermal Tumor. Post-surgery, patient was given VAC/IE-based adjuvant chemotherapy. In view of highly aggressive nature of this tumor, prompt diagnosis and imparting effective chemotherapy regimen to the patient is required, and it is important to differentiate PNET from other small round-cell tumors because of different therapeutic approach.

  6. Calcifying epithelial odontogenic tumor (Pindborg tumor without calcification: A rare entity

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    Seema Kaushal

    2012-01-01

    Full Text Available The calcifying epithelial odontogenic tumor is a rare benign odontogenic tumor that was first described by Pindborg in 1955. It accounts for less than 1% of all odontogenic neoplasms. The tumor is characterized histologically by the presence of polygonal epithelial cells, calcification, and eosinophilic deposits resembling amyloid. Noncalcifying Pindborg tumor is very rare and only three cases have been documented in the English language literature so far. We present an additional case of noncalcifying Pindborg tumor and review the previously reported cases. Because noncalcifying Pindborg tumor is believed to be an aggressive variant, a definitive resection of the tumor with tumor-free surgical margins and long-term follow-up is recommended.

  7. Calcifying epithelial odontogenic tumor (Pindborg tumor) without calcification: A rare entity.

    Science.gov (United States)

    Kaushal, Seema; Mathur, Sandeep R; Vijay, Maneesh; Rustagi, Ankur

    2012-01-01

    The calcifying epithelial odontogenic tumor is a rare benign odontogenic tumor that was first described by Pindborg in 1955. It accounts for less than 1% of all odontogenic neoplasms. The tumor is characterized histologically by the presence of polygonal epithelial cells, calcification, and eosinophilic deposits resembling amyloid. Noncalcifying Pindborg tumor is very rare and only three cases have been documented in the English language literature so far. We present an additional case of noncalcifying Pindborg tumor and review the previously reported cases. Because noncalcifying Pindborg tumor is believed to be an aggressive variant, a definitive resection of the tumor with tumor-free surgical margins and long-term follow-up is recommended.

  8. 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. PMID:28217164

  9. Sporadic Multifocal Malignant Peripheral Nerve Sheath Tumor-A Rare Presentation: Multifocal MPNST.

    Science.gov (United States)

    Leena, J B; Fernandes, Hilda; Swethadri, G K

    2013-06-01

    Malignant peripheral nerve sheath tumors(MPNST) are uncommon neoplasms with an incidence of 0.001% in general population. Multifocality is a rare manifestation of MPNST . A case of a 65 year old patient who presented with multiple swellings involving the neck, extremity and back without associated neurofibromatosis is reported for its rarity of presentation.. Diagnosis was made by FNAC and confirmed by peroperative findings and histopathology.

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

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    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

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    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. Papillary tumor of pineal region: A rare entity

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    Meena Patil

    2013-01-01

    Full Text Available Pineal tumors comprise 0.4-1.0% of intracranial space occupying lesions in adults. Papillary tumor of pineal region (PTPR is a very rare entity. It has been newly described in WHO 2007 classification of brain tumors. Only a few case reports are available in the literature. We report a case of a 60-year-old female presenting with headache, giddiness and reduced vision. Imaging studies showed a pineal mass with areas of hemorrhage. All ventricles were normal. There was a past history of a pineal gland tumor excised 2 years ago. This case is being reported for its rarity and aggressiveness in the form of recurrence. Limited/available immunohistochemistry workup has been done.

  13. A Rare Case of Phyllodes Tumor Metastasis to the Stomach Presenting as Anemia.

    Science.gov (United States)

    Choi, Do Il; Chi, Ho Seok; Lee, Sang Ho; Kwon, Youngmee; Park, Seog Yun; Sim, Sung Hoon; Park, In Hae; Lee, Keun Seok

    2016-09-01

    Metastasis of a phyllodes tumor to the stomach is an extremely rare condition with important clinical implications. A 44-year-old woman was initially diagnosed with a phyllodes tumor in her right breast in 2008, and subsequently presented to an out-patient clinic with dizziness on December 16, 2013. We found that she had severe anemia (hemoglobin levels: 6.7 g/dL), and we quickly performed esophagogastroduodenoscopy to identify the cause. This procedure revealed large ulcerofungating masses with active bleeding in the stomach. Histopathological examination revealed that the masses were consistent with phyllodes tumor metastases. In patients with a metastatic phyllodes tumor presenting as anemia, gastric metastasis should be considered as one of the differential diagnoses because overlooking the possibility might have dire consequences if cytotoxic chemotherapy were administered.

  14. Syringocystadenoma Papilliferum of the Bony External Auditory Canal: A Rare Tumor in a Rare Location

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    Anastasija Arechvo

    2013-01-01

    Full Text Available Tumors originating from ceruminous glands are rare lesions of the external auditory canal. The lack of specific clinical and radiological signs makes their diagnosis challenging. We report the case of an exceptionally rare benign tumor, a syringocystadenoma papilliferum (SCAP, in an atypical location in the bony segment of the external auditory canal with uncommon clinical signs. The special traits of the case included the following: the most lateral component of the tumor was macroscopically cystic and a granular myringitis with an obstructing keratin mass plug was observed behind the mass. The clinical, audiological, radiological, and histological characteristics of the neoplasm are consequently presented. Intraoperative diagnosis of the epidermal cyst was proposed. The final diagnosis of SCAP was determined only by histological analysis after the surgical excision. The educational aspects of the case are critically discussed.

  15. WARTHIN TUMOR LIKE PAPILLARY CARCINOMA OF THYROID: A RARE OCCURENCE

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    Shikha

    2015-12-01

    Full Text Available INTRODUCTION Among the thyroid cancers, papillary carcinoma is the most common type. Warthin tumor like papillary carcinoma of thyroid is a rare variant of papillary carcinoma. The distinguishing feature of this rare variant is papillary formations lined by tumor cells with oncocytic cytoplasm with nuclear features of papillary carcinoma and lymphoplasmacytic infiltrate in the papillary stalks with striking histological resemblance to Warthin’s tumor of salivary glands. A 46 years old female with complaints of painless swelling of the neck for four years and gradually increasing in size, measuring 3x2.5 cm on the right lobe of the thyroid gland. The swelling moved with deglutition, non-tender and firm to hard in consistency. Thyroid function was within normal limits. FNAC suggested a diagnosis of oxyphilic variant of papillary carcinoma of thyroid. It showed syncytial aggregates, sheets of cells and few papillary structures with focal nuclear crowding. The patient underwent bilateral total thyroidectomy and neck dissection. Microscopic examination showed predominantly follicles and small papillary structures lined by cells having eosinophilic cytoplasm and clear nucleus. There was lymphoid stroma in the core of papillae and interfolllicular areas. Hyalinized collagen, dystrophic calcification and follicles without colloid matter infiltrating the hyalinised area were seen. No lymphovascualr tumour embolization were noted. This tumor is found more commonly in women with Hashimoto’s thyroiditis. The prognosis is favourable as conventional papillary carcinoma. About 8% of Warthin’s tumor are detected in extraparotid locations.

  16. CONGENITAL EPULIS: A RARE BENIGN JAW TUMOR OF NEWBORN

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    Mahavir Singh

    2012-04-01

    Full Text Available A congenital epulis is a rare benign jaw tumor of neonate and is also known as “congenital gingival granular cell tumor”. The Greek word ‘‘epulis’’ means ‘‘swelling of the gingiva’’. It was first described in 1871 by Neumann; hence the alternative name is Neumann’s Tumor. It usually presents at birth with an obvious mass arising from the gingival mucosa of the maxilla or mandible. It commonly presents in neonates, although prenatal diagnosis with ultrasound has been reported as early as 26 weeks gestation.

  17. CONGENITAL EPULIS: A RARE BENIGN JAW TUMOR OF NEWBORN

    OpenAIRE

    2012-01-01

    A congenital epulis is a rare benign jaw tumor of neonate and is also known as “congenital gingival granular cell tumor”. The Greek word ‘‘epulis’’ means ‘‘swelling of the gingiva’’. It was first described in 1871 by Neumann; hence the alternative name is Neumann’s Tumor. It usually presents at birth with an obvious mass arising from the gingival mucosa of the maxilla or mandible. It commonly presents in neonates, although prenatal diagnosis with ultrasound has been reported as early as 26 we...

  18. Supratentorial cortical ependymoma: An unusual presentation of a rare tumor

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    Mohammad Reza Mohaghegh

    2015-01-01

    Full Text Available Ependymomas are glial tumors derived from ependymal cells lining the ventricles and the central canal of the spinal cord. Two thirds of ependymomas arise in the infratentorial or intraventricles, whereas one-third are located in supratentorial space. But supratentorial "cortical" ependymomas are very rare. We report a case of a cortical ependymoma in a 17-year-old boy. The patient presented with transient recurrent right weakness and diplopia. This tumor was located in the left parieto-occipital region and he had gross total excision. Microscopy and immunohistochemistry showed grade III differentiation ependymoma.

  19. Supratentorial cortical ependymoma: An unusual presentation of a rare tumor.

    Science.gov (United States)

    Mohaghegh, Mohammad Reza; Chitsaz, Ahmad; Okhovat, Ali Asghar; Pour, Elnaz Babaei

    2015-01-01

    Ependymomas are glial tumors derived from ependymal cells lining the ventricles and the central canal of the spinal cord. Two thirds of ependymomas arise in the infratentorial or intraventricles, whereas one-third are located in supratentorial space. But supratentorial "cortical" ependymomas are very rare. We report a case of a cortical ependymoma in a 17-year-old boy. The patient presented with transient recurrent right weakness and diplopia. This tumor was located in the left parieto-occipital region and he had gross total excision. Microscopy and immunohistochemistry showed grade III differentiation ependymoma.

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

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    Shrikant V Rege

    2016-01-01

    Full Text Available 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.

  1. Orbital Chondroma: A rare mesenchymal tumor of orbit

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    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.

  2. Congenital Granular Cell Tumor – A Rare Entity

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    Monal Yuwanati

    2015-03-01

    Full Text Available Congenital granular cell tumor is a rare benign neoplastic growth affecting the gingival mucosa of neonates. Prenatal ultrasound diagnosis has recently come to focus and in spite of several reports on immune-histochemical and other advanced marker studies, the cause and origin of the lesion remains debatable till date. Review of literature on prenatal diagnosis and histopathology along with immunohistochemistry is discussed.

  3. Carcinosarcoma of the Pancreas: How a Common Blood Disorder Can Hide an Extremely Rare Tumour

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    Anastasios Katsourakis

    2015-05-01

    Full Text Available Context Sarcomas represent a relatively rare malignancy. Primary sarcomas of the pancreas represent an extremely rare pathology. Case report We report a case of primary pancreatic carcinoma that presented with anaemia. The patient underwent a Kausch-Whipple operation, and, 16 months after the operation, the patient is disease free. Conclusion This unique case describes an extremely rare gastrointestinal tumour that was found during the patient's anaemia assessment.

  4. Incidentally Discovered Extensive Squamous Metaplasia within Borderline Phyllodes Tumor: Presentation of a Rare Tumor.

    Science.gov (United States)

    Uğraş, Nesrin; Tolunay, Şahsine; Öz Atalay, Fatma; Gökgöz, Şehsuvar

    2016-01-01

    Phyllodes tumors are uncommon biphasic fibroepithelial neoplasms of breast, comprising less than 1% of all breast neoplasms. We therefore aimed to present the case with its microscopic findings. In this article, we report a 59-year-old female admitted to the general surgery department with a rapidly, enlarging, palpable mass in right breast. After histopathological examination, it was diagnosed as borderline phyllodes tumor with extensive squamous metaplasia. Metaplastic changes are infrequent in the stromal and epithelial component of these tumors. Extensive squamous metaplasia within phyllodes tumor is rare and may occur in benign, borderline and malign subtypes.

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

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    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.

  6. Extensive squamous metaplasia in a benign phyllodes tumor: A rare case report

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    Harsh Kumar

    2015-01-01

    Full Text Available Phyllodes tumors (PTs with extensive squamous metaplasia is an uncommon biphasic fibroepithelial neoplasms of breast, comprising of <1% of all breast neoplasm′s. In this article, we report a case of a 55-year-old female patient admitted to the General Surgery Department with a rapidly enlarging, palpable mass in right breast. After histopathological examination, it was diagnosed to be as benign PT with extensive squamous metaplasia. Metaplastic changes are known, but infrequent in these tumors. Extensive squamous metaplasia within PT is rare and may occur in benign, borderline and malignant subtypes. There are only a few cases reported in the literature. We therefore, aimed to present this case in view of its extremely rare nature.

  7. Malignant Peripheral Nerve Sheath Tumor of Prostate: A Rare Case Report and Literature Review

    Science.gov (United States)

    Lu, Chih-Cheng; Li, Chien-Feng

    2016-01-01

    A mid-aged male presented with progressive lower urinary tract symptoms (LUTS) for years. Huge prostate with low serum prostate-specific antigen (PSA) level was detected. The specimen from transurethral resection revealed surprising pathology finding as malignant peripheral nerve sheath tumor (MPNST). Considering its huge size (more than 300 gm) and location, we prescribed neoadjuvant chemotherapy firstly. The tumor became regressive and then radical surgical resection was achieved. Adjuvant multimodality treatment including concurrent chemoradiotherapy (CCRT) and target therapy was given. However, he expired about one year later. MPNST originating from prostate is very rare and seldom reported before. We here present this extremely rare disease and share our treatment experience. PMID:27872789

  8. Malignant Peripheral Nerve Sheath Tumor of Prostate: A Rare Case Report and Literature Review

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    Kun-Lin Hsieh

    2016-01-01

    Full Text Available A mid-aged male presented with progressive lower urinary tract symptoms (LUTS for years. Huge prostate with low serum prostate-specific antigen (PSA level was detected. The specimen from transurethral resection revealed surprising pathology finding as malignant peripheral nerve sheath tumor (MPNST. Considering its huge size (more than 300 gm and location, we prescribed neoadjuvant chemotherapy firstly. The tumor became regressive and then radical surgical resection was achieved. Adjuvant multimodality treatment including concurrent chemoradiotherapy (CCRT and target therapy was given. However, he expired about one year later. MPNST originating from prostate is very rare and seldom reported before. We here present this extremely rare disease and share our treatment experience.

  9. Lymphangioma of epidydimis: An extremely rare cause of scrotal swelling

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    K K Pai

    2006-01-01

    Full Text Available Lymphangioma of epidydimis is a very rare cause of scrotal swelling. We report a case of lymphangioma of epidydimis in a 24 year old male. This case is being presented in view of its rarity.

  10. Peripheral primitive neuroectodermal tumors:a rare case report

    Institute of Scientific and Technical Information of China (English)

    Kun Yan; Yanyan Ge; Youhong Ding; Shiguang Yan; Xiangyang Li

    2014-01-01

    We-aimed-to-explore-the-diagnosis-and-treatment-of-peripheral-primitive-neuroectodermal-tumors-(pPNETs).-We-retrospectively-analyzed-the-diagnosis-and-treatment-process-of-a-patient-who-was-diagnosed-with-pPNETs-by-pathology.-This-case-was-a-man-with-soft-masses-arising-from-the-left-chest-wal-near-the-armpit-and-left-supraclavicular-of-a-47-year-old-man.-The-patient-mainly-presented-with-the-masses-which-increasing-gradual-y-with-obvious-pain.-Needle-biopsy-showed-that-they-were-both-metastatic-adenocarcinoma.-Ultrasonography-B-revealed-blood-flow-of-these-two-low-density-placeholders-can-be-seen-in-the-signal,-not-oppression-axil-ary-and-vein.-Radical-resection-of-the-masses-were-performed.-Histopathologic-study-and-immunohistochemistry-(IHC)-confirmed-the-masses-to-be-peripheral-primitive-neuroectodermal-tumors.-pPNETs-is-a-rare-malignant-smal-round-cel-tumor.-CT-and-MRI-examination-can-estimate-the-resectability-of-the-tumor;Ultrasound-B-can-make-sure-its-inside-blood-supply-and-the-positional-relationship-between-the-mass-and-the-surrounding-vasculature.-The-diagnosis-of-pPNETs-is-based-primarily-on-histopathologic-study-and-IHC,-especial-y-those-with-the-characteristics-of-the-Homer-Wright-and-neuroendocrine-markers.-Radical-resection-of-the-tumor-is-the-most-ef-ective-therapeutic-method.-The-ef-ect-of-adjuvant-chemo-radiation-is-worth-af-irmation.-Autologous-stem-cel-rescue-besides-adjuvant-chemotherapy-has-been-associated-with-prolonged-survival.

  11. 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

  12. Keratocystic odontogenic tumor involving the maxillary sinus: A rare entity

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    Mohammad Asif Kiresur

    2016-01-01

    Full Text Available The keratocystic odontogenic tumor (KCOT is a frequently encountered developmental cyst of the jaws. The occurrence of KCOT in the maxillary sinus is rare. The mucosa of the maxillary sinus is susceptible to infections, allergic diseases, and neoplasm. The anatomic position of maxillary premolar and molar teeth is in close contact with the sinus predispose to spreading of pulp and periodontal infection, odontogenic cyst, and tumors to the sinus. Diagnosis and treating KCOT in maxillary sinus is challenging as treatment has to be rendered for sinusitis because of pathology in the sinus and for KCOT. We report a case of 35-year-old female with KCOT involving the lining of the maxillary sinus and put forward hypotheses for the origin of KCOT in the maxillary sinus.

  13. [Solitary fibrous tumor of the pelvis: a rare extrathoracic manifestation].

    Science.gov (United States)

    Gessmann, J; Seybold, D; Helwing, M; Muhr, G; Schildhauer, T A

    2009-07-01

    Solitary fibrous tumors (SFT) are rare spindle cell neoplasms. To date only very few cases of pelvic SFT have been reported in the literature. SFT are characterized by unique microscopic and immunohistochemical findings. Complete local resection is the treatment of choice. Recurrence and metastasis may be related to infrequent malignant histological features, but histology is not always a reliable predictor for prognosis. Therefore long-term follow-up is necessary.We report about a male patient with a malignant pelvic SFT. After complete resection the tumor recurred after a short period of 6 months posterior to the original location in the pelvis. The differential diagnoses and the therapy options are discussed with a review of the present literature.

  14. Congenital granular cell lesion: A rare tumor of new born

    Directory of Open Access Journals (Sweden)

    Rajesh Kumar

    2013-01-01

    Full Text Available Congenital granular cell tumor (CGCT, or congenital epulis, is an uncommon benign soft tissue lesion that usually arises from the alveolar mucosa of neonates and may cause respiratory and feeding problems. The histogenesis and clinical history of the lesion remains obscure. Treatment involves surgical excision and recurrences are rare. The present report describes a case of congenital granular cell lesion (CGCL in the anterior segment of maxillary alveolar ridge of a 2-month-old female. This lesion was causing feeding problem and was excised under local anesthesia, with no recurrence even after 3 years.

  15. Vulvar malignant melanoma: a rare tumor with worse prognosis

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    Swati Singh

    2013-06-01

    Full Text Available Malignant melanoma, which has a highly malignant potential, is a tumor of the skin and mucosal membranes. Malignant melanomas of the female genital tract, including the vulva and vagina, are rare. Their overall prognosis is worse. A 75 year old woman presented with complaint of growth in vulvar region since 4 months. There was history of itching in vulvar region over growth. Surgery is still the best available treatment for the control and potential cure of malignant melanomas [Int J Reprod Contracept Obstet Gynecol 2013; 2(3.000: 494-496

  16. Verrucous carcinoma in external auditory canal: Presentation of an extremely rare case

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    Md Zillur Rahman

    2013-01-01

    Full Text Available Verrucous carcinoma is a variant of squamous cell carcinoma. It is of low grade malignancy and rarely present with distant metastasis. Oral cavity is the commonest site of this tumor, other sites are larynx, oesophagus, and genitalia. Verrucous carcinoma in external auditory canal is extremely rare. This is the presentation of a 45-year-old woman who came to the ENT and Head Neck Surgery department of Delta Medical College with discharg from left ear and impairment of hearing on the same side for 7 years. Otoscopic examination showed that the skin of external auditory canal was thickened, papillary and blackish. External auditory canal bone was found eroded. Cytology from external auditory canal scrap showed hyperkeratosis and parakeratosis. Excision of the external auditory canal mass was done under G/A. Whole skin from external auditory canal was excised under microscope. Split thickness skin grafting was done in external auditory canal. The specimen was sent for histopathological examination which revealed as verrucous carcinoma. Subsequently, she was treated by radiotherapy. Six months follow-up shows no recurrence.

  17. Analogues of atmospheric circulation to probe extreme and rare events

    Science.gov (United States)

    Yiou, P.

    2015-12-01

    Analogues of atmospheric circulation have had many applications, from weather prediction to the downscaling of climate variables. The main assumptions behind this methodology are that climate variables (such as temperature or precipitation) are linked a large-scale atmospheric predictand, which is usually taken as sea-level pressure, and that such predictands recur through time. They offer a possibility to estimate probability distributions of a climate variable, conditional to patterns of atmospheric circulation. In addition, this methodology allows the quantification of unusual weather patterns that have been observed. I will represent a way to use analogues of circulation for the detection/attribution of extreme events of precipitation and temperature. This approach will be illustrated on test cases, including the warm European winter of 2006/2007, the extremes of precipitation over Southern UK and northwestern France in January 2014, and the European summer of 2015. I will show how this analysis provides a low-cost estimate of the fraction of attributable risk (FAR) for extreme events that verify the above mentioned hypotheses. Such an analysis can be performed in continuous time with reanalysis data and meteorological observations.

  18. Gaussians Rarely Extremize Adjoint Fourier Restriction Inequalities For Paraboloids

    CERN Document Server

    Christ, Michael

    2010-01-01

    It was proved independently by Foschi and Hundertmark, Zharnitsky that Gaussians extremize the adjoint Fourier restriction inequality for L^2 functions on the paraboloid in the two lowest-dimesional cases. Here we prove that Gaussians are critical points for the L^p to L^q adjoint Fourier restriction inequalities if and only if p=2. Also, Gaussians are critial points for the L^2 to L^r_t L^q_x Strichartz inequalities for all admissible pairs (r,q) in (1,infinity)^2.

  19. Primitive neuroectodermal tumor in a mixed germ cell tumor - A rare case report

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    Khushboo Dewan

    2015-01-01

    Full Text Available A rare case of testicular tumor in a 20-year-old male with Primitive Neuroectodermal Tumor (PNET was reported. Imaging studies showed a large heterogenous mass in the right scrotal sac and a large retroperitoneal mass with metastasis in the lung and liver. Serum alpha fetoprotein (AFP was markedly elevated with moderate increase in serum β-human chorionic gonadotropin (hCG levels. After orchidectomy, a histological diagnosis of mixed germ cell tumor-teratoma with primitive neuroectodermal, embryonal, and yolk sac components was made. Some scattered embryoid bodies representative of primitive germ cell tumor were also present. Morphological diversity including PNET prompted the authors to report this case as PNET points toward a poor prognosis.

  20. Proliferative ameloblastomatous calcifying cystic odontogenic tumor of the mandible: A rare histological variant

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    Kamala Rawson

    2015-01-01

    Full Text Available Calcifying cystic odontogenic tumor (CCOT is an extremely rare clinical entity. It was considered as a cyst until 2005, when World Health Organization included it among the odontogenic tumors. It presents as a slow-growing, painless swelling of the jaw. Radiographically, it may be seen as a unilocular or multilocular radiolucency. Here, we present a case of a proliferative ameloblastomatous type of CCOT of the left mandible. Conventional radiography and cone beam computed tomography were performed, in which the lesion was found to be unicystic without any sign of calcification. The lesion was also associated with an impacted 37 tooth, which was displaced to the inferior border of the mandible.

  1. A Rare Presternal Soft Tissue Tumor: Ectopic Hamartomatous Thymoma (Branchial Anlage Mixed Tumor).

    Science.gov (United States)

    Reusens, H; Van den Broecke, C; Creytens, D; Fierens, K

    2015-01-01

    Ectopic hamartomatous thymoma is a rare benign tumor in adults, mostly located in the lower neck region. It was first reported in 1982 by Smith and McClure. Histopathologically these tumors are typically well marginated and composed of a mixture of spindle cells, mature adipose tissue, and epithelial cells, including both glandular and squamous elements. The histogenesis of this tumor is controversial. Recently, an origin from a remnant of the cervical sinus of His was proposed. Ectopic hamartomatous thymoma needs to be differentiated from malignant lesions such as synovial sarcomas or malignant peripheral nerve sheath tumors. These tumors can have similar clinical features and radiologic images. Recognition of this tumor is important because it follows a benign clinical course and conservative surgical excision is the treatment of choice. To our knowledge, 61 cases have previously been reported. We present the case of a 45-year-old women with an uncomplicated presternal located ectopic hamartomatous thymoma. The morphological and immunohistochemical findings are discussed and a review of literature is made.

  2. 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.)

  3. Diffuse Hepatic Hemangiomatosis in the Adult without Extra-hepatic Involvement: An Extremely Rare Occurrence.

    Science.gov (United States)

    Batista, Alexandre; Matos, António Pedro; Neta, Jorge Oliveira E; Ramalho, Miguel

    2014-01-01

    We present a case of a pathologically proven multinodular diffuse hepatic hemangiomatosis (DHH) with no extra-hepatic involvement in a 68-year-old male. Cavernous hemangioma is the most common hepatic tumor. However, DHH, which is characterized by extensive replacement of liver parenchyma with hemangiomatous lesions, has been rarely reported in adults. The etiology and clinical course are not completely understood because of its rareness, although the diagnosis might be suggested by the magnetic resonance imaging findings.

  4. 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

  5. Malignant peripheral nerve sheath tumor of the anterior mediastinum: a rare presentation

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    Babusha Kalra

    2014-12-01

    Full Text Available Malignant peripheral nerve sheath tumor (MPNST is a rare tumor that accounts for 5% of all thoracic neoplasm usually located in the posterior mediastinum and is generally associated with a poor outcome. We present a case of MPNST of the anterior mediastinum presenting in a rare location leading to diagnostic dilemmas and treated primarily by surgical resection.

  6. SCHWANNOMA OF THE CERVICAL VAGUS NERVE: A RARE BENIGN NEUROGENIC TUMOR

    Directory of Open Access Journals (Sweden)

    Gokce SIMSEK

    2013-06-01

    Full Text Available A schwannoma, also known as an acoustic neuroma, is a benign nerve sheath tumor composed of schwann cells, which normally produce the insulating myelin sheath covering the peripheral nerves. Schwannoma, originating from the cervical vagus nerve, is an extremely rare neoplasm that usually occurs in men between the 3rd and 6th decades of life. The most common presentation is a painless, slow-growing, lateral neck mass;this appears in a large proportion of cases. Complete surgical resection with care to protect the nerve of origin is the recommended treatment of choice. Here, we report a case of cervical vagal schwannoma in a 55-year-old male who admitted with the complaint of a firm and painless mass lesion on the right side of the neck. The management of the case is discussed along with the relevant literature.

  7. Solitary fibrous tumor of the hard palate: a rare entity in oral cavity

    OpenAIRE

    Pinto, Antonione Santos Bezerra; Department of Oral Pathology – São Leopoldo Mandic Institute and Research Center – Campinas – SP – Brazil.; De Araújo, Vera Cavalcanti; Department of Oral Pathology – São Leopoldo Mandic Institute and Research Center – Campinas – SP – Brazil.; Santos, Fabrício Passador; Department of Oral Pathology – São Leopoldo Mandic Institute and Research Center – Campinas – SP – Brazil.; Filho, José Ferreira de Menezes; Antonio Carlos Institute of Tocantins – Araguaína – TO – Brazil.; Siqueira, Viviane; Department of Oral Pathology – São Leopoldo Mandic Institute and Research Center – Campinas – SP – Brazil.; Soares, Andresa Borges; Department of Oral Pathology – São Leopoldo Mandic Institute and Research Center – Campinas – SP – Brazil.

    2016-01-01

    A case of solitary fibrous tumor is reported. Solitary fibrous tumor is a rare neoplasia characterized by the proliferation of fusiform cells of mesenchymal origin accounting for at least 2% of all soft tissue tumors. In this present case, the initial diagnosis was salivary gland tumor because of the location in the hard palate. Histologically, the tumor was composed by conjunctive tissue with proliferation of oval and fusiform cells. The immunohistochemical analysis was positive for CD34 and...

  8. Cerebellar metastases of recurrent phyllodes tumor breast; a rare phenomenon reflecting the unpredictable outcome.

    Science.gov (United States)

    Singh, Jyotsna; Majumdar, Kaushik; Gupta, Rahul; Batra, Vineeta Vijay

    2015-01-01

    Carcinomas of lung, breast, colon, kidney, and malignant melanomas are the most common malignancies that metastasize to the central nervous system (CNS). Phyllodes tumor is a rare fibroepithelial tumor of the breast, often having unpredictable recurrences, with increasing histological grade and distant metastasis. Malignant forms exist, which may develop distant metastases usually to the lung, pleura, bone, and liver. CNS metastasis of phyllodes tumor is rare and associated with a poor prognosis, with resistance to chemotherapy and radiation. We present a rare case of cerebellar metastasis in recurrent phyllodes tumor breast with subsequent rapid downhill course.

  9. The inside mystery of jejunal gastrointestinal stromal tumor: a rare case report and review of the literature.

    Science.gov (United States)

    Dhull, A K; Kaushal, V; Dhankhar, R; Atri, R; Singh, H; Marwah, N

    2011-01-01

    Gastrointestinal stromal tumors (GISTs) are malignant and rare form of soft tissue sarcoma of the digestive tract. The incidence of gastrointestinal stromal tumors is very low Kramer et al. 2005 Jejunal GISTs are extremely rare. Here we present a rare case of jejunal GIST with unusually large size at presentation. The patient presented with severe abdomen pain, exophytic growth, and dimorphic anemia. Surgical resection of the tumor was carried out, and operative findings revealed a 15 × 10 cm growth, arising from serosal surface of jejunum, at the antimesenteric surface. Diagnosis in this case was made by subjecting the resected specimen to immunohistochemical analysis. In view of large size of the resected tumor, and high-risk histopathological features, imatinib mesylate 400 mg once daily was given as adjuvant chemotherapy. Patient is asymptomatic without any evidence of tumor recurrence after six months of postoperative followup. Imatinib as such is recommended in metastatic, residual or recurrent cases of GISTs or which are surgically not removable; however, recent recommendations suggests the use of imatinib mesylate after radical surgery in high-risk cases, because it has shown a significant decrease in the recurrence rate, and the Food and Drug Administration (FDA) has also approved the use of imatinib as adjuvant therapy after complete resection of localized, primary GIST.

  10. The Inside Mystery of Jejunal Gastrointestinal Stromal Tumor: A Rare Case Report and Review of the Literature

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    A. K. Dhull

    2011-01-01

    Full Text Available Gastrointestinal stromal tumors (GISTs are malignant and rare form of soft tissue sarcoma of the digestive tract. The incidence of gastrointestinal stromal tumors is very low Kramer et al. 2005 Jejunal GISTs are extremely rare. Here we present a rare case of jejunal GIST with unusually large size at presentation. The patient presented with severe abdomen pain, exophytic growth, and dimorphic anemia. Surgical resection of the tumor was carried out, and operative findings revealed a 15 × 10 cm growth, arising from serosal surface of jejunum, at the antimesenteric surface. Diagnosis in this case was made by subjecting the resected specimen to immunohistochemical analysis. In view of large size of the resected tumor, and high-risk histopathological features, imatinib mesylate 400 mg once daily was given as adjuvant chemotherapy. Patient is asymptomatic without any evidence of tumor recurrence after six months of postoperative followup. Imatinib as such is recommended in metastatic, residual or recurrent cases of GISTs or which are surgically not removable; however, recent recommendations suggests the use of imatinib mesylate after radical surgery in high-risk cases, because it has shown a significant decrease in the recurrence rate, and the Food and Drug Administration (FDA has also approved the use of imatinib as adjuvant therapy after complete resection of localized, primary GIST.

  11. 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

  12. Well differentiated neuroendocrine tumor of the kidney: Report of a rare case with review of literature

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    Kavita Mardi

    2017-01-01

    Full Text Available Neuroendocrine tumors (NETs are uncommon tumors that exhibit a wide range of neuroendocrine differentiation and biological behavior. Primary NETs of the kidney, including carcinoid tumor, small cell carcinoma (SCC, and large cell neuroendocrine carcinoma (LCNEC are exceedingly rare. Renal carcinoids are typically slow-growing tumors and pursue a variable clinical course. In contrast, SCC and LCNEC often present with locally advanced or metastatic disease and carry a poor prognosis. We herein report a rare cases of well-differentiated NET (carcinoid in a 39-year-old male along with the immunohistochemical features. The rarity of these tumors poses a diagnostic and therapeutic challenge.

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

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    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.

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

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    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

  15. 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.

  16. An Extremely Rare Anatomical Variation: Abnormal Drainage of the Anterior Interventricular Coronary Vein

    Science.gov (United States)

    Okur, Aylin; Sade, Recep; Ogul, Hayri; Karaca, Leyla; Kantarci, Mecit

    2016-01-01

    Variation of anterior interventricular vein draining into the left atrium is an extremely rare occurrence. Multidetector computed tomography (MDCT) coronary angiography has recently become the gold standard for depicting anatomical variations and anomalies of coronary arteries and veins. We herein have reported the case of a 36-year-old male whose anterior interventricular vein draining into the left atrium was demonstrated by MDCT coronary angiography. PMID:27122958

  17. Intestinal anisakiasis can cause intussusception in adults:An extremely rare condition

    Institute of Scientific and Technical Information of China (English)

    Tomofumi; Miura; Akira; Iwaya; Takao; Shimizu; Junpei; Tsuchiya; Junichiro; Nakamura; Satoshi; Yamada; Tsutomu; Miura; Masahiko; Yanagi; Hiroyuki; Usuda; Iwao; Emura; Toru; Takahashi

    2010-01-01

    We report an extremely rare case of adult intussusception caused by anisakiasis. A 41-year-old man was admitted into our hospital for right lower abdominal colicky pain. Ultrasonography and computed tomography revealed the presence of intussusception. As pneumodynamic resolution by colonoscopy failed, surgery was performed. The anisakis body was found in the submucosal layer of the resection specimen. The patient was discharged 9 d after the operation. Anisakiasis may cause intussusception in any country wh...

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

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    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.

  19. Primary Dural Spinal Lymphoma Presentation of a Rare Spinal Tumor Case

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    Dilber Ayçiçek Çeçen

    2015-01-01

    Full Text Available Background. Primary spinal dural lymphomas (PSDL are tumors with characteristic histopathology of a lymphoma, which are completely in the spinal epidural space without any other systemic involvement. Extranodal primary lymphoma involving nervous system prefers thalamus/basal ganglia, periventricular region, cerebellum, eyes, meninges/dura, and cranial nerves or spinal cord. Rare spinal localization with acute spinal cord compression is worth attention. Case Presentation. A 48-year-old male presented with a several-month-long history of upper back pain. Lately, he had numbness and weakness at both lower extremities and was unable to walk for one week. A spinal MRI showed a thoracic lesion with cord compression at T2–T4 levels. The patient underwent surgical decompression, with his final histopathology showing diffuse large B-cell lymphoma. Systemic work-up was negative for nodal disease. Following surgery, he received radiotherapy combined with chemotherapy. He experienced a good outcome after four years. Conclusion. The upper thoracic cord is a rare location for primary spinal lesions/metastases, both of which prefer the lower thoracic and upper lumbar regions. In cases of progressive paraparesis, there should be immediate surgical intervention in the case of denovo disease, followed by combined radiotherapy and chemotherapy procedures.

  20. Cystic tumor of papillary muscle of heart: a rare finding in sudden death.

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    Murty, O P

    2009-06-01

    Primary cystic tumors of papillary muscles of the heart are extremely rare. Here, one case of unusual cystic tumor in papillary muscle of the heart in a 37-year-old Myanmar migrant worker has been reported. He came to Malaysia 2 weeks before and one morning was found dead in sleep. Autopsy revealed cystic lesion in the papillary muscle of the mitral valve of heart, which was prolapsing into ventricular cavity. The cyst had white-jelly like sticky mucus material. The cyst was present in papillary muscle with slight invasion in septum area; it was lined by cuboidal-columnar epithelium and contained mucinous contents. There was no evidence of an inflammatory reaction in the cyst and in cardiac muscles. In addition to cystic neoplasm, the deceased also had histoplasmosis of the lungs. The case is presented with macroscopic and microscopic photographs of the cyst and histoplasmosis of the lungs. This case is reported because of its rarity, unique position, and unusual appearance.

  1. Squash preparation of a malignant triton tumor in a rare location

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    Sundaram Sandhya

    2008-01-01

    Full Text Available Malignant peripheral nerve sheath tumors (MPNST are rare malignant mesenchymal neoplasms of neural origin. Malignant peripheral nerve sheath tumors arising in a cranial nerve are rare with only a few cases being reported in literature. An MPNST with rhabdomyosarcomatous differentiation is also known as malignant triton tumor (MTT. MTT has a worse prognosis than the classic MPNST. The cytomorphological patterns of these tumors are insufficiently documented in literature. We present here the cytohistological features of an MPNST with focal rhabdomyomatous differentiation arising in the trigeminal nerve, which was confirmed by immunohistochemistry.

  2. Rare Solid Tumors of the Pancreas as Differential Diagnosis of Pancreatic Adenocarcinoma

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    Sabine Kersting

    2012-05-01

    Full Text Available Context Rare solid tumors of the pancreas can be misinterpreted as primary pancreatic cancer. Objective The aim of this study was to report our experience in the treatment of patients with rare tumor lesions of the pancreas and to discuss clinical and pathological characteristics in the context of the role of surgery. Design Data from patients of our prospective data-base with rare benign and malignant tumors of the pancreas, treated in our division from January 2004 to August 2010, were analyzed retrospectively. Results One-thousand and ninety-eight patients with solid tumors of the pancreas underwent pancreatic surgery. In 19 patients (10 women, 9 men with a mean age of 57 years (range: 20-74 years rare pancreatic tumors (metastasis, solid pseudopapillary tumor, teratoma, hemangioma, accessory spleen, lymphoepithelial cyst, hamartoma, sarcoidosis, yolk sac tumor were the reason for surgical intervention. Conclusion If rare benign and malignant pancreatic tumors, intrapancreatic metastasis, as well as pancreatic malformations or other abnormalities, present themselves as solid masses of the pancreas, they constitute an important differential diagnosis to primary pancreatic neoplasia, e.g. pancreatic ductal adenocarcinoma. Clinical imaging techniques cannot always rule out malignancy, thus operative exploration often remains the treatment of choice to provide the correct diagnosis and initiate adequate surgical therapy.

  3. Malignant Peripheral Nerve Sheath Tumor in the Maxilla: Report of a Rare Case

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    Jahanshah Salehinejad

    2013-12-01

    Full Text Available Malignant peripheral nerve sheath tumor (MPNST is a rare malignant tumor that develops either from a preexisting neurofibroma or de novo. The cell of origin is believed to be the Schwann cell and possibly other nerve sheath cells. In this report, we describe a rare case of MPNST that arise from the socket of second left maxillary molar that has been already extracted in a young man. He was referred to a dentist’s office with a tumor-like mass of soft tissue on his left maxillary gingiva. Biopsy and histopathologic examination was performed and based on histologic and immuno-histochemical findings, the diagnosis of MPNST was made. MPNST is a rare malignant tumor in the oral cavity. Dentists must be careful and conscious because this rare malignancy can occur in gingiva and can mimic the clinical feature of any benign gingival enlargements.

  4. Malignant Peripheral Nerve Sheath Tumor in the Maxilla: Report of a Rare Case

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    Jahanshah Salehinejad

    2013-01-01

    Full Text Available Malignant peripheral nerve sheath tumor (MPNST is a rare malignant tumor that develops either from a preexisting neurofibroma or de novo. The cell of origin is believed to be the Schwann cell and possibly other nerve sheath cells. In this report, we describe a rare case of MPNST that arise from the socket of second left maxillary molar that has been already extracted in a young man. He was referred to a dentist’s office with a tumor-like mass of soft tissue on his left maxillary gingiva. Biopsy and histopathologic examination was performed and based on histologic and immuno-histochemical findings, the diagnosis of MPNST was made. MPNST is a rare malignant tumor in the oral cavity. Dentists must be careful and conscious because this rare malignancy can occur in gingiva and can mimic the clinical feature of any benign gingival enlargements.

  5. Sinonasal Phosphaturic Mesenchymal Tumor: A Rare and Misinterpreted Entity

    Science.gov (United States)

    Arnaoutakis, Demetri; Naseri, Iman

    2015-01-01

    Objectives Oncogenic osteomalacia is a paraneoplastic syndrome in which the tumor secretes a peptide-like hormone, fibroblast growth factor, resulting in urinary loss of phosphates. Methods We present the case of a 50-year-old woman with a benign phosphaturic mesenchymal tumor (PMT) involving the ethmoid sinus with obstruction of the ostiomeatal complex causing unilateral nasal airway obstruction. Results The tumor was initially thought to be an esthesioneuroblastoma based on primary pathology interpretation and on clinical and radiographic appearance. However, a benign PMT was later confirmed by further testing. Conclusion The tumor was removed entirely by the endoscopic transnasal approach, leading to a full resolution of symptoms. PMID:26623233

  6. Nivolumab and Ipilimumab in Treating Patients With Rare Tumors

    Science.gov (United States)

    2016-08-24

    Acinar Cell Carcinoma; Adrenal Cortex Carcinoma; Adrenal Gland Pheochromocytoma; Anal Canal Neuroendocrine Carcinoma; Anal Canal Undifferentiated Carcinoma; Appendix Mucinous Adenocarcinoma; Bladder Adenocarcinoma; Bronchioloalveolar Carcinoma; Cervical Adenocarcinoma; Cervical Squamous Cell Carcinoma, Not Otherwise Specified; Cholangiocarcinoma; Chordoma; Colorectal Squamous Cell Carcinoma; Endometrial Adenocarcinoma; Endometrioid Adenocarcinoma; Esophageal Neuroendocrine Carcinoma; Esophageal Undifferentiated Carcinoma; Extrahepatic Bile Duct Carcinoma; Fallopian Tube Adenocarcinoma; Fibromyxoid Tumor; Gastric Neuroendocrine Carcinoma; Gastric Squamous Cell Carcinoma; Giant Cell Carcinoma; Intestinal Neuroendocrine Carcinoma; Intrahepatic Cholangiocarcinoma; Lung Carcinoid Tumor; Lung Sarcomatoid Carcinoma; Major Salivary Gland Carcinoma; Malignant Odontogenic Neoplasm; Malignant Peripheral Nerve Sheath Tumor; Malignant Skin Neoplasm; Malignant Testicular Sex Cord-Stromal Tumor; Metastatic Malignant Neoplasm of Unknown Primary Origin; Mixed Mesodermal (Mullerian) Tumor; Mucinous Adenocarcinoma; Mucinous Cystadenocarcinoma; Nasal Cavity Adenocarcinoma; Nasal Cavity Carcinoma; Nasopharyngeal Carcinoma; Nasopharyngeal Papillary Adenocarcinoma; Nasopharyngeal Undifferentiated Carcinoma; Oral Cavity Carcinoma; Oropharyngeal Undifferentiated Carcinoma; Ovarian Adenocarcinoma; Ovarian Germ Cell Tumor; Ovarian Mucinous Adenocarcinoma; Ovarian Squamous Cell Carcinoma; Pancreatic Acinar Cell Carcinoma; Pancreatic Neuroendocrine Carcinoma; Paraganglioma; Paranasal Sinus Adenocarcinoma; Paranasal Sinus Carcinoma; Parathyroid Gland Carcinoma; Pituitary Gland Carcinoma; Placental Choriocarcinoma; Placental-Site Gestational Trophoblastic Tumor; Primary Peritoneal High Grade Serous Adenocarcinoma; Pseudomyxoma Peritonei; Scrotal Squamous Cell Carcinoma; Seminal Vesicle Adenocarcinoma; Seminoma; Serous Cystadenocarcinoma; Small Intestinal Adenocarcinoma; Small Intestinal Squamous

  7. Diffuse Cavernous Hemangioma of the Penis, Scrotum, Perineum, and Rectum - A rare tumor

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    Rastogi Rajul

    2008-01-01

    Full Text Available Hemangiomas are benign lesions that occur in any part of the body. Genital hemangioma involving the entire penis and scrotum are extremely rare. More rarely they can extend in to the pelvis making preoperative imaging imperative and decisive in treatment. Very few cases have been reported in the medical literature. Hereby, a rare cavernous hemangioma that involves the entire penis, scrotum and extends into perineum and rectum in an 18-year-old male is presented with review of literature.

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

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    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.

  9. Epithelioid trophoblastic tumor and its diagnostic dilemmas: A rare case report.

    Science.gov (United States)

    Madhu, Bagaria; Gerbi, Rada; Nabila, Rasool

    2011-01-01

    ►This case is an unusual presentation of a rare disease entity. ►The diagnosis of epithelioid trophoblastic tumor can be challenging resulting in delay in diagnosis. ►Epithelioid trophoblastic tumor can have a very aggressive course, especially in metastatic disease.

  10. Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Kidney: A Rare and Lethal Entity.

    Science.gov (United States)

    Celli, Romulo; Cai, Guoping

    2016-03-01

    Ewing sarcoma/primitive neuroectodermal tumor represents a spectrum of undifferentiated tumors with similar biology that together represent the second most common sarcoma in the pediatric-young adult age range. Very rarely, this tumor presents as a primary neoplasm of the kidney. The clinical presentation of this tumor is not specific, and other renal tumors may present with a similar histologic appearance. Establishing the correct diagnosis is critical because renal Ewing sarcoma/primitive neuroectodermal tumor carries a strikingly dismal prognosis and thus dictates a specific treatment strategy. A low threshold for the use of ancillary molecular tests is recommended, particularly in diagnostically problematic cases. Important considerations with regards to morphology, immunohistochemistry, and molecular alterations will be reviewed here and should be taken into account before rendering this rare and lethal diagnosis.

  11. 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

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

    Directory of Open Access Journals (Sweden)

    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

  13. Gastrointestinal stromal tumor (GIST of the Treitz’s angle– a very rare cause of high bowel obstruction

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    Mădălina Elena Tobă

    2016-10-01

    Full Text Available Gastrointestinal stromal tumors (GIST are somewhat rare gastrointestinal tumors - approximately 1% to 3% incidence, but they are the most common mesenchymal neoplasms of the gastrointestinal tract. GISTs are usually found in the stomach or small intestine but can occur anywhere within the gastrointestinal tract, even in extremely uncommon locations like duodeno-jejunal flexure. Only 3% – 5% of GISTs are located in the duodenum and tumors occurring in the angle of Treitz are even rarer, most published studies being case reports. These tumors have a size ranging from small lesions to large masses and can cause digestive bleeding or high bowel obstruction. This paper is a case presentation illustrating an emergency situation involving a high bowel obstruction caused by a small tumor with an unusual location in the Treitz’s angle. A large percentage of duodenal GISTs are localized in the third and fourth part of the duodenum and may not be found through standard upper endoscopy; only the barium study of the upper gastrointestinal tract highlights the obstruction point. Preoperative diagnosis is difficult but non-invasive imaging techniques like ultrasonography and computed tomography of the abdomen can be helpful. Recently, targeted therapy with inhibitors of tyrosine kinase receptors (IMATINIB has been introduced for the management of advanced and metastatic tumors. In our opinion the surgical resection with curative intent is the treatment of choice.

  14. Retroperitoneal primitive neuroectodermal tumor in an adult: A rare case report and review of the literature

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    Kavita Mardi

    2016-01-01

    Full Text Available Primitive neuroectodermal tumor (PNET and Ewing's sarcoma (EWS are small round cell tumors occurring mainly in children and adolescents. Their occurrence in adults is rare. The abdominal cavity and retroperitoneal PNET/EWS are also relatively rare, grow rapidly in size, compressing surrounding organs/large vessels, and make surgical resection difficult. We report one such rare occurrence of a retroperitoneal PNET in 41-year-old male who presented with abdominal pain and constipation. Contrast enhanced computed tomography abdomen showed large lobulated necrotic hypodense enhancing lesion extending from epigastrium to hypogastrium and involving entire abdomen. Excision of retroperitoneal mass with omentectomy was done. Microscopic examination revealed a malignant small round cell tumor with homer wright rosettes and the tumor cells were positive for CD99.

  15. Synchronous multicentric desmoid tumors (aggressive fibromatosis) of the extremities

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    Sundaram, M.; Duffrin, H.; Vas, W.; McGuire, M.H.

    1988-01-01

    Synchronous multicentric aggressive fibromatosis does not appear to have been previously reported. Two such cases are described. The tumors were identified by magnetic resonance (MR) imaging. The incidence of synchronous multicentric aggressive fibromatosis is not known. It is anticipated that increased use of coronal MR imaging will reveal more tumors of this type, both synchronous and metachronous. In a patient with known or suspected aggressive fibromatosis, every other soft tissue nodule or mass in the same limb has to be regarded as an additional tumor of the same histology. (orig.)

  16. Malignant rhabdoid tumour of kidney - a rare aggressive tumor

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    Krishna Shetty MV

    2016-01-01

    Full Text Available Malignant rhabdoid tumour of kidney is a rare highly aggressive neoplasm of childhood. We present the case of a 18-months old girl presenting with decreased appetite, abdominal distention of 20 days duration and 3 episodes of haematuria. The patient underwent left radical nephrectomy and histopathological examination of the excised specimen confirmed the diagnosis of malignant rhabdoid tumour of the kidney. This case highlights the need to consider malignant rhabdoid tumour of the kidney of possibility young children in presenting with a renal mass.

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

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    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.

  18. Marrow signal mimicking tumor on MRI T1-weighted imaging after neoadjuvant chemotherapy in extremity osteosarcomas

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    Zhiping Deng

    2017-03-01

    Conclusions: Neoadjuvant chemotherapy for extremity osteosarcoma can result in a variety of changes of the MRI appearance of tumor and adjacent bone and marrow. Areas of signal change beyond the tumor that represent marrow conversion and not tumor progression appear on T1 weighted imaging to be lower in signal than subcutaneous fat and higher in signal than muscle. Recognizing the existence of the effect of neoadjuvant chemotherapy on the MR appearance of the tumor and surrounding bone and myeloid elements is important so as to plan for oncological sound tumor resections while avoiding resecting more normal bone than necessary.

  19. Lower Extremity Arterial Occlusive Disease As a Rare Complication of Crohn's Disease

    Institute of Scientific and Technical Information of China (English)

    Wei-wei Wu; Xue-ying Jiang; Chang-wei Liu; Yong-jun Li; Rong Zeng

    2009-01-01

    Objective To investigate the dinical characteristics and treatment strategy of lower extremity arterial occlusive disease in patients with Crohn's disease(CD).Methods Clinical information of 9 cases suffering from lower extremity arterial occlusion and CD was investigated retrospectively.Results All the cases were less than 50 years old and the most were females(8/9).Arterial occlusions occurred in either active(5/9)or inactive(4/9)stage of CD.Besides the arteries of lower extremities,other arteries could also be involved such as aorta,iliac artery,renal artery or mesentery artery.Seven cases had atherosclerotic imaging findings(4 had aortic plaques and 6 had iliac artery stenoses).Embolectomy or thromboendarterectomy were mostly performed.Four(44.4% )cases had recurrent lower limb ischemia.Conclusions Arterial occlusive disease is a rare extraintestinal manifestation of CD.A thorough inspection of aorta is necessary.Embolectomy is mostly preferred.Anticoagulation treatment is highly recommended after the operation.

  20. A Rare Malignant Peripheral Nerve Sheath Tumor of the Maxilla Mimicking a Periapical Lesion

    Science.gov (United States)

    Álvares, Pamella; Silva, Luciano; Pereira dos Santos Neto, Alexandrino; Rodrigues, Cleomar Donizeth; Caubi, Antônio; Silveira, Marcia; Sayão, Sandra; Sobral, Ana Paula

    2016-01-01

    Malignant peripheral nerve sheath tumor is a malignant neoplasm that is rarely found in the oral cavity. About 50% of this tumor occurs in patients with neurofibromatosis type I and comprises approximately 10% of all soft tissue sarcomas of head and neck region. Intraosseous malignant peripheral nerve sheath tumor of the maxilla is rare. This article is the first to address malignant peripheral nerve sheath tumor of the maxilla presenting as a periapical radiolucency on nonvital endodontically treated teeth in the English medical literature. Surgical approaches to malignant soft tissue tumor vary based on the extent of the disease, age of the patient, and pathological findings. A rare case of intraosseous malignant peripheral nerve sheath tumor is reported in a 16-year-old woman. The patient presented clinically with a pain involving the upper left incisors region and with defined unilocular periapical radiolucency lesion involved between the upper left incisors. An incisional biopsy was made. Histological and immunohistochemical examination were positive for S-100 protein and glial fibrillary acidic protein showed that the lesion was an intraosseous malignant peripheral nerve sheath tumor of the maxilla. Nine years after the surgery, no regional recurrence was observed. PMID:27994888

  1. A Rare Malignant Peripheral Nerve Sheath Tumor of the Maxilla Mimicking a Periapical Lesion

    Directory of Open Access Journals (Sweden)

    José Alcides Arruda

    2016-01-01

    Full Text Available Malignant peripheral nerve sheath tumor is a malignant neoplasm that is rarely found in the oral cavity. About 50% of this tumor occurs in patients with neurofibromatosis type I and comprises approximately 10% of all soft tissue sarcomas of head and neck region. Intraosseous malignant peripheral nerve sheath tumor of the maxilla is rare. This article is the first to address malignant peripheral nerve sheath tumor of the maxilla presenting as a periapical radiolucency on nonvital endodontically treated teeth in the English medical literature. Surgical approaches to malignant soft tissue tumor vary based on the extent of the disease, age of the patient, and pathological findings. A rare case of intraosseous malignant peripheral nerve sheath tumor is reported in a 16-year-old woman. The patient presented clinically with a pain involving the upper left incisors region and with defined unilocular periapical radiolucency lesion involved between the upper left incisors. An incisional biopsy was made. Histological and immunohistochemical examination were positive for S-100 protein and glial fibrillary acidic protein showed that the lesion was an intraosseous malignant peripheral nerve sheath tumor of the maxilla. Nine years after the surgery, no regional recurrence was observed.

  2. A CRISPR/Cas9 Functional Screen Identifies Rare Tumor Suppressors

    Science.gov (United States)

    Katigbak, Alexandra; Cencic, Regina; Robert, Francis; Sénécha, Patrick; Scuoppo, Claudio; Pelletier, Jerry

    2016-01-01

    An enormous amount of tumor sequencing data has been generated through large scale sequencing efforts. The functional consequences of the majority of mutations identified by such projects remain an open, unexplored question. This problem is particularly complicated in the case of rare mutations where frequency of occurrence alone or prediction of functional consequences are insufficient to distinguish driver from passenger or bystander mutations. We combine genome editing technology with a powerful mouse cancer model to uncover previously unsuspected rare oncogenic mutations in Burkitt’s lymphoma. We identify two candidate tumor suppressors whose loss cooperate with MYC over-expression to accelerate lymphomagenesis. Our results highlight the utility of in vivo CRISPR/Cas9 screens combined with powerful mouse models to identify and validate rare oncogenic modifier events from tumor mutational data. PMID:27982060

  3. Malignant Triton tumor: a rare cause of sciatic pain and foot drop.

    Science.gov (United States)

    Gomes, Maribel R; Sousa, Alexandre M P; Couto, Roberto J A; Oliveira, Marco M B; Moura, João L M; Vilela, Carlos A

    2017-01-01

    Malignant peripheral nerve sheath tumors (MPNST) are very rare and are frequently localized in the buttocks, thigh, arm, or paraspinal region; one variant is the malignant Triton tumor, with rhabdomyosarcomatous differentiation. The authors present a challenging differential diagnosis of a sciatic pain and foot drop in a woman with history of lumbar disk herniation, which was found to be caused by a Triton tumor of the sciatic nerve. She underwent surgical excision, followed by radiation and chemotherapy. Malignant Triton tumor cases have rarely been described and reported in the literature. The recommended treatment is radical excision followed by high-dose radiotherapy and chemotherapy. The prognosis, although poor, depends on the location, grade, and completeness of surgical margins.

  4. Non-functional parathyroid gland carcinoma, a rare malignant tumor of the head and neck.

    Science.gov (United States)

    Kotromanović, Zeljko; Birtić, Darija; Vceva, Andrijana; Medić, Darija; Zubcić, Zeljko; Mihalj, Hrvoje; Kotromanović, Zdenka; Erić, Suzana; Dmitrović, Branko; Stefanić, Mario

    2012-11-01

    Carcinoma of the parathyroid gland is a very rare tumor of the head and neck. The largest number of carcinomas are discovered by chance. (intraoperatively, during surgery removal of the parathyroid gland are adenomas). Around 1% of the primary parathyreoidism is caused by the cancer of parathyroid glands. Only 10% of these rare tumors make up dysfunctional cancer of parathyroid glands. There have been 24 cases reported of this disease in the literature. The focus of our study is to present a case of this disease and to review the published literature to date.

  5. Importance of rare gene copy number alterations for personalized tumor characterization and survival analysis.

    Science.gov (United States)

    Seifert, Michael; Friedrich, Betty; Beyer, Andreas

    2016-10-03

    It has proven exceedingly difficult to ascertain rare copy number alterations (CNAs) that may have strong effects in individual tumors. We show that a regulatory network inferred from gene expression and gene copy number data of 768 human cancer cell lines can be used to quantify the impact of patient-specific CNAs on survival signature genes. A focused analysis of tumors from six tissues reveals that rare patient-specific gene CNAs often have stronger effects on signature genes than frequent gene CNAs. Further comparison to a related network-based approach shows that the integration of indirectly acting gene CNAs significantly improves the survival analysis.

  6. 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

  7. Squamous Odontogenic Tumor with Unusual Localization and Appearance: A Rare Case Report

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    Sucheta Bansal

    2013-01-01

    Full Text Available Squamous odontogenic tumor is a rare benign neoplasm and may affect multiple sites in the mouth. SOT was first described by Pullon et al. (1975. Since then, there have been less than 50 reported cases. The tumor is often asymptomatic, although it can present with symptoms of pain and tooth mobility. We report a case of SOT occurring in a 26-year-old female in the anterior mandible with unusual localization and appearance.

  8. CBS 78 - A second extremely rare DBZ degenerate star with accreted calcium

    Science.gov (United States)

    Sion, E. M.; Shipman, H. L.; Wagner, R. M.; Starrfield, S. G.; Liebert, J.

    1986-09-01

    The paper reports the discovery of a second, extremely rare DBZ degenerate CBS 78 which reveals photospheric calcium in its nearly pure helium atmosphere. The presence of calcium strongly suggests accretion of interstellar matter as its origin, but hydrogen is not detected. The properties of CBS 78 are compared with other cool helium-rich degenerates with metals. The suggested existence of an empirical correlation between the hydrogen-to-calcium abundance ratio and effective temperature is confirmed. This correlation shows that above a critical temperature of about 11,000 K, a screening mechanism is operative which prevents accretion of interstellar hydrogen onto white dwarfs; this mechanism apparently does not operate at lower temperatures.

  9. 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

    2016-01-01

    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.

  10. Urethral protrusion of the abdominal catheter of ventriculoperitoneal shunt: Case report of extremely rare complication

    Directory of Open Access Journals (Sweden)

    Ugur Yazar

    2012-01-01

    Full Text Available Hydrocephalus in its various forms constitutes one of the major problems in pediatric neurosurgical practice. The placement of a ventriculoperitoneal (VP shunt is the most common form of treatment for hydrocephalus, so that all neurosurgeons struggle with shunt malfunctions and their complications. Well-known complications are connected with the use of the valve systems (malfunction, infectious, overdrainage, secondary craniosynostosis, etc.. We report an unusual case of protruding abdominal catheter from the urethra. This girl had received a VP shunt for hydrocephalus following surgery of posterior fossa medulloblastoma 4 years ago. After admission, the entire system was removed, antibiotic treatment was administered for 2 weeks, and a new VP shunt was placed. The postoperative course was uneventful. This complication is extremely rare.

  11. New approaches for rainfall ensemble post-processing with a focus on extreme and rare events

    Science.gov (United States)

    Taillardat, Maxime; Mestre, Olivier; Fougères, Anne-Laure; Naveau, Philippe

    2017-04-01

    Ensemble forecasts of rainfall are very important for decision making such as storm warnings or flood risk downstream watersheds. We present a statistical post-processing method based on an extension of Quantile Regression Forests (QRF) for heavy-tailed distributions. Our proposed method is applied to daily 51-h forecasts of 6-h accumulated precipitation from 2012 to 2015 over France using the Météo-France ensemble prediction system called PEARP. It provides calibrated predictive distributions and competes favourably with methods like Analog method or EMOS, which we try to improve using some new predictors and distributions derived from hydrology. We also discuss about evaluation of ensemble forecasts for extreme and rare events. Our goal is to improve drastically ensemble quality for these events subject to a good overall performance.

  12. Eosinophilia as the presenting sign in pancreatic cancer: an extremely rare occurrence.

    Science.gov (United States)

    Ibrahim, Uroosa; Asti, Divya; Saqib, Amina; Mudduluru, Bindu Madhavi; Ayaz, Sadaf; Odaimi, Marcel

    2017-04-01

    A case of pancreatic adenocarcinoma diagnosed following work up for eosinophilia is reported. A 68-year-old female was referred to our Hematology clinic for an absolute eosinophil count of 1869 per microliter. No allergic signs or symptoms were reported. Laboratory studies for parasitic infestations autoimmune disease and collagen vascular disease were negative. Computed tomography of the abdomen revealed a mass in the neck of the pancreas with fine needle aspiration biopsy consistent with adenocarcinoma. The patient received one cycle of modified FOLFIRINOX with complete resolution of the eosinophilia. There are rare case reports of tumor-associated blood eosinophilia in solid malignancies. The finding may be indicative of rapid disease progression and poor prognosis. Our case is the third in published English literature with eosinophilia being the initial finding in pancreatic cancer.

  13. 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.

  14. Foreign body granuloma mimicking recurrent intracranial tumor: a very rare clinical entity.

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    Askin Esen Hasturk

    2013-11-01

    Full Text Available Gelatin sponge, oxidized cellulose and microfibrillar collagen are used to achieve hemostasis during neurosurgical procedures. Hemostatic agents may produce clinically symptomatic, radiologically apparent mass lesions. The differential diagnosis should include the foreign body along with recurrent tumor. We present a case of intracranial hemostatic agents found in a 56-year-old male patient seven years after undergoing a craniotomy for a left posterior parietal convexity meningioma. Preoperative magnetic resonance imaging (MRI suggested the presence of a recurrent tumor. We emphasize that although it is rare, a granuloma due to a foreign body reaction can result in a false image of tumor recurrence.

  15. SHIELD II: AGC 198507 - An Extremely Rare Low-Mass Galaxy Interaction?

    Science.gov (United States)

    Nikolina Borg Stevens, Karin; Cannon, John M.; McNichols, Andrew; McQuinn, Kristen B.; Teich, Yaron; SHIELD II Team

    2016-01-01

    The "Survey of HI in Extremely Low-mass Dwarfs II" ("SHIELD II") is a multiwavelength, legacy-class observational campaign that is facilitating the study of both internal and global evolutionary processes in low-mass dwarf galaxies discovered by the Arecibo Legacy Fast ALFA (ALFALFA) survey. New HST imaging of one of these sample galaxies, AGC 198507, has revealed it to be a very rare interacting system; to our knowledge this is one of only a few known interactions in this extreme mass range. WSRT imaging indicates that the bulk of the HI is associated with the more luminous AGC 198507, while low surface brightness gas extends toward and coincides with the less luminous companion, which is separated by roughly 1.5 kpc from AGC 198507. Here we present new VLA B configuration HI imaging that allows us to localize the HI gas, to examine the rotational dynamics of AGC 198507, and to study the nature of star formation in this unique low-mass interacting system.Support for this work was provided by NSF grant AST-1211683 to JMC at Macalester College, and by NASA through grant GO-13750 from the Space Telescope Science Institute, which is operated by AURA, Inc., under NASA contract NAS5-26555.

  16. Pindborg Tumor Presenting as a Nasal Polyp: Immunohistology and Ultrastructural Features of a Rare Case, With Review of the Literature.

    Science.gov (United States)

    Mitra, Suvradeep; Kaur, Gurwinder; Nada, Ritambhra; Mohindra, Satyawati

    2016-09-01

    Calcifying epithelial odontogenic tumor, also known as Pindborg tumor, is a rare benign odontogenic tumor. This tumor predominantly involves the mandible, though the maxilla can also be involved in some cases. The involvement of maxillary sinuses, however, has been previously reported in only 8 case reports English literature. These patients chiefly presented with nasal obstruction along with epistaxis or proptosis in rare cases. However, a primary presentation as nasal polyp is unheard of. We present a rare case of calcifying epithelial odontogenic tumor presenting as a nasal polyp, posing a diagnostic difficulty.

  17. Fine-needle aspiration cytology of ovarian steroid cell tumor: A rare case report

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    Nidhi Agrawal

    2015-01-01

    Full Text Available Steroid cell tumors (SCTs of the ovary are a rare subgroup of sex cord tumors that account for less than 0.1% of all ovarian tumors. These tumors can produce steroids, especially testosterone, which produces symptoms such as hirsutism, amenorrhea/oligomenorrhea, and male patterned voice. For evaluation of the androgen excess, testosterone and dehydroepiandrosterone sulfate (DHEA-S are the first laboratory tests to be measured. Abdominal ultrasound and magnetic resonance imaging (MRI are useful radiologic imaging techniques. Although SCTs are generally benign, the risk of malignant transformation is always present. Surgical excision of tumor is the most important and hallmark treatment. The present case signifies the early preoperative diagnosis of a virilizing SCT, based on cytological features and its careful correlation with clinicopathological and radiological findings.

  18. Steroid Cell Tumor of the Ovary in an Adolescent: A Rare Case Report

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    Gokhan Boyraz

    2013-01-01

    Full Text Available Steroid cell tumors (SCTs of the ovary are a rare subgroup of sex cord tumors, account for less than 0.1% of all ovarian tumors, and also will present at any age. These tumors can produce steroids, especially testosterone, and may give symptoms like hirsutism, hair loss, amenorrhea, or oligomenorrhea. For the evaluation of androgen excess, testosterone and dehydroepiandrosterone sulfate (DHEA-S are the first laboratory tests to be measured. A pelvic ultrasound and a magnetic resonance imaging are useful radiologic imaging techniques. Although steroid cell tumors are generally benign, there is a risk of malignant transformation and clinical malignant formation. Surgery is the most important and hallmark treatment.

  19. Ghost cell odontogenic tumor associated with odontoma--report of two rare cases.

    Science.gov (United States)

    Hogge, Maritzabel; Velez, Ines; Kaltman, Steven; Movahed, Reza; Yeh, Frank

    2012-01-01

    The ghost cell odontogenic tumor (GCOT) is a neoplastic/cystic lesion with a diverse histopathological and clinical behavior It was formerly known as calcified odontogenic cyst, but in 2005 the World Health Organization categorized this lesion as an odontogenic, benign tumor rather than a cyst; nominating this neoplasm as calcifying cystic odontogenic tumor. A later comprehensive classification named it ghost cell odontogenic tumor because the most remarkable histopathologic characteristic is the presence of a mass of ghost cells embedded in the epithelium. We report two cases of a rare variant of a ghost cell odontogenic tumor associated with odontoma; to our knowledge, one is the youngest patient (four month old) reported in the English literature.

  20. Isolation of rare tumor cells from blood cells with buoyant immuno-microbubbles.

    Directory of Open Access Journals (Sweden)

    Guixin Shi

    Full Text Available Circulating tumor cells (CTCs are exfoliated at various stages of cancer, and could provide invaluable information for the diagnosis and prognosis of cancers. There is an urgent need for the development of cost-efficient and scalable technologies for rare CTC enrichment from blood. Here we report a novel method for isolation of rare tumor cells from excess of blood cells using gas-filled buoyant immuno-microbubbles (MBs. MBs were prepared by emulsification of perfluorocarbon gas in phospholipids and decorated with anti-epithelial cell adhesion molecule (EpCAM antibody. EpCAM-targeted MBs efficiently (85% and rapidly (within 15 minutes bound to various epithelial tumor cells suspended in cell medium. EpCAM-targeted MBs efficiently (88% isolated frequent tumor cells that were spiked at 100,000 cells/ml into plasma-depleted blood. Anti-EpCAM MBs efficiently (>77% isolated rare mouse breast 4T1, human prostate PC-3 and pancreatic cancer BxPC-3 cells spiked into 1, 3 and 7 ml (respectively of plasma-depleted blood. Using EpCAM targeted MBs CTCs from metastatic cancer patients were isolated, suggesting that this technique could be developed into a valuable clinical tool for isolation, enumeration and analysis of rare cells.

  1. This is the full PDF of Rare Tumors 2009, volume 1, issue 1.

    Directory of Open Access Journals (Sweden)

    the Editors

    2010-03-01

    Full Text Available This is the full PDF of Rare Tumors 2009, volume 1, issue 1. This file is a low-resolution one (about 6.5 Mb: if you would like a high-definition version (about 96 Mb, you can download it here.

  2. [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.

  3. Carcinoid tumor of the common bile duct: A rare complication of von Hippel-Lindau syndrome

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    Van Hippel-Lindau syndrome (VHL) is a rare autosomal dominant, inherited familial cancer syndrome. Hemangioblastomas, pheochromocytomas and renal carcinoma are the frequent reported VHL tumors. Neuroendocrine tumors have also been described, mostly in the pancreas and rarely in the biliary trees. We report the second case of bile duct carcinoid in a 31-year-old VHL woman. She underwent right adrenalectomy for a pheochromocytoma in the past. She also had a positive family history of phenotypic expression of VHL syndrome. The patient presented with biliary colic. Endoscopic retrograde cholangio-pancreatography showed intra luminal bile duct mass. Surgical exploration identified a beige nodular lesion that was a carcinoid tumor on histology. This new association should be clarified by further genetic investigations.

  4. Magnetic resonance imaging of soft-tissue tumors of the extremities: A practical approach

    Institute of Scientific and Technical Information of China (English)

    Wing; P; Chan

    2013-01-01

    Diagnosis of extremity soft-tissue tumors can be challenging.Characteristics of tumor margins can help precisely identify locally aggressive or non-aggressive behavior for surgical planning,but cannot differentiate benign from malignant lesions.Most malignant tumors can have inhomogeneous signals on T2-weighted images.Although a uniform signal on T2-weighted images can be a reliable indication of a benign lesion,a well-defined mass with homogeneous internal signal intensity does not definitively identify a benign lesion.Some common and distinctive soft-tissue lesions can have specific clinical and imaging features allowing a diagnosis without biopsy.These are known as determinate lesions.This illustrative report presents a diagnostic guide for extremity soft-tissue tumors based on tissue signal and morphological characteristics on magnetic resonance images.It is important for clinicians to be familiar with the imaging characteristics of common determinate lesions.

  5. Synchronous papillary urothelial carcinoma of urinary bladder and adenocarcinoma of stomach in a middle-aged man: An extremely rare association with therapeutic dilemma

    Directory of Open Access Journals (Sweden)

    Dodul Mondal

    2013-01-01

    Full Text Available Synchronous occurrence of urinary bladder carcinoma and gastric carcinoma is very rare. A middle-aged Asian man presented with complaints of hematuria which was diagnosed due to muscle invasive papillary urothelial carcinoma of urinary bladder. Metastatic work-up revealed simultaneous presence of locally advanced gastric adenocarcinoma. He was treated with TURBT for the bladder cancer and was planned for radical gastric resection followed by radiation to urinary bladder and stomach with concurrent chemotherapy. However, due to very advanced nature of the gastric tumor patient was treated only with palliative gastric resection followed by palliative radiation to both urinary bladder and stomach due to his poor performance status. Lack of published English literature and evidence related to such clinical entity made this an extremely rare clinical entity and treatment decision difficult.

  6. A rare cause of acute abdomen: tumor rupture of nonpalpable testis

    Science.gov (United States)

    Yıldız, Turan; İlçe, Zekeriya; Gündüz, Yasemin; Çakırsoy, Gözde Çakar

    2016-01-01

    Undescended testicle is the most common congenital anomaly among males. Testicular tumor develops in 3–5% of the boys with a complaint of undescended testicle. The clinical presentation of malignant intra-abdominal testicular tumors ranges from asymptomatic cases to acute abdomen. In this study, we present a child with testicular tumor rupture which is observed very rarely. A 16-year-old boy presented 24 hours after the sudden onset of right lower quadrant pain, nausea, vomiting and fever. On physical examination, extensive tenderness in the abdomen and abdominal guarding were found. The right testicle was not palpable. The serum white blood cell count was elevated. Ultrasonography and abdominal computerized tomography scan demonstrated a perforated, hyperdense mass with free fluid in the abdomen. The preoperative alpha fetoprotein level was found to be increased. We performed surgery with laparoscopy and a perforated right intraabdominal testicle was found in the right iliac fossa. The mass was excised. Histopathological examination revealed a yolk sac tumor. Ruptured nonpalpable testicular tumors are very rare. To our knowledge, this is the first adolescent case reported so far. Testicular tumor rupture should be considered in patients with nonpalpable testicle and acute abdomen. Laparoscopy may be performed in differential diagnosis and treatment of these patients.

  7. A rare cause of acute abdomen: tumor rupture of nonpalpable testis.

    Science.gov (United States)

    Yıldız, Turan; İlçe, Zekeriya; Gündüz, Yasemin; Çakırsoy, Gözde Çakar

    2016-09-01

    Undescended testicle is the most common congenital anomaly among males. Testicular tumor develops in 3-5% of the boys with a complaint of undescended testicle. The clinical presentation of malignant intra-abdominal testicular tumors ranges from asymptomatic cases to acute abdomen. In this study, we present a child with testicular tumor rupture which is observed very rarely. A 16-year-old boy presented 24 hours after the sudden onset of right lower quadrant pain, nausea, vomiting and fever. On physical examination, extensive tenderness in the abdomen and abdominal guarding were found. The right testicle was not palpable. The serum white blood cell count was elevated. Ultrasonography and abdominal computerized tomography scan demonstrated a perforated, hyperdense mass with free fluid in the abdomen. The preoperative alpha fetoprotein level was found to be increased. We performed surgery with laparoscopy and a perforated right intraabdominal testicle was found in the right iliac fossa. The mass was excised. Histopathological examination revealed a yolk sac tumor. Ruptured nonpalpable testicular tumors are very rare. To our knowledge, this is the first adolescent case reported so far. Testicular tumor rupture should be considered in patients with nonpalpable testicle and acute abdomen. Laparoscopy may be performed in differential diagnosis and treatment of these patients.

  8. Pancreatic Extra-Gastrointestinal Stromal Tumor: An Unusual Presentation of a Rare Diagnosis

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    Joshi, Jitesh; Rustagi, Tarun

    2010-01-01

    Background: Gastrointestinal stromal tumors (GISTs) rarely develop outside the digestive tract and such tumors are designated extra-GISTs (EGISTs). The majority of EGISTs are located in the mesentery, omentum, and retroperitoneum, and the primary localization in the pancreas has been reported in only about six cases. We describe a patient with a large metastatic pancreatic EGIST that had metastasized to the liver at time of presentation. Case: An 84-year-old male presented with worsening confusion and agitation for the past few days. He also reported progressively increasing abdominal distension for the past 3 years, more so in the past few months. He denied any abdominal pain, nausea, or vomiting. He mentioned one episode of melena 2 months ago. There was a history of unintentional weight loss of 30 pounds over the past few months. Review of systems was otherwise negative. Past medical history was significant for diabetes mellitus and lactose intolerance. Pertinent examination findings included a cachectic appearance, altered mentation without any focal neurologic deficit, and marked abdominal distension with dullness on percussion. Investigations were significant for elevated ammonia level (168 ug/dL), AST/ALT/Alk. phosphatase (424/153/102 U/L), and total bilirubin of 1.7 mg/dL. CEA and CA19-9 were within normal limits. Computed tomography (CT) scan of the abdomen showed an extremely large central heterogeneous mass of 34 × 24 × 27 cm replacing the entire pancreatic tissue and multiple hepatic metastases. Subsequently, a CT-guided liver biopsy demonstrated a spindle cell neoplasm with CD117 (c-kit), CD34, and vimentin-positive cells, consistent with liver metastasis from an EGIST. On day 3, he had massive hematemesis, for which he was transferred to the intensive care unit. His condition rapidly deteriorated with hemodynamic instability and further worsening of mental status. After a thorough discussion about treatment options and prognosis, his family

  9. Hematemesis, a very rare presentation of solid pseudo-papillary tumors of the pancreas: a case report

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    Apostolidis Stylianos

    2008-08-01

    Full Text Available Abstract Introduction Solid pseudo-papillary tumors of the pancreas are rare and typically present in young female patients. They are slowly growing masses that may attain large size, and are of low malignant potential. Surgical resection is usually curative. Case presentation A 71-year-old woman presented to the emergency department with an episode of hematemesis but was otherwise hemodynamically stable. Emergency gastroscopy revealed a bleeding mass projecting to the duodenum. Fluid, blood and electrolyte resuscitation followed. Computed tomography revealed a small mass in the head of the pancreas. A Whipple operation was performed. Pathology revealed a solid pseudo-papillary tumor. The postoperative course of the patient was uneventful and no recurrence was present a year after the operation. Conclusion In our case, the most noteworthy observations concern the small size of the tumor, the age of the patient and the presenting symptom. However, pancreaticoduodenectomy in a 71-year-old woman is a major effort and should only be undertaken by centers and surgeons experienced in complex hepatobiliary surgery. Furthermore, the unique nature of this case reminds every clinician that each patient has to be considered separately and with extreme caution.

  10. Urothelial carcinoma with oncocytic features: an extremely rare case presenting a diagnostic challenge in urine cytology.

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    Tajima, Shogo

    2015-01-01

    Recognizing histological variants in urothelial carcinoma (UC) is important because some may be associated with different clinical outcomes and/or therapeutic approaches; being aware of unusual histological variants may also be crucial in preventing diagnostic misinterpretations. Histological variants based on cytoplasmic features, such as clear-cell, plasmacytoid, rhabdoid, and lipoid-rich variants, are described in invasive UC; however, these cytoplasmic features are not formally defined and not usually encountered in non-invasive UC. Oncocytic cytoplasm has not been well described in either invasive or non-invasive UC. Herein, we report an exceedingly rare case of UC with oncocytic features arising in the right renal pelvis, which presented a diagnostic challenge in urine cytology due to the relatively low nuclear-to-cytoplasmic ratio; however, it could definitively be diagnosed using histological specimens. UC diagnosis is based on the presence of papillary architecture and widespread p53 nuclear accumulation, suggesting malignancy. An oncocytic tumor is generally considered to be not actively dividing, as shown by the low Ki-67 labeling index in this case. In spite of the low proliferative activity, the possibility of intravesicle recurrence (IVR) should be considered since positive preoperative cytology of upper tract UC is a risk factor for IVR after nephroureterectomy.

  11. Schwannoma of the vagus nerve, a rare middle mediastinal neurogenic tumor: case report

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    Foroulis Christophoros N

    2009-11-01

    Full Text Available Abstract Schwannoma originating from the vagus nerve within the mediastinum is a rare, usually benign tumor. A 44-year old male was presented with chest pain. Chest radiography, CT scan and MRI showed a well circumscribed mass, 5 × 4 cm located in the aortopulmonary window. The mass was found at surgery to be in close proximity with the aortic arch and the left pulmonary hilum, alongside the left vagus nerve. The encapsulated tumor was completely resected through a left thoracotomy incision and it was found to be a benign schwannoma in pathology. The patient is free of recurrence 6 years after surgery.

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

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    Tepeler, Abdulkadir; Erdem, Mehmet Remzi; Kurt, Omer; Topaktas, Ramazan; Kilicaslan, Isin; Armağan, Abdullah; Önol, Şinasi Yavuz

    2011-01-01

    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. PMID:22110514

  13. Breast malignant phyllodes tumor with rare pelvic metastases and long-term overall survival

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    Shan, Jinlan; Zhang, Shizhen; Wang, Zhen; Fu, Yanbiao; Li, Ling; Wang, Xiaochen

    2016-01-01

    Abstract Background: Malignant phyllodes tumor (PT) is a rare fibro epithelial neoplasm of the breast, which is poor prognosis due to high risk of recurrence and distant metastasis. Methods: We report a case of malignant PT. It had recurred locally five times, and the sixth relapse was occurred 54 months after first diagnosis, presenting a huge pelvic mass (14 cm × 11 cm) by CT scan. Histopathological examination has demonstrated a metastatic phyllodes tumor. After postoperative chemotherapy treatment, a longer survival has been achieved, which is more than 72 months. Results: Our case report describes a breast PT with several local recurrences and a rare metastasis (pelvic cavity), but long-term overall survival was achieved after surgery and chemotherapy. Conclusion: We conclude that trustworthy prognosticators that identify patients with excessive potential of aggressive clinical course should be explored. Moreover, proper treatment could prolong overall survival of metastatic PT patients. PMID:27661051

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

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    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.

  15. Dentigerous cyst associated with Adenomatoid Odontogenic tumor (AOT A Rare Case Report and Review of Literature

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    Bhagyashri. R. Latti

    2016-09-01

    Full Text Available Adenomatoid Odontogenic tumor (AOT is a benign (hamartomatous lesion of Odontogenic origin, which is relatively uncommon and affects young individuals with a female predominance, mainly in the second decade. This lesion is most commonly located in the anterior maxilla and is usually associated with an impacted canine tooth. The present case report is a 15year old female patient, who presented with a large AOT in the anterior mandible associated with an impacted canine – a very rare situation.

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

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    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. 

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

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    Fabio Cesar Miranda Torricelli

    2015-02-01

    Full Text Available Main findings 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.

  18. Warthin-like papillary thyroid carcinoma: A rare tumor of the thyroid

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    Nuray Can

    2011-12-01

    Full Text Available Warthin-like papillary thyroid carcinoma is a rare variant of papillary thyroid carcinoma with favorable prognosis. The tumor is named “Warthin-like papillary thyroid carcinoma” because of bearing a striking morphological resemblance to Warthin’s tumor occurring in the salivary glands. Thyroid ultrasonography of 65 years old female patient with a history of bilateral subtotal thyroidectomy 30 years ago in another center with unknown histopathological diagnosis revealed a 20 mm isoechoic solid nodule in the left lobe and this nodule was hypoactive in thyroid scintigraphy. Grossly, a grey-tan colored, solid nodule with 1 cm diameter was seen in the left lobe. Histologically, the tumor was composed of papillary structures surrounded by marked lymphocytic stroma and oncocytic cells with papillary carcinoma’s nuclear features were lining the papillae. Hashimoto’s thyroiditis was present throughout the thyroid. Immunohistochemically, tumor cells exhibited cytokeratin 19, Thyroid transcription factor-1 (TTF-1, Galectin-3, HBME-1(Mesothelioma antibody and thyroglobulin positivity, proliferative index with Ki-67 was low. Warthin-like papillary thyroid carcinoma is a rare variant of papillary thyroid carcinoma and other oncocytic cell lesions especially Hurthle cell carcinoma should be kept in mind in the differential diagnosis of this neoplasm.

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

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    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.)

  20. 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.

  1. Primary Cervical Placental Site Trophoblastic Tumor: A Rare Entity with an Unusual Presentation

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    Kanthilatha Pai

    2013-10-01

    Full Text Available Placental site trophoblastic tumour (PSTT is the least common form of gestational trophoblastic neoplasia accounting for only 1-2% of trophoblastic tumors. Approximately 200 cases are reported in English literature. PSTT presenting as a cervical growth is even less common. Differentiation of PSTT from other types of GTN, non-neoplastic gestational trophoblastic disease and non-trophoblastic tumors is important clinically due to differences in their therapeutic approaches.Appreciation of the morphologic features and immunophenotype allows their accurate diagnosis.Although most of the cases of PSTT behave in a benign fashion,the clinical behavior of PSTT can sometimes be variable and several prognostic factors can help to predict the biological behavior of this condition. We report a rare case of placental site trophoblastic tumor, presenting as a cervical mass, in a 38 year old female, and review the literature. [J Interdiscipl Histopathol 2013; 1(5.000: 286-289

  2. Pure yolk sac tumor of testis in a 50 years old : A rare case report

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    Pradhan M Pagaro

    2016-01-01

    Full Text Available Yolk sac tumor (YST is most common in infants and children in age range of 3-8 years in pure form; however in adults, YST rarely occurs in pure form, but commonly occurs in association with other germ cell tumors as mixed forms. Less than 10 adult testicular pure YSTs have been reported in literature. We report a 50-year-old male with testicular neoplasm involving the left testis, with pain following trauma. Alpha-fetoprotein was markedly raised 5726 IU/ml, and on histopathology, characteristic features of YST with abundant Schiller–Duval bodies associated with reticular and microcystic pattern, and focal solid areas of nests and trabeculae of cuboidal cells, with prominent nucleoli and mitotic figures, were noted, and extensive histopathological search did not reveal presence of any other type of tumor.

  3. Detecting the Common and Individual Effects of Rare Variants on Quantitative Traits by Using Extreme Phenotype Sampling

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    Ya-Jing Zhou

    2016-01-01

    Full Text Available Next-generation sequencing technology has made it possible to detect rare genetic variants associated with complex human traits. In recent literature, various methods specifically designed for rare variants are proposed. These tests can be broadly classified into burden and nonburden tests. In this paper, we take advantage of the burden and nonburden tests, and consider the common effect and the individual deviations from the common effect. To achieve robustness, we use two methods of combining p-values, Fisher’s method and the minimum-p method. In rare variant association studies, to improve the power of the tests, we explore the advantage of the extreme phenotype sampling. At first, we dichotomize the continuous phenotypes before analysis, and the two extremes are treated as two different groups representing a dichotomous phenotype. We next compare the powers of several methods based on extreme phenotype sampling and random sampling. Extensive simulation studies show that our proposed methods by using extreme phenotype sampling are the most powerful or very close to the most powerful one in various settings of true models when the same sample size is used.

  4. Hemangioendotelioma: tumor raro de mediastino Hemangioendothelioma: a rare tumor of the mediastinum

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    Marcelo Loze de Queiroz

    2004-04-01

    Full Text Available Apresentou-se para atendimento um homem de 30 anos, branco, natural e procedente de São Paulo, com quadro de dor em hemitórax esquerdo, na região anterior e lateral, constante e de leve intensidade havia três meses, associado a dispnéia aos grandes esforços havia um mês. Durante a investigação foi visualizada em radiograma, tomografia e ressonância magnética de tórax grande tumoração em mediastino anterior e médio, com possível invasão dos vasos da base. Ele foi submetido à mediastinotomia paraesternal esquerda com biópsia da massa mediastinal, a qual complicou por sangramento intenso. Optou-se pela esternotomia mediana total e toracotomia ântero-lateral esquerda de urgência, com controle do sangramento e ressecção completa do tumor. Houve boa evolução, com alta hospitalar no nono dia pós-operatório. O exame anatomopatológico mostrou tratar-se de hemangioendotelioma de mediastino.A 30-year-old Caucasian male from São Paulo was admited to the hospital. He had been complaining about constant, moderate pain in the anterior and lateral left hemi-thoracic region for the last three mouths as well as associatede great effort dyspnea over the last mounth. Investigation with chest X-rays, CT scans and MRI revealed an large vessel invasion. The patient was submitted to a left side parasternal madiastinostomy and a biopsy of the mediastinal mass which was complicated by severe bleeding. An immediate median full sternotomy was elected in addition to a left anterior-lateral thoracotomy for total tumor resection and control of the bleeding. Evolution was good, with hospital discharge on the ninth postoperative day. The anatomical-pathological essay disclosed a hemangioendothelioma of the mediastinum.

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

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    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.

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

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    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.

  7. Molecular Profiling of a Rare Rosette-Forming Glioneuronal Tumor Arising in the Spinal Cord.

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    Lucas Tadeu Bidinotto

    Full Text Available Rosette-forming glioneuronal tumor (RGNT of the IV ventricle is a rare and recently recognized brain tumor entity. It is histologically composed by two distinct features: a glial component, resembling pilocytic astrocytoma, and a component forming neurocytic rosettes and/or perivascular rosettes. Herein, we describe a 33-year-old man with RGNT arising in the spinal cord. Following an immunohistochemistry validation, we further performed an extensive genomic analysis, using array-CGH (aCGH, whole exome and cancer-related hotspot sequencing, in order to better understand its underlying biology. We observed the loss of 1p and gain of 1q, as well as gain of the whole chromosomes 7, 9 and 16. Local amplifications in 9q34.2 and 19p13.3 (encompassing the gene SBNO2 were identified. Moreover, we observed focal gains/losses in several chromosomes. Additionally, on chromosome 7, we identified the presence of the KIAA1549:BRAF gene fusion, which was further validated by RT-PCR and FISH. Across all mutational analyses, we detected and validated the somatic mutations of the genes MLL2, CNNM3, PCDHGC4 and SCN1A. Our comprehensive molecular profiling of this RGNT suggests that MAPK pathway and methylome changes, driven by KIAA1549:BRAF fusion and MLL2 mutation, respectively, could be associated with the development of this rare tumor entity.

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

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    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.

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

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    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

  10. Phyllodes tumor showing intraductal growth.

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    Makidono, Akari; Tsunoda, Hiroko; Mori, Miki; Yagata, Hiroshi; Onoda, Yui; Kikuchi, Mari; Nozaki, Taiki; Saida, Yukihisa; Nakamura, Seigo; Suzuki, Koyu

    2013-07-01

    Phyllodes tumor of the breast is a rare fibroepithelial lesion and particularly uncommon in adolescent girls. It is thought to arise from the periductal rather than intralobular stroma. Usually, it is seen as a well-defined mass. Phyllodes tumor showing intraductal growth is extremely rare. Here we report a girl who has a phyllodes tumor with intraductal growth.

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

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    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.

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

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    Arslan, Deniz; Gündüz, Şeyda; Tural, Deniz; Uysal, Mükremin; Tatlı, Ali Murat; Başsorgun, Cumhur İbrahim; Elpek, Gülsüm Özlem; Coşkun, Hasan Şenol; Bozcuk, Hakan Şat; Savaş, Burhan

    2013-01-01

    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. PMID:23573435

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

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    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.

  14. Sarcoma-like mural nodule in a borderline mucinous tumor of the ovary: A rare entity.

    Science.gov (United States)

    Ghosh, Prithwijit; Saha, Kaushik; Bhowmik, Sourav

    2014-10-01

    Sarcoma-like mural nodule (SLMN) is a very uncommon and misleading benign entity which may be associated with benign, borderline or malignant mucinous neoplasm of the ovary. It should be distinguished from other malignant mural nodules with sarcoma, carcinosarcoma or anaplastic carcinoma for proper management. We report a rare case of SLMN in a borderline mucinous tumor of the ovary in a 30-year-old lady. In spite of having confusing histopathological features the final diagnosis was made depending on the younger age of the patient, well circumscription of the nodule, absence of vascular invasion and immunohistochemical profile.

  15. Pott's puffy tumor: a rare complication of acute otitis media in child: a case report.

    Science.gov (United States)

    Urík, Milan; Machač, Josef; Šlapák, Ivo; Hošnová, Dagmar

    2015-09-01

    To describe a rare case of Potts' puffy tumor (PPT) in the zygomatic area, which developed as a complication of acute otitis media in a 6-year-old child. To date, only one case of PPT has been described in the literature as a complication of latent mastoiditis in an adult, and one case of PPT as a complication of acute mastoiditis in a 10-year-old child. Urgent surgical intervention, including evacuation of the purulent lesion, removal of inflamed soft tissue and osteolysis of the involved bone, and antromastoidectomy, intravenous treatment with broad-spectrum antibiotics, including G+, G-, anaerobes and fungi, and local therapy.

  16. One Case of Pulmonary Chondroma, Rare Benign Tumor of the Lung

    Institute of Scientific and Technical Information of China (English)

    YANG Kun; YANG Guanghai; LI Jinsong; WANG Jianjun

    2006-01-01

    @@ Pulmonary chondroma is a rare form of benign neoplasms of the lung, of which the cartilage is the only constituent. Most of the patients with lung chondroma are asymptomatic at the time of diagnosis and the occupying lesion in lung is mostly detected in the routine health examination. The image of pulmonary chondroma is similar to the other benign solitary neoplasms of the lung and the definitive diagnosis can only be established under a microscope after the resection of the tumor. In the present report, a 33-years-old woman with chondroma in the left lower lung was described.

  17. Primary primitive neuroectodermal tumor of kidney: A rare case report with diagnostic challenge

    Directory of Open Access Journals (Sweden)

    Sunita Kakkar

    2014-01-01

    Full Text Available Primary primitive neuroectodermal tumors (PNETs of the kidney are quite rare and can be mistaken for a wide variety of other small round blue cell tumors which includes rhabdomyosarcoma, Wilm′s tumor, carcinoid, neuroblastoma, clear cell sarcoma of the kidney, lymphoma etc. Renal Ewings/PNET can occur in the age group from 4 to 61 years. Approximately, 90% of Ewing sarcoma (ES/PNET have a specific t(11;22 which results in a chimeric EWS-FLI-1 fusion protein. Immunohistochemical for the carboxy-terminus of FLI-1 is sensitive and highly specific for the diagnosis of ES/PNET. Herein, we have an interesting presentation in a 23-year-old male who came with neck pain and progressive quadriparesis and was diagnosed as a case of poorly differentiated malignant tumor with a differential of lymphoma versus metastatic renal cell carcinoma. The patient′s condition deteriorated fast and he had a rapid downhill course. The final diagnosis of Ewings/PNET was confirmed at autopsy.

  18. Myoepithelial carcinoma of the posterior mediastinum: An uncommon site for a rare tumor

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    Eman Abdulfatah, M.D.

    2016-09-01

    Full Text Available Myoepithelial carcinoma (MC is a rare tumor that arises from myoepithelial cells; most commonly in the salivary glands, but other infrequent body sites such as the breast, lung, lower limb, upper limb, head and neck, vulva, and vagina can be involved. We report the first case of myoepithelial carcinoma arising in the posterior mediastinum of a 51 year-old male who presented with a mediastinal mass and subsequently underwent tumor debulking surgery. Grossly, the specimen consisted of multiple tan–gray firm fragments of tissue with an overall measurement of 7.0 cm in greatest dimension. Histologic examination revealed an ill-defined, infiltrative lesion with a biphasic cell population. The tumor cells were diffusely positive for epithelial and myoepithelial markers, confirming the above diagnosis. Recognition of this entity at an uncommon site may present a diagnostic challenge due to its morphologic heterogeneity and the differential diagnosis includes benign and malignant tumors, which could lead to over or under-treatment, respectively.

  19. Atypical teratoid rhabdoid tumor arising in a pleomorphic xanthoastrocytoma: a rare entity.

    Science.gov (United States)

    Uner, Meral; Saglam, Arzu; Meydan, Bilge Can; Aslan, Kerim; Soylemezoglu, Figen

    Atypical teratoid rhabdoid tumor (AT/RT) is a rare and aggressive tumor usually occurring at younger ages. Pleomorphic xanthoastrocytomas (PXA) on the other hand are quiescent tumors with benign behavior. AT/RTs arising in the setting of PXA are exceptional. We present the case of a 23-year-old female patient, the fourth in the literature, speculated as having AT/RT arising within a PXA, as demonstrated by the presence of INI1 mutation. The patient presented with a short history of headache, which increased over time, and emerging seizures. She had a contrast-enhancing mass in the left temporal area demonstrated by MRI. Pathological examination demonstrated a dimorphic tumor containing a spindle-pleomorphic component reminiscent of PXA and a rhabdoid component with INI1 loss showing features of AT/RT. Both components shared the same BRAF mutation, supporting their common origin, and hence the case was speculated as an AT/RT arising in the setting of a PXA by secondary genetic change of inactivation of INI1. She had a poor outcome despite surgery and died 8 months after her diagnosis.
.

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

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    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.

  1. 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.

  2. Malignant extrarenal rhabdoid tumour (MERT) with liver metastases as a rare cause of an esophageal tumor in a 57 years old patient.

    Science.gov (United States)

    Kaechele, V; Vogelpohl, J; Boeck, W; Riecke, A; Eisele, R; Barth, T

    2015-07-01

    Tumors with a rhabdoid phenotype are aggressive neoplasms with a dismal prognosis. Malignant extrarenal rhabdoid tumor (MERT) of the esophagus is an extremely rare disease with so far only 6 cases reported. We report on a 57-year-old male patient with rhabdoid tumor situated in the esophagus with metastases to the liver and local lymph nodes. Assuming an undifferentiated esophageal adenocarcinoma a palliative chemotherapy with 5-FU/folinic acid, oxaliplatin, and docetaxel (FLOT) was initiated which was changed towards a combination of doxorubicin and ifosphamide as immunohistochemistry of the primary and the liver metastases revealed a rhabdoid tumor. This treatment with doxorubicin and ifosphamide resulted in a short clinical and radiological response which lasted only for 2 months. Due to the bad general condition at the time of progression no further chemotherapy was initiated. The patient died due to tumor progression 6 months after initial diagnosis which is consistent with other reports on malignant extrarenal rhabdoid tumors (median survival of metastatic disease less than 6 months). Thus, metastatic MERT represents a disease with a poor prognosis and no established standard therapy. © Georg Thieme Verlag KG Stuttgart · New York.

  3. Primitive neuroectodermal tumor of the uterine cervix diagnosed during pregnancy: a rare case with review of literature.

    Science.gov (United States)

    Khosla, Divya; Rai, Bhavana; Patel, Firuza D; Sreedharanunni, Sreejesh; Dey, Pranab; Sharma, Suresh C

    2014-03-01

    Primitive neuroectodermal tumors of the cervix are very rare. A 28-year-old pregnant woman presented with a cervical mass. The tumor was staged as IB2. The biopsy from tumor was suggestive of malignant small round cell tumor. She then underwent termination of pregnancy followed by radical hysterectomy. Based on morphologic and immunohistochemical profile, a diagnosis of peripheral primitive neuroectodermal tumor of the cervix was made. The patient received adjuvant chemotherapy and radiotherapy. The patient is alive and disease-free 33 months post-surgery. The present case highlights the importance of keeping primitive neuroectodermal tumors in the differential diagnosis of small cell neoplasms of the uterine cervix. Pregnancy should not be a barrier to early detection and treatment of this potentially aggressive tumor. The optimal treatment methods have not yet been established because of the rarity of the tumor.

  4. Well-differentiated neuroendocrine tumor of tailgut cyst. A rare entity with controversial medical opportunities.

    Science.gov (United States)

    Damato, Angela; Pusceddu, Sara; Milione, Massimo; Mazzaferro, Vincenzo; Magli, Michelle; Seregni, Ettore; De Braud, Filippo; Buzzoni, Roberto

    2013-01-01

    The incidence of neuroendocrine tumors is rising, and this rise is explained by more than just better diagnostic procedures. About 85% of these neoplasms arise in gastrointestinal or pulmonary sites, but cases where the location is more unusual also occur in clinical practice. The tailgut cyst is a rare entity well described in the medical literature, but a neuroendocrine tumor within such a cyst is a very rare event, with about 30 cases described in the literature to date. In this report we present the case of a young woman with this unusual diagnosis. The characteristics of the case differ from most previous case reports in a few respects: the patient was a young rather than middle-aged female; she had a presacral mass with a significant solid component; at diagnosis, there was evidence of a lytic lesion in the coccyx. Despite this particular medical presentation, radical surgery was accomplished. In this disease the greatest risk is local relapse, but adjuvant radiotherapy may compromise the patient's fertility. We therefore opted for strict control only, but this decision might be debatable.

  5. Intramedullary meningioma of spinal cord: case report of a rare tumor highlighting the differential diagnosis of spinal intramedullary neoplasms.

    Science.gov (United States)

    Pant, Ishita; Chaturvedi, Sujata; Gautam, Vinod Kumar Singh; Kumari, Rima

    2014-01-01

    A 15-year-old male presented with progressive weakness of both lower limbs with urinary incontinence. Magnetic resonance imaging revealed a spinal intramedullary mass at D7-D8 level. The child was operated with a preliminary diagnosis of an intramedullary tumor. Atypical ependymoma and astrocytoma were considered in the differential diagnosis. Per- and post-operative histopathological examination reported the case as transitional meningioma (WHO Grade I). Spinal intramedullary meningiomas being a rare entity may be confused with other common intramedullary tumors. Though, rare still the possibility of an intramedullary spinal mass of being a meningioma does exist and therefore should be considered in the differential diagnosis of intramedullary tumors.

  6. Heavily calcified gastrointestinal stromal tumors: Pathophysiology and implications of a rare clinicopathologic entity

    Science.gov (United States)

    Salati, Massimiliano; Orsi, Giulia; Reggiani Bonetti, Luca; Di Benedetto, Fabrizio; Longo, Giuseppe; Cascinu, Stefano

    2017-01-01

    Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract, and are characterized by a broad spectrum of clinical, histological and molecular features at presentation. Although focal and scattered calcifications are not uncommon within the primary tumor mass, heavy calcification within a GIST is rarely described in the literature and the clinical-biological meaning of this feature remains unclear. Cases with such an atypical presentation are challenging and may be associated with diagnostic pitfalls. Herein, we report a gastric GIST with the unusual presentation of prominent calcifications that was identified incidentally on imaging during a post-trauma diagnostic work-up. The patient underwent laparoscopic surgery with a radical resection of the mass, which was subsequently characterized by histological analysis as spindle-shaped tumor cells, positive for CD117/c-KIT, CD34 and DOG1, and with calcified areas. Given the intermediate risk of recurrence, no adjuvant therapy was recommended and the patient underwent regular follow-up for 22 mo, with no evidence of relapse. Our case can be considered of interest because of the rarity of clinical presentation and the uniquely large size of the GIST at diagnosis (longest diameter exceeding 9 cm). In closing, we discuss the pathophysiology and clinical implications of calcifications in GISTs by reviewing the most up-to-date relevant literature. PMID:28344749

  7. Population sequencing of two endocannabinoid metabolic genes identifies rare and common regulatory variants associated with extreme obesity and metabolite level

    Science.gov (United States)

    2010-01-01

    Background Targeted re-sequencing of candidate genes in individuals at the extremes of a quantitative phenotype distribution is a method of choice to gain information on the contribution of rare variants to disease susceptibility. The endocannabinoid system mediates signaling in the brain and peripheral tissues involved in the regulation of energy balance, is highly active in obese patients, and represents a strong candidate pathway to examine for genetic association with body mass index (BMI). Results We sequenced two intervals (covering 188 kb) encoding the endocannabinoid metabolic enzymes fatty-acid amide hydrolase (FAAH) and monoglyceride lipase (MGLL) in 147 normal controls and 142 extremely obese cases. After applying quality filters, we called 1,393 high quality single nucleotide variants, 55% of which are rare, and 143 indels. Using single marker tests and collapsed marker tests, we identified four intervals associated with BMI: the FAAH promoter, the MGLL promoter, MGLL intron 2, and MGLL intron 3. Two of these intervals are composed of rare variants and the majority of the associated variants are located in promoter sequences or in predicted transcriptional enhancers, suggesting a regulatory role. The set of rare variants in the FAAH promoter associated with BMI is also associated with increased level of FAAH substrate anandamide, further implicating a functional role in obesity. Conclusions Our study, which is one of the first reports of a sequence-based association study using next-generation sequencing of candidate genes, provides insights into study design and analysis approaches and demonstrates the importance of examining regulatory elements rather than exclusively focusing on exon sequences. PMID:21118518

  8. Population sequencing of two endocannabinoid metabolic genes identifies rare and common regulatory variants associated with extreme obesity and metabolite level.

    Science.gov (United States)

    Harismendy, Olivier; Bansal, Vikas; Bhatia, Gaurav; Nakano, Masakazu; Scott, Michael; Wang, Xiaoyun; Dib, Colette; Turlotte, Edouard; Sipe, Jack C; Murray, Sarah S; Deleuze, Jean Francois; Bafna, Vineet; Topol, Eric J; Frazer, Kelly A

    2010-01-01

    Targeted re-sequencing of candidate genes in individuals at the extremes of a quantitative phenotype distribution is a method of choice to gain information on the contribution of rare variants to disease susceptibility. The endocannabinoid system mediates signaling in the brain and peripheral tissues involved in the regulation of energy balance, is highly active in obese patients, and represents a strong candidate pathway to examine for genetic association with body mass index (BMI). We sequenced two intervals (covering 188 kb) encoding the endocannabinoid metabolic enzymes fatty-acid amide hydrolase (FAAH) and monoglyceride lipase (MGLL) in 147 normal controls and 142 extremely obese cases. After applying quality filters, we called 1,393 high quality single nucleotide variants, 55% of which are rare, and 143 indels. Using single marker tests and collapsed marker tests, we identified four intervals associated with BMI: the FAAH promoter, the MGLL promoter, MGLL intron 2, and MGLL intron 3. Two of these intervals are composed of rare variants and the majority of the associated variants are located in promoter sequences or in predicted transcriptional enhancers, suggesting a regulatory role. The set of rare variants in the FAAH promoter associated with BMI is also associated with increased level of FAAH substrate anandamide, further implicating a functional role in obesity. Our study, which is one of the first reports of a sequence-based association study using next-generation sequencing of candidate genes, provides insights into study design and analysis approaches and demonstrates the importance of examining regulatory elements rather than exclusively focusing on exon sequences. © 2010 Harismendy et al.; licensee BioMed Central Ltd.

  9. GIANT FIBROADENOMA MIMICKING PHYLLOIDES TUMOR: A RARE OCCURRENCE IN A POSTMENOPAUSAL FEMAL E

    Directory of Open Access Journals (Sweden)

    Monika

    2015-04-01

    Full Text Available OBJECTIVE: Fibroadenomas are the most common solid lesions of the breast. They typically present as firm, mobile, painless, easily palpable breast nodules. Fibroadenomas measuring 5 cm or more in diameter are conside red as giant fibroadenomas which can grow to huge proportions and compress the surrounding normal breast tissue. They can occur in any part of reproductive life of female but are more common before the age of 30 years. CASE REPORT: we report a rare case of Giant fibroadenoma in a postmenopausal female of 52 yr of age. The diagnosis was made on fine needle aspiration cytology and was subsequently confirmed on histopathology. CONCLUSION: Fibroadenoma is an estrogen induced benign tumor common in young female, but in our case it occurred in postmenopausal female, even without any documented estrogen therapy.

  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. Sigmoid schwannoma: A rare case

    Institute of Scientific and Technical Information of China (English)

    Constantine I. Fotiadis; Ilias A. Kouerinis; Ioannis Papandreou; George C. Zografos; George Agapitos

    2005-01-01

    Schwannomas are rare tumors derived from the cells of Schwann that form the neural sheath. When located in the gastrointestinal tract, they constitute together with leiomyoma, leiomyoblastoma, and leiomyosarcoma, the gastrointestinal stromal tumors (GIST). Peripheral nerve sheath tumors represent 2-6% GIST with most common location, the stomach and the small intestine. Schwannomas of the colon and rectum are extremely rare and radical excision with wide margins is mandatory, due to their tendency to recur locally and become malignant, if left untreated. In the present study, we report a rare case of a sigmoid schwannoma, which was successfully treated in our department and reviewed the literature.

  12. Lung mass in a 28-year-old male: a case report of a rare tumor.

    Science.gov (United States)

    Mroz, Robert M; Korniluk, M; Swidzinska, E; Dzieciol, J; Czaban, J; Panek, B; Chyczewska, E

    2010-11-01

    A twenty eight-year-old male presented with a two week history of dyspnea, cough, hemoptysis, chest pain, and fever 38-39°C. He also complained of loss of appetite, general weakness and left leg pain for two months preceding admission. He was referred with suspicion of lung tumor to our institution. Chest X-ray showed almost total atelectasis of the right lung with compensatory overinflation of the contralateral lung. Using computed tomography (CT), a lesion of diameter of 19.3 x 14.1 x 19.1 cm in the right lung, pleuritis, Th3 osteolysis, and compensatory overinflation of the left lung was seen. Bronchoscopy revealed a total obstruction of the right main bronchus due to submucosal infiltration and compression of the right main bronchus with negative histology of bronchial biopsy specimens. Transthoracic fine needle aspiration revealed celullae suspectae probabiliter neoplasmaticae suggesting tumor fusocellularis. USG of the abdomen revealed liver with numerous heterogeneous, solid areas hypo- and hyperechogenic, some of them with features of liquid or the disintegration up to diameter of 74 mm. Subsequent fine needle aspirations of the thorax and liver revealed fibrolamellar hepatocarcinoma and carcinoma adenoides of the lung. Patient underwent chemotherapy with 5-FU/DDP/VCR with no response. This report presents a case of a rare lung metastasis from FL-HCC.

  13. 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.

  14. Extremely Rare Form of Impaction Bilateral Kissing Molars: Report of a Case and Review of the Literature.

    Science.gov (United States)

    Zerener, Tamer; Bayar, Gurkan Rasit; Altug, Hasan Ayberk; Kiran, Serkan

    2016-01-01

    Kissing molars (KM) or rosette formation is a term that is used to describe impacted teeth contacting occlusal surfaces in a single follicular space and their roots pointing in opposite directions. In some cases kissing molars can be seen but occurrence of bilateral kissing molars is extremely rare phenomenon in the dental literature and the aetiology of this phenomenon is still unknown. In this paper we describe a case and review of the literature and discuss the management of this pathology. In our case, extremely rare form of impacted bilateral kissing molars was extracted surgically. The decision of extraction of asymptomatic kissing molars represents surgical dilemma. There may be many surgical complications; on the other hand in some cases surgical intervention is unavoidable. Few treatment options were described in the literature. This phenomenon can be sign of various medical conditions that may require further investigation. In this paper, our treatment option is in agreement with the literature suggesting the surgical removal of both teeth at either side of the mandible.

  15. 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

  16. Bilateral Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Breast: A Very Rare Entity and Review of the Literature

    Directory of Open Access Journals (Sweden)

    N. Majid

    2013-01-01

    Full Text Available Peripheral primitive neuroectodermal tumors (PNET are rare malignant tumors, affecting mostly children and adolescents and have been described in breast in eight case reports only. In this paper, we present a case of bilateral mammary ES/PNET where distinction between primary and metastatic diseases was discussed through a literature review. The aim of this work is to demonstrate that although rare, the possibility of PNET should be kept in mind while evaluating a palpable breast abnormality in a young female.

  17. Keratocystic odontogenic tumor involving the maxillary antrum with displacement of the third molar: A rare case report

    Directory of Open Access Journals (Sweden)

    Ashwini Kumar Mengji

    2014-01-01

    Full Text Available Keratocystic odontogenic tumor (KCOT is a benign intraosseous neoplasm of the jaw with a high rate of recurrence. The lesion commonly occurs in the mandibular molar-ramus area and is rarely seen in the maxilla. Its occurrence in the maxillary sinus along with a displaced third molar is very rare. This article reports a case of KCOT in a 15-year-old boy with a displaced third molar involving the right maxillary antrum.

  18. Recurrent acute pancreatitis and persistent hyperamylasemia as a presentation of pancreatic osteoclastic giant cell tumor: an unusual presentation of a rare tumor.

    Science.gov (United States)

    Rustagi, Tarun; Rampurwala, Murtuza; Rai, Mridula; Golioto, Michael

    2011-01-01

    Giant cell tumors of the pancreas are rare neoplasms divided into three forms: osteoclastic, pleomorphic, and mixed. We report an unusual case of a 62-year-old male presenting with recurrent acute pancreatitis and found to have a mass in the head of the pancreas on routine imaging. Endoscopic retrograde cholangiopancreatography showed a main pancreatic duct stricture, with brush cytology revealing the diagnosis of osteoclastic giant cell tumor of the pancreas. Whipple's procedure was successfully performed for resection of this tumor. and IAP.

  19. 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.

  20. Craniofacial brown tumor as a result of secondary hyperparathyroidism in chronic renal disease patient: A rare entity

    Science.gov (United States)

    Verma, Pradhuman; Verma, Kanika Gupta; Verma, Dinesh; Patwardhan, Nitin

    2014-01-01

    Brown tumors are erosive bony lesions caused by rapid osteoclastic activity and peritrabecular fibrosis due to primary or secondary hyperparathyroidism resulting in a local destructive phenomenon. The differential diagnosis based on histological examination is only presumptive. Clinical, radiological and laboratory data are necessary for definitive diagnosis. Here, we report a very rare case of brown tumor involving maxilla and mandible, which is the result of secondary hyperparathyroidism in 30-year-old female patient with chronic renal disease. PMID:25328310

  1. Two Rare Magnetic Cataclysmic Variables with Extreme Cyclotron Features Identified in the Sloan Digital Sky Survey

    CERN Document Server

    Szkody, P; Schmidt, G; Hall, P B; Margon, B; Miceli, A; Subba-Rao, M; Frith, W J; Harris, H; Szkody, Paula; Anderson, Scott F.; Schmidt, Gary; Hall, Patrick B.; Margon, Bruce; Miceli, Antonino; Rao, Mark Subba; Frith, James; Harris, Hugh

    2003-01-01

    Two newly identified magnetic cataclysmic variables discovered in the Sloan Digital Sky Survey (SDSS), SDSSJ155331.12+551614.5 and SDSSJ132411.57+032050.5, have spectra showing highly prominent, narrow, strongly polarized cyclotron humps with amplitudes that vary on orbital periods of 4.39 and 2.6 hrs, respectively. In the former, the spacing of the humps indicates the 3rd and 4th harmonics in a magnetic field of ~60 MG. The narrowness of the cyclotron features and the lack of strong emission lines imply very low temperature plasmas and very low accretion rates, so that the accreting area is heated by particle collisions rather than accretion shocks. The detection of rare systems like these exemplifies the ability of the SDSS to find the lowest accretion rate close binaries.

  2. 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.

  3. PET imaging of blood flow and glucose metabolism in localized musculoskeletal tumors of the extremities

    Energy Technology Data Exchange (ETDEWEB)

    Lindholm, Paula, E-mail: paula.lindholm@tyks.f [Department of Oncology and Radiotherapy, Turku University Hospital, Turku FI-20521 (Finland); Turku PET Centre, Turku (Finland); Sutinen, Eija [Department of Oncology and Radiotherapy, Turku University Hospital, Turku FI-20521 (Finland); Turku PET Centre, Turku (Finland); Oikonen, Vesa [Turku PET Centre, Turku (Finland); Mattila, Kimmo [Department of Radiology, Turku University Hospital, Turku FI-20521 (Finland); Tarkkanen, Maija [Department of Oncology, Helsinki University Central Hospital, Helsinki (Finland); Kallajoki, Markku [Department of Pathology, Turku University Hospital, Turku FI-20521 (Finland); Aro, Hannu [Department of Orthopaedic Surgery, Turku University Hospital, Turku FI-20521 (Finland); Boehling, Tom [Department of Pathology, Helsinki University Central Hospital, Helsinki (Finland); Kivioja, Aarne [Department of Orthopaedic Surgery, Helsinki University Central Hospital, Helsinki, FI-00029 (Finland); Elomaa, Inkeri [Department of Oncology, Helsinki University Central Hospital, Helsinki (Finland); Minn, Heikki [Department of Oncology and Radiotherapy, Turku University Hospital, Turku FI-20521 (Finland); Turku PET Centre, Turku (Finland)

    2011-02-15

    Introduction: Little is known about blood flow in sarcomas. Our purpose was to study glucose metabolism and blood flow in untreated localized musculoskeletal tumors of the extremities using [{sup 18}F]fluorodeoxyglucose (FDG), oxygen-15 labeled water ([15O]H{sub 2}O) and positron emission tomography (PET). Methods: Six patients with high-grade osteosarcoma (OS), two with soft-tissue sarcoma (STS) and one with aneurysmal bone cyst had PET studies with [15O]H{sub 2}O and FDG. Arterial blood sampling and autoradiography calculation method were used to define blood flow as milliliters per 100 g times minutes. Tumor FDG uptake was measured as standardized uptake values (SUVs) and regional metabolic rates for FDG (rMRFDG). Two patients also had FDG PET studies during (one patient) and after (two patients) preoperative chemotherapy. All patients underwent dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI). The PET findings were compared with the clinical follow-up data and results of DCE-MRI. Results: Blood flow in bone tumors was 31.7-75.2 ml/(100 gxmin) and in STS 9.0-45.9 ml/(100 gxmin). [{sup 18}F]-Fluorodeoxyglucose uptake and rMRFDG in untreated bone tumors were 5.4-18.4 and 10.9-57.4 {mu}mol/100 g/min, respectively. [{sup 18}F]-Fluorodeoxyglucose uptake and rMRFDG in STS were 2.6-11.5 and 5.6-32.2 {mu}mol/100 g/min, respectively. Four of five sarcomas with SUV>9.0 have already relapsed. High blood flow in untreated OS was related to long overall survival, while the predictive power of glucose metabolism was less apparent. Good histopathological response to therapy was not associated with long survival. Conclusions: Measurement of blood flow in musculoskeletal tumors appears to be feasible by PET and [{sup 15}O]H{sub 2}O. The influence of tumor blood flow and glucose metabolism on the final outcome in sarcoma is variable and needs further research.

  4. Coexistence of tuberculous axillary lymphadenitis and giant borderline malignant phyllodes tumor of the breast: A rare case report

    Directory of Open Access Journals (Sweden)

    Sunder Goyal

    2015-04-01

    Full Text Available Cystosarcoma phyllodes are uncommon breast tumors which rarely metastasize to axillary lymph nodes. The tumor is similar to fibroadenoma in structure, but it is different histologically. Although surgery (excision vs. mastectomy is the mainstay of treatment, the need for adjuvant therapies such as radiotherapy for a malignant variety is unclear. Its association with ipsilateral tubercular axillary lymph nodes has not been reported in literature so far. We report a 35-year-old female that presented with a giant borderline malignant phyllodes tumor of the right breast along with ipsilateral tubercular granulomatous axillary lymph nodes. [Arch Clin Exp Surg 2015; 4(2.000: 114-117

  5. The lipoma of tongue - A rare site for a tumor: Case report and review of the literature

    Science.gov (United States)

    Baonerkar, Hemant A.; Vora, Meena; Sorathia, Rakesh; Shinde, Swapnil

    2015-01-01

    Lipoma is the most common tumor of the human body, but their presences in the oral cavity are very rare. Reported cases of lipoma of tongue in English literature are very few. Here, we report a case of lipoma of tongue in 63-year-old male patient, with its clinical presentation, the histological picture, classification, and brief review of the literature. PMID:26752882

  6. Outcome and Prognostic Factors in Endometrial Stromal Tumors: A Rare Cancer Network Study

    Energy Technology Data Exchange (ETDEWEB)

    Schick, Ulrike, E-mail: Ulrike.schick@icr.ac.uk [Department of Radiation Oncology, University Hospital, Geneva (Switzerland); Bolukbasi, Yasmin [Department of Radiation Oncology, Ege University Hospital, Izmir (Turkey); Thariat, Juliette [Department of Radiation Oncology, Antoine Lacassagne Center, Nice (France); Abdah-Bortnyak, Roxolyana; Kuten, Abraham [Department of Radiation Oncology, Rambam Medical Center, Haifa (Israel); Igdem, Sefik [Department of Radiation Oncology, Metropolitan Hospital, Istanbul (Turkey); Caglar, Hale [Department of Radiation Oncology, Marmara University Hospital, Istanbul (Turkey); Ozsaran, Zeynep [Department of Radiation Oncology, Ege University Hospital, Izmir (Turkey); Loessl, Kristina [Department of Radiation Oncology, University Hospital, Bern (Switzerland); Schleicher, Ursula [Department of Radiation Oncology, Dueren Hospital, Dueren (Germany); Zwahlen, Daniel [Department of Radiation Oncology, William Buckland Radiotherapy Centre, Melbourne (Australia); Villette, Sylviane [Department of Radiation Oncology, Rene Huguenin Center, Saint-Cloud (France); Vees, Hansjoerg [Department of Radiation Oncology, University Hospital, Geneva (Switzerland); Department of Radiation Oncology, Sion Hospital, Sion (Switzerland)

    2012-04-01

    Purpose: To provide further understanding regarding outcome and prognostic factors of endometrial stromal tumors (EST). Methods and Materials: A retrospective analysis was performed on the records of 59 women diagnosed with EST and treated with curative intent between 1983 and 2007 in the framework of the Rare Cancer Network. Results: Endometrial stromal sarcomas (ESS) were found in 44% and undifferentiated ESS (UES) in 49% of the cases. In 7% the grading was unclear. Of the total number of patients, 33 had Stage I, 4 Stage II, 20 Stage III, and 1 presented with Stage IVB disease. Adjuvant chemotherapy was administered to 12 patients, all with UES. External-beam radiotherapy (RT) was administered postoperatively to 48 women. The median follow-up was 41.4 months. The 5-year overall survival (OS) rate was 96.2% and 64.8% for ESS and UES, respectively, with a corresponding 5-year disease-free survival (DFS) rate of 49.4% and 43.4%, respectively. On multivariate analysis, adjuvant RT was an independent prognostic factor for OS (p = 0.007) and DFS (p = 0.013). Locoregional control, DFS, and OS were significantly associated with age ({<=}60 vs. >60 years), grade (ESS vs. UES), and International Federation of Gynecology and Obstetrics stage (I-II vs. III-IV). Positive lymph node staging had an impact on OS (p < 0.001). Conclusion: The prognosis of ESS differed from that of UES. Endometrial stromal sarcomas had an excellent 5-year OS, whereas the OS in UES was rather low. However, half of ESS patients had a relapse. For this reason, adjuvant treatment such as RT should be considered even in low-grade tumors. Multicenter randomized studies are still warranted to establish clear guidelines.

  7. A extremely rare case of cervical intramedullary granuloma due to Brucella accompanied by Chiari Type-1 malformation.

    Science.gov (United States)

    Tufan, Kadir; Aydemir, Fatih; Sarica, Feyzi Birol; Kursun, Ebru; Kardes, Özgür; Cekinmez, Melih; Caner, Hakan

    2014-01-01

    Chiari Type-1 malformation is displacement of the cerebellar tonsils through the foramen magnum into the cervical spine and usually does not exceed the level of C2. It is 50-70% associated with syringomyelia. Nervous system involvement due to brucellosis is called neurobrucellosis, and neurological involvement rate has been reported an average of 3-5%, ranging between 3% and 25% at different series. Intramedullary abscess or granuloma due to Brucella is extremely rare. Hence far, six cases have been reported in the literature and only two of these cases were reported as intramedullary granuloma. This case is presented in order to remind the importance of the cervical cord granuloma which was presented once before in the literature and to emphasize the importance of evaluation of patient history, clinical and radiological findings together in the evaluation of a patient.

  8. Gallstone ileus of the sigmoid colon: an extremely rare cause of large bowel obstruction detected by multiplanar CT.

    Science.gov (United States)

    Carlsson, Tarryn; Gandhi, Sanjay

    2015-12-18

    Gallstone ileus of the sigmoid colon is an important, though extremely rare, cause of large bowel obstruction. The gallstone often enters the large bowel through a fistula formation between the gallbladder and colon, and impacts at a point of narrowing, causing large bowel obstruction. We describe the case of an 80-year-old woman who presented with features of bowel obstruction. Multiplanar abdominal CT demonstrated a cholecystocolonic fistula in exquisite detail. The scan also showed obstruction of the colon due to a large gallstone impacted just proximal to a stricture in the sigmoid. Owing to inflammatory adhesions and a stricture from extensive diverticular disease, the gallstone could not be retrieved. This frail and elderly woman was treated with a loop colostomy to relieve bowel obstruction. The patient made an uneventful recovery.

  9. 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.

  10. Mediastinal tuberculous lymphadenitis presenting as an esophageal intramural tumor: A very rare but important cause for dysphagia

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    Dysphagia associated with esophageal mechanical obstruction is usually related to malignant esophageal diseases. Benign lesions are rarely a cause for this type of dysphagia, and usually occur either as an intramural tumor or as an extrinsic compression.Mediastinal tuberculous lymphadenitis is rare in adults, and even more rarely causes dysphagia. We report two cases of dysphagia in adult patients caused by mediastinal tuberculous lymphadenitis, presenting radiologically and endoscopically as an esophageal submucosal tumor.Based on the clinical and imaging diagnosis, the patients underwent a right thoracotomy, and excision of the mass attached to and compressing the esophagus. Pathological examination of the specimens showed a chronic granulomatous inflammation with caseous necrosis,which was consistent with tuberculous lymphadenitis.

  11. 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.

  12. Primary extraskeletal peripheral primitive neuroectodermal tumor of subcutaneous tissue neck in a young adult: A rare case report

    Directory of Open Access Journals (Sweden)

    Sumeet Aggarwal

    2016-01-01

    Full Text Available Primitive neuroectodermal tumors (PNETs are poorly differentiated small round cell neoplasms which primarily affect childhood age and very rarely seen in adults. Peripheral PNET (pPNET cases are very rare compared to central PNET, and most of them originate from neural crest cells located outside the central nervous system. We report a case of large extraosseous pPNET arising from subcutaneous tissue of left side neck in a young male patient. Despite aggressive inherent nature of histology, structural complexity of neck area and poor predictors like tumor size >6 cm, extraosseous nature, this case advocate that complete resolution is possible with aggressive multimodal treatment including surgery, radiation therapy, and chemotherapy. Other major concerns in such cases of pPNET are difficulty in diagnosis due to low incidence, unpredictable site involvement, histological similarity with other round cell tumors and lack of established treatment guidelines.

  13. A rare cause of intraabdominal tumors: An analysis of 21 patients with mesenteric cyst

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    Akın Önder

    2011-12-01

    Full Text Available Objectives: Mesenteric cysts are rare intraabdominal tumors. In this study, we aimed to investigate the clinical findings, pathological features and surgical approaches of mesenteric cysts.Materials and methods: Between January 1985 - December 2010 at Dicle University Medical Faculty General Surgery and Pediatric Surgery Department, 21 patients with mesenteric cyst were retrospectively analyzed.Results: Sixteen patients (76.2% were women and 5 (23.8% were men, with a mean age of 32.62 ± 22:19 (4-79 years. The most common symptoms were abdominal mass and pain. Mesenteric cysts were often located on the small bowel mesentery (61.9%. Cyst sizes ranged from 4-25 cm in diameters. The most common performed surgical procedures in patients were enucleation (90.5%. The histopathological diagnosis was confirmed as cystic lymphangioma in 17 (80.9% patients. The mean length of hospitals stay was 5.29 ± 2.28 (2-11 days. Postoperative morbidity rate was 33.3% and the most common complication was wound infection. There was no postoperative mortality.Conclusion: When physical examination revealed a palpable mass with regular margins and intraabdominal cystic mass is detected with imaging techniques, mesenteric cyst should be considered in differential diagnosis in patients presenting with abdominal pain. J Clin Exp Invest 2011; 2 (4: 380-383Key words: Mesenteric cyst, abdominal pain, palpabl mass, surgery

  14. Computer-Aided Treatment Design of a Distal Upper Extremity Soft Tissue Tumor with Electron Beam Radiotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Li, Chun; Crawford, Susan; Mundt, A.J.; Vijayakumar, S.

    2015-01-15

    We present here a novel approach for the planning and treatment of a distal upper extremity soft tissue tumor. Utilizing computed tomography (CT) based electron dose calculations, a customized compensating wax bolus was designed in order to deliver sufficient dose coverage to the tumor volume while sparing the draining lymphatics of the patient’s hand and digits. A clinical case is presented as well as the design and construction of the compensator. Outcome and clinical implications are discussed.

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

    Science.gov (United States)

    Nadrowski, Karin; Pietsch, Katherina; Baruffol, Martin; Both, Sabine; Gutknecht, Jessica; Bruelheide, Helge; Heklau, Heike; Kahl, Anja; Kahl, Tiemo; Niklaus, Pascal; Kröber, Wenzel; Liu, Xiaojuan; Mi, Xiangcheng; Michalski, Stefan; von Oheimb, Goddert; Purschke, Oliver; Schmid, Bernhard; Fang, Teng; Welk, Erik; Wirth, Christian

    2014-01-01

    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.

  16. Intradural tumoral calcinosis of the foramen magnum region: A case report.

    Science.gov (United States)

    Wang, Hao; Zhao, Yuanli; Yang, Jun

    2016-10-11

    Tumoral calcinosis is a rare disorder characterized by tumor-like calcified masses commonly occurring in the periarticular regions of the extremities. Conversely, intradural tumoral calcinosis of the spine is extremely rare, with only two previously reported cases. To our knowledge, this is the first intradural case located in the foramen magnum region.

  17. Rare Skin Adnexal and Melanocytic Tumors Arising in Ovarian Mature Cystic Teratomas: A Report of 3 Cases and Review of the Literature.

    Science.gov (United States)

    Moulla, Alexandra A; Magdy, Nesreen; Francis, Nicholas; Taube, Janis; Ronnett, Brigitte M; El-Bahrawy, Mona

    2016-09-01

    Mature teratoma of the ovary is the most common primary ovarian tumor accounting for 15% (10%-20%) of all ovarian neoplasms. Skin and skin adnexal structures are the most common elements identified in mature teratomas. Benign and malignant skin tumors can arise in ovarian teratomas, the most common being epithelial tumors. Melanocytic and adnexal tumors developing in a teratoma are rare and can be easily overlooked. We report 3 cases and review melanocytic and skin adnexal tumors encountered in ovarian teratomas.

  18. A mixed neoplasm of intraosseous hemangioma with an ameloblastoma: a case of collision tumor or a rare variant?

    Directory of Open Access Journals (Sweden)

    Harshvardhan Shridhar Jois

    2011-12-01

    Full Text Available Hemangiomas of the head and neck are considered to be benign tumors of infancy that are characterized by a rapid growth phase with endothelial cell proliferation, followed by gradual involution. Central hemangiomas are a rare occurrence and even rarer are the hybrid tumors of central hemangiomas with odontogenic tumors such as ameloblastomas. This paper reports a case of one such hybrid tumor in a middle aged adult clinical presenting as a mandibular swelling with indistinct mixed radiographic presentation and histopathologically comprising of intimately associated hemangiomatous vascular channels and typical ameloblastic areas. To the authors’ knowledge this is the sixth case of such a hemangiomatous ameloblastoma which has been reported till date.

  19. An odontogenic tumor mimicking a dentigerous cyst in a 13-year-old female: A rare clinical presentation

    Directory of Open Access Journals (Sweden)

    Leelavathy Jegadeesan

    2016-01-01

    Full Text Available Ameloblastic fibroma is a relatively rare benign odontogenic tumor in which both the epithelial and ectomesenchymal components are neoplastic. A 14-year-old girl presented with a complaint of unerupted maxillary right premolar. The panoramic radiograph revealed a well-circumscribed unilocular radiolucency involving an unerupted maxillary right first premolar. The lesion was enucleated and the material was sent for histopathologic examination. This case emphasized the importance of careful differential diagnosis of intraosseous oral lesions and reported a rare lesion as well as its atypical location.

  20. 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.

  1. Pleomorphic liposarcoma arising in a malignant phyllodes tumor of breast: A rare occurrence.

    Science.gov (United States)

    Sancheti, Sankalp M; Sawaimoon, Satyakam K; Ahmed, Rosina

    2015-01-01

    Primary malignant phyllodes tumor of the breast accounts for 0.3-1% of all the tumors of breast and only a couple of cases of pleomorphic liposarcoma (PL) arising in a malignant phyllodes (MP) tumor have been reported. A thorough sampling is most essential in phyllodes tumor, not only to detect high grade component of the neoplasm but also to diagnose heterologous elements in the same lesion elsewhere, as it may affect the prognosis adversely and may have a greater metastatic potential.

  2. Testicular tumors with tumor thrombosis within the inferior vena cava: Two case reports

    Energy Technology Data Exchange (ETDEWEB)

    Park, Ji Eun; Moon, Sung Kyoung; Lim, Joo Won; Park, Seong Jin [Kyung Hee Univ. Hospital/College of Medicine/Kyung Hee Univ. Seoul (Korea, Republic of)

    2012-09-15

    Testicular tumors are almost all malignant tumors and can develop in younger age groups. Testicular tumors are mostly curable, with reported cases of tumor thrombosis within the inferior vena cava being rare. Two patients, aged 35 years and 37 years old complaining of testicular pain and lower abdominal pain were diagnosed with testicular tumors by ultrasound. In addition, tumor thrombus of the inferior vena cava was diagnosed concomitantly at the time of the diagnosis by computed tomography and magnetic resonance imaging. Here, we report testicular tumors accompanied by tumor thrombus, which is an extremely rare finding, with limited reports available. Pathologic diagnoses were seminoma and mixed germ cell tumors, respectively.

  3. Review of pineal anlage tumor with divergent histology.

    Science.gov (United States)

    Berns, Stephen; Pearl, Gary

    2006-08-01

    Pineal anlage tumor is an extremely rare tumor that is not listed in the 2000 World Health Organization Classification of nervous system tumors. It has been defined as a primary pineal tumor with both neuroepithelial and ectomesenchymal differentiation and without endodermal differentiation. We review the literature on this tumor, including the clinical presentation, gross pathology, histopathology, immunohistochemistry, differential diagnosis, and prognosis.

  4. Differentiated Malignant Myoepithelioma of the Parotid Gland: A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Jagannath Dev Sharma

    2014-12-01

    Full Text Available Malignant myoepithelioma (MM is an epithelial malignant tumor in which predominant differentiation of the tumor cells are myoepithelial in nature. Dedifferentiated MM is an extremely rare entity. We report here a rare case of the parotid tumor of differentiated MM with its many morphological and immunohistochemistry features. [Natl J Med Res 2014; 4(4.000: 380-382

  5. A rare case of repeated anastomotic recurrence due to tumor implantation after curative surgery for sigmoid colon cancer

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    Shiokawa Hiroyuki

    2007-08-01

    Full Text Available Abstract Background Anastomotic recurrence is often experienced at colocolic or colorectal anastomoses. Tumor cell implantation has been reported as the mechanism of anastomotic recurrence. However, anastomotic recurrence occurring repeatedly after curative surgery is rare. We herein report a rare case of repeated anastomotic recurrence after curative surgery for sigmoid colon cancer. Case presentation A 51-year-old man underwent radical surgery for sigmoid colon cancer. However, anastomotic recurrence developed three times during three years and six months after the initial operation in spite of irrigation with 5% povidone-iodine before anastomosis. The serum carcinoembryonic antigen (CEA level had been within normal limits after sigmoidectomy. Finally, the patient underwent abdominoperineal resection. The clinico-pathological findings revealed that possible tumor cell implantation caused these anastomotic recurrences. The patients survived without recurrence during the follow-up period of seven years and nine months. Conclusion We experienced a rare case of repeated anastomotic recurrence due to possible tumor implantation after curative surgery for sigmoid colon cancer; however the prognosis was ultimately very good. CEA monitoring was insensitive for detection of anastomotic recurrence in this case.

  6. Identification of a Novel NLRP12 Nonsense Mutation (Trp408X in the Extremely Rare Disease FCAS by Exome Sequencing.

    Directory of Open Access Journals (Sweden)

    Xiaoru Xia

    Full Text Available Familial cold autoinflammatory syndrome (FCAS is an extremely rare autosomal dominant inherited disease. Although there are four genes that have been linked with FCAS, its molecular diagnosis has been challenging in a relatively large proportion of cases. In this study, we aimed to investigate the genetic defect of a recruited FCAS family using exome sequencing followed by in-depth bioinformatics analysis. As a result, a novel heterozygous stop-gain mutation (Trp408X in NLRP12 was identified in autosomal dominant inherited FCAS with clinical features of recurrent fever and skin urticaria due to cold conditions. When combined with previous studies, all of the reported mutations were found to have occurred in a highly conserved region in the NACHT domain coding sequence in NLRP12 exon 3, suggesting that a screening strategy for FCAS should focus on this area of the gene. In conclusion, this study demonstrates the importance of exome sequencing for clinical diagnosis of genetic disorders and provides molecular insight into FCAS treatment and diagnosis.

  7. 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.

  8. 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.

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

    Directory of Open Access Journals (Sweden)

    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.

  10. OUR EXPERIENCE WITH RARE PRESENTATION OF GASTROINTESTINAL STROMAL TUMORS IN A RURAL MEDICAL COLLEGE HOSPITAL

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    Jigar Vipul

    2013-10-01

    Full Text Available ABSTRACT : Gastrointestinal stromal tumors (GIST - are one of the most common mesenchymal tumors of the gastrointestinal tract [1 - 3% of all gastrointestinal malignancies]. Their behaviour is driven by mutations in the kit gene or PDGFRA gene and may or may not positively stain for kit. We report fo ur additional cases of a GIST presenting as an abdominal mass along with a pertinent review of the literature. All four patients received surgical resection. The mean tumor size was 10.5 with an average mitotic index of 6.25 per 50 high power fields. Three patients were disease free and one patient came with recurrence. In conclusion, symptomatic patents have an increased incidence of high - risk tumors and metastases at presentation. Adjuvant therapy with imatinib improves disease - free survival in patients w ith large abdominal GIST tumors, but no change in overall survival was noted. KEY WORD: Gastrointestinal stromal tumors; Imatinib; mitotic index;Meckel’s Diverticulum

  11. Giant cell tumor of the tendon sheath: a rare periungual location simulating myxoid cyst*

    Science.gov (United States)

    Minotto, Renan; Rodrigues, Camila Britto; Grill, Aline Barcellos; Furian, Roque

    2017-01-01

    Giant cell tumor of the tendon sheath is a benign soft tissue tumor most frequent between the third and fifth decades of life. It can mimic and make differential diagnoses with several hand tumors. Definitive diagnosis and the treatment of choice are reached with complete resection and histopathological examination. Here we describe a case with clinical presentation similar to that of a myxoid cyst. PMID:28225971

  12. Perineurial malignant peripheral nerve sheath tumor in the setting of multiple soft tissue perineuriomas: A rare presentation of an uncommon tumor

    Directory of Open Access Journals (Sweden)

    Bharat Rekhi

    2013-01-01

    Full Text Available Perineurioma is an uncommon soft tissue tumor with characteristic histological and immunohistochemical features. Herein, this tumor is presented within a rare clinical setting, in a 45-year-old gentleman, with multiple soft tissue swellings and a previous history of surgical excision of a thigh mass. Four years back, he developed multiple soft tissue tumor swellings for which he lately underwent multiple wide excisions, elsewhere that were reported as multiple dermatofibrosarcoma protuberans (DFSPs. Histopathological review of the thigh and axillary tumor showed a perineurioma (EMA+, S100-P-ve, CD34-ve, low MIB1, whereas the recurrent leg mass disclosed a high-grade perineurial malignant peripheral nerve sheath tumor (MPNST (EMA+, CD56+, S100-P-, high MIB1, CD34-ve, p53+ve. Within four months of post-excision, he developed additional swellings, and died of respiratory insufficiency. This case forms the first documented case of perineurial MPNST with multiple, metachronous soft tissue perineuriomas. Available literature review of perineurial MPNSTs and diagnostic implications are discussed herewith

  13. Radiotherapy for marginally resected, unresectable or recurrent giant cell tumor of the bone: a rare cancer network study

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    Robert C. Miller

    2011-10-01

    Full Text Available The role of radiotherapy for local control of marginally resected, unresectable, and recurrent giant cell tumors of bone (GCToB has not been well defined. The number of patients affected by this rare disease is low. We present a series of 58 patients with biopsy proven GCToB who were treated with radiation therapy. A retrospective review of the role of radiotherapy in the treatment of GCToB was conducted in participating institutions of the Rare Cancer Network. Eligibility criteria consisted of the use of radiotherapy for marginally resected, unresectable, and recurrent GCToB. Fifty-eight patients with biopsy proven GCToB were analyzed from 9 participating North American and European institutions. Forty-five patients had a primary tumor and 13 patients had a recurrent tumor. Median radiation dose was 50 Gy in a median of 25 fractions. Indication for radiation therapy was marginal resection in 33 patients, unresectable tumor in 13 patients, recurrence in 9 patients and palliation in 2 patients. Median tumor size was 7.0 cm. A significant proportion of the tumors involved critical structures. Median follow- up was 8.0 years. Five year local control was 85% . Of the 7 local failures, 3 were treated successfully with salvage surgery. All patients who received palliation achieved symptom relief. Five year overall survival was 94%. None of the patients experienced grade 3 or higher acute toxicity. This study reports a large published experience in the treatment of GCToB with radiotherapy. Radiotherapy can provide excellent local control for incompletely resected, unresectable or recurrent GCToB with acceptable morbidity.

  14. Dacarbazine-Doxorubicin therapy ameliorated an extremely aggressive mesenteric desmoid tumor associated with familial adenomatous polyposis: report of a case.

    Science.gov (United States)

    Ezumi, Koji; Yamamoto, Hirofumi; Takemasa, Ichiro; Nomura, Masaya; Ikeda, Masataka; Sekimoto, Mitsugu; Monden, Morito

    2008-03-01

    A 30-year-old man with familial adenomatous polyposis (FAP) underwent prophylactic proctocolectomy by laparoscopy-assisted surgery. After 10 months, we found an intra-abdominal tumor, which grew rapidly to 25 cm in diameter. We performed an emergency operation, which revealed that it was a desmoid tumor derived mainly from colorectal mesenterium. The tumor was removed with three short segments of intestine and the left ureter. A computed tomography (CT) scan done 3 months later showed a 10 cm mesenteric desmoid tumor at the beginning of jejunum, approaching the root of the superior mesenteric artery (SMA). Fortunately, we were able to remove the tumor without injuring the SMA. To our distress, however, another recurrent mesenteric desmoid tumor was discovered in the pelvis one month later, which grew rapidly from 5 cm to 16 cm within 4 months. During this period, we gave the patient several regimens, including antiestrogen (tamoxifen), a nonsteroidal anti-inflammtory drug and imatinib mesylate (Gleevec), which had little or no effect. Finally, when the desmoid occupied the pelvic space, we gave the patient dacarbazine (DTIC) and doxorubicin (DOX). After seven courses, the mesenteric tumor showed an almost complete response (CR). The chemotherapy caused grade 3 to 4 leukocytopenia, but without any hazardous events. No evidence of further recurrence of mesenteric desmoid has been seen for 4 years. This combination chemotherapy is a promising strategy, even against an extremely aggressive, life-threatening mesenteric desmoid associated with FAP.

  15. Cystic adenomatoid tumor of the uterus

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    Varsha Manucha

    2015-01-01

    Full Text Available We present a case of a cystic adenomatoid tumor in a 40-year-old woman. The tumor was an intramural multicystic mass, histologically similar to a multicystic mesothelioma. Cystic adenomatoid tumors of the uterus are extremely rare. They present with a wide differential diagnosis in radiology. The tumors are known to be benign and awareness of this rare entity is the key to its diagnosis for a pathologist.

  16. Gliosarcomas arising from the pineal gland region: uncommon localization and rare tumors.

    Science.gov (United States)

    Sugita, Yasuo; Terasaki, Mizuhiko; Tanigawa, Ken; Ohshima, Koichi; Morioka, Motohiro; Higaki, Koichi; Nakagawa, Setsuko; Shimokawa, Shoko; Nakashima, Susumu

    2016-02-01

    Gliosarcomas are a variant of glioblastomas and present a biphasic pattern, with coexisting glial and mesenchymal components. In this study, two unusual cases are presented. Case 1 is a 52-year-old woman with a headache and memory disturbance for a month. Case 2 is an 18-year-old man with a headache lasting two weeks. In both cases, an MRI revealed enhancing T1-low to iso, T2-iso to high intensity lesions in the pineal gland region. Histologically, in case 1, the tumor showed spindle cell proliferation with disorganized fascicles and cellular pleomorphism. Tumor cells variously exhibited oncocytic transformation. Immunohistochemically, most of the spindle tumor cells were positive for myoglobin and desmin. Some of the tumor cells were positive for GFAP and S-100 protein. On the other hand, all tumor cells were positive for CD133, Musashi1, and SOX-2 which are the markers of neural stem cells. In case 2, the tumor showed monotonous proliferation of short spindle cells with disorganized fascicles and cellular atypism. The morphological distinction between glial and mesenchymal components was not apparent. Immunohistochemically, most of the spindle tumor cells were positive for desmin. Glial tumor cells that were dispersed within the sarcoma as single cells were positive for GFAP. In addition, all tumor cells were positive for CD133, Musashi1 and SOX-2. Based on these microscopic appearances, and immunohistochemical findings, these cases were diagnosed as gliosarcomas arising from the pineal gland region. These results also indicated that pluripotential cancer stem cells differentiated into glial and muscle cell lines at the time of tumor growth. In a survey of previous publications on gliosarcoma arising from the pineal gland, these cases are the second and third reports found in English scientific writings.

  17. Renal cell carcinoma with t(6:11 (p21;q12. A case report highlighting distinctive immunohistologic features of this rare tumor

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    Sarabjeet Kaur Arneja

    2015-01-01

    Conclusion: Knowledge of distinctive morphological and immuno-histochemical features of this tumor can help in establishing a diagnosis of this rare subset of translocation associated RCC on routine hematoxylin and eosin (H and E staining and immunophenotyping.

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

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    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.

  19. Um caso raro de tumores torácicos malignos sincrônicos A rare case of synchronous malignant thoracic tumors

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    Benoit Jacques Bibas

    2009-02-01

    Full Text Available Tumores neurogênicos malignos do mediastino em adultos são raros e extremamente agressivos. Este artigo relata o caso de um paciente de 61 anos com a ocorrência simultânea de schwannoma maligno de mediastino e carcinoma bronquíolo-alveolar. Apesar do carcinoma bronquíolo-alveolar estar presente em 4-7% dos tumores torácicos sincrônicos ressecados, essa associação nunca foi apresentada na literatura. É, no entanto, um achado frequente em pacientes com infiltrados aparentemente inflamatórios e com opacidades em vidro fosco, como apresentado neste caso.Malignant neurogenic mediastinal tumors in adults are uncommon and extremely aggressive. We report the case of a 61-year-old male patient with the simultaneous occurrence of malignant mediastinal schwannoma and bronchioloalveolar carcinoma. Although bronchioloalveolar carcinoma is present in 4-7% of the resected synchronous thoracic tumors, this association has never been reported in the literature. However, it is a common finding in patients presenting apparently inflammatory infiltrates and ground-glass opacities, as in the case presented here.

  20. Small cell carcinoma of the submandibular gland: a rare small round blue cell tumor.

    Science.gov (United States)

    Walters, David M; Little, Stewart C; Hessler, Richard B; Gourin, Christine G

    2007-01-01

    The presence of small cell carcinoma within the submandibular gland is an uncommon clinical entity. However, other small round blue cell tumors are encountered in the head and neck with greater frequency. These include lymphoma, Ewing's sarcoma, melanoma, esthesioneuroblastoma, and neuroblastoma. A basic knowledge of the immunohistochemical studies available to distinguish each these tumors from one another significantly improves the frequency of accurate and timely initial diagnosis. We report a case of small cell carcinoma of the submandibular gland and review the other common small round blue cell tumors that occur within the head and neck. We utilize an acronym, LEMONS, to organize our review and facilitate improved retention of the differential diagnosis for small round blue cell tumors of the head and neck.

  1. A rare case of recurrent malignant phyllodes tumor of the breast in a young nulliparous woman

    Directory of Open Access Journals (Sweden)

    Zeeshanuddin Ahmad

    2014-01-01

    Full Text Available Phyllodes tumors (PT are fibroepithelial neoplasm characterized by a combination of hypercellular stroma and cleft-like or cystic spaces lined by epithelium, into which the stroma classically project in a leaf-like fashion and have a potential to recur and metastasize. These tumors are more common in 3 rd to 4 th decade. Here we present a case of recurrent malignant PT of the breast in a young nulliparous woman.

  2. Vertebral Artery Transposition Via an Extreme-Lateral Approach for Anterior Foramen Magnum Meningioma or Craniocervical Junction Tumors.

    Science.gov (United States)

    Park, Hun Ho; Lee, Kyu-Sung; Hong, Chang-Ki

    2016-04-01

    Vertebral artery (VA) transposition in the extreme-lateral transcondylar approach can minimize the manipulation of the low cranial nerves and the brain stem. The authors describe the surgical technique of VA transposition. From March 2000 to December 2014, 28 of 48 patients underwent VA transposition for anterior foramen magnum meningioma (16 patients) and craniocervical junction (CCJ) tumors (12 patients). Tumor was resected via an extreme-lateral approach with partial condylectomy to expose the anterior portion of the brain stem. For intradural tumors, the VA was mobilized caudomedially after circumferential dural incision around the VA at the level of the foramen magnum. For extradural tumors involving the CCJ, VA was transposed medially from the transverse foramen of C1 without any dural incision. Gross total resection was achieved in 26 of 28 patients (92.9%) with VA transposition. Histologically, meningioma and schwannoma were most common. The origin of the tumors was foramen magnum (57.1%), C1 nerve root (17.9%), clivus (10.7%), jugular foramen (7.1%), posterior skull base (3.6%), and hypoglossal canal (3.6%). VA transposition was performed intradurally in 19 patients (67.9%) and extradurally in 9 patients (32.1%). Surgical morbidity was 17.9% including 4 patients with hypoglossal nerve palsy and 1 patient with quadriparesis. The mean follow-up duration after surgery was 4.2 years (range, 0.1-14.8 years). VA transposition can provide a wide surgical window for anterior foramen magnum meningioma or tumors involving the CCJ with the least manipulation of the neuraxis. Copyright © 2016 Elsevier Inc. All rights reserved.

  3. 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.

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

    Directory of Open Access Journals (Sweden)

    Hyunsik Jang

    2016-04-01

    Full Text Available Purpose: 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. Methods: 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. Results: 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%. Conclusion: 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.

  5. Peripheral primitive neuroectodermal tumor of the adrenal gland: A rare entity

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    Chandan Phukan

    2013-01-01

    Full Text Available Peripheral primitive neuroectodermal tumor (PNET is an uncommon tumor and the overall incidence is 1% of all sarcomas. PNET of the adrenal gland is an even rarer entity. A 37-year-old female was evaluated for an episode of loin pain. Ultrasonography showed a large heterogenous left adrenal mass with internal echogenic components. Computed tomography did not show any fat density within to suggest a myelolipoma. Biopsy suggested a poorly differentiated neoplasm with a possibility of PNET of the adrenal gland.

  6. Tumorer

    DEFF Research Database (Denmark)

    Prause, J.U.; Heegaard, S.

    2005-01-01

    oftalmologi, øjenlågstumorer, conjunctivale tumorer, malignt melanom, retinoblastom, orbitale tumorer......oftalmologi, øjenlågstumorer, conjunctivale tumorer, malignt melanom, retinoblastom, orbitale tumorer...

  7. 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

  8. 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

  9. Cytotoxicity, tumor targeting and PET imaging of sub-5 nm KGdF4 multifunctional rare earth nanoparticles

    Science.gov (United States)

    Cao, Xinmin; Cao, Fengwen; Xiong, Liqin; Yang, Yang; Cao, Tianye; Cai, Xi; Hai, Wangxi; Li, Biao; Guo, Yixiao; Zhang, Yimin; Li, Fuyou

    2015-08-01

    Ultrasmall sub-5 nm KGdF4 rare earth nanoparticles were synthesized as multifunctional probes for fluorescent, magnetic, and radionuclide imaging. The cytotoxicity of these nanoparticles in human glioblastoma U87MG and human non-small cell lung carcinoma H1299 cells was evaluated, and their application for in vitro and in vivo tumor targeted imaging has also been demonstrated.Ultrasmall sub-5 nm KGdF4 rare earth nanoparticles were synthesized as multifunctional probes for fluorescent, magnetic, and radionuclide imaging. The cytotoxicity of these nanoparticles in human glioblastoma U87MG and human non-small cell lung carcinoma H1299 cells was evaluated, and their application for in vitro and in vivo tumor targeted imaging has also been demonstrated. Electronic supplementary information (ESI) available: Details of the experimental section as well as EDXA, XRD, zeta potential, FTIR, TGA, stability, TEM, Z scanning, ICP-MS, and MicroPET/CT images. See DOI: 10.1039/c5nr03374h

  10. Peripheral papillary tumor of type-II pneumocytes: a rare neoplasm of undetermined malignant potential.

    Science.gov (United States)

    Dessy, E; Braidotti, P; Del Curto, B; Falleni, M; Coggi, G; Santa Cruz, G; Carai, A; Versace, R; Pietra, G G

    2000-03-01

    Peripheral papillary adenomas of the lung are uncommon neoplasms (only ten cases have been described so far in the English literature) composed predominantly of type-II pneumocytes and generally considered benign. We describe here two additional cases of this lung tumor. In both cases histological examination revealed an encapsulated papillary neoplasm with invasion of the capsule and, in one case, invasion of the adjacent alveoli and visceral pleura too. The proliferative index (Ki67) was less than 2% and the epithelial cells were positive for cytokeratins, surfactant apoproteins (SP), and nuclear thyroid transcription factor-1 (TTF- 1). Ultrastructurally, the epithelial cells showed the characteristic surface microvilli and cytoplasmic lamellar inclusions of type-II cells. Review of the literature has revealed two other cases of peripheral papillary adenoma of type-II pneumocytes with infiltrative features. Thus, we propose replacing the term peripheral papillary adenoma with peripheral papillary tumor of undetermined malignant potential.

  11. The Rare Association of Spina Bifida and Extrarenal Wilms Tumor: A Case Report and Review of the Literature.

    Science.gov (United States)

    Igbaseimokumo, Usiakimi; Cartwright, Cathy; Lewing, Karen; Hutchison, Lisa; Habeebu, Sultan

    2017-08-01

    The diagnosis of nephroblastoma outside of the kidneys, in the absence of a renal primary tumor, is known as extrarenal Wilms tumor (ERWT). ERWT is an uncommon entity that typically involves the embryonic path of the developing kidneys and gonads. The occurrence of ERWT in a dysraphic spine is uncommon, with no reported cases of preoperative diagnosis, with all cases diagnosed at pathology. These tumors are malignant and ideally should be completely excised. Thus, preoperative diagnosis would be highly desirable. A newborn female was found to have a lumbar lipoma. Magnetic resonance imaging (MRI) was performed to rule out lipomyelomeningocele. The MRI showed a dorsal lipoma on the terminal spinal cord, as well as a 2 × 2 cm uniformly enhancing mass abutting the bifid posterior elements of L5. The lesion was completely excised, and the pathological diagnosis was ERWT. We report this case with a review of the literature to raise awareness of this association, illustrate the key imaging findings, and document the clinical outcome. The lack of pathognomonic radiologic features makes the preoperative diagnosis extremely difficult, but a diagnosis of ERWT should be considered in the context of a soft tissue mass without the typical imaging features of a hemangioma or teratoma. Copyright © 2017 Elsevier Inc. All rights reserved.

  12. Intraabdominal Intravascular Papillary Endothelial Hyperplasia (Masson’s Tumor: A Rare and Novel Cause of Gastrointestinal Bleeding

    Directory of Open Access Journals (Sweden)

    Michael C. Meadows

    2010-03-01

    Full Text Available Intravascular papillary endothelial hyperplasia (IPEH, or Masson’s tumor, a rare benign vascular lesion, occurs mainly in the head, neck, and hands in the human population. Aberrant tumor locations have been rarely reported. We present a case of a patient with chronic abdominal pain and melena of variable severity due to a Masson’s tumor, with no apparent Masson’s tumor-associated comorbidities, along with a comprehensive review of the literature. Using PubMed, a search engine provided by the U.S. National Library of Medicine and the National Institutes of Health, we searched for all reports of Masson’s tumor limited within the abdominal cavity. Furthermore, keywords such as ‘intravascular papillary endothelial hyperplasia’, ‘renal’, ‘gastrointestinal’, ‘hepatic’ and ‘intraabdominal’ were used to facilitate the search. We thus found fourteen cases of intraabdominal Masson’s tumors published. Six (42.9% of these were located in the renal vein, 4 (28.6% were reported in the gastrointestinal tract, 1 (7.1% in the adrenal gland, 1 (7.1% in the liver, and 1 (7.1% instance with multiple lesion sites including the renal hilum and retroperitoneum. Among these patients, 9 (64.3% were female and 5 (35.7% male, with a mean age of 38.9 years (7–69. IPEH is a reactive process, having three subtypes, all involving the proliferation of epithelial cells around a thrombus in the setting of venous stasis. In its pure form, the organized thrombus is solely localized within the vascular lumen. Mixed-form IPEH is formed in preexisting vascular lesions (such as arteriovenous malformation, hemangioma, pyogenic granuloma, etc.. The rarest form is the extravascular variety, which arises in hematomas often from recent trauma to the area. In its pure form, IPEH has a zero recurrence rate when an R0 resection is performed; all mixed and extravascular forms show the highest recurrence rates. The exact histogenesis of these epithelial cells

  13. Pseudoangio-matous stromal hyperplasia: A rare tumor of the breast.

    Science.gov (United States)

    Shahi, Kedar Singh; Bhandari, Geeta; Gupta, Rakesh Kumar; Sawai, Malvika

    2015-01-01

    Pseudoangiomatous stromal hyperplasia (PASH) is a benign breast entity described first by Vuitch et al., in 1986. PASH is a benign stromal lesion containing complex anastomosing channels lined by slender spindle cells. It can be mistaken with fibroadenoma on ultrasound examination and histologically with low-grade angiosarcoma and phyllodes tumor. Here, presented is a case report of a 30-year-old female who presented with huge palpable lump in left breast. Ultrasonography revealed the lesion as giant fibroadenoma and fine needle aspiration cytology report was suggestive of cystosarcoma phyllodes. Excision and reduction mammoplasty was done and histopathology report was suggestive of PASH.

  14. A rare case of massif Adenomatoid Odontogenic Tumor in the anterior region of mandible: Mimicking as dentigerous cyst

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    Aris Munandar

    2017-08-01

    Full Text Available Background. Adenomatoid Odontogenic Tumor (AOT is a rare tumor of epithelial origin. AOT appears in three clinico-topographic variants: follicular, extrafollicular and peripheral. The AOT was predominantly found in the upper jaw, and rarely found in mandible, especially at anterior mandible. AOT is a tumor of odontogenic epithelium having duct like structures, which may be partly cystic, and in some cases the solid lesion may be present only as masses in the wall of a large cyst. The surgical management of this lesion would be enucleation along with removal of associated impacted tooth. The prognosis for both of them is good and recurrences are very rare after complete removal of the lesion. Purpose. It is important to define final diagnose for AOT due to mimicking with DC in clinically and radiographically finding. Biopsy is still obviously necessary to the final diagnosis. Case. 15-year-old female patients reported with chief complain of swelling in anterior mandible. The swelling beginning 4 years ago, gradually progressed, with no history pain, discharge and patient is complaint about loss of sensation around anterior mandible. Aspiration revealed straw colored fluid thinking in the way of DC. The provisional diagnosis of DC was given due to clinical presentation and radiographic imaging. But the biopsy examination showed AOT due to duct-like epithelial cells was being found. Discussion. The case report illustrates characteristic clinical and radiographic features of follicular variant of AOT mimicking a DC at unusual site that is anterior mandible. AOT is thought to arise from odontogenic epithelium and associated with the impacted tooth. Rightfully AOT is a perfect imitator of DC radiographically as well as histopathologically. It usually clinically misdiagnosed as DC as both have a unilocular, well-defined radiolucency surrounding the crown of an impacted tooth. The mass was enucleated, involved teeth were extracted, and titanium plates

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

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    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.

  16. 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

  17. [Is Malherbe calcifying epithelioma in the ENT area of a rare tumor?].

    Science.gov (United States)

    Ganz, H

    1986-07-01

    The calcifying epithelioma (pilomatrixoma) is a benign tumour of the skin originating from hair sheath cells. The head, especially the cheek and preauricular region with the parotid gland, is a very common site (about 50%). The tumour arises predominantly in younger people, with a 2:1 female to male ratio. It is not so rare as suggested by the few ENT publications. The histologic picture shows two characteristic epithelial cells, the basophilic and ghost cell. Calcification is present in most cases. The prognosis after removal of the tumour is good. Two personal cases are reported, multiple pilomatrixomas on the cheek of an 12 year old girl, and a tumour on the auricle, an unusual site.

  18. Anaesthetic Management of a Neuroectodermal Tumor of Infancy: A Rare Case Report

    Science.gov (United States)

    Ubale, Pravin; Baldwa, Namita; Gujjar, Pinakin

    2017-01-01

    Melanotic neuroectodermal tumor of infancy is a relatively uncommon osteolytic-pigmented neoplasm that primarily affects the jaws of infants. We report a 5-month-old male child who presented with a swelling in the right upper tooth region of upper jaw in which we face difficult mask ventilation as well as difficult intubation. Wide surgical excision was performed under general anesthesia. The uneventful course of anesthesia in the presented case was due to the thorough systemic evaluation and careful anesthetic strategy. Patients of congenital epulis continue to pose challenge to anesthesiologist as a consequence of the potential difficult mask ventilation and intubation. We hereby present a case of congenital epulis repair using diode laser under general anesthesia.

  19. Computer-aided detection of rare tumor populations in flow cytometry: an example with classic Hodgkin lymphoma.

    Science.gov (United States)

    Ng, David P; Wu, David; Wood, Brent L; Fromm, Jonathan R

    2015-09-01

    Diagnosing classical Hodgkin lymphoma (cHL) by flow cytometry (FC) relies on an observer gating rare populations of Hodgkin/Reed Sternberg (HRS) cells. Here, we apply machine-learning methods to aid in the detection of rare tumor cell populations using data derived from clinical FC analysis of cHL as a model disease. FC data from 144 clinical cases using a nine-color FC reagent panel were analyzed using Python 2.7 and the "scikit-learn" module. Seventy-eight 50 × 50 two-dimensional histograms were generated from routine FC data and a reciprocal power function applied to favor rare events. Data were classified by support vector machine (SVM), gradient boosting, and random forest classifiers. All three classifiers showed no statistical difference in performance, with 89%-92% accuracy on cross-validation. Nearly all classifiers misclassified the same set of cases, with more false-positive than false-negative cases. Dimensionality reduction by ensemble methods selected for data points in a CD5+/ CD40+/CD64- region. All classifiers provide probabilistic confidences for each result, and diagnostic cutoffs can be chosen to minimize false negatives and serve as a screening tool. Computational exclusion of manually gated HRS cells had little impact on the overall performance of selected support vectors in SVM or dimensionality reduction, suggesting that features of the immune response in cHL may dictate the method accuracy. We hypothesize there are distinct inflammatory cells that suggest cHL. Copyright© by the American Society for Clinical Pathology.

  20. Surgical treatment of double primary liver cancer: An observational study for a rare type of tumor.

    Science.gov (United States)

    Li, Aijun; Ma, Senlin; Pawlik, Timothy; Wu, Bin; Yang, Xiaoyu; Cui, Longjiu; Wu, Mengchao

    2016-08-01

    Double primary liver cancer (DPLC) is a special type of clinical situation. As such, a detailed analysis of the surgical management and prognosis of patients with DPLC is lacking. The objective of the current study was to define the management and outcome of patients undergoing surgery for DPLC at a major hepatobiliary center.A total of 87 patients treated by surgical resection at the Eastern Hepatobiliary Surgery Hospital from January 1st, 2007 to October 31st, 2013 who had DPLC demonstrated by final pathological diagnosis were identified. Among these, 50 patients had complete clinical and prognostic data. Demographic and tumor characteristics as well as the prognosis were analyzed.The proportion of hepatitis B surface antigen (HBsAg) (+) and hepatitis B virus e antigen (HBeAg) (+), HBsAg (+), and HBeAg (-) hepatocirrhosis in all patients was 21.84%, 67.82%, and 63.22%, respectively. Incidental findings accounted for 58.62% of patients; among those who had symptoms, the main symptom was abdominal pain (31.03%). Nonanatomic wedge resection was the main operative approach (62.07%). Postoperatively, the main complications included seroperitoneum (11.49%), hypoproteinemia (10.34%), and pleural effusion (8.05%). Factors associated with disease-free survival (DFS) included intrahepatic cholangiocarcinoma (ICC) tumor size (P = 0.002) and use of postoperative prophylactic transcatheter arterial chemoembolization (TACE) treatment (P = 0.015). Meanwhile, hepatocellular carcinoma (HCC) size (P = 0.045), ICC size (P < 0.001), and liver function (including aspartate aminotransferase [P = 0.001] and r-glutamyl transferase [P < 0.001]) were associated with overall survival (OS).Hepatitis B virus (HBV)-related hepatitis or cirrhosis is also an important factor in the pathogenesis of DPLC and surgical treatment is safe for it with low complication rates. In addition, it is effective to prolong DFS that DPLC patients undergo postoperative prophylactic TACE

  1. A case of idiopathic colonic varices: A rare cause of hematochezia misconceived as tumor

    Institute of Scientific and Technical Information of China (English)

    Joung-Ho Han; Won-Joong Jeon; Hee-Bok Chae; Seon-Mee Park; Sei-Jin Youn; Seok-Hyung Kim; Il-Hun Bae; Sang-Jeon Lee

    2006-01-01

    Colonic varices are a very rare cause of lower gastrointestinal bleeding. Fewer than 100 cases of colonic varices, and 30 cases of idiopathic colonic varices (ICV) have been reported in the English literature.Among these 30 cases of ICV, 19 cases were diagnosed by angiography, and 7 operated cases were diagnosed later as ileocecal vein deficit, hemangioma, and idiopathic in 1, 1, 5 cases, respectively. We report the case of a 24-year-old man who suffered from multiple episodes of hematochezia of varying degree at the age of 11 years. He had severe anemia with hemoglobin of 21 g/L. On colonoscopy, tortuously dilated submucosal vein and friable ulceration covered with dark necrotic tissues especially at the rectosigmoid region were seen from the rectum up to the distal descending colon. It initially appeared to be carcinoma with varices. Mesenteric angiographic study suggested a colonic hemangioma.Low anterior resection was done due to medically intractable and recurrent hematochezia. Other bowel and mesenteric vascular structures appeared normal.Microscopic examination revealed normal colonic mucosa with dilated veins throughout the submucosa and serosa without representing new vessel growth. Taken all of these findings together, the patient was diagnosed as ICV. His postoperative course was uneventful.

  2. A Rare Case of Aggressive Digital Adenocarcinoma of the Lower Extremity, Masquerading as an Ulcerative Lesion that Clinically Favored Benignancy

    Directory of Open Access Journals (Sweden)

    Ryan Vazales

    2014-08-01

    Full Text Available A rare case report of Aggressive Digital Adenocarcinoma (ADPCa is presented complete with a literature review encompassing lesions that pose potential diagnostic challenges. Similarities between basal cell carcinoma (BCC, marjolin’s ulceration/squamous cell carcinoma (MSCC and ADPCa are discussed. This article discusses potential treatment options for ADPCa and the need for early biopsy when faced with any challenging lesion. An algorithmic approach to ADPCa treatment based on the most current research is recommended.

  3. Tracheal and bronchial tumors.

    Science.gov (United States)

    Varela, Patricio; Pio, Luca; Brandigi, Elisa; Paraboschi, Irene; Khen-Dunlop, Nazhia; Hervieux, Erik; Muller, Cecile; Mattioli, Girolamo; Sarnacki, Sabine; Torre, Michele

    2016-12-01

    Although primary tracheobronchial tumors are extremely rare in children, recurrent respiratory symptoms resistant to conventional therapy require further investigations to exclude possible malignant obstructive causes. As the matter of fact, early diagnosis may allow minimally invasive surgeries, improving the standard of living and the globally survival rate. The aim of this article is to provide an overview of diagnosis and management of tracheobronchial tumors in the early age, since only few reports are reported in the worldwide literature.

  4. A rare form of extremely wide QRS complex due to reversed homologous electrical ventricular separation of acute heart failure.

    Science.gov (United States)

    Yan, Sujuan; Yu, Jianhua; Xia, Zhen; Zhu, Bo; Hu, Jinzhu; Li, Juxiang

    2017-06-29

    Electrical ventricular separation, as a special complete intraventricular block, denotes that ventricles be electrically separated into two or more parts caused by severe and wide damage of myocardium and conduction. Electrical ventricular separation can be divided into homologous and heterologous, homologous electrical ventricular separation is a rare phenomenon, literally the excitement of whole ventricle originate from supraventricle, on ECG, there are two different QRS waves which connect with an isoelectric line, one ST segment and T wave. We report a valve heart disease presented with complicated electrophysiological characteristics, which has reversed complex homologous electrical ventricular separation with second degree intraventricular block. © 2017 Wiley Periodicals, Inc.

  5. Sinonasal epithelial-Myoepithelial carcinoma-A rare entity

    OpenAIRE

    Pradhan, Sultan A.; Khannan, Rajan; Hazarika, Biswajyoti; Desai, Meena

    2007-01-01

    Epithelial-myoepithelial carcinoma is a rare salivary gland tumor. It comprises less than 1% of all salivary gland tumors. It generally arises from the parotid gland. Unusual sites of occurrence include sinonasal tract, lung, trachea, lacrimal gland and breast. Histopathologically epithelial-myoepithelial carcinoma comprises a dual population of ductal and myoepithelial cells. We report an extremely rare case of epithelial-myoepithelial carcinoma occurring in the sinonasal tract of young man.

  6. Extreme chemical conditions of crystallisation of Umbrian Melilitolites and wealth of rare, late stage/hydrothermal minerals

    Science.gov (United States)

    Stoppa, F.; Schiazza, M.

    2014-12-01

    Melilitolites of the Umbria Latium Ultra-alkaline District display a complete crystallisation sequence of peculiar, late-stage mineral phases and hydrothermal/cement minerals, analogous to fractionated mineral associations from the Kola Peninsula. This paper summarises 20 years of research which has resulted in the identification of a large number of mineral species, some very rare or completely new and some not yet classified. The progressive increasing alkalinity of the residual liquid allowed the formation of Zr-Ti phases and further delhayelitemacdonaldite mineral crystallisation in the groundmass. The presence of leucite and kalsilite in the igneous assemblage is unusual and gives a kamafugitic nature to the rocks. Passage to non-igneous temperatures (T<600 °C) is marked by the metastable reaction and formation of a rare and complex zeolite association (T<300 °C). Circulation of low-temperature (T<100 °C) K-Ca-Ba-CO2-SO2-fluids led to the precipitation of sulphates and hydrated and/or hydroxylated silicate-sulphate-carbonates. As a whole, this mineral assemblage can be considered typical of ultra-alkaline carbonatitic rocks.

  7. Vaginal superficial myofibroblastoma: a rare mesenchymal tumor of the lower female genital tract and a study of its association with viral infection.

    Science.gov (United States)

    Liu, Jing-Lan; Su, Tzu-Cheng; Shen, Ko-Hung; Lin, Shu-Hui; Wang, Hsin-Kai; Hsu, Jui-Chang; Chen, Chih-Jung

    2012-06-01

    Superficial myofibroblastoma is a rare mesenchymal tumor in the lower female genital tract. The exact etiology of superficial myofibroblastoma remains unclear. The association of viral infection and mesenchymal tumors has been well established in some particular types of soft tissue tumors. In the lower female genital tract, the intimate correlation of viral infection and tumor pathogenesis has been also proposed. We present a 59-year-old woman with postcoital bleeding for 1 month. The pelvic examination revealed a 2-cm polypoid mass mimicking leiomyoma at the vaginal fornix. Local excision was performed, and the pathological examination revealed a superficial myofibroblastoma. No tumor recurrence was noted during the 12-month follow-up. Pathological differential diagnosis of this tumor from other mesenchymal tumors is essential because of its distinct clinicopathological features. Furthermore, fluorescence in situ hybridization of human papilloma virus (HPV) and Epstein-Barr virus (EBV), as well as immunohistochemical staining of human herpesvirus 8 (HHV8), was negative in tumor cells. To the best of our knowledge, we are the first group to study the possible relationship of viral infection and the occurrence of this mesenchymal tumor. Our results suggested no association of vaginal superficial myofibroblastoma and infection with HPV, EBV, or HHV8.

  8. Primary Epidural Peripheral Primitive Neuroectodermal Tumor of the Lumbar Spine: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Choi, Woo Jung; Lee, Seung Hun; Joo, Kyung Bin [Dept. of Radiology, Hanyang University Hospital, Seoul (Korea, Republic of); Paik, Seung Sam; Jun, Young Jin [Dept. of Pathology, Hanyang University Hospital, Seoul (Korea, Republic of); Lee, Young Ho [Dept. of Pediatrics, Hanyang University Hospital, Seoul (Korea, Republic of)

    2011-05-15

    A primitive neuroectodermal tumor (PNET) is a highly malignant tumor in children and young adults, and extremely rare in the spine. We report a case of a primary epidural peripheral PNET of the lumbar spine. The present extremely rare case of primary epidural peripheral PNET of the lumbar spine illustrates the unexpected occurrence and should be included in differential diagnoses for patients with spinal tumors.

  9. The Challenge of Cancer Genomics in Rare Nervous System Neoplasms: Malignant Peripheral Nerve Sheath Tumors as a Paradigm for Cross-Species Comparative Oncogenomics.

    Science.gov (United States)

    Carroll, Steven L

    2016-03-01

    Comprehensive genomic analyses of common nervous system cancers provide new insights into their pathogenesis, diagnosis, and treatment. Although analogous studies of rare nervous system tumors are needed, there are major barriers to performing such studies. Cross-species comparative oncogenomics, identifying driver mutations in mouse cancer models and validating them in human tumors, is a promising alternative. Although still in its infancy, this approach is being applied to malignant peripheral nerve sheath tumors (MPNSTs), rare Schwann cell-derived malignancies that occur sporadically, after radiotherapy, and in neurofibromatosis type 1. Studies of human neurofibromatosis type 1-associated tumors suggest that NF1 tumor suppressor loss in Schwann cells triggers cell-autonomous and intercellular changes, resulting in development of benign neurofibromas; subsequent neurofibroma-MPNST progression is caused by aberrant growth factor signaling and mutations affecting the p16(INK4A)-cyclin D1-CDK4-Rb and p19(ARF)-Mdm2-p53 cell cycle pathways. Mice with Nf1, Trp53, and/or Cdkn2a mutations that overexpress the Schwann cell mitogen neuregulin-1 or overexpress the epidermal growth factor receptor validate observations in human tumors and, to various degrees, model human tumorigenesis. Genomic analyses of MPNSTs arising in neuregulin-1 and epidermal growth factor receptor-overexpressing mice and forward genetic screens with Sleeping Beauty transposons implicate additional signaling cascades in MPNST pathogenesis. These studies confirm the utility of mouse models for MPNST driver gene discovery and provide new insights into the complexity of MPNST pathogenesis.

  10. Lhermitte-Duclos disease associated to Cowden syndrome: de novo diagnosis and management of these extremely rare syndromes in a patient.

    Science.gov (United States)

    Gama, Ivo; Almeida, Leonor

    2017-01-30

    A 36-year-old woman, with history of cutaneous papilomatosis and thyroid carcinoma presented with headache, transitory visual blurring and nausea. Funduscopy showed papilloedema. MRI showed a tumour of the right cerebellar hemisphere with a striated, tigroid pattern, typical of Lhermitte-Duclos disease (LDD). Significant clinical and perimetric improvements were noted after surgery and the follow-up did not reveal recurrences of the tumour. LDD is an extremely rare differential diagnosis of posterior fossa tumours. LDD and the history of thyroid carcinoma permitted us to diagnose Cowden syndrome (CS). We present a clinical case that supports the possibility of performing a preoperative diagnosis of LDD based on MRI features. We review the diagnosis and management of LDD and CS. This report highlights the importance of excluding CS after LDD diagnosis, of monitoring the optic nerve postoperatively using optical coherence tomography and of prompt treatment that can potentially prevent visual function loss.

  11. Wilms Tumor

    Science.gov (United States)

    Wilms tumor is a rare type of kidney cancer. It causes a tumor on one or both kidneys. It usually affects ... are at risk should be screened for Wilms tumor every three months until they turn eight. Symptoms ...

  12. Nonadenomatous tumors of the pituitary and sella turcica.

    Science.gov (United States)

    Huang, Benjamin Y; Castillo, Mauricio

    2005-07-01

    While pituitary adenomas make up over 90% of all sellar masses, there are a number of less known tumors, both malignant and benign, which may arise within the sella turcica. These include relatively common tumors such as meningiomas and craniopharyngiomas, as well as extremely rare tumors such as pituitary astrocytomas and granular cell tumors. Unfortunately, many of these tumors lack characteristic imaging features, often making it extremely difficult to distinguish them by imaging alone from the more common pituitary adenoma. In this article, we review several nonadenomatous tumors of the sella, with a focus on their clinical features and typical MR imaging characteristics.

  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. Intramedullary spinal cord primitive neuroectodermal tumor presenting with hydrocephalus.

    Science.gov (United States)

    Alexiou, George A; Siozos, George; Stefanaki, Kalliopi; Moschovi, Maria; Prodromou, Neofytos

    2013-02-01

    Spinal primitive neuroectodermal tumors are exceedingly rare. Herewith, we present the first case of an intramedullary spinal cord tumor associated with hydrocephalus in a 2-month-old boy that presented with left hemiparesis. The patient had been diagnosed on prenatal ultrasound with enlarged ventricular system. At his current admission, a brain magnetic resonance imaging (MRI) revealed hydrocephalus and an intramedullary lesion extending from the second cervical to the first thoracic vertebrae. Dissemination of the tumor was revealed intracranially and in the spinal canal. After a ventriculoperitoneal shunt placement a radical resection of the tumor was performed, however some small tumor remnants could not be safely removed. Postoperative there was no neurologic deterioration. The tumor was diagnosed as a central nervous system primitive neuroectodermal tumor (World Health Organization grade IV). Spinal intramedullary primitive neuroectodermal tumors are extremely rare. In such rare tumors, multiinstitutional studies are needed for treatment guidelines to be established.

  15. Collision tumor with inflammatory breast carcinoma and malignant phyllodes tumor: a case report and literature review.

    Science.gov (United States)

    Shin, Young Duck; Lee, Seul Kee; Kim, Kyu Sun; Park, Mi Ja; Kim, Joo Heon; Yim, Hyun Sun; Choi, Young Jin

    2014-01-08

    There have been some reports of coincidental presentation of breast carcinoma and phyllodes tumor in the same breast. Most of the cases were carcinoma that arose from a phyllodes tumor with a histologically identified transitional area, and they behaved less aggressively than the usually encountered carcinoma. Collision tumors are rare clinical entities in which two histologically distinct tumor types show involvement at the same site. The occurrence of these tumors in the breast is extremely rare. Here, we report a case of 45-year-old woman who had both invasive ductal carcinoma as the finding of inflammatory carcinoma and a malignant phyllodes tumor in the same breast. There was no evidence of a transitional area between the phyllodes tumor and the invasive ductal carcinoma. To our knowledge, this is the first report of a collision tumor of inflammatory breast carcinoma coincident with a malignant phyllodes tumor in same breast.

  16. Primitive Neuroectodermal Tumor with Glioblastoma Multiforme Components in an Adult: A Collision Tumor.

    Science.gov (United States)

    Forbes, Victoria; Vredenburgh, James

    2016-01-11

    We report a rare case of a central nervous system collision tumor in a 40-year-old woman. Histopathological examination of her large temporal tumor revealed two different components making up the tumor tissue. The predominant component of the tumor was found to be a primitive neuroectodermal tumor. The other component was glioblastoma multiforme. Both of these tumors carry a poor prognosis, and primitive neuroectodermal tumors are extremely uncommon in adults. Central nervous system neoplasms with the combined features of both primitive neuroectodermal tumor and malignant glioma are very rare and represent a diagnostic and treatment predicament. The patient underwent surgical resection, radiation therapy, and chemotherapy targeting both the primitive neuroectodermal tumor and glioblastoma. Our patient has been fortunate in not showing any sign of recurrence and will celebrate the third anniversary since her diagnosis this January.

  17. Giant myxoinflammatory fibroblastic sarcoma with bone invasion:a very rare clinical entity and literature review

    Institute of Scientific and Technical Information of China (English)

    Guray Togral; Murat Arikan; Elif Aktas; Safak Gungor

    2014-01-01

    Myxoinflammatory fibroblastic sarcoma (MIFS) is a rare low-grade, malignant soft tissue tumor that is usually observed in the extremities of adult patients. Magnetic resonance imaging findings for this tumor type have rarely been reported. We report a case involving the distal left femur of a middle-aged man and tumoral invasion of the bone, which, to our knowledge, has been previously described only once. He was treated with distal femoral tumor resection and reconstruction with a modular prosthesis. Histopathologic diagnosis confirmed MIFS. We reviewed literature of the diagnostic imaging and bone invasion findings associated with this tumor type.

  18. PRIMITIVE NEUROECTODERMAL TUMOR OF URINARY BLADDER

    Directory of Open Access Journals (Sweden)

    Jagadeeshwar

    2014-12-01

    Full Text Available PNET of the urinary bladder is extremely rare. Only 12 cases are reported till now in the world. Primitive Neuro Ectodermal Tumor (PNET is a malignant small round blue cell tumor exhibiting a variable degree of neural differentiation, which arises outside the brain, spinal cord and sympathetic nervous system. (1,2 PNET is a very aggressive tumor with rapid local infiltration combined with widespread metastasis.

  19. Tumor

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    2008479 Preliminary study of MR elastography in brain tumors. XU Lei(徐磊), et al.Neurosci Imaging Center, Beijing Tiantan Hosp, Capital Med Univ, Beijing 100050.Chin J Radiol 2008;42(6):605-608. Objective To investigate the potential values of magnetic resonance elastography (MRE) for evaluating the brain tumor consistency in vivo. Methods Fourteen patients with known solid brain tumor (5 male, 9 female; age range: 16-63 years)

  20. Extreme light rare earth element mobilization by diagenetic fluids in the geological environment of the Oklo natural reactor zones, Franceville basin, Gabon

    Science.gov (United States)

    Cuney, Michel; Mathieu, Régis

    2000-08-01

    The anomalously high Th/La ratio (˜1.14) of the Early Proterozoic silicified sandstones of the Franceville basin (Gabon), compared to Archean and Proterozoic metasedimentary rocks (Th/La ˜0.27), results from extreme light rare earth element (REE) migration during diagenesis. Monazite, which represents the main light REE-bearing phase in the sandstones, was altered by diagenetic brines at 140 °C and 1 kbar. The alteration phase is a microcrystalline Th-silicate phase, indicating low Th solubility at these conditions. Light REEs are simultaneously leached out together with P and U. The increase in Th/La from detrital monazite to residual Th-silicate phase indicates that about 76% of the light REEs were leached out, corresponding to a global amount of 2.01 × 109 metric tons at the scale of the FA Formation in the Franceville basin. Uranium was also leached during monazite alteration and may have contributed significantly to the genesis of the high-grade uranium deposits of the Franceville basin that host the natural nuclear reaction zones.

  1. Testicular tumors

    Directory of Open Access Journals (Sweden)

    Giovanni Rosti

    2011-12-01

    Full Text Available Germ cell tumors of the testes represent a unique paradigm of diseases which can be cured even in extremely advanced phase. Unfortunately, this makes them unique among adult solid tumors. Seminoma and non seminoma are relatively rare with approximatively 25,000 patients in Europe per year, but numbers are increasing world wide. Different strategies are needed depending on stage and prognostic scores. Seminoma is extremely sensitive to radiation therapy and chemotherapy, while all germ cell tumors show a very good response to chemotherapy. Clinical stage I seminoma is currently treated with radiation, single course carboplatin or surveillance policy. Clinical stage I non seminoma can also be approached with different strategies such as retroperitoneal lymph node dissection, observation or one-two courses of standard chemotherapy. Stage II seminoma may be treated with either radiation or chemotherapy, while for all advanced stages chemotherapy is mandatory. Since the mid-eighties PEB (Cisplatin, Etoposide and Bleomycin is the regimen of choice and no other schedule has proved superior in terms of efficacy. Surgery on the residual disease is crucial to the whole strategy and should be performed or attempted in all cases. Consequently, the correct treatment strategy for these tumors does not depend only on the ability of a single physician, but on a skilled team specialized in this particular tumor. Second line therapies (VeIP, PEI, TIP can cure 25%–40% of patients, but improved strategies for resistant tumors are desperately needed. High-dose chemotherapy has shown very good results in some studies while being less impressive in others. In any case, it should remain an option for relapsing patients and could be used in some cases of upfront chemotherapy in patients with slow marker decline, but this should only be considered in referring centers.

  2. Imaging findings of desmoplastic fibroma rarely involoving the clavicle: Case report

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Dong Min; Juhng, Seon Kwan; Sohn, Young Jun; Kim, Hun Soo [Wonkwang University School of Medicine and Hospital, Iksan (Korea, Republic of)

    2014-02-15

    Desmoplastic fibroma of bone is a rare locally aggressive, but non-metastatic tumor. In this case report, we present a desmoplastic fibroma in an unusual location, the clavicle. Desmoplastic fibroma involving the clavicle is extremely rare, with only 2 reported cases before 1985. We report the imaging findings of a desmoplastic fibroma of the clavicle with a review of the relevant literature.

  3. Anatomic Tumor Location Influences the Success of Contemporary Limb-Sparing Surgery and Radiation Among Adults With Soft Tissue Sarcomas of the Extremities

    Energy Technology Data Exchange (ETDEWEB)

    Korah, Mariam P., E-mail: mariam.philip@gmail.com [Department of Radiation Oncology, Emory University, Atlanta, GA (United States); Deyrup, Andrea T. [Department of Pathology, Emory University, Atlanta, GA (United States); Monson, David K.; Oskouei, Shervin V. [Department of Orthopedics, Emory University, Atlanta, GA (United States); Weiss, Sharon W. [Department of Pathology, Emory University, Atlanta, GA (United States); Landry, Jerome; Godette, Karen D. [Department of Radiation Oncology, Emory University, Atlanta, GA (United States)

    2012-02-01

    Purpose: To examine the influence of anatomic location in the upper extremity (UE) vs. lower extremity (LE) on the presentation and outcomes of adult soft tissue sarcomas (STS). Methods and Materials: From 2001 to 2008, 118 patients underwent limb-sparing surgery (LSS) and external beam radiotherapy (RT) with curative intent for nonrecurrent extremity STS. RT was delivered preoperatively in 96 and postoperatively in 22 patients. Lesions arose in the UE in 28 and in the LE in 90 patients. Patients with UE lesions had smaller tumors (4.5 vs. 9.0 cm, p < 0.01), were more likely to undergo a prior excision (43 vs. 22%, p = 0.03), to have close or positive margins after resection (71 vs. 49%, p = 0.04), and to undergo postoperative RT (32 vs. 14%, p = 0.04). Results: Five-year actuarial local recurrence-free and distant metastasis-free survival rates for the entire group were 85 and 74%, with no difference observed between the UE and LE cohorts. Five-year actuarial probability of wound reoperation rates were 4 vs. 29% (p < 0.01) in the UE and LE respectively. Thigh lesions accounted for 84% of the required wound reoperations. The distribution of tumors within the anterior, medial, and posterior thigh compartments was 51%, 26%, and 23%. Subset analysis by compartment showed no difference in the probability of wound reoperation between the anterior and medial/posterior compartments (29 vs. 30%, p = 0.68). Neurolysis was performed during resection in (15%, 5%, and 67%, p < 0.01) of tumors in the anterior, medial, and posterior compartments. Conclusions: Tumors in the UE and LE differ significantly with respect to size and management details. The anatomy of the UE poses technical impediments to an R0 resection. Thigh tumors are associated with higher wound reoperation rates. Tumor resection in the posterior thigh compartment is more likely to result in nerve injury. A better understanding of the inherent differences between tumors in various extremity sites will assist in

  4. A rare case of fever of unknown origin: inflammatory myofibroblastic tumor of the liver. Case report and review of the literature.

    Science.gov (United States)

    Kruth, Jens; Michaely, Henrik; Trunk, Markus; Niedergethmann, Marco; Rupf, Ann-Katrin; Krämer, Bernhard K; Göttmann, Uwe

    2012-12-01

    We present the case of a rare cause of fever of unknown origin (FUO). FUO is challenging for patients as well as for physicians as there are more than 200 differential diagnoses of FUO (1,2). Pointing out a diagnosis often requires numerous noninvasive and invasive procedures that sometimes even fail to explain the fever. Our patient was admitted twice to our hospital due to remitting fever rising up to 40 degrees C without any subjective discomfort. At the first presentation no clinical focus could be identified. This included the examination of multiple blood and urine cultures, serology, autoimmune serology, transesophageal echocardiography, CT-scan of the lung and the abdomen, and bone scintigraphy. Elevated C-reactive protein (268 mg/l) decreased spontaneously and fever disappeared after 4 weeks. However, the patient was re-admitted 4 months later with identical symptoms. Multiple blood and urine cultures, serology, bone marrow examination, CT-scan of the lung and the abdomen, esophago-gastro-duodenoscopy and colonoscopy still showed no pathological findings. MRI-scan of the abdomen identified a liver tumor of 3.3 cm in diameter in segment 6 without typical signs of an adenoma, focal nodular hyperplasia or hepatocellular carcinoma. Biopsy of the suspect liver lesion revealed an inflammatory myofibroblastic tumor (inflammatory pseudotumor). After surgical resection of the tumor elevated inflammation markers as C-reactive protein normalized and fever disappeared. One year after surgery no more episodes of fever re-occurred. An inflammatory myofibroblastic tumor of the liver can be a rare cause of fever of unknown origin. MRI-scan can be an additional imaging tool to identify previously not recognized liver tumors.

  5. Retroperitoneal calcifying fibrous tumor mimicking an adrenal tumor.

    Science.gov (United States)

    Prochaska, Erica C; Sciallis, Andrew P; Miller, Barbra S

    2016-06-01

    Establishing the etiology of a retroperitoneal tumor may be difficult due to close proximity of multiple organs. Evaluation of retroperitoneal tumors often leads to surgery, many times to obtain a definitive diagnosis and rule out malignancy. Calcifying fibrous tumors (CFT) are very rare soft tissue tumors occurring most often in young patients. They are most often found arising in the thoracic cavity, mediastinum, abdominal cavity and extremities and usually have a benign clinical course. Macrocscopically, the tumors are well circumscribed and firm with a white-tan appearance. Histologically, CFT comprised a hypocellular proliferation of bland spindle cells, densely hyalinized collagen, chronic lymphoplasmacytic inflammation and dystrophic calcifications. Other considerations in the pathologic differential diagnosis include solitary fibrous tumor and inflammatory myofibroblastic tumor.

  6. Unusual Radiologic Finding of Intracranial Inflammatory Myofibroblastic Tumor Presenting a Cyst with Mural Nodule.

    Science.gov (United States)

    Park, Jong-Heok; Yoon, Wan-Soo; Chung, Dong-Sup

    2015-10-01

    An intracranial cyst tumor with a mural nodule can be representative of some types of brain tumors, but is a rare presentation of intracranial inflammatory myofibroblastic tumor (IMT). Herein, we report the case of an intracranial IMT in a 48-year-old woman presenting with the extremely unusual radiologic findings of a cyst with a mural nodule.

  7. A case series of transformation of teratoma to primitive neuroectodermal tumor: evolving management of a rare malignancy

    Directory of Open Access Journals (Sweden)

    Richard F. Dunne

    2014-03-01

    Full Text Available Primitive neuroectodermal tumor (PNET is a pathologic diagnosis that encompasses several different tumor types, including central nervous system tumors and Ewing’s sarcomas. Teratoma, a common element of germ cell tumor (GCT, has the ability to transform to malignant PNET in a small number of patients. Making a definitive diagnosis of PNET is difficult given its deviation from elements of GCT and its non-specific pathologic findings. Establishing the diagnosis is crucial as PNETs respond poorly to standard platinum-based chemotherapy used for treatment of GCT. Primary treatment for PNET is surgical, though this is often not feasible in many patients due to extensive disease at diagnosis. As an alternative, chemotherapy regimens traditionally used for Ewing’s sarcoma, such as vincristine, doxorubicin and cyclophosphamide alternating with ifosfamide and etoposide, have shown limited efficacy in the neoadjuvant, adjuvant, and palliative settings. Future research should delineate the genetic underpinnings of PNET and develop therapeutic options accordingly.

  8. Skeletal metastasis of carcinoid tumors: Two case reports and review of the literature

    OpenAIRE

    HORI, TAKESHI; YASUDA, TAKETOSHI; Suzuki, Kayo; KANAMORI, MASAHIKO; KIMURA, TOMOATSU

    2012-01-01

    Carcinoid tumors are neuroendocrine neoplasms derived from enterochromaffin cells. Skeletal metastases from carcinoid tumors are considered to be extremely rare. In this study, we present two cases of carcinoid tumors that metastasized to the bone. Furthermore, we review 50 published case reports and reveal the features of skeletal metastasis of carcinoid tumors. The first case involved a 59-year-old man with a history of multiple metastases of a lung carcinoid tumor. The patient complained o...

  9. Scrotal Lymphangioma – A Rare Cause of Scrotal Swelling

    Directory of Open Access Journals (Sweden)

    Pradeep Kajal

    2013-01-01

    Full Text Available Lymphangioma is an extremely rare cause of scrotal swelling. We are reporting such a tumor in a one and half year old child presenting with a painless, progressive scrotal swelling. The mass was evaluated and excised completely. Histopathology confirmed it as Lymphangioma.

  10. [Thymoma in children: a rare and difficult diagnosis].

    Science.gov (United States)

    Juricic, M; Labouret, G; Castex, M-P; Baunin, C; Galinier, P; Abbo, O

    2015-08-01

    Thymoma is extremely rare within the pediatric age range, which could lead to delayed diagnosis. Based on the clinical case of a mediastinal tumor in an 8-year-old patient, we detail the key points in the management of this disease highlighted by our recent experience.

  11. Original report of bilateral carotid body tumors with 2 rare concomitant anatomic findings, an ectopic parathyroid gland and cervical thymus, with literature review.

    Science.gov (United States)

    Westbrook, Benjamin J; Harsha, Wayne J; Strenge, Karen

    2013-03-01

    Carotid body tumors are uncommon neoplasms with unique epidemiology and management demands. Cervical embryology is complex. Developmental abnormalities can result in ectopic displacement of native tissues. We present the case of a 21-year-old female with bilateral carotid body paragangliomas who presented to our clinic seeking excision of the symptomatic right-sided tumor. The patient was successfully treated with surgical excision. Two rare anatomic variants were identified in her surgical specimen: a carotid sheath parathyroid gland and ectopic thymus tissue. This patient represents an unreported combination of pathologic and anatomic phenomena. The paraganglioma resulted from a familial genetic mutation that is well studied in this patient's ethnic population (Dutch), and the literature on this topic is reviewed herein. The 2 anatomic variants likely represent a single, embryologic glitch that will carry no physiologic sequelae. The clinical application of this ectopic anatomy and the common embryologic origins are discussed. Copyright © 2011 Wiley Periodicals, Inc.

  12. Tumor Seeding With Renal Cell Carcinoma After Renal Biopsy

    OpenAIRE

    M.F.B. Andersen; Norus, T.P.

    2016-01-01

    Tumor seeding following biopsy of renal cell carcinoma is extremely rare with an incidence of 1:10.000. In this paper two cases with multiple recurrent RRC metastasis in the biopsy tract following biopsy of renal tumor is presented and the current literature is shortly discussed.

  13. High endothelial venules are rare in colorectal cancers but accumulate in extra-tumoral areas with disease progression.

    Science.gov (United States)

    Bento, Diana Costa; Jones, Emma; Junaid, Syed; Tull, Justyna; Williams, Geraint T; Godkin, Andrew; Ager, Ann; Gallimore, Awen

    2015-03-01

    Prolonged patient survival after surgical resection, is associated with a higher cytotoxic and memory T cell density within colorectal cancers (CRC). High endothelial venules (HEVs) are specialized blood vessels present in secondary lymphoid organs (SLO) that allow ingress of naïve and central memory T cells from the blood. It has been proposed that HEVs in tumors might serve as a similar route of entry for lymphocytes into the tumor and result in an improved prognosis. The present study aimed to characterize HEVs and their microenvironment in resected tumors from colorectal cancer patients (n = 62). We observed HEVs in association with lymphoid aggregates in 49 out of 62 patients. However, these HEV(+) lymphoid aggregates were largely at the invasive margin of the tumor and although there was an association with lymphocytes and HEVs at the invasive margin (p = 0.002) there was only a very weak association with tumor infiltrating lymphocytes. Indeed, lymphoid aggregates were associated with more advanced disease (Dukes' stage C) and did not indicate a favorable prognosis.

  14. 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.

    Science.gov (United States)

    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.

  15. A Rare Case of a Symptomatic Tumor Found in the Groin Area: An Atypical Location Unexposed to the Known Causes

    Directory of Open Access Journals (Sweden)

    Ellen Toyonaga

    2015-11-01

    Full Text Available Nevoid basal cell carcinoma syndrome (NBCCS, also known as Gorlin syndrome, is a rare hereditary condition characterized by a wide range of developmental abnormalities and a predisposition to neoplasms. The syndrome consists of early-onset and/or multiple BCC. Herein we report a rare NBCCS case in which the first BCC onset occurred in the groin area. To the best of our knowledge, there have been no reports of first-onset BCC in the groin area in an NBCCS patient of any race.

  16. A rare benign genitourinary tumor in a Japanese male: urinary retention owing to aggressive angiomyxoma of the prostate

    Directory of Open Access Journals (Sweden)

    Hayakazu Nakazawa

    2010-03-01

    Full Text Available Close examination of a 67-year-old Japanese man, who complained of persistent nocturia, revealed that a semitransparent polypoid tumor had developed from the bladder neck to the prostatic urethra obstructing the internal urethral meatus, which resulted in excessive urinary retention and post-renal dysfunction. The tumor was resected by a transurethral procedure and a pathological examination of specimens revealed aggressive angiomyxoma (AAM of the prostate. AAM usually develops in the intrapelvic and perineal organs of females. So far as we know, this is the second case of primary prostatic AAM reported in the English literature, and is the first case where the patient encountered urethral obstruction.

  17. Fibroblastic Type Osteosarcoma of the Ulna: a Case Report of a Tumor in a Rare Location with Atypical Imaging Findings

    Science.gov (United States)

    Joo, Ijin; Chung, Jin-Haeng; Oh, Joo Han; Hong, Sung Hwan; Kang, Heung Sik

    2009-01-01

    The ulna is a rare site of origin for osteosarcoma, and purely osteolytic osteosarcomas are uncommonly noted on conventional radiographs. We present a patient with a lytic lesion of the distal ulna for which imaging findings suggested an aneurysmal bone cyst. The lesion was histologically confirmed to be a fibroblastic osteosarcoma. PMID:19182508

  18. Fibroblastic Type Osteosarcoma of the Ulna: a Case Report of a Tumor in a Rare Location with Atypical Imaging Findings

    Energy Technology Data Exchange (ETDEWEB)

    Joo, I Jin; Choi, Jung Ah; Hong, Sung Hwan; Kang, Heung Sik [Seoul National University College of Medicine, Seoul (Korea, Republic of); Chung, Jin Haeng; Oh, Joo Han [Seoul National University Bundang Hospital, Seongnam (Korea, Republic of)

    2009-02-15

    The ulna is a rare site of origin for osteosarcoma, and purely osteolytic osteosarcomas are uncommonly noted on conventional radiographs. We present a patient with a lytic lesion of the distal ulna for which imaging findings suggested an aneurysmal bone cyst. The lesion was histologically confirmed to be a fibroblastic osteosarcoma.

  19. Hypothalamic tumor

    Science.gov (United States)

    Complications of brain surgery may include: Bleeding Brain damage Death (rarely) Infection Seizures can result from the tumor or from any surgical procedure on the brain. Hydrocephalus can occur with some tumors and ...

  20. MRI Diagnosis and Differential Diagnosis of Rare Tumors in Pineal Region%松果体区少见肿瘤MRI诊断与鉴别诊断

    Institute of Scientific and Technical Information of China (English)

    李松涛; 汪文胜; 黄泽春

    2014-01-01

    目的探讨松果体区少见肿瘤的MRI诊断与鉴别诊断情况。方法回顾性分析我院于2007年1月至2013年7月收治的15名松果体区肿瘤患者的临床资料,总结这些患者的临床表现和MRI检查结果。结果诊断结果显示,这些患者患有松果体实质瘤、松果体细胞瘤、成熟与未成熟的畸胎瘤、胚胎发育不良性神经上皮瘤、胶质母细胞瘤、节细胞胶质瘤、神经母细胞瘤、精原细胞瘤、松果体母细胞瘤、胆脂瘤和胚胎癌等松果体区肿瘤。结论单一靠MRI检查无法明确诊断松果体区少见肿瘤, DWI及MRS等功能磁共振检查可以帮助诊断,确诊依旧需要依靠病理检查结果。%Objective To discuss the MRI diagnosis and differential diagnosis of rare tumors in pineal region. Methods Clinical data of 15 patients with pineal region tumor were retrospectively analyzed in our hospital from 2007 January to 2013 July, and their clin-ical manifestations and MRI findings were summarized. Results The diagnosis results showed that the tumors included pineal paren-chymal tumor, pineocytoma, mature or immature teratoma, dysembryoplastic neuroepithelial tumor, glioblastoma, ganglioglioma, neu-roblastoma, seminoma, pinealoblastoma, cholesteatoma and embryonal carcinoma. Conclusion Rare tumor in pineal region could not be definitely diagnosed only by MRI check;DWI and MRS functional magnetic resonance imaging examination could also contribute to its diagnosis. However, the diagnosis still depends on the pathological examination results.

  1. Molecular Characterization of Inflammatory Myofibroblastic Tumors With Frequent ALK and ROS1 Gene Fusions and Rare Novel RET Rearrangement

    NARCIS (Netherlands)

    Antonescu, Cristina R.; Suurmeijer, Albert J. H.; Zhang, Lei; Sung, Yun-Shao; Jungbluth, Achim A.; Travis, William D.; Al-Ahmadie, Hikmat; Fletcher, Christopher D. M.; Alaggio, Rita

    2015-01-01

    Approximately 50% of conventional inflammatory myofibroblastic tumors (IMTs) harbor ALK gene rearrangement and overexpress ALK. Recently, gene fusions involving other kinases have been implicated in the pathogenesis of IMT, including ROS1 and in 1 patient PDGFRB. However, it remains uncertain whethe

  2. Retroperitoneal sclerosing PEComa with melanin pigmentation and granulomatous inflammation-A rare association within an uncommon tumor

    Directory of Open Access Journals (Sweden)

    Bharat Rekhi

    2012-01-01

    Full Text Available PEComa, defined as a perivascular epithelioid cell tumor, displays a wide clinicopathological spectrum. Lately, a sclerosing PEComa has been identified as its distinct variant, but with limited documentation, in view of its rarity. Herein, we describe an uncommon case of a 53-year-old lady, who was referred to us with pain abdomen. Radiological imaging disclosed a well-defined, hypodense retroperitoneal mass. The excised tumor was a round, encapsulated soft tissue mass measuring 7 cm with a tan-brown cut surface. Microscopy showed uniform, epithelioid cells with clear cytoplasm, focal melanin pigmentation and mild nuclear atypia, arranged in sheets and nests around capillary-sized vessels in a dense sclerotic stroma. Additionally, co-existing epithelioid granulomas were noted. On immunohistochemistry (IHC, tumor cells were diffusely positive for HMB45; focally for desmin and smooth muscle actin (SMA, while negative for EMA, CD10, S100-P, Melan A, CD34, AMACR and CK MNF116. This case reinforces sclerosing PEComa as an uncommon, but a distinct clinicopathological entity and exemplifies diagnostic challenge associated with it; necessitating application of IHC markers for its correct identification. Presence of melanin pigment and granulomatous inflammation in the present tumor constitute as novel histopathological findings in a sclerosing PEComa.

  3. Huge malignant phyllodes breast tumor: a real entity in a new era of early breast cancer.

    Science.gov (United States)

    Testori, Alberto; Meroni, Stefano; Errico, Valentina; Travaglini, Roberto; Voulaz, Emanuele; Alloisio, Marco

    2015-02-27

    Phyllodes tumor is an extremely rare tumor of the breast. It occurs in females in the third and fourth decades. The difficulty in distinguishing between phyllodes tumors and benign fibroadenoma may lead to misdiagnosis. Lymph node involvement is rarely described in phyllodes tumors; for this reason, sentinel node biopsy may be warranted. We present a case of a 33-year-old woman affected by huge tumor of the right breast with ulceration in the skin with a rapid tumor growth and with omolateral axillary metastasis.

  4. [A rare case of laryngeal leiomyoma].

    Science.gov (United States)

    Burzej-Uchto, Małgorzata; Nowak-Makieła, Zuzanna; Lapińska-Skowron, Kalina; Kosowski, Bogdan

    2006-01-01

    To report a rare case of laryngeal leiomioma. Tumors of smooth muscle origin are rare in the upper aerodigestive tract, due to the paucity of smooth muscle in the area. A review of the literature confirm that this kind of neoplasm is extremely rare in the larynx and not so many cases were described in the literature so far. We described classification, treatment and clinical course of laryngeal leiomioma. We encountered such a tumor in a 68-year-old men who was admitted to our Hospital. Hoarseness since 2 years was a main symptom. The tumor was removed endoscopically and was located under left vocal cord. The patient recovered well but after 6 months was admitted again to our Hospital due to recurrence of ailments. Tracheotomy and enlarged chordectomy on the left side of larynx was performed under general anesthesia. The tumor was encapsulated and could be completely resected. No complications were observed in the postoperative course and patient was discharged from the hospital in 5th day after operation. There was no evidence of loco regional recurrence 4 years post surgery and laryngeal function was preserved. All histological pictures of the specimens from the first and second operation indicated tumor as a laryngeal leiomioma. Laryngeal leiomioma is a very infrequent neoplasm and to our knowledge, this is the 38th case of laryngeal smooth muscle tumor to be reported in the world literature.

  5. 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.

  6. Xanthogranulomatous Prostatitis, a Rare Prostatic Entity.

    Science.gov (United States)

    Noyola, Alejandro; Gil, José Fernando; Lujano, Heriberto; Piñon, Omar; Muñoz, Gabriel; Michel, José Manuel; Garcia, Jorge; Valdez, Jorge; Morales, Omar

    2017-01-01

    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.

  7. A rare case of multiple myeloma initially presenting with pseudoachalasia.

    Science.gov (United States)

    Lazaraki, Georgia; Nakos, Andreas; Katodritou, Eirini; Pilpilidis, Ioannis; Tarpagos, Anestis; Katsos, Ioannis

    2009-01-01

    Pseudoachalasia is a rare clinical entity with clinical, radiographic, and manometric features often indistinguishable from achalasia. Primary adenocarcinomas arising at the gastroesophageal junction or a tumor of the distal esophagus are the most frequent causes of pseudoachalasia. Rarely, processes other than esophagogastric cancers including chronic idiopathic intestinal pseudo-obstruction, amyloidosis, sarcoidosis, Chagas' disease, vagotomy, antireflux surgery, pancreatic pseudocysts, von Recklinghausen's neuroinomatosis, gastrointestinal stromal tumor, and other malignancies and rare genetic syndromes, may lead to the development of pseudoachalasia. Secondary achalasia is extremely rare, with less than 100 cases reported in the literature so far. Gastrointestinal manifestations in primary or secondary amyloidosis include abdominal pain, diarrhea, constipation, malabsorption, obstruction, motility disturbance, intestinal infarction, perforation, and hemorrhage; however, gastrointestinal tract involvement is asymptomatic in most instances. We present here a rare case of multiple myeloma initially presenting with dysphagia because of esophageal amyloidosis and manometric findings typical of achalasia.

  8. 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.

  9. Extremely high genetic diversity in a single tumor points to prevalence of non-Darwinian cell evolution.

    Science.gov (United States)

    Ling, Shaoping; Hu, Zheng; Yang, Zuyu; Yang, Fang; Li, Yawei; Lin, Pei; Chen, Ke; Dong, Lili; Cao, Lihua; Tao, Yong; Hao, Lingtong; Chen, Qingjian; Gong, Qiang; Wu, Dafei; Li, Wenjie; Zhao, Wenming; Tian, Xiuyun; Hao, Chunyi; Hungate, Eric A; Catenacci, Daniel V T; Hudson, Richard R; Li, Wen-Hsiung; Lu, Xuemei; Wu, Chung-I

    2015-11-24

    The prevailing view that the evolution of cells in a tumor is driven by Darwinian selection has never been rigorously tested. Because selection greatly affects the level of intratumor genetic diversity, it is important to assess whether intratumor evolution follows the Darwinian or the non-Darwinian mode of evolution. To provide the statistical power, many regions in a single tumor need to be sampled and analyzed much more extensively than has been attempted in previous intratumor studies. Here, from a hepatocellular carcinoma (HCC) tumor, we evaluated multiregional samples from the tumor, using either whole-exome sequencing (WES) (n = 23 samples) or genotyping (n = 286) under both the infinite-site and infinite-allele models of population genetics. In addition to the many single-nucleotide variations (SNVs) present in all samples, there were 35 "polymorphic" SNVs among samples. High genetic diversity was evident as the 23 WES samples defined 20 unique cell clones. With all 286 samples genotyped, clonal diversity agreed well with the non-Darwinian model with no evidence of positive Darwinian selection. Under the non-Darwinian model, MALL (the number of coding region mutations in the entire tumor) was estimated to be greater than 100 million in this tumor. DNA sequences reveal local diversities in small patches of cells and validate the estimation. In contrast, the genetic diversity under a Darwinian model would generally be orders of magnitude smaller. Because the level of genetic diversity will have implications on therapeutic resistance, non-Darwinian evolution should be heeded in cancer treatments even for microscopic tumors.

  10. 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.

  11. 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.)

  12. Tumor neuroectodérmico primitivo da bexiga urinária: uma rara neoplasia Primitive neuroectodermal tumor of the urinary bladder: a rare neoplasm

    Directory of Open Access Journals (Sweden)

    Daniel Cury Ogata

    2010-02-01

    Full Text Available Os autores relatam o caso de paciente do sexo feminino, 52 anos, com queixas de hematúria macroscópica e dor pélvica. Foi realizado exame ultrassonográfico, que mostrou massa expansiva em assoalho vesical. A cistoscopia confirmou a presença dessa lesão, sendo realizada biópsia. O exame histológico revelou neoplasia de pequenas células. A análise imuno-histoquímica foi necessária para elucidação diagnóstica. O referido exame demonstrou positividade para os marcadores EWS-FLI1, CD99 e S-100. O diagnóstico foi de tumor neuroectodérmico primitivo. A paciente foi tratada com quimioterapia adjuvante. Depois de sete meses de seguimento, a paciente encontra-se livre de doença.The authors report the case of a 52 year-old woman that presented macroscopic hematuria and pelvic pain. Ultrasound examination was performed, which showed an expansive mass in the bladder floor. A cystoscopy confirmed the presence of this lesion and a biopsy was carried out. Histological analysis showed a small cell neoplasm. The immunohistochemical analysis was required to establish diagnosis. This analysis revealed positivity for EWS-FLI1, CD99 and S-100. The conclusive diagnosis was primitive neuroectodermal tumor. The patient was treated with chemotherapy. After a seven month follow-up period, the patient is disease-free.

  13. Osteochondromyxoma: Review of a rare carney complex criterion.

    Science.gov (United States)

    Golden, Todd; Siordia, Juan A

    2016-11-01

    Osteochondromyxoma is an extremely rare bone tumor associated with 1% of Carney complex patients and constitutes one of its 11 diagnostic criteria. This narrative review of osteochondromyxoma is based on a search of all references to the topic in PubMed, Web Of Science, SCOPUS, ScienceDirect, and JSTOR databases. Special attention was focused on case reports, leading to a review encompassing the case reports to date, as well as related animal model studies. This review covers the current understanding of osteochondromyxoma, highlighting its variability while providing consensus on the most common clinical presentation, pathological findings, and genetic features of this rare bone tumor.

  14. Basal cell adenoma-clinicopathological, immunohistochemical analysis and surgical considerations of a rare salivary gland tumor with review of literature

    Directory of Open Access Journals (Sweden)

    A D Bhagat Singh

    2015-01-01

    Full Text Available Introduction: Basal cell adenoma (BCA of the salivary glands is a rare benign salivary gland tumour. Differentiation of BCA from varied entities involving maxillofacial area is mandatory. Aim: To analyze the clinicopathological, histopathologic features, immunohistochemcal analysis and surgical considerations of this rare entity. Materials and Methods: This study included 12 cases of BCA from archives of department reported over the period of 13 years. All the pertaining clinicopathologic features such as incidence, age, sex and site of lesions were assessed. Tissue sections were stained by using panel of immunohistochemical markers, i.e. Pan CK, CK 5/6 and S100, Calponin, p63, CD 117 and smooth muscle actin. Results: BCA was observed in 26-52 years age group (mean age, 38.75 years with female propensity of 7:5 male to female ratio. It is seen more commonly in parotid gland, followed by upper lip, buccal mucosa and palate. Solid type is the most common histopathologic type followed by tubular, membranous and trabecular. Only one case of membranous type of BCA showed recurrence. Pan CK, CK 5/6 showed strong immunoreactivity, calponin showed moderate staining, p63 and Ki-67 mild staining, whereas CD 117 and SMA showed negative immunostaining. Conclusion: Vigilant comprehensive analysis of all the pertaining clinicopathologic and histopathologic features and immunohistochemical analysis are required for differentiating from other lesions with basaloid differentiation having varying prognosis.

  15. Triple independent primaries of female genital tract: A rare event

    Directory of Open Access Journals (Sweden)

    Suprio Ray Chaudhury

    2014-01-01

    Full Text Available The synchronous presence of endometrioid carcinoma of ovary and uterus in a single patient is an uncommon occurrence; but, finding a patient having a third simultaneous primary in cervix along with carcinoma ovary and endometrium is an extremely rare entity. In this article we describe a 40-year-old female who presented with gradual distension of abdomen, and underwent extended radical hysterectomy operation following diagnosis of an ovarian tumor on ultrasonography. On examination of the specimen, it was found to harbor endometrioid carcinoma of uterus and endometrioid carcinoma of ovary along with a small focus of well-differentiated squamous cell carcinoma of cervix. The presence of three coexistent primary tumors in female genital tract is an extremely rare event. Further molecular and genetic studies may throw light on the probable etiology of such rare cases.

  16. 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

  17. Intercalary Reconstruction after Wide Resection of Malignant Bone Tumors of the Lower Extremity Using a Composite Graft with a Devitalized Autograft and a Vascularized Fibula

    Directory of Open Access Journals (Sweden)

    Koichi Ogura

    2015-01-01

    Full Text Available Introduction. Although several intercalary reconstructions after resection of a lower extremity malignant bone tumor are reported, there are no optimal methods which can provide a long-term reconstruction with fewest complications. We present the outcome of reconstruction using a devitalized autograft and a vascularized fibula graft composite. Materials and Methods. We conducted a retrospective review of 11 patients (7 males, 4 females; median age 27 years undergoing reconstruction using a devitalized autograft (pasteurization (n=6, deep freezing (n=5 and a vascularized fibula graft composite for lower extremity malignant bone tumors (femur (n=10, tibia (n=1. Results. The mean period required for callus formation and bone union was 4.4 months and 9.9 months, respectively. Four postoperative complications occurred in 3 patients: 2 infections (1 pasteurized autograft, 1 frozen autograft and 1 fracture and 1 implant failure (both in pasteurized autografts. Graft removal was required in 2 patients with infections. The mean MSTS score was 81% at last follow-up. Conclusions. Although some complications were noted in early cases involving a pasteurized autograft, our novel method involving a combination of a frozen autograft with a vascularized fibula graft and rigid fixation with a locking plate may offer better outcomes than previously reported allografts or devitalized autografts.

  18. A rare case of recurrent malignant triton tumor in a male with NF1: Case report and mini-review

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    B. Aykut

    2016-01-01

    Conclusion: Clinically, MTTs are characterized as highly aggressive tumors that are fast-growing and prone to local recurrence and distant metastasis. To date, there is no treatment consensus available yet and many patients succumb to the disease shortly after diagnosis. This is because the pathogenesis of MTT remains unknown and patients with MTT are often diagnosed at a late stage of disease. Our case presents valuable teaching points in terms of providing a possible progression model based on the coexistence of a low-grade MPNST and MTT in the context of NF1 and an atypical neurofibroma in this patient. Close monitoring of patients with NF1 and atypical neurofibromas or MPNST might therefore help to diagnose MTT at an earlier stage.

  19. Intracranial subdural osteoma: a rare benign tumor that can be differentiated from other calcified intracranial lesions utilizing MR imaging.

    Science.gov (United States)

    Barajas, Ramon F; Perry, Arie; Sughrue, Michael; Aghi, Manish; Cha, Soonmee

    2012-10-01

    We report the magnetic resonance (MR) imaging characteristics of subdural osteoma and other benign calcified intracranial lesions to highlight imaging features that differentiate between these disease entities. A 63-year-old woman presented with progressively altered mental status. Non-contrast CT demonstrated a densely calcified right middle cranial fossa extra-axial mass. MR imaging of the lesion demonstrated T1 and T2 hypointensity without evidence of contrast enhancement, parenchymal abnormality, or connection to adjacent venous structures. Diffusion weighted imaging demonstrated markedly decreased signal intensity and artificially reduced diffusion on apparent diffusion coefficient map. Histologically, the tumor was predominantly composed of lamellar bone and small fragments of residual dura consistent with subdural osteoma. This case demonstrates that radiological examination can provide additional insight into the origin of intracranial osteomas (extradural versus subdural versus sinonasal) and help distinguish from other diagnostic considerations including benign meningeal ossification and calcified meningioma prior to surgical resection.

  20. Primary intraoral granulocytic sarcoma: A rare case presenting as generalized gingival enlargement

    Directory of Open Access Journals (Sweden)

    Thayalan Dineshkumar

    2016-01-01

    Full Text Available Granulocytic sarcoma (GS is an extremely rare condition involving infiltration of myeloblasts or immature myeloid cells in an extramedullary site. It is also known as chloroma, myeloid sarcoma or extramedullary myeloid tumor. It usually occurs concomitantly with acute myelogenous leukemia or with the onset of blastic phase of chronic myelogenous leukemia. On rare occasions, it evolves even before the onset of leukemias, and when it precedes leukemias without any overt signs, it is referred to as the primary type. Although GSs can involve any body part, localization in the oral cavity is extremely rare. The recognition of this rare primary entity is important because early aggressive chemotherapy can cause regression of the tumor and improve survival. Here, we report a rare case of GS in a nonleukemic 62-year-old female who presented with generalized gingival enlargement involving both maxilla and mandible.

  1. 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.

  2. 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.)

  3. 表现为下肢深静脉血栓的盆腔肿瘤四例报告%Pelvic tumors misdiagnosed as lower extremity deep venous thrombosis:4 cases report

    Institute of Scientific and Technical Information of China (English)

    刘铖; 李鼎锋; 郭钧; 崔秋

    2015-01-01

    Objective To explore the reasons for the misdiagnosis of pelvic tumors as lower extremity deep venous thrombosis ( DVT ), as well as the diagnostic and treatment methods for pelvic tumors.Methods Four patients with pelvic tumors were misdiagnosed as lower extremity DVT. The reasons of misdiagnosis and diagnosis and treatment for pelvic tumors were analyzed based on the relevant literatures.Results There were one case of ifbroneuroma, one cases of moderately differentiated squamous-cell carcinoma, one case of myxo-lfuidity liposarcoma and one case of synovial sarcoma. Tumors in the patient with fibroneuroma were completely removed. Malignant tumors in the other 3 patients were resected, when the tumor size was shrinked with a clear boundary and the blood supply was decreased after 3 cycles of intra-arterial chemotherapy via an implanted pump from the femoral artery to the common iliac artery. No recurrence or metastasis was found in the follow-up.Conclusions The clinical manifestations are untypical in the patients with pelvic tumors, which are easily misdiagnosed as DVT. Therefore, great attention should be paid in the diagnosis. Resection is preferred for benign tumors, and intra-arterial intervention chemotherapy should be applied ifrst for malignant tumors, followed by surgery.

  4. 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....

  5. Testicular Seminoma With Pseudocyst and Coagulation Necrosis Like Burned-out Tumor: A Case Report

    Directory of Open Access Journals (Sweden)

    Tatsuhiko Hoshii

    2016-07-01

    Full Text Available Testicular seminoma is a relatively common testicular cancer; however, testicular seminoma with pseudocyst is an extremely rare. The ‘burned-out’ phenomenon in germ cell tumors refers to a germ cell tumor in extra-gonadal tissues with spontaneous regression of an intra-gonadal tumor. We present a case of the testicular seminoma with pseudocyst and coagulation necrosis like burned-out tumor without metastasis.

  6. Benign multicystic peritoneal mesothelioma mimicking recurrence of an ovarian borderline tumor: a case report

    OpenAIRE

    Takemoto Shuji; Kawano Ryosuke; Honda Kazumi; Nakazono Aki; Shimamatsu Kazuhide

    2012-01-01

    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 ...

  7. Giant Cell Tumor: A Rare Condition in the Immature Skeleton—A Retrospective Study of Symptoms, Treatment, and Outcome in 16 Children

    Science.gov (United States)

    Skeie, Anette Torød; Lobmaier, Ingvild Koren; Zaikova, Olga

    2016-01-01

    Background. Pediatric giant cell tumor (GCT) of bone is rare and the course of the disease in the immature skeleton is sparsely described. We performed a retrospective study addressing symptoms, treatment, and outcome in children with GCT. Methods. Review of medical records and images of patients with GCT. Patients were detected from our hospital prospective database and those with open epiphyseal cartilages were included. Results. 16 children (75% girls) from 6 to 15 years old were identified. Eight lesions (50%) were in long bones and 4 (25%) in flat bones. One lesion appeared to be purely epiphyseal. All patients had pain as the initial symptom. Local recurrence developed in 2 patients. 14 of 16 patients returned to normal activity with no sequelae. One patient developed anisomelia after surgery. Conclusions. The biological tumor behavior in children does not seem to differ from what is reported in adults. Lesions in flat bones are very unusual, but our data alone do not provide enough evidence to conclude that this is more common in the immature skeleton. Literature review showed only one previous case report describing a purely epiphyseal GCT. Intralesional curettage is appropriate treatment and gives good functional results with acceptable recurrence rates. PMID:27999474

  8. Giant Cell Tumor: A Rare Condition in the Immature Skeleton—A Retrospective Study of Symptoms, Treatment, and Outcome in 16 Children

    Directory of Open Access Journals (Sweden)

    Thale M. Asp Strøm

    2016-01-01

    Full Text Available Background. Pediatric giant cell tumor (GCT of bone is rare and the course of the disease in the immature skeleton is sparsely described. We performed a retrospective study addressing symptoms, treatment, and outcome in children with GCT. Methods. Review of medical records and images of patients with GCT. Patients were detected from our hospital prospective database and those with open epiphyseal cartilages were included. Results. 16 children (75% girls from 6 to 15 years old were identified. Eight lesions (50% were in long bones and 4 (25% in flat bones. One lesion appeared to be purely epiphyseal. All patients had pain as the initial symptom. Local recurrence developed in 2 patients. 14 of 16 patients returned to normal activity with no sequelae. One patient developed anisomelia after surgery. Conclusions. The biological tumor behavior in children does not seem to differ from what is reported in adults. Lesions in flat bones are very unusual, but our data alone do not provide enough evidence to conclude that this is more common in the immature skeleton. Literature review showed only one previous case report describing a purely epiphyseal GCT. Intralesional curettage is appropriate treatment and gives good functional results with acceptable recurrence rates.

  9. Giant Cell Tumor: A Rare Condition in the Immature Skeleton-A Retrospective Study of Symptoms, Treatment, and Outcome in 16 Children.

    Science.gov (United States)

    Strøm, Thale M Asp; Skeie, Anette Torød; Lobmaier, Ingvild Koren; Zaikova, Olga

    2016-01-01

    Background. Pediatric giant cell tumor (GCT) of bone is rare and the course of the disease in the immature skeleton is sparsely described. We performed a retrospective study addressing symptoms, treatment, and outcome in children with GCT. Methods. Review of medical records and images of patients with GCT. Patients were detected from our hospital prospective database and those with open epiphyseal cartilages were included. Results. 16 children (75% girls) from 6 to 15 years old were identified. Eight lesions (50%) were in long bones and 4 (25%) in flat bones. One lesion appeared to be purely epiphyseal. All patients had pain as the initial symptom. Local recurrence developed in 2 patients. 14 of 16 patients returned to normal activity with no sequelae. One patient developed anisomelia after surgery. Conclusions. The biological tumor behavior in children does not seem to differ from what is reported in adults. Lesions in flat bones are very unusual, but our data alone do not provide enough evidence to conclude that this is more common in the immature skeleton. Literature review showed only one previous case report describing a purely epiphyseal GCT. Intralesional curettage is appropriate treatment and gives good functional results with acceptable recurrence rates.

  10. The ectopic expression of the gastric inhibitory polypeptide receptor is frequent in adrenocorticotropin-independent bilateral macronodular adrenal hyperplasia, but rare in unilateral tumors.

    Science.gov (United States)

    Groussin, Lionel; Perlemoine, Karine; Contesse, Vincent; Lefebvre, Hervé; Tabarin, Antoine; Thieblot, Philippe; Schlienger, Jean Louis; Luton, Jean Pierre; Bertagna, Xavier; Bertherat, Jérôme

    2002-05-01

    Control of cortisol secretion by the abnormal expression of the gastric inhibitory polypeptide receptor (GIP-R) have been observed in some rare cases of ACTH-independent, food-dependent Cushing's syndrome (FD-ACS) due to adrenal adenoma (AA) or bilateral macronodular hyperplasia (AIMAH). This study was performed to determine the prevalence of GIP-R ectopic expression in ACS and its correlation with fasting cortisol levels. GIP-R expression was studied by RT-PCR in 30 unilateral adrenal tumors [16 AA and 14 adrenocortical cancer (AC)] and 8 AIMAH tissues. Fasting and postprandial cortisol levels were assayed, respectively, at 0800 and 1200 h in AA, AC, and AIMAH, and 1 h after a morning standard meal in 6 AIMAH patients. Similar expression of 2 GIP-R isoforms was observed in 1 of 16 AA, 0 of 14 AC, and 4 of 8 AIMAH as well as in the 4 insulinomas used as positive controls. In vitro study of the GIP-R-expressing AA showed stimulation of cortisol secretion and cAMP production by GIP. The fasting 0800-h plasma cortisol level was above 276 nmol/liter in all patients except 1 AA case and 1 AIMAH case, both of whom expressed GIP-R. In the 3 additional AIMAH cases that expressed the GIP-R, fasting plasma cortisol levels were above 276 nmol/liter. This study demonstrates that ectopic expression of GIP-R is rare in AA and is usually associated with the low fasting plasma cortisol levels that characterize FD-ACS. In contrast, GIP-R expression is frequent in AIMAH and might not always be associated with a low fasting plasma cortisol level. This suggests that maintenance of hypercortisolemia in GIP-R- expressing AIMAH does not always depend solely on GIP-R, and that simultaneous abnormal expression of other membrane receptors might be present. The expression of GIP-R could not be observed during malignant transformation of the adrenal cortex. This study highlighted the major role of cAMP alterations secondary to GIP-R ectopic expression in the pathophysiology of AIMAH and in

  11. Pediatric cervical sympathetic chain schwannoma with Horner syndrome: a rare case presentation.

    Science.gov (United States)

    Bhagat, Sanjeev; Varshney, Saurabh; Bist, Sampan S; Gupta, Nitin

    2014-03-01

    Schwannomas are rare, benign, slowly growing neurogenic tumors that originate in peripheral, spinal, or cranial nerves other than the optic and olfactory nerves. In the head and neck, these tumors usually arise from the vagus nerve and the cervical sympathetic chain in the parapharyngeal space. Cervical sympathetic chain schwannomas represent a rare subgroup of schwannomas; fewer than 60 cases have been reported in the literature. These tumors are rarely seen in children and adolescents. Because patients typically present with an asymptomatic neck mass and vague complaints, clinical suspicion is important in making the diagnosis. Neural deficits at presentation are uncommon. The presence of features of Horner syndrome before excision is very rare, having been previously reported in only 10 cases of cervical sympathetic chain schwannoma. We report an extremely rare case of a cervical sympathetic chain schwannoma in a child who presented with Horner syndrome. The lesion was successfully excised.

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

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    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.

  13. Sclerosing Stromal Tumor of Ovary: A Case Report

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    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.

  14. Imaging findings of rare tumors or tumor-like lesions in liver grafts after liver transplantation%移植肝少见肿瘤或肿瘤样病变的影像学表现

    Institute of Scientific and Technical Information of China (English)

    陈炳辉; 谢佩怡; 全力; 谢斯栋; 孟晓春; 单鸿

    2014-01-01

    Objective To investigate the imaging findings of rare tumors or tumor-like lesions in liver grafts after liver transplantation.Methods Imaging data of 4 patients with rare tumors or tumor-like lesions in the liver grafts of patients after liver transplantation from December 2006 to August 2013 in the Third Affiliated Hospital of Sun Yat-sen University were reviewed.Findings of computerized tomography ( CT ) and ( or ) magnetic resonance imaging ( MRI) plain scan and dynamic enhanced MRI of liver lesions in these patients were analyzed specially.Results In the 4 patients, 3 cases were misdiagnosed , including undifferentiated liver sarcoma , secondary lymphoma and eosinophilic granuloma.One case was diagnosed correctly with hepatic metastases of gallbladder carcinoma.The undifferentiated liver sarcomas appeared as cystic and solid space occupying masses with significant and persistent enhancement in the surrounding solid and separating parts .No enhancement was observed in the cystic parts , which increased rapidly in a short term.No edema area around the lesions was observed , but many calcifications were detected.The secondary hepatic lymphoma appeared as intrahepatic multiple nodules , most of which presented uniform signal intensity with moderate enhancement in every phase.No intrahepatic vascular invasion was observed but extrahepatic lymphadenectasis was detected .Bleeding was observed in a few lesions.The liver eosinophilic granuloma appeared as multiple liver lesions with variable signal intensity and enhancement modes , which suggested that the lesions were consisted of variety of elements and in the different disease stages.Most of the lesions were observed with progressive circled enhancement.The recurrence of gallbladder carcinoma and hepatic metastasis after liver transplantation appeared as a intrahepatic hypodensity lesion with slight enhancement in arterial phase and slight hypodensity in portal phase.Retroperitoneal lymph nodes were observed

  15. Improvement of long-term blindness caused by compression from inner-third sphenoid wing meningioma after optic canal decompression: An extremely rare case report

    Science.gov (United States)

    Tamura, Ryota; Takahashi, Satoshi; Horikoshi, Tomo; Yoshida, Kazunari

    2016-01-01

    Background: There has been no previous case report of a patient whose visual acuity improved after long-term blindness caused by tumor invasion into the optic canal. Case Description: A 65-year-old Asian woman presented with a 6-month history of blindness caused by a meningioma located on the inner third of the sphenoid ridge. An operation was performed to prevent further tumor invasion into the cavernous sinus and contralateral optic nerve. During surgery, optic canal decompression was performed using an epidural approach. Subtotal removal of the tumor was achieved. Two days after the surgery, her left visual acuity recovered from blindness. Conclusion: Normally, long-term blindness caused by optic nerve compression by a brain tumor is regarded as irreversible, and even a surgical excision of the optic nerve is performed in some cases. However, because we experienced a case in which the patient recovered from long-term blindness after optic canal decompression, we believe that this surgical procedure should definitely be considered as an option. PMID:27413579

  16. Germ cell tumor located in the midline of the anterior neck.

    Science.gov (United States)

    Pirdopska, Tatyana; Terziev, Ivan; Hristova, Sv; Mladenovsky, W; Petkov, R

    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.

  17. 腮腺少见肿瘤的影像学特点分析%Imaging characteristics of rare tumor of parotid gland

    Institute of Scientific and Technical Information of China (English)

    赵柳燕; 陈祖华

    2015-01-01

    目的:探讨腮腺少见肿瘤的影像学特点。方法回顾性分析13例经手术病理证实腮腺少见肿瘤(淋巴上皮癌4例,肌上皮瘤3例,淋巴瘤及转移瘤各2例,恶性黑色素瘤及肌上皮癌各1例)的C T、M RI影像学表现。结果均为腮腺单侧发病,其中左侧9例,右侧6例。淋巴上皮癌位于腮腺浅叶,可累及深叶,以单发为主(3/4);沿腮腺浅叶铸形生长(3/4)可能具有一定特点;密度和信号均匀,增强轻、中度均匀强化。肌上皮瘤均为单发,位于腮腺浅叶,呈椭圆形,边缘清晰锐利,密度和信号均匀或不均匀,显著强化(2/3)可能具有提示作用。淋巴瘤具有一定特点,常表现为多发类圆形肿块(2/2),边缘光整,密度均匀,增强后中度强化,常伴颈部淋巴结肿大。恶性黑色素瘤M RI具有一定特点:T1 WI呈多发结节状高信号,T2WI呈低信号;CT及MRI增强不具特点。肌上皮癌与腮腺常见恶性肿瘤相仿。转移瘤表现与原发肿瘤密切相关。结论腮腺上述少见肿瘤部分具有一定影像学特点,有助于鉴别诊断,确诊仍依靠组织病理学和免疫组化。%Objective To study the imaging characteristics of rare tumor of parotid gland .Methods CT ,MRI imaging findings of 13 cases of rare tumor (4 cases of lymphoepithelial carcinoma ,3 cases of myoepithelioma ,2 cases of lymphoma and metastatic tumors in each 1 case of malignant melanoma and myoepithelial carcinoma in each in the parotid gland) con‐firmed by operation and pathology were retrospectively analysed .Results The lesions were located in the unilateral parot‐id gland ,in the left side (n=8) ,in the right side (n=5) .Lymphoepithelial carcinomas were located in the superficial lobe of parotid gland ,which may violate the deep lobe ,majority of single lesion (3/4) .Casting growth (3/4) along the parotid gland may have certain feature:homogeneous density

  18. 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

  19. Improvement of long-term blindness caused by compression from inner-third sphenoid wing meningioma after optic canal decompression: An extremely rare case report

    Directory of Open Access Journals (Sweden)

    Ryota Tamura

    2016-01-01

    Conclusion: Normally, long-term blindness caused by optic nerve compression by a brain tumor is regarded as irreversible, and even a surgical excision of the optic nerve is performed in some cases. However, because we experienced a case in which the patient recovered from long-term blindness after optic canal decompression, we believe that this surgical procedure should definitely be considered as an option.

  20. 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. ...

  1. Clinicopathological features and treatment of extremity bone metastasis in patients with endometrial carcinoma: a case report and review

    Institute of Scientific and Technical Information of China (English)

    JIANG Guo-qing; GAO Yu-nong; GAO Min; ZHENG Hong; YAN Xin; WANG Wen; AN Na; CAO Kun

    2011-01-01

    Unlike other non-gynecologic solid tumors, such as breast cancer, lung cancer, metastasis to bone from endometrial carcinoma is rare, metastasis to extremity is extremely rare. We report a 51-year-old multiparous woman with FIGO Stage IVb Grade 2 endometrial adenocarcinoma which metastasized to left lower extremity bone. She received an amputation of left lower extremity below the knees, and a total abdominal hysterectomy and bilateral salpingo-oophorectomy, and followed by systemic chemotherapy, radiation therapy to the pelvis and progestational agent. She had a complete response to above treatments, and disease-free survival for 10 months. After recurrence, she received chemotherapy, radiotherapy and progestational agent once again. She had lived 56 months and is still alive by the time of report. Metastasis of endometrial carcinoma to extremity bone can rarely occur and should be considered when the patient with endometrial carcinoma complained of unexplained pain and swelling associated with extremity bone.

  2. Intraosseous malignant peripheral nerve sheath tumor of maxilla: A case report with review of the literature

    Directory of Open Access Journals (Sweden)

    Sandhya Tamgadge

    2014-01-01

    Full Text Available Malignant peripheral nerve sheath tumor (MPNST, the principle malignancy of peripheral nerve origin, though rare in the general population, occurs with excessive frequency among patients with neurofibromatosis. This tumor always arises in soft-tissues, usually found in the lower extremities and only 10-12% of all lesions occur in the head and neck region, which makes it a rare entity. The primary intraosseous MPNST is rare and has been reported most frequently in the mandible. This article discusses a case report of MPNST of the left maxilla without a history of benign nerve tissue tumor and the diagnostic difficulties associated with MPNST.

  3. Extremely rare coincidence of non-radiographic axial spondyloarthropathy HLA-B27 positive and Stiff Person Syndrome--rheumatologist point of view.

    Science.gov (United States)

    Marinović, Ivanka; Pivalica, Dinko; Aljinović, Jure; Vlak, Tonko; Škorić, Ela; Martinović Kaliterna, Dušanka

    2016-01-01

    Stiff Person Syndrome (SPS) is a rare autoimmune neurological disorder characterized by progressive stiffness and rigidity of truncal muscles accompanied with co-contraction of agonist-antagonist muscles. Our 51-year-old female patient was presented for the first time to physiatrists in 2006 and diagnosed with axial-spondyloarthropathy (SpA) HLA-B27 positive. SPS was diagnosed 7 years after initial symptoms. SPS should be taken into consideration in HLA-B27 positive patients if stiffness of paravertebral and abdominal muscles progresses during SpA therapy.

  4. 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.

  5. 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.)

  6. Synchronous dual primary malignancy of urinary bladder and hypopharynx in a 62-year-old male patient: An extremely rare phenomenon

    Directory of Open Access Journals (Sweden)

    Kuna J

    2016-04-01

    Full Text Available The diagnosis of multiple primary malignancy (MPM is not uncommon. Nevertheless synchronous MPM involving urinary bladder and hypopharynx is an extremely unusual event.No such cases have been reported so far in English literature to our review. We report the case of 62-year-old male patient who presented with haematuria and later dysphagia to solids. Whole body 18F-fluro deoxy glucose positron emission tomography- computed tomography (18FFDG PET-CT revealed two distinct lesions one in hypopharynx and another in urinary bladder. Biopsy from the urinary bladder mass and the hypopharyngeal lesion were suggestive of urothelial carcinoma and poorly differentiated squamous cell carcinoma respectively. The patient was treated with concurrent chemo-radiotherapy to hypopharynx. Following treatment he had improvement of dysphagia. Haematuria had also stopped. Because of severe comorbidities the planned radical cystectomy or radiotherapy were deferred. Currently the patient is in symptomatic remission six months following treatment.

  7. 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.

  8. Solitary eccrine syringofibroadenoma with nail involvement: A rare entity

    Directory of Open Access Journals (Sweden)

    Pooja Arora

    2015-01-01

    Full Text Available Eccrine syringofibroadenoma (ESFA is a rare, benign tumor of eccrine sweat gland origin that usually presents as a nodule on the extremities of an elderly person. It can also present as an ulcerative plaque, verrucous lesion, papular or nodular lesion or as palmoplantar keratoderma. Although the clinical features are variable, histology is characteristic in the form of anastomosing strands, cords and columns of epithelial cells embedded in a fibrovascular stroma. We report the case of a 62-year-old male with a nodular lesion on the extremity that caused secondary involvement of the nail in the form of complete nail dystrophy. The histology showed features consistent with ESFA. Nail involvement by ESFA is a rare presentation and is rarely described in the literature.

  9. Analysis of rare categories

    CERN Document Server

    He, Jingrui

    2012-01-01

    In many real-world problems, rare categories (minority classes) play essential roles despite their extreme scarcity. The discovery, characterization and prediction of rare categories of rare examples may protect us from fraudulent or malicious behavior, aid scientific discovery, and even save lives. This book focuses on rare category analysis, where the majority classes have smooth distributions, and the minority classes exhibit the compactness property. Furthermore, it focuses on the challenging cases where the support regions of the majority and minority classes overlap. The author has devel

  10. 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.

  11. 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.

  12. Rare Skeletal Complications in the Setting of Primary Hyperparathyroidism

    Science.gov (United States)

    Sabanis, Nikos; Gavriilaki, Eleni; Paschou, Eleni; Kalaitzoglou, Asterios; Papanikolaou, Dimitrios; Ioannidou, Pinelopi; Vasileiou, Sotirios

    2015-01-01

    Parathyroid carcinoma represents an extremely rare neoplasm with diverse clinical manifestations which vary from asymptomatic patients to severe complications of hypercalcemia or parathyrotoxicosis while skeletal involvement is rather common. Herein we aimed at presenting a unique case of a young patient with rare aggressive skeletal complications of parathyroid cancer that initially were misdiagnosed. Ossification of the cervical ligamentum flavum and skull tumor illustrates erosive bonny lesions of hyperparathyroidism that in association with previous medical history of recurrent nephrolithiasis and biochemical findings guide the diagnosis. We suggest that increased awareness and holistic approach are needed in order to recognize and further investigate signs and symptoms of hyperparathyroidism. PMID:26664767

  13. Rare Skeletal Complications in the Setting of Primary Hyperparathyroidism

    Directory of Open Access Journals (Sweden)

    Nikos Sabanis

    2015-01-01

    Full Text Available Parathyroid carcinoma represents an extremely rare neoplasm with diverse clinical manifestations which vary from asymptomatic patients to severe complications of hypercalcemia or parathyrotoxicosis while skeletal involvement is rather common. Herein we aimed at presenting a unique case of a young patient with rare aggressive skeletal complications of parathyroid cancer that initially were misdiagnosed. Ossification of the cervical ligamentum flavum and skull tumor illustrates erosive bonny lesions of hyperparathyroidism that in association with previous medical history of recurrent nephrolithiasis and biochemical findings guide the diagnosis. We suggest that increased awareness and holistic approach are needed in order to recognize and further investigate signs and symptoms of hyperparathyroidism.

  14. 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.

  15. Pindborg tumor

    Science.gov (United States)

    Caliaperoumal, Santhosh Kumar; Gowri, S.; Dinakar, J.

    2016-01-01

    Calcifying epithelial odontogenic tumor (CEOT), also known as Pindborg tumor, is a rare odontogenic epithelial neoplasm. So far, nearly 200 cases have been reported in the literature. We are reporting a case of CEOT in a 42-year-old male patient with painless bony swelling in the mandible. The clinical, radiographic, and histopathologic features are discussed with relevant references. PMID:27041911

  16. Pindborg tumor

    Directory of Open Access Journals (Sweden)

    Santhosh Kumar Caliaperoumal

    2016-01-01

    Full Text Available Calcifying epithelial odontogenic tumor (CEOT, also known as Pindborg tumor, is a rare odontogenic epithelial neoplasm. So far, nearly 200 cases have been reported in the literature. We are reporting a case of CEOT in a 42-year-old male patient with painless bony swelling in the mandible. The clinical, radiographic, and histopathologic features are discussed with relevant references.

  17. 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.

  18. Intramedullary thoracic spinal cord meningioma: a rare case report and review of the literature.

    Science.gov (United States)

    Yuan, Dun; Liu, Dingyang; Yuan, Xian-rui; Xi, Jian; Ding, Xi-ping

    2013-12-01

    A 33-year-old male presented with a thoracic spinal intramedullary meningioma manifesting as bilateral asymmetric progressive weakness in the lower extremities. Preoperative magnetic resonance imaging (MRI) showed an intramedullary mass at the T1-T3 level. Intraoperative inspection found that the spinal cord was markedly swollen with a normal surface while dural attachment was not confirmed. Gross total removal of the tumor was achieved. The morphologic and immunohistochemical findings were compatible with the diagnosis of meningioma. Postoperatively, the patient recovered from preoperative paraplegia. Although extremely rare, meningiomas should be considered when diagnosing intramedullary tumors. Georg Thieme Verlag KG Stuttgart · New York.

  19. How extreme are extremes?

    Science.gov (United States)

    Cucchi, Marco; Petitta, Marcello; Calmanti, Sandro

    2016-04-01

    High temperatures have an impact on the energy balance of any living organism and on the operational capabilities of critical infrastructures. Heat-wave indicators have been mainly developed with the aim of capturing the potential impacts on specific sectors (agriculture, health, wildfires, transport, power generation and distribution). However, the ability to capture the occurrence of extreme temperature events is an essential property of a multi-hazard extreme climate indicator. Aim of this study is to develop a standardized heat-wave indicator, that can be combined with other indices in order to describe multiple hazards in a single indicator. The proposed approach can be used in order to have a quantified indicator of the strenght of a certain extreme. As a matter of fact, extremes are usually distributed in exponential or exponential-exponential functions and it is difficult to quickly asses how strong was an extreme events considering only its magnitude. The proposed approach simplify the quantitative and qualitative communication of extreme magnitude

  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. Rare ovarian lesion in an adolescent girl

    Directory of Open Access Journals (Sweden)

    Ramasamy Senthilnathan

    2008-01-01

    Full Text Available Large solid ovarian lesions are considered malignant in nature in pediatric and adolescent age group. We present an adolescent girl who had large solid ovarian lesion, with negative tumor markers. She underwent laparotomy and right oopherectomy. Histopathology revealed that the lesion was massive ovarian edema. This is an extremely rare lesion of ovary and is benign in nature. Very few case reports are available in English literature. Hence we suggest that massive ovarian edema should be considred as one of the differential diagnosis in all the patients having large solid ovarian lesions with ngative tumor marker assay. Ovarian preservation with the help of frozen section analysis should always be considred in these patients.

  2. 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.

  3. [Rare ovarian tumours: therapeutic strategies in 2010, national website observatory for rare ovarian cancers and delineation of referent centers in France].

    Science.gov (United States)

    Ray-Coquard, I; Pautier, P; Pujade-Lauraine, E; Méeus, P; Morice, P; Treilleux, I; Duvillard, P; Alexandre, J; Lhommé, C; Selle, F; Guastalla, Jp

    2010-01-01

    Majorities of the rare ovarian cancers were represented by germ cell tumours and sex cords ovarian tumours with borderline tumours, clear cell carcinoma and mucinous carcinoma and are extremely rare malignant diseases of the ovaries. Tumors of the stromal (Leydig cells) and/or sex cords (Sertoli cells) represent approximately 7% of ovarian cancers and develop from the conjunctive tissue (respectively, interstitial and nurse cells) of the ovaries. All together, they represented less than 5% of the adult malignant and non malignant ovarian tumours. Treatment of rare ovarian tumors is currently as follows. Surgery is the same as that for ovarian adenocarcinoma, with one major difference: conservation of reproductive function in women of reproductive age is usual case for this type of tumor. Chemotherapy for germ cell and sex cords tumors, based on data reported in the literature is the same as that prescribed for testicular germ-cell tumors. For rare epithelial carcinoma, carboplatin plus paclitaxel remains the standard attitude with a well-known less efficiency than for other epithelial subtypes. Surgery, chemotherapy and possible surgical intervention for residual lesions are highly complex. Too rare to be included in randomized studies, treatment of these tumors has benefited from the therapeutic advancements made against testicular germ-cell tumors or with publications using retrospective data. Effectively, some prognostic factors such stage, histology, number of managed patients seems to be prognostic for survival. Because of the rarity of these tumours a specialized website (www.ovaire-rare.org) was developed in France in 2002. Objectives were: to delineate prognostic factors of these very rare diseases, to favour patient inclusion in a clinical trial available online, to provide access to online medical expert forum (disease-related) for complex cases, and finally to demonstrate the impact of these tools on improving medical practice. The website provides very

  4. Imaging findings of atypical teratoid/rhabdoid tumor of infancy and childhood in CNS: report of two cases

    Energy Technology Data Exchange (ETDEWEB)

    Shin, Hee Jung; Park, Dong Woo; Lee, Seung Roh; Hahm, Chang Kok; Hong, Eun Kyung [Hanyang Univ. College of Medicine, Seoul (Korea, Republic of)

    2001-05-01

    Atypical teratoid/rhabdoid tumor rarely occurs in the CNS, though is most common in infants under two years of age. It is characterized by unique histologic features, has an extremely aggressive natural course, and is located mainly in the infratentorial region. Radiologically, it is difficult to distinguish from primitive neuroectodermal tumor or medulloblastoma. We report the radiologic findings of two cases of atypical teratoid/rhabdoid tumor.

  5. An ancillary CT finding of intrapulmonary solitary fibrous tumor: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Dong Jun; Rho, Ji Young; Kwon, Ah Young [CHA Bundang Medical Center, CHA University, Seongnam (Korea, Republic of)

    2017-02-15

    Intrapulmonary solitary fibrous tumor is extremely rare. A few reports have presented typical CT findings such as well-defined, variable—sized, heterogeneously or homogenously well-enhanced intrapulmonary nodules. We report herein a rare case of intrapulmonary solitary fibrous tumor that showed typical clinical and CT features, and we also provide an ancillary CT finding that shows a distinguishable tubular vascular structure within the nodule. The tubular vascular structure was conjoined to the proximal pulmonary vein. In this study, we highlight an ancillary CT finding reported for the first time for the diagnosis of a patient with intrapulmonary solitary fibrous tumor.

  6. Metaphyseal giant cell tumor

    Energy Technology Data Exchange (ETDEWEB)

    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.

    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.

  7. Composite pheochromocytoma with a malignant peripheral nerve sheath tumor: Case report and review of the literature.

    Science.gov (United States)

    Namekawa, Takeshi; Utsumi, Takanobu; Imamoto, Takashi; Kawamura, Koji; Oide, Takashi; Tanaka, Tomoaki; Nihei, Naoki; Suzuki, Hiroyoshi; Nakatani, Yukio; Ichikawa, Tomohiko

    2016-07-01

    Adrenal tumors with more than one cellular component are uncommon. Furthermore, an adrenal tumor composed of a pheochromocytoma and a malignant peripheral nerve sheath tumor is extremely rare. A composite pheochromocytoma with malignant peripheral nerve sheath tumor in a 42-year-old man is reported here. After adequate preoperative control, left adrenalectomy was performed simultaneously with resection of the ipsilateral kidney for spontaneous rupture of the left adrenal tumor. Pathological findings demonstrated pheochromocytoma and malignant peripheral nerve sheath tumor in a ruptured adrenal tumor. To date, there have been only four reported cases of composite pheochromocytoma with malignant peripheral nerve sheath tumor, so the present case is only the fifth case in the world. Despite the very poor prognosis of patients with pheochromocytoma and malignant peripheral nerve sheath tumors reported in the literature, the patient remains well without evidence of recurrence or new metastatic lesions at 36 months postoperatively. Copyright © 2012. Published by Elsevier Taiwan.

  8. A rare case of dermatofibrosarcoma protuberans of the forefoot

    Directory of Open Access Journals (Sweden)

    Cameron Wales

    2016-06-01

    Full Text Available Dermatofibrosarcoma protuberans is an extremely rare, potentially malignant tumor type that usually presents on the trunk or proximal extremities. The clinical presentation includes a gradually enlarging painless plaque-like or nodular lesion of the skin with surrounding red to blue discoloration. The diagnosis is based on clinical presentation, computed tomography or magnetic resonance imaging, and biopsy with histologic analysis. An early and timely diagnosis improves chances of complete surgical resection thus improving prognosis. Herein, we present a rare case of dermatofibrosarcoma protuberans with the hopes that its addition to the literature will aid in the earlier recognition of future patients and help prevent this potentially curable disease from becoming deadly.

  9. 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.

  10. Myxoinflammatory Fibroblastic Sarcoma: A Radiographical, Pathological, and Immunohistochemical Report of Rare Malignancy

    Directory of Open Access Journals (Sweden)

    Michitaka Kato

    2015-01-01

    Full Text Available Myxoinflammatory fibroblastic sarcoma (MIFS is a rare, painless, and intermediate (rarely metastasizing fibroblastic tumor, which commonly occurs in the extremities, with an equal sex predilection. This sarcoma is composed of a mixed inflammatory infiltrate along with spindled, epithelioid, and bizarre tumor cells in a background of hyaline and myxoid areas. In spite of such a distinctive morphology, the tumor can be a diagnostic challenge, simulating inflammatory conditions as well as neoplastic nature. For accurate diagnosis, the tumor requires extensive clinical, radiological, and pathological investigations. We present a case of MIFS in a 19-year-old female who presented with a mass in the left ankle. After appropriate excision and postoperative radiation therapy, she is free of disease, including recurrence and metastasis, at 12 years postoperatively.

  11. Primary rhabdomyosarcoma of the fallopian tube: A very rare case

    Directory of Open Access Journals (Sweden)

    Aramita Saha

    2013-01-01

    Full Text Available Sarcomas of the fallopian tube are extremely rare malignancies, primary rhabdomyosarcoma (RMS of the fallopian tube being exceedingly rare entity. We present here a case of primary RMS of the fallopian tube in a 72-year-old female who presented with occasional intermittent colicky abdominal pain and watery per vaginal discharge for 1.5 months. Macroscopic examination of the operated specimen of uterus and ovaries showed that the ampullary end of the right fallopian tube had a 9 cm tumor in its greatest axis. Microscopic examination revealed pleomorphic sarcoma of the right fallopian tube. Immunohistochemical examination revealed the tumor cells expressed desmin, myogenin, and smooth muscle actin (SMA; and are immunonegative for cytokeratin, epithelial membrane antigen (EMA, human melanoma black (HMB-45, S-100 protein, and h-caldesmon; which was in favor of pleomorphic sarcoma, RMS. Considering the age, performance status of the patient and histology (RMS, adjuvant chemotherapy with single agent doxorubicin was considered.

  12. 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.

  13. Neurofibroma of the maxillary antrum: A rare case

    Directory of Open Access Journals (Sweden)

    Deepali Jain

    2014-01-01

    Full Text Available Neurofibromas are benign tumors of peripheral nerve tissue, frequently associated with neurofibromatosis type 1. Their isolated occurrence in the maxillary antrum is rare, with only 6 cases described in the English literature to the best of our knowledge. Primary neurogenic tumors in the maxillary sinus are unusual entities. The majority of the reported cases that have dealt with neurilemmomas and isolated neurofibromas are extremely rare. Here, a case of neurofibroma of the maxillary sinus. We present the case of a 60-year-old female patient with the chief complain of growth in the upper right back region of the jaw, which was preceded by exfoliation of teeth in the same region 1 month back.

  14. Malignant Peripheral Nerve Sheath Tumor - A Case Report

    Directory of Open Access Journals (Sweden)

    Anju N Duttargi

    2007-01-01

    Full Text Available Malignant Peripheral Nerve Sheath Tumor [MPNST] is an extremely rare tumor affecting the oral cavity. It refers to sarcomas that arise from nerve or display features of neural differentiation. Here we present a case of 30-year old male patient with MPNST of right side of the mandible. There was a family history of neurotibromatosis in this case. Histologically, pleomorphic spindle cells with wavy nuclei, light stained cytoplasm, and mitotic activity were observed. The clinical presentation, radiological findings, and light microscopic findings are described in detail. The criteria for diagnosing these tumors and recent advances for diagnosis have also been highlighted.

  15. Recurrent Renal Cell Carcinoma with Synchronous Tumor Growth in Azygoesophageal Recess and Duodenum: A Rare Cause of Anemia and Upper Gastrointestinal Bleeding

    Directory of Open Access Journals (Sweden)

    Vamshidhar R. Vootla

    2015-01-01

    Full Text Available Renal cell carcinoma (RCC has potential to present with distant metastasis several years after complete resection. The common sites of metastases include the lungs, bones, liver, renal fossa, and brain. RCCs metastasize rarely to the duodenum, and duodenal metastasis presenting with acute gastrointestinal bleed is infrequently reported in literature. We present a case of synchronous presentation of duodenal and azygoesophageal metastasis manifesting as acute upper gastrointestinal bleeding, four years after undergoing nephrectomy for RCC. The patient underwent further workup and was treated with radiation. The synchronous presentation is rare and stresses the importance of searching for recurrence of RCC in patients presenting with acute gastrointestinal bleeding.

  16. [Solitary fibrous tumor of endometrium--a case report].

    Science.gov (United States)

    Dvořák, O; Dvořáková, E; Laco, J; Spaček, J

    2013-06-01

    Solitary fibrous tumor (SFT) is an uncommon mesenchymal tumor. We present a case of SFT occurring in endometrium. Case report. Department of Obstetrics and Gynecology, The Fingerland Department of Pathology, Medical Faculty of Charles University and Faculty Hospital Hradec Králové. We report a case of 57 years old woman with SFT arising from the endometrium, which was diagnosed and treated at our department. Histological finding was supported by typical immunohistochemical profile of the tumor. Aggressive nature of the tumor wasnt showed. The patient underwent abdominal hysterectomy with bilateral adnexectomy and is followed up in regular periods. Occurence of solitary fibrous tumor (SFT) in the female genital tract is extremely rare. To the best of our knowledge, we report the first case of SFT occurring in endometrium. Because of potencial aggressive behaviour of the tumor complete surgical excision and close follow-up is highly recommended.

  17. 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.

  18. Primitive Neuroectodermal Tumor of the Stomach: A Case Report.

    Science.gov (United States)

    Song, Min Jeong; An, Soyeon; Lee, Seung Soo; Kim, Beom Su; Kim, Jihun

    2016-09-01

    Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) is a highly aggressive small round cell tumor that mainly occurs in the bone or soft tissue of children or young adults but is extremely rare in the stomach. A 55-year-old man presented with melena and anemia. On endoscopy, an ulcerofungating mass was observed in the high body and total gastrectomy was performed. Histologically, the mass consisted of small round cells with scanty cytoplasm and inconspicuous nucleoli. They often formed perivascular pseudorosettes and multinucleated giant cells were frequently observed. The tumor cells strongly expressed CD99, FLI1, and chromogranin and weakly expressed synaptophysin and CD56. EWS-FLI1 fusion transcript was confirmed by reverse transcription-polymerase chain reaction. ES/PNET is frequently misdiagnosed because of its similarity with small cell carcinoma. Although gastric ES/PNET is very rare, it should be included in differential diagnoses of small round cell tumor in the stomach.

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

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    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.

  20. Tumor carcinoide apendicular Appendiceal carcinoid tumor

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    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.

  1. A New Type of Uterine Trophoblastic Tumor: Epithelioid Trophoblastic Tumor

    Institute of Scientific and Technical Information of China (English)

    Langdi Fan; Zhanhong Wang; Xiurong Wang; Yingge Xing

    2005-01-01

    OBJECTIVE To describe a case of a patient with an epithelioid trophoblastic tumor (ETT) and review the literature regarding this new type of uterine trophoblastic tumor which is being reported with increasing frequency.There have been only 42 cases described in the world literature.METHODS Routine sections of the tumor were prepared and stained with H&E. Using the SP method, immunohistochemical staining, for AE1/AE3,hPL, PLAP, and α-inhibin antigens was conducted.RESULTS The patient was a 34 years old female who had delivered 12 years previously. She presented with amenorrhea for three months and vaginal irregular bleeding for 2 months. Her serum hCG level was 2,240 IU/L. After diagnostic curettage, an ETT was identified, and total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAHBSO) performed.On microscopic examination it was found that the tumor was composed of chorionic-type intermediate- trophoblastic cells. The tumor cell nests were distributed in a geographical pattern. Some cells were filled with an eosinophilic hyalinized degenerative material. Study of the immunophenotype of the tumor showed that AE1/AE3, hPL, hCG, and α-inhibin were positively expressed.CONCLUSION This is the 4th case report of an ETT in China. The tumor was identified as a new type of trophoblastic tumor combined with a focal chorioepithelioid carcinoma, a condition that is extremely rare. It consists of chorionic-type intermediate-trophoblastic cells, and is considered to have a Iowgrade of malignancy. ETT should be differentiated from a placenta-site trophoblastic tumor, placenta-site nodule, chorioepithelioid carcinoma, and cervical squamous cell carcinoma.

  2. Fifty tumor necrosis factor-based isolated limb perfusions for limb salvage in patients older than 75 years with limb-threatening soft tissue sarcomas and other extremity tumors

    NARCIS (Netherlands)

    B. van Etten (Boudewijn); A.N. van Geel (Albert); J.H.W. de Wilt (Johannes); A.M.M. Eggermont (Alexander)

    2003-01-01

    textabstractBACKGROUND: Isolated limb perfusion (ILP) with tumor necrosis factor (TNF) and melphalan is highly effective in treating limb-threatening soft tissue sarcoma (STS) and other bulky tumors. Because of fear of TNF-associated toxicity, ILP with TNF is not offered to older p

  3. Primary pulmonary synovial sarcoma: a rare case report

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    Sumalatha Kasturi

    2014-08-01

    Full Text Available Primary lung sarcoma is an extremely rare tumor, accounting for less than 0.5% of all lung tumors. Histological subtypes are differentiated on the basis of immunohistochemical markers, such as vimentin, desmin, actin, CD99, and epithelial membrane antigen. A 50-year-old male presented with progressively increasing shortness of breath with cough for 2 months. On Contrast Enhanced Computed Tomography (CECT of thorax a large heterogeneous mass with multiple areas of necrosis, occupying almost whole of left hemithorax was seen. CT-guided Fine Needle Aspiration Cytology (FNAC revealed spindle cell neoplasm. Histopathological examination revealed a spindle cell sarcoma. On immunohistochemistry the tumor cells expressed both epithelial membrane antigen and vimentin. Hence, final impression from immunohistochemistry was primary monophasic synovial sarcoma of lung. [Int J Res Med Sci 2014; 2(4.000: 1729-1731

  4. Primitive Neuroectodermal Tumors of Hand and Foot: Report of Two Cases

    OpenAIRE

    DEMİR, Pelin; Arikan, Murat; ÇİLEDAĞ, Nazan; AKTAŞ, ELIF; Güngör, Şafak; Arda, Kemal

    2009-01-01

    Primitive neuroectodermal tumor (PNET) of hand and foot is extremely rare. To our knowiedge, limited number of cases of typicat PNET has been reported, involving the hand orfoot. İn this report, we presented magnetic resonance imaging findings of a PNET case localized in the proximal phalanx of left foot and a case localized in distal phalanx of the right hand.

  5. Desmoplastic small round cell tumor of the lung:case report

    Institute of Scientific and Technical Information of China (English)

    WANG Zhao-ming; XIAO Wen-bo; ZHENG Shu-sen

    2007-01-01

    @@ Desmoplastic small round cell tumor(DSRCT)is a clinically and morphologically well-defined neoplasm.1 This highly aggressive malignant small cell neoplasm tends to affect adolescents and young adults and occurs predominantly in the abdomen,pelvis,and omentum.1,2 DSRCT in the lung is extremely rare.

  6. Primitive neuroectodermal tumor of the kidney in a young male: Case report and review of literature.

    Science.gov (United States)

    Patnaik, Nivedita; Mishra, Kiran; Saini, Pradeep; Agarwal, Nitin

    2015-01-01

    Primitive neuroectodermal tumor of the kidney is a rare tumor. A total of approximately 79 primary renal cases have been reported to date. Primitive neuroectodermal tumors occur preferentially in the soft-tissues of the paravertebral region and chest wall, less frequently in extremities, with a slight male predominance. We report a case of primitive neuroectodermal tumor of the kidney in a 17-year-old male with a pre-operative diagnosis of renal cell carcinoma-stage 4. The patient underwent radical nephrectomy and histopathological examination revealed a highly aggressive tumor of monotonous sheets of round cells with focal areas of rosette formations and high mitotic rate with Ki67 index of 25-30%. Tumor cells were positive for CD 99 confirming the diagnosis of primitive neuroectodermal tumor. Primitive neuroectodermal tumor of the kidney needs to be kept in mind as a differential diagnosis in young adults presenting with a large kidney mass.

  7. 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.

  8. Peripheral primitive neuroectodermal tumor of the posterior mediastinum:A case report

    Institute of Scientific and Technical Information of China (English)

    Yu Liu; Weigang Zhao; Yusheng Shu

    2014-01-01

    Peripheral primitive neuroectodermal tumor (pPNET) is an extremely rare disease entity of malignant tumors belonging to the Ewing sarcoma family that usualy occurs in children and adolescents. We describe a 41-year-old female who presented with right upper abdominal pain. Surgical resection and biopsy revealed smal round-celltumor. Combined with immunohistochemical analysis, pPNET was diagnosed. No evidence of recurrence was noted at 18 months postoperatively. Even thought pPNET is a highly malignant tumor, Wide tumor-free resection and multi-agent chemotherapy can also obtain good clinical outcomes.

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

    Directory of Open Access Journals (Sweden)

    Tatyana PIRDOPSKA

    2011-09-01

    Full Text Available 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.

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

    OpenAIRE

    Manjula Jain; Aparna Harbhajanka; S Roy Choudhary

    2011-01-01

    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 eosin...

  11. Dentinogenic ghost cell tumor: A variant of Gorlin′s cyst

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    Patil Karthikeya

    2008-01-01

    Full Text Available Calcifying odontogenic cyst (COC was described as a distinct entity for the first time by Gorlin and his associates in 1962. Dentinogenic ghost cell tumor (DGCT was described by Praetorius et al. in 1981 as a neoplastic variety of COC. DGCT is an extremely rare odontogenic tumor and accounts for only 2% to 14% of all COCs. A case of DGCT in a 40-year-old male patient is being reported.

  12. 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.

  13. Symplastic glomus tumor of the urinary bladder treated by robot-assisted partial cystectomy: a case report and literature review.

    Science.gov (United States)

    Palmisano, Franco; Gadda, Franco; Spinelli, Matteo G; Maggioni, Marco; Rocco, Bernardo; Montanari, Emanuele

    2017-01-16

    Glomus tumors arising in the urinary bladder are extremely rare, and only two cases have been reported in the English Literature. We present a case of a 58-year-old man with an asymptomatic mass of the anterior wall of the bladder that measured 2.5 × 2.5 cm. Endoscopic excision was performed, and the tumor was diagnosed as symplastic glomus tumor. The patient finally underwent robotic-assisted partial cystectomy, and he remains healthy without any recurrence to date.After reviewing this case and previous reports, we analyzed the clinicopathologic features and treatment options for this rare neoplasm.

  14. Nonfunctional Islet Cell Tumor of the Pancreas in a Patient with Tuberous Sclerosis: A Case Report with Literature Review

    Directory of Open Access Journals (Sweden)

    Aysegul Cansu

    2014-01-01

    Full Text Available Islet cell tumors (ICTs are rare tumors of the pancreas. Association of this type of tumor with tuberous sclerosis is extremely rare. Only 13 cases of pancreatic ICT with tuberous sclerosis have so far been documented in the literature. However, awareness of the association of tuberous sclerosis and ICT is important for early diagnosis and appropriate treatment of this condition. This article presents the case of a 63-year-old female with angiomyolipoma (AML of the kidney and liver, calcified subependymal nodules and a large mass in the pancreas, which was proven to be an ICT on histopathological examination.

  15. A Rare Case of an Osteoid Osteoma of the Rib Treated under Computed Tomography Guidance: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Sakiko Mizuno

    2015-11-01

    Full Text Available Osteoid osteoma (OO usually occurs in the extremities of young adults. The tumor can arise in any part of the skeletal tissue; however, it is rarely found in the rib, with limited reports to date. In this report, we present a rare case of OO arising in the rib, which was successfully treated under computed tomography guidance with minimal invasiveness. At the final follow-up after 4 years, no local recurrence was observed.

  16. The clinical features of rare thymic primitive neuroectodermal tumor%罕见胸腺原始神经外胚层肿瘤的临床特点

    Institute of Scientific and Technical Information of China (English)

    杜亚奇; 刘东屏; 孙明军; 赵梅芬

    2016-01-01

    Objective To explore primitive neuroectodermal tumor (PNET) clinical characteristics and method of diagnosis and treatment. Methods The clinical data of a case of thymic primitive neural ectodermal tumor was retrospectively analyzed. Results By ultrasound guided puncture biopsy, immunohistochemistry was performed, and histological evidence was obtained, for the diagnosis of thymic primitive neuroectodermal tumors (stage IV). Conclusion This disease is highly malignant tumor. It is prone to distant metastasis and the prognosis is poor. The clinical and pathological doctors know little about this disease and could be easily misdiagnosed.%目的:探讨原始神经外胚层肿瘤(PNET)的临床特点和诊疗方法。方法回顾性分析中国医科大学附属第一医院收治的1例胸腺原始神经外胚层肿瘤的临床资料,分析其临床、病理及治疗的特点。结果经超声引导下穿刺取病理,行免疫组化,获得组织学证据,诊断为胸腺原始神经外胚层肿瘤(IV期)。结论 PNET属于高度恶性肿瘤,易发生远处转移,且预后差,因发病罕见,临床及病理医师对其认识不足,易造成误诊或漏诊。

  17. Highly efficient and selective isolation of rare tumor cells using a microfluidic chip with wavy-herringbone micro-patterned surfaces.

    Science.gov (United States)

    Wang, Shunqiang; Thomas, Antony; Lee, Elaine; Yang, Shu; Cheng, Xuanhong; Liu, Yaling

    2016-04-07

    Circulating tumor cells (CTCs) in peripheral blood have been recognized as a general biomarker for diagnosing cancer and providing guidance for personalized treatments. Yet due to their rarity, the challenge for their clinical utility lies in the efficient isolation while avoiding the capture of other non-targeted white blood cells (WBCs). In this paper, a wavy-herringbone (HB) microfluidic chip coated with antibody directly against epithelial cell adhesion molecule (anti-EpCAM) was developed for highly efficient and selective isolation of tumor cells from tumor cell-spiked whole blood samples. By extending the concept of the hallmark HB-Chip in the literature, the wavy-HB chip not only achieves high capture efficiency (up to 85.0%) by micro-vortexes induced by HB structures, but also achieves high purity (up to 39.4%) due to the smooth wavy microstructures. These smooth wavy-HB structures eliminate the ultra-low shear rate regions in the traditional grooved-HB structures that lead to non-specific trapping of cells. Compared with the grooved-HB chip with sharp corners, the wavy-HB chip shows significantly higher purity while maintaining similarly high capture efficiency. Furthermore, the wavy-HB chip has up to 11% higher captured cell viability over the grooved-HB chip. The distributions of tumor cells and WBCs along the grooves and waves are investigated to help understand the mechanisms behind the better performance of the wavy-HB chip. The wavy-HB chip may serve as a promising platform for CTC capture and cancer diagnosis.

  18. 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 ext

  19. 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

  20. A rare case of TFE-related pigmented renal tumor with overlapping features between melanotic Xp11 translocation renal cancer and Xp11 renal cell carcinoma with melanotic features.

    Science.gov (United States)

    Cardili, Leonardo; Wrublevsky Pereira, Gregório; Viana, Cristiano Ribeiro

    2017-02-16

    In recent years, an increasing number of TFE3 rearrangement-associated tumors with melanotic features have been reported as primary neoplasm in different anatomical sites, including the kidney. Melanotic Xp11 translocation renal cancer (MXTRC) and Xp11 renal cell carcinoma with melanotic features (XRCCM) have been proposed to be main categories for pigmented lesions in the microophthalmia-associated transcription factor (MiTF/TFE3) family of renal tumors that may show variable degrees of melanocytic differentiation. Herein we report a rare case of TFE3-related pigmented renal tumor showing unusual immunoexpression of cytokeratins (AE1/AE3) and renal cell carcinoma markers (RCC, CD10). Cathepsin-K and Vimentin were diffusely positive whereas melanocytic markers (HMB-45 and Melan-A) displayed weak and patchy expression. We found no labelling for PAX-8, muscle markers (desmin, smooth muscle actin, muscle-specific actin and caldesmon) and S-100. TFE3 fusion was confirmed by break-apart fluorescence in situ hybridization (FISH). This case corroborates previous evidence for overlap in the TFE3-associated cancer family and illustrates that it may not be possible to set a clear cutoff between epithelial (XRCCM) and mesenchymal (MXTRC) subgroups.

  1. A primary spinal extradural atypical teratoid/rhabdoid tumor of the cervical spine with bony involvement.

    Science.gov (United States)

    Xin, Xiaoyan; Zhu, Bin; Shen, Jingtao; Tian, Chuanshuai; Fan, Xiangshan; Liu, Bao-rui

    2014-05-01

    Primary spinal atypical teratoid/rhabdoid tumors are extremely rare and most commonly occur as intramedural or extramedural intradural. The location of extradural type is rarely reported. A 10-year-old girl presented with a 2-month history of nape pain. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed an extradural tumor from C2-C5 with bony destruction of the C3 vertebral body. Subtotal removal of the tumor was performed and atypical teratoid/rhabdoid tumor was proven histologically. But 2 months after surgery, the neck pain became worse. There were metastasic lesions in bilateral lung fields and multiple enlarged lymph nodes around the carotid sheath. She died 8 months after the initial symptoms. The present case is the third detailed report of spinal extradural. We describe the CT and MRI findings of this case and review the literature describing this rare disease.

  2. Endodermal cyst in pineal region: Rare location

    Science.gov (United States)

    Lopez-Gonzalez, Miguel Angel; Dolan, Eugen

    2016-01-01

    Background: Pineal tumors are very uncommon intracranial lesions, and endodermal cysts in this location are extremely rare. Case Description: A 49-year-old right-handed female presented with 3 weeks history of progressive dizziness and imbalance. Imaging studies showed 1.8 cm × 1.7 cm × 1.8 cm pineal lesion with small enhancing mural component displacing ventrally the quadrigeminal plate and narrowing of aqueduct of Sylvius without hydrocephalus. In addition, she was found with small interhemispheric lipoma, and small posterior falx possible meningioma. Cerebrospinal fluid markers obtained by lumbar puncture were all negative. She underwent tumor resection, and final pathology reported endodermal cyst. No new deficits were encountered, and her gait imbalance improved significantly by 3 months follow-up. Conclusions: With evidence of enlargement or symptomatic pineal lesions, surgical consideration is necessary. Among pineal lesions, endodermal cysts are extremely uncommon and although benign pathology, long-term follow-up is advised due to unknown chronic behavior. PMID:27217965

  3. Primary renal carcinoid tumor: A radiologic review

    Directory of Open Access Journals (Sweden)

    Leslie Lamb, MD, Msc, Bsc

    2014-01-01

    Full Text Available Carcinoid tumor is the classic famous anonym of neuroendocrine neoplasms. Primary renal carcinoid tumors are extremely rare, first described by Resnick and colleagues in 1966, with fewer than a total of 100 cases reported in the literature. Thus, given the paucity of cases, the clinical and histological behavior is not well understood, impairing the ability to predict prognosis. Computed tomography and (occasionally octreotide studies are used in the diagnosis and followup of these rare entites. A review of 85 cases in the literature shows that no distinctive imaging features differentiate them from other primary renal masses. The lesions tend to demonstrate a hypodense appearance and do not usually enhance in the arterial phases, but can occasionally calcify. Octreotide scans do not seem to help in the diagnosis; however, they are more commonly used in the postoperative followup. In addition, we report a new case of primary renal carcinoid in a horseshoe kidney.

  4. Rare Power

    Institute of Scientific and Technical Information of China (English)

    2011-01-01

    China begins to reinvigorate the rare earth industry after decades of underselling the valuable metals On May 19,the State Council released Guidelines to Promote Sustainable and Sound Development of the Rare Earth Industry,delivering a strong boost to the fragmented industry.

  5. Rare Security

    Institute of Scientific and Technical Information of China (English)

    2011-01-01

    China’s regulation on rare earth accords with WTO rules Worries abound Western countries may use a recent WTO ruling on China’s exports restriction on nine raw materials to launch actions against China’s curbs on rare earth exports.

  6. 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.

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

    Science.gov (United States)

    Johnson, Eric D.; Gulbahce, Evin; McNally, Joseph; Buys, Saundra S.

    2016-01-01

    Introduction Phyllodes tumors (PTs) are rare fibroepithelial tumors of the breast which are classified as benign, borderline, or malignant. Malignant PTs account for 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. PMID:28203179

  8. Benign fibrous histiocytoma: A rare case involving jaw bone

    Directory of Open Access Journals (Sweden)

    Hitesh Shoor

    2015-01-01

    Full Text Available Benign fibrous histiocytoma (BFH is a soft tissue neoplasm which occurs mostly on the skin of extremities. BFH rarely occurs in bone and may affect femur, tibia, and pelvic bone. Jaw bone involvement is very unusual with only 11 cases reported till date. This report describes a case of BFH occurring in a 30-year-old female patient affecting left mandibular posterior region. Computed tomography revealed a well-defined expansile lytic lesion in the posterior mandible. Gross examination of the tumor revealed an admixture of fibroblasts and histiocytes in a fascicular and storiform pattern. Immunohistochemical staining was positive for CD68.

  9. Benign fibrous histiocytoma: A rare case involving jaw bone.

    Science.gov (United States)

    Shoor, Hitesh; Pai, Keerthilatha M; Shergill, Ankur Kaur; Kamath, Abhay Taranath

    2015-09-01

    Benign fibrous histiocytoma (BFH) is a soft tissue neoplasm which occurs mostly on the skin of extremities. BFH rarely occurs in bone and may affect femur, tibia, and pelvic bone. Jaw bone involvement is very unusual with only 11 cases reported till date. This report describes a case of BFH occurring in a 30-year-old female patient affecting left mandibular posterior region. Computed tomography revealed a well-defined expansile lytic lesion in the posterior mandible. Gross examination of the tumor revealed an admixture of fibroblasts and histiocytes in a fascicular and storiform pattern. Immunohistochemical staining was positive for CD68.

  10. Infratentorial angioleiomyoma: a new location for a rare neoplastic entity.

    Science.gov (United States)

    Gasco, Jaime; Franklin, Brodus; Rangel-Castilla, Leonardo; Campbell, Gerald A; Eltorky, Mahmoud; Salinas, Paul

    2009-04-01

    Angioleiomyomas are benign neoplasms most often located in the subcutaneous tissue of middle-aged individuals and usually confined to the subcuticular and deep dermal layers of the lower extremities. An intracranial site for this tumor is exceedingly rare, with very few reports documenting locations in the neuraxis. To the authors' knowledge the present case represents the first reported instance of an infratentorial angioleiomyoma. The authors conducted a review of selected English-language papers published since 1960 describing well-documented cases of intracranial vascular leiomyomas, with detailed information on the clinical presentation, radiology, pathology, and particulars of surgical management in each case.

  11. A rare case of white pearls in brainstem

    Directory of Open Access Journals (Sweden)

    Dipanker Singh Mankotia

    2016-01-01

    Full Text Available Intrinsic brainstem epidermoid is extremely rare, and only 14 cases have been reported. Authors report a classic case of brainstem epidermoid in a 14-year-old male child presenting with symptoms of brainstem involvement. The child underwent a successful surgical excision. The lesion was intrinsic and caused diagnostic dilemma based on conventional radiological images. Based on our experience in this case and a thorough review of literature, we are of the opinion that diffusion-weighted images are very important in establishing the diagnosis. Such lesions are challenging and attempt to remove adherent tumor capsule may produce additional neurological deficits.

  12. [Management of complications in anal and transanal tumor surgery].

    Science.gov (United States)

    Sailer, M; Eisoldt, S; Möllmann, C

    2015-08-01

    Anal and transanal tumor operations are safe and are associated with a very low morbidity. Perianal and anal lesions as well as low rectal tumors can be excised by direct exposure using an anal retractor. For lesions situated in the middle or upper third of the rectum, special instrumentation, such as transanal endoscopic microsurgery (TEM) and transanal endoscopic operation (TEO) should be used to avoid unnecessary R1 resections. Fatal complications are extremely rare and most complications, such as urinary retention or temporary subfebrile temperatures, are minor. Suture line dehiscences are usually clinically unremarkable. Major complications comprise significant hemorrhage and opening of the peritoneal cavity. The latter must be recognized intraoperatively and can usually be managed by primary suturing. Infections, abscess formation, rectovaginal fistula, injury of the prostate or even urethra are extremely rare complications.

  13. Epithelioid hemangioendothelioma of the proximal phalanx of the left second toe – a rare occurence

    Directory of Open Access Journals (Sweden)

    Bharath Raju G

    2013-07-01

    Full Text Available Introduction: Epithelioid hemangioendothelioma (EHE of the bone is extremely rare and occurs predominantly in males. It most frequently occurs during the second and third decades of life. The lower extremities are most commonly involved. We describe a diagnostically challenging case of epithelioid hemangioendothelioma of proximal phalanx of 2nd toe of left foot with histological features reminiscent of osteoblastomatosis. Case Report: A 52 year old man presented with history of intermittent pain with swelling in second toe since 6 months. Radiograph showed a lytic lesion in proximal phalanx of the great toe. CT and MRI reported non specific lesion in the toe. Pain was quite severe and as the patient was a labourer and wanted to get back to his work as soon as possible a decision of disarticulation of the second toe at metatarsophalnageal joint was taken. Histopathology confirmed the diagnosis of Epithelioid hemangioendothelioma and patient was called for regular follow up. There are no complications and recurrence at two year follow up. Conclusion: EHE of the bone is extremely rare vascular tumor. To our knowledge, this is the first case of EHE with such features. EHE should be kept as one of the important differential diagnosis while diagnosing vascular tumors. Careful attention to the histopathological features is necessary for the confirmation of the diagnosis. Keywords: Epitheloid haemangioma; epitheloid haemangioendothelioma; rare occurrence; vascular tumor.

  14. Isolated limb perfusion with tumor necrosis factor and melphalan for limb salvage in 186 patients with locally advanced soft tissue extremity sarcomas. The cumulative multicenter European experience

    NARCIS (Netherlands)

    A.M.M. Eggermont (Alexander); P.M. Schlag; D. Lienard; A.N. van Geel (Albert); F.J. Lejeune; H.J. Hoekstra; I. Meller; J.C. Pector; O.E. Nieweg (Omgo); G. Ben-Ari; C. Kettelhack; H. Schraffordt Koops; J.M. Klausner; B.B. Kroon

    1996-01-01

    textabstractOBJECTIVE: The objective of the study was to achieve limb salvage in patients with locally advanced soft tissue sarcomas that can only be treated by amputation or functionally mutilating surgery by performing an isolated limb perfusion (ILP) with tumor necro

  15. Functional Ability and Physical Activity in Children and Young Adults After Limb-Salvage or Ablative Surgery for Lower Extremity Bone Tumors

    NARCIS (Netherlands)

    Bekkering, W. Peter; Vlieland, Theodora P. M. Vliet; Koopman, Hendrik M.; Schaap, Gerard R.; Schreuder, H. W. Bart; Beishuizen, Auke; Jutte, Paul C.; Hoogerbrugge, Peter M.; Anninga, Jacob K.; Nelissen, Rob G. H. H.; Taminiau, Antonie H. M.

    2011-01-01

    Background: Aim of our study was to compare functional ability and physical activity in children and young adults who underwent surgery for a malignant bone tumor that was located around the knee. Methods: This cross-sectional study included 82 patients aged 8-25 years with a follow-up of 1-5 years.

  16. Functional ability and physical activity in children and young adults after limb-salvage or ablative surgery for lower extremity bone tumors

    NARCIS (Netherlands)

    Bekkering, W.P.; Vliet Vlieland, T.P.M.; Koopman, H.M.; Schaap, G.R.; Schreuder, H.W.B.; Beishuizen, A.; Jutte, P.C.; Hoogerbrugge, P.M.; Anninga, J.K.; Nelissen, R.G.; Taminiau, A.H.M.

    2011-01-01

    BACKGROUND: Aim of our study was to compare functional ability and physical activity in children and young adults who underwent surgery for a malignant bone tumor that was located around the knee. METHODS: This cross-sectional study included 82 patients aged 8-25 years with a follow-up of 1-5 years.

  17. Spinal schwannomatosis in the absence of neurofibromatosis: A very rare condition

    Science.gov (United States)

    Landi, A.; Dugoni, D.E.; Marotta, N.; Mancarella, C.; Delfini, R.

    2010-01-01

    Schwannomatosis is defined as an extremely rare tumors syndrome characterized by the presence of multiple schwannomas in the absence of typical signs of NF1 and NF2 syndromes. The genetic and molecular analysis performed on these tumors makes it possible to name schwannomatosis as distinct clinical and genetic syndrome. The treatment in the case of symptomatic lesions is surgical removal; if the lesions are asymptomatic it is better to perform serial MRI studies. Given the high incidence of developing additional lesions in patients with schwannomatosis, it remains imperative to perform serial brain and spinal cord MRI studies during follow-up. The differential diagnosis is important including clinical and radiological criteria plus molecular genetic analysis of tumor cells and lymphocyte DNA. We report a rare case of spinal schwannomatosis in which genetic analysis performed on surgical samples showed two different mutations in the cells of the two lesions. PMID:22096683

  18. Endometrial stromal sarcoma together with leiomyosarcoma in a single patient: a rare case report

    Directory of Open Access Journals (Sweden)

    Reema Jain

    2016-04-01

    Full Text Available Uterine sarcomas are relatively rare tumors of mesodermal origin. The three most common histological variants of uterine sarcoma are Leiomyosarcomas, Endometrial stromal sarcomas, and Carcinosarcomas. It is extremely uncommon to find the 2 variants in a single patient. Given the rarity of these tumors there are limited reports in the literature referring to the clinical management and final outcome of these cases. Our patient was a 60 year old post-menopausal women presented in the opd with post-menopausal bleeding. Ultrasound report showed a fibroid of around 8 by 8 cm fundal fibroid. Endometrial biopsy report showed low grade endometrial sarcoma while the CECT report showed leiomyosarcoma creating a dilemma in the diagnosis. Patient was then taken up for surgical exploration. The biopsy report confirmed the existence of both the types of tumor, a very rare finding. [Int J Reprod Contracept Obstet Gynecol 2016; 5(4.000: 1239-1241

  19. Extreme Heat

    Science.gov (United States)

    ... Landslides & Debris Flow Nuclear Blast Nuclear Power Plants Power Outages Pandemic Radiological Dispersion Device Severe Weather Snowstorms & Extreme ... Landslides & Debris Flow Nuclear Blast Nuclear Power Plants Power Outages Pandemic Radiological Dispersion Device Severe Weather Snowstorms & Extreme ...

  20. Rhabdoid Tumor Mimicking Hemangioma

    NARCIS (Netherlands)

    Assen, Yvette J.; Madern, Gerard C.; de Laat, Peter C. J.; den Hollander, Jan C.; Oranje, Arnold P.

    2011-01-01

    We report a young boy with a malignant tumor, which remained unrecognized for 8 months because it was assumed to be a hemangioma. The presentation of a rhabdoid tumor mimicking hemangioma is very rare. It was reported only on two earlier occasions. Rhabdoid tumors are one of the most aggressive type

  1. Rhabdoid Tumor Mimicking Hemangioma

    NARCIS (Netherlands)

    Assen, Yvette J.; Madern, Gerard C.; de Laat, Peter C. J.; den Hollander, Jan C.; Oranje, Arnold P.

    2011-01-01

    We report a young boy with a malignant tumor, which remained unrecognized for 8 months because it was assumed to be a hemangioma. The presentation of a rhabdoid tumor mimicking hemangioma is very rare. It was reported only on two earlier occasions. Rhabdoid tumors are one of the most aggressive

  2. Intrasellar malignant peripheral nerve sheath tumor (MPNST).

    Science.gov (United States)

    Krayenbühl, N; Heppner, F; Yonekawa, Y; Bernays, R L

    2007-02-01

    Intracranial malignant peripheral nerve sheath tumors (MPNST) and intrasellar schwannomas are rare tumors. We describe a case of an intrasellar schwannoma with progression to a MPNST, a finding that, although very rare, extends the differential diagnosis of intrasellar lesions.

  3. Intravenous Glomus Tumor Masquerading as Lateral Antebrachial Cutaneous Neuroma.

    Science.gov (United States)

    Chim, Harvey; Al-Qattan, Husain; Valencia, Herbert; Brathwaite, Carole; Price, Andrew; Grossman, John A I

    2017-03-01

    Background: Intravenous glomus tumors are extremely rare. Methods: We report a patient with an intravenous glomus tumor within a venous aneurysm misdiagnosed as a neuroma of the lateral antebrachial cutaneous nerve, based on clinical exam, electrodiagnostic studies, and findings on a magnetic resonance imaging neurogram. Results: After surgical resection, the patient's symptoms, including pain and localized hypersensitivity, totally resolved. Conclusions: This case illustrates 2 important points. First, unlike extradigital glomus tumors, magnetic resonance imaging is not reliable in diagnosing intravenous glomus tumors. Second, in the presence of chronic localized neuroma type pain and sensitivity in the upper limb without a clear cause, an extradigital cutaneous or intravenous glomus tumor must be considered in the differential diagnosis.

  4. 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.

  5. 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.

  6. Primary de novo malignant giant cell tumor of kidney: a case report

    Directory of Open Access Journals (Sweden)

    Torkian Bahman

    2004-06-01

    Full Text Available Abstract Background Osteoclast-like giant cell tumors are usually observed in osseous tissue or as tumors of tendon sheath, characterized by the presence of multinucleated giant cells and mononuclear stromal cells. It has been reported in various extraosseous sites including breast, skin, soft tissue, salivary glands, lung, pancreas, female genital tract, thyroid, larynx and heart. However, extraosseus occurrence of such giant cell tumors in the kidney is extremely rare and is usually found in combination with a conventional malignancy. De-novo primary malignant giant cell tumors of the kidney are unusual lesions and to our knowledge this is the second such case. Case Presentation We report a rare case of extraosseous primary denovo malignant giant cell tumor of the renal parenchyma in a 39-year-old Caucasian female to determine the histogenesis of this neoplasm with a detailed literature review. Conclusion Primary denovo malignant giant cell tumor of the kidney is extremely rare. The cellular origin of this tumor is favored to be a pluripotential mesenchymal stromal cell of the mononuclear/phagocytic cellular lineage. Awareness of this neoplasm is important in the pathological interpretation of unusual findings at either fine needle aspiration or frozen section of solid renal masses.

  7. Conservative Resectoscopic Surgery, Successful Delivery, and 60 Months of Follow-Up in a Patient with Endometrial Stromal Tumor with Sex-Cord-Like Differentiation

    Directory of Open Access Journals (Sweden)

    Pasquale De Franciscis

    2016-01-01

    Full Text Available Uterine tumors with sex-cord-like differentiation are extremely rare types of uterine stromal neoplasm. These tumors were classified into two groups with considerable practical relevance because clinical behaviour of uterine tumor resembling ovarian sex cord tumor (UTROSCT differs widely from its closely related endometrial stromal tumors with sex-cord-like elements (ESTSCLE. Treatment and prognosis of these tumors are unresolved issues because of the exiguous number of reported cases. We describe a rare case of endometrial stromal tumor with sex-cord-like differentiation successfully treated by resectoscopic surgery and conservation of the uterus, in an infertile patient affected by metrorrhagia. This procedure resulted in a pregnancy immediately after treatment and in a successful delivery. During 60 months of follow-up no evidence of recurrence was observed.

  8. 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.

  9. Study of malignant peripheral nerve sheath tumor in cerebellopontine angle.

    Science.gov (United States)

    Hong, WenMing; Cheng, HongWei; Wang, XiaoJie; Hu, XiaoPeng; Feng, ChunGuo

    2014-01-01

    Malignant peripheral nerve sheath tumors (MPNSTs) are very rare soft tissue sarcomas, usually arising from somatic soft tissues or peripheral nerves. Primary MPNST of the cerebellopontine angle is extremely rare, with only a single case reported so far. Here, we report an unusual case of MPNST in cerebellopontine angle in a 25-year-old man presented with dizziness, left facial numbness, and tinnitus. After hospitalization, the tumor was treated with complete surgical excision followed by adjuvant chemotherapy and radiotherapy. Histologically, the tumor showed malignant spindle cells, which were with focal S-100 positivity on immunohistochemistry, and a diagnosis of the MPNST was made. This case is being reported for its rarity and presence in cerebellopontine and illustrated the difficulties in the diagnosis and treatment of MPNST, which to the best of our knowledge, has not been described before in the soft tissue sarcomas.

  10. Growth hormone-releasing hormone (GRH)-producing pancreatic tumor with no evidence of multiple endocrine neoplasia type 1.

    Science.gov (United States)

    Kawa, S; Ueno, T; Iijima, A; Midorikawa, T; Fujimori, Y; Tokoo, M; Oguchi, H; Kiyosawa, K; Imai, Y; Kaneko, G; Kuroda, T; Hashizume, K; Osamura, R Y; Katakami, H

    1997-07-01

    The characteristic features of a 48-year-old male presenting with isolated acromegaly caused by a GRH-producing pancreatic endocrine tumor bearing no relation to MEN1 was reported. The clinical features, laboratory findings, and sellar enlargement were improved after removal of the pancreatic tumor. The resected pancreatic tumor showed positive GRH immunoreactivity and contained abundant GRH mRNA. This tumor is extremely rare and to date only 10 cases have been reported. In the management of acromegaly, the measurement of GRH is recommended and the search for an ectopic source will prevent unnecessary and potentially ineffective pituitary surgery.

  11. Infantile pericardial round cell tumor

    Directory of Open Access Journals (Sweden)

    K H Sridevi

    2015-01-01

    Full Text Available Cardiac malignancies presenting in infancy are rare. Desmoplastic small round cell tumor (DSRCT is a rare occurrence in this age group. No case of intrapericardial DSRCT has been reported in the literature in infants.

  12. Aggressive malignant phyllodes tumor

    OpenAIRE

    Nathan Roberts; Dianne M. Runk

    2015-01-01

    Introduction: Originally described in 1838 by Muller, phyllodes tumor is a rare fibroepithelial neoplasm which represents roughly 0.3–0.9% of all breast cancers. Phyllodes tumor are divided into benign, borderline and malignant histologic categories. Malignant phyllodes tumor represent anywhere from 10–30% of all phyllodes tumors. This group has both the potential to recur locally and metastasize, however not all malignant phyllodes behave this way. The challenge lays in predicting which tumo...

  13. Solitary Fibrous Tumor in Bladder:A Case Report

    Institute of Scientific and Technical Information of China (English)

    王涛; 陈瑞宝; 乔建坤; 胡涛; 刘继红; 杨为民; 叶章群

    2010-01-01

    Solitary fibrous tumor(SFT) in bladder is extremely rare.In this study,we reported one case of bladder SFT and reviewed the only ten cases of the disease that had been reported so far.The patient suffered from residual urine sensation and urethral pain.Cystoscopy revealed a 7-cm protruding mass at the dome of the bladder,and bladder mucosa biopsy showed normal differentiation of the bladder mucosa with a small amount of inflammatory cells.Radical resection of the tumor was performed in this patient.Patholog...

  14. A case of simultaneous triple primary gastrointestinal tumor

    Institute of Scientific and Technical Information of China (English)

    Xinglong Qu; Yu Han; Yi Zhang; Bing Wang

    2014-01-01

    Multiple primary carcinoma which is the same organ of the same patient or multiple organs, tissues has occurred two or more than two kinds of the primary malignant tumor. Al cancer at the same time or 6 months from diagnosis is caled simultaneous multiple primary carcinoma. In this case the patient sufering from cancer including rectal cancer, colon cancer and appendix gastrointestinal stromal tumor(GIST) three primary carcinoma, is simultaneous multiple primary carcinoma and it’s extremely rare on the clinical cases. This report address that the incidence of the patient with operation and pathological diagnosis.

  15. 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.

  16. 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.

  17. A rare Cervical Nerve Root, C2-C3 Schwannoma

    Directory of Open Access Journals (Sweden)

    Nilesh Chordia

    2014-04-01

    Full Text Available Schwannomas, neurilemmomas or neurinomas are benign nerve sheath tumors deriving from Schwann cells that occur in the head and neck region in 25-45% of cases 1 .About 10% of schwannoma that occur in the head and neck region generally originate from the vagus or sympathetic nervous system, those arising from C2 nerve root are extremely rare. 2 Preoperative imaging studies such as magnetic resonance imaging (MRI and computed tomography (CT are used to distinguish its location and origin. The treatment of schwannoma is surgical resection, with several surgical modalities have been introduced to preserve the neurological function. We present a rare case of Cervical nerve (C2-C3 root schwannoma of 70 years old male who presented with lateral neck swelling with no neurological deficit ,swelling which also had intervertebral part was removed successfully through neck incision with no post-operative neurological symptoms

  18. A Rare Case of Gastric Carcinoma with Oral Metastasis

    Directory of Open Access Journals (Sweden)

    Manjunath KV

    2013-11-01

    Full Text Available Oral region is an uncommon site for metastatic tumour cell colonization and usually evidence of wide spread disease, It accounts for only 1% of all oral malignant neoplasm’s. They mainly involve the bony structures (particularly the mandible, whereas primary metastases to soft tissues are extraordinarily rare (only 0.1% of oral malignancies. The breast is the most common primary site for tumors that metastasize to the jawbones, whereas the lung is the most common source for cancers that metastasize to the oral soft tissues. We are reporting an extremely rare case of carcinoma stomach with oral soft tissue metastatic lesion in the upper alveolar ridge extending to involving the upper labial mucosa extending upto the premolars on right side on the gingio- buccal sulcus.

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

    Science.gov (United States)

    Jain, Manjula; Harbhajanka, Aparna; Choudhary, S Roy

    2011-01-01

    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 eosinophilic 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 enolase NSE, 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.

  1. A Rare Case of Esophageal Adenocarcinoma with Urinary Bladder Metastasis

    Science.gov (United States)

    Saad, Rahoma E.; Denning, Krista; Pacioles, Toni O.

    2017-01-01

    Metastatic esophageal adenocarcinoma to the urinary bladder is extremely rare. We describe a previously healthy 49-year-old female with recent diagnosis of adenocarcinoma of the gastroesophageal junction with metastatic disease to the liver. Biopsy was positive for human epidermal growth factor receptor 2 (HER2) by Fluorescence In Situ Hybridization (FISH). She received six cycles of Cisplatin, 5-Fluorouracil, and Herceptin and subsequently developed symptomatic anemia and hematuria. Cystoscopy with retroflexion was performed and she received a transurethral resection of bladder tumor with fulguration. Pathology of the bladder tumor revealed similar morphology to her liver metastasis and immunohistochemical stains were consistent with metastatic esophageal cancer. Three weeks after being diagnosed with metachronous urinary bladder metastasis from esophageal adenocarcinoma primary, she expired. She only received her first cycle of palliative chemotherapy with Ramucirumab and Paclitaxel. PMID:28642830

  2. A Rare Case of Esophageal Adenocarcinoma with Urinary Bladder Metastasis

    Directory of Open Access Journals (Sweden)

    Heather Katz

    2017-01-01

    Full Text Available Metastatic esophageal adenocarcinoma to the urinary bladder is extremely rare. We describe a previously healthy 49-year-old female with recent diagnosis of adenocarcinoma of the gastroesophageal junction with metastatic disease to the liver. Biopsy was positive for human epidermal growth factor receptor 2 (HER2 by Fluorescence In Situ Hybridization (FISH. She received six cycles of Cisplatin, 5-Fluorouracil, and Herceptin and subsequently developed symptomatic anemia and hematuria. Cystoscopy with retroflexion was performed and she received a transurethral resection of bladder tumor with fulguration. Pathology of the bladder tumor revealed similar morphology to her liver metastasis and immunohistochemical stains were consistent with metastatic esophageal cancer. Three weeks after being diagnosed with metachronous urinary bladder metastasis from esophageal adenocarcinoma primary, she expired. She only received her first cycle of palliative chemotherapy with Ramucirumab and Paclitaxel.

  3. Osteosarcoma of breast: A rare case of extraskeletal osteosarcoma

    Directory of Open Access Journals (Sweden)

    Ashwin A Kallianpur

    2013-01-01

    Full Text Available Primary osteogenic sarcomas of the breast are exceptionally uncommon. We describe such a case occurring in a 50 year-old woman who presented with a large painful mass in her left breast. Simple mastectomy of the left breast was performed. Microscopical and immunohistochemical findings established the diagnosis of primary osteogenic sarcoma. Similar to extremity osteosarcoma, adjuvant adriamycin and cisplatin based chemotherapy and external beam radiotherapy was given to the present case. She remained well 57 months later, without tumor recurrence. The current article made a literature search proving the rarity of this lesion type and discusses in detail the diagnostic implications and the treatment of this rare site tumor entity.

  4. Bilateral sandwiched scapulae: A rare presentation of hereditary multiple exostoses.

    Science.gov (United States)

    Mozaffarian, Kamran; Farahani, Mohammad Javad; Vosoughi, Amir Reza

    2016-01-01

    Hereditary multiple exostoses, an autosomal dominant condition, is a common benign tumor which is characterized by the development of multiple osteochondromas. Bilateral dorsal and ventral involvement of scapulae is extremely rare without any reported case in the literature. An 18-year-old girl was referred because of bilateral prominent scapulae and left-sided pain on shoulder girdle motion especially at more than 90° abduction and forward flexion. Radiograph evaluation showed multiple exostoses on ventral and dorsal surfaces of body of both scapulae; hence simple excision of the lesion was impossible. The patient was symptom-free about 18 months after partial scapulectomy of left side. Partial scapulectomy seems to be an effective treatment for the sandwiched scapula between dorsal and ventral benign tumors.

  5. Cardiac Tumors; Tumeurs cardiaques

    Energy Technology Data Exchange (ETDEWEB)

    Laissy, J.P.; Fernandez, P. [Centre Hospitalier Universitaire Bichat Claude Bernard, Service d' Imagerie, 76 - Rouen (France); Mousseaux, E. [Hopital Europeen Georges Pompidou (HEGP), Service de Radiologie Cardio Vasculaire et Interventionnelle, 75 - Paris (France); Dacher, J.N. [Centre Hospitalier Universitaire Charles Nicolle, 75 - Rouen (France); Crochet, D. [Centre Hospitalier Universitaire, Hopital Laennec, Centre Hemodynamique, Radiologie Thoracique et Vasculaire, 44 - Nantes (France)

    2004-04-01

    Metastases are the most frequent tumors of the heart even though they seldom are recognized. Most primary cardiac tumors are benign. The main role of imaging is to differentiate a cardiac tumor from thrombus and rare pseudo-tumors: tuberculoma, hydatid cyst. Echocardiography is the fist line imaging technique to detect cardiac tumors, but CT and MRl arc useful for further characterization and differential diagnosis. Myxoma of the left atrium is the most frequent benign cardiac tumor. It usually is pedunculated and sometimes calcified. Sarcoma is the most frequent primary malignant tumor and usually presents as a sessile infiltrative tumor. Lymphoma and metastases are usually recognized by the presence of known tumor elsewhere of by characteristic direct contiguous involvement. Diagnosing primary and secondary pericardial tumors often is difficult. Imaging is valuable for diagnosis, characterization, pre-surgical evaluation and follow-up. (author)

  6. Malignant Solitary Fibrous Tumor of the Kidney: Report of the First Case Managed with Interferon

    Directory of Open Access Journals (Sweden)

    Javier Cuello

    2013-01-01

    Full Text Available Solitary fibrous tumors of the kidney are extremely rare tumors with unpredictable behavior. We describe a case of a patient with a solitary fibrous tumor of kidney with malignant findings with distant metastasis and nephrectomy managed with subcutaneous interferon achieving 23 months of progression-free survival. To date there is no prospective evaluation of any specific modality of treatment, but the surgical management and long-term followup are the only ones so far recommended strategies in the management of these patients. Studies are awaited with more patients to evaluate the different strategies of systemic therapy reported so far to allow adding survival benefit.

  7. Malignant solitary fibrous tumor of the kidney: report of the first case managed with interferon.

    Science.gov (United States)

    Cuello, Javier; Brugés, Ricardo

    2013-01-01

    Solitary fibrous tumors of the kidney are extremely rare tumors with unpredictable behavior. We describe a case of a patient with a solitary fibrous tumor of kidney with malignant findings with distant metastasis and nephrectomy managed with subcutaneous interferon achieving 23 months of progression-free survival. To date there is no prospective evaluation of any specific modality of treatment, but the surgical management and long-term followup are the only ones so far recommended strategies in the management of these patients. Studies are awaited with more patients to evaluate the different strategies of systemic therapy reported so far to allow adding survival benefit.

  8. Primary mixed germ cell tumor of the liver with sarcomatous components

    Institute of Scientific and Technical Information of China (English)

    2010-01-01

    Germ cell tumor(GCT)of the liver is extremely rare. Here,we describe a case of hepatic mixed GCT with significant sarcomatous components and elevated serum α-fetoprotein(AFP)in a 34-year-old man.Histopathologically,the tumor was composed of two GCTs components:yolk sac tumor and immature teratoma.The predominant components of immature teratoma consisted of several types of tissue that represented different germinal layers(endoderm,mesoderm and ectoderm) and showed varying degrees of differentiation with sig...

  9. 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.

  10. Ependymal tumors.

    Science.gov (United States)

    Moynihan, Timothy J

    2003-12-01

    Ependymal tumors are rare malignancies that arise from the cells that line the ventricles and central canal of the spinal cord. Although they are more common in children, adults may also be effected by ependymal tumors. Prognosis is dependent on tumor location, histology, especially for myxopapillary tumors that tend to occur in the lumbar spine, extent of surgical resection, and stage of disease. Standard therapy consists of complete resection when feasible. The exact role of adjuvant radiotherapy in patients with radiographically confirmed complete resection is poorly defined. Patients with known residual disease may benefit from local radiation therapy, but the extent of radiation field and total dose are controversial. Even in patients treated with involved field radiotherapy, most relapses occur within the original tumor bed, thus local control remains the biggest obstacle to effective therapy. Chemotherapy has little impact against this tumor and has no role in the adjuvant setting, outside of a well designed clinical trial, with the possible exception of children younger than 5 years in an effort to delay radiation. A minority of patients may respond to one of several chemotherapy regimens at the time of recurrence, but the impact of this therapy is limited. Newer treatment strategies are needed.

  11. Concurrent spinal schwannoma and meningioma mimicking a single cervical dumbbell-shaped tumor: case report.

    Science.gov (United States)

    Oichi, Takeshi; Chikuda, Hirotaka; Morikawa, Teppei; Mori, Harushi; Kitamura, Daisuke; Higuchi, Junya; Taniguchi, Yuki; Matsubayashi, Yoshitaka; Oshima, Yasushi; Tanaka, Sakae

    2015-12-01

    Dumbbell-shaped tumors consisting of 2 different tumors are extremely rare. Herein, the authors present a case of concurrent spinal schwannoma and meningioma mimicking a single cervical dumbbell-shaped tumor. A 64-year-old man presented with a 5-year history of gradually exacerbating left occipital pain without clinical evidence of neurofibromatosis. Magnetic resonance imaging showed an extradural tumor along the left C-2 nerve root with a small intradural component. The tumor was approached via a C-1 hemilaminectomy. The intradural tumor was resected together with the extradural tumor after opening the dura mater. The intradural tumor was attached to the dura mater around the exit point of the C-2 nerve root. Intraoperative biopsy revealed that the extradural tumor was a schwannoma and that the intradural tumor was a meningioma. The dura mater adjacent to the tumor was then coagulated and resected. Postoperative pathological examination confirmed the same diagnoses with no evidence of continuity between the intra- and extradural components. The patient's postoperative clinical course was uneventful. Clinicians should be aware that cervical dumbbell-shaped tumors can consist of 2 different tumors.

  12. 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.

  13. Uterine primitive neuroectodermal tumor with adenosarcoma: a case report

    Directory of Open Access Journals (Sweden)

    Chawla Indu

    2010-06-01

    Full Text Available Abstract Introduction Primitive neuroectodermal tumor of the uterus is extremely rare. They occur as either pure primitive neuroectodermal tumors or admixed with neoplasms of mullerian origin. Case presentation A case of uterine primitive neuroectodermal tumor with adenosarcoma in a 50-year-old Asian Indian woman is presented. Histologically, the neoplasm displayed perivascular pseudorosettes and occasional Homer-Wright rosettes. A strong positivity for neuronspecific enolase and synaptophysin was noted, while chromogranin and CD99 were negative. Merging imperceptibly with the neuroectodermal components were the areas of adenosarcoma. Conclusion To the best of our knowledge, this report represents the second case of a uterine primitive neuroectodermal tumor with an admixed adenosarcoma.

  14. An unusual presentation of "silent" disseminated pancreatic neuroendocrine tumor

    Institute of Scientific and Technical Information of China (English)

    Dragomir Marisavljevic; Natasa Petrovic; Nikola Milinic; Vesna Cemerikic; Miodrag Krstic; Olivera Markovic; Dragoljub Bilanovic

    2004-01-01

    To present a patient diagnosed with pancreatic carcinoid that was extremely rare and produced an atypical carcinoid syndrome.We reported a 58-year old male patient who presented with long standing,prominent cervical lymphadenopathy and occasional watery diarrhea.Pathohistological and immunohistochemical examination of lymph node biopsy showed a metastatic neuroendocrine tumor,which was histological type A of carcinoid (EMA+,cytokeratin+,CEA-,NSE+,chromogranin A+,synaptophysin+,insulin-).Bone marrow biopsy showed identical findings.Primary site of the tumor was pancreas and diagnosis was made according to cytological and immunocytochemical analysis of the tumor cells obtained with aspiration biopsy of pancreatic mass (12 mm in diameter) under endoscopic ultrasound guidance.However,serotonin levels in blood and urine samples were normal.It is difficulty to establish the precise diagnosis of a "functionally inactive" pancreatic carcinoid and aspiration biopsy of pancreatic tumor under endoscopic ultrasound guidance can be used as a new potent diagnostic tool.

  15. 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.

  16. Nerve sheath tumors of the head and neck - a radiological review; Tumores da bainha nervosa em cabeca e pescoco - estudo revisional

    Energy Technology Data Exchange (ETDEWEB)

    Souza, Ricardo Pires de; Carramao, Cintia Fernandes; Soares, Aldemir Humberto [Hospital Heliopolis, Sao Paulo, SP (Brazil). Servico de Diagnostico por Imagem; Chacra Junior, Jose; Rapoport, Abrao [Hospital Heliopolis, Sao Paulo, SP (Brazil). Servico de Cirurgia de Cabeca e Pescoco

    1997-01-01

    Peripheral nerve sheath tumors of the head and neck - a review. Peripheral nerve sheath tumors are derived from neural crest and rare classified as neuroectodermal in origin. They can be divided into neurofibroma, schwannoma and neurogenic sarcoma. Neurofifromas are benign well circumscribed, nonencapsulated tumors which involve all elements of normal peripheral nerves. Schwannomas are beginning encapsulated tumors composed fundamentally by Schwann cells. Neurogenic sarcomas are malignant tumors which can be de novo or arise from preexisting neurofibroma or schwannoma. Peripheral nerve sheath tumors can arise from any nerve that contain myelin sheath, but are more frequent in extremities and trunk, being rare on cervical region. neurogenic tumors of head and neck can arise from cranial nerves, especially vagus nerve, brachial plexus and other small nervous plexus. Computed tomography and magnetic resonance imaging are the methods of choice in the evaluation of those tumors and can demonstrate lesions with several patterns. Areas of cystic degeneration are frequent in schwannomas, while neurofibromas are usually homogeneous. About 1/3 of those tumors are hyper vascularized and those who arise nervous spinal; roots can have an aspect of dumbbell which contain cervical and intravertebral components. (author) 51 refs., 5 figs.

  17. Stromal tumor of colon: Case report

    Directory of Open Access Journals (Sweden)

    Nićiforović Dijana

    2008-01-01

    Full Text Available Introduction Gastrointestinal stromal tumor is relatively new term, it can be localized anywhere inside the gastrointestinal system. It has formerly been called leiomyoma, leiomyoblastoma, and/or leiomyosarcoma. Case report Case report is about a female patient with indefinite difficulties described as 'bother', mild anemia and anamnesis data of her mother who had been operated on for colon tumor. After blood examination, which had shown values within referential limits except for mild anemia, patient underwent radiological examination. Primarily, an abdominal cavity ultrasound had been performed, where a suspicious formation in the right hemiabdomen was found, but without distinctive anatomical localization in the abdominal cavity. Secondly, a checkup by Duplex Doppler ultrasound was made, as well as radiological examination with double contrast of colon and computed tomography, where tumor was visualized on ascendant colon with extraluminal localization. Discussion Radiological findings were confirmed by surgery. Histopathological findings were positive for gastrointestinal stromal colon tumor. Conclusion Gastrointestinal stromal tumors represent extremely rare tumors of gastrointestinal system, especially when localized at the colon, but they should be included in a differential diagnosis for their malignant potential.

  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. Rectal carcinoid tumor metastasis to a skull base meningioma

    Science.gov (United States)

    Huang, Jennifer; Gupta, Amit; Badve, Chaitra; Cohen, Mark L; Wolansky, Leo J

    2016-01-01

    Carcinoid tumors are rare, slow-growing neuroendocrine tumors that most frequently develop in the gastrointestinal tract or lungs and have high potential for metastasis. Metastasis to the brain is rare, but to another intracranial tumor is extremely rare. Of the intracranial tumors, meningiomas are the most common to host metastases, which may be related to its rich vascularity and E-cadherin expression. We describe the case of a 65-year-old female with active chemotherapy-treated neuroendocrine carcinoma who presented with left-sided facial numbness, headaches, and blurry vision. Initial imaging revealed a 1 cm irregular dural-based left petrous apex mass suggestive of a meningioma that was re-imaged four months later as a rapidly enlarging, extra-axial, mass extending into the cavernous sinus, effacing Meckel’s cave that resembled a trigeminal schwannoma. Pathology revealed a carcinoid tumor metastatic to meningioma. While the mass displayed characteristic imaging findings of a schwannoma, rapid growth in the setting of known active malignancy should prompt the clinician to consider mixed pathology from metastatic disease or a more aggressive meningioma. PMID:26825133

  20. 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.

  1. 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.

  2. Large calcifying epithelial odontogenic tumor with extension into the maxillary sinus: a case report.

    Science.gov (United States)

    da Rosa, Marize Raquel Diniz; de Oliveira, James Maxwell Souza; Dias-Ribeiro, Eduardo; Ferreira-Rocha, Julierme; de Barros, Iolanda Maria Cariry Carvalho Lacet; Lopes, Patricia de Medeiros Loureiro

    2011-01-01

    Calcifying epithelial odontogenic tumor (CEOT) is a rare, locally invasive neoplasm characterized by the presence of amyloid material that can become calcified. It often is found in the posterior region of the mandible. Such tumors in the maxilla and those that invade the maxillary sinus are extremely rare. This article presents the sixth reported clinical case of a CEOT that invaded the maxillary sinus and extended to the interior of the nasal cavity. The tumor had grown toward the sinus roof, but there was no association with an impacted tooth. Histopathologically, the tumor was composed of plates of polyhedral epithelial cells with highly eosinoplilic cytoplasm, nuclear polymorphism, clear-cell contours, and intercellular bridges in fibrous conjunctive tissue. Amorphous eosinophilic material and diverse calcifications permeated the epithelial cells.

  3. Giant cell tumor of the greater wing of the sphenoid: an unusual presentation.

    Science.gov (United States)

    Pelaz, Andrés Coca; Llorente Pendás, José L; Rodrigo Tapia, Juan P; Suárez Nieto, Carlos

    2008-05-01

    We report a very unusual presentation of giant cell tumor probably originated on the greater wing of the sphenoid and show a review about the knowledge and the treatment of the lesion in this rare localization. We treated a 48-year-old man with a giant cell tumor of the infratemporal fossa. He presented with a right-side hearing loss and facial pain. The tumor was resected by means of a subtemporal-preauricular approach, and after 12 months of follow-up, the patient is free of recurrence. Giant cell tumors of the skull base are an extremely rare neoplasm, and there is not much information on the literature about the treatment and the prognostic. Wide resection ought to be made, and at the follow-up, the clinician must try to diagnose not only local recurrence but also the possibility of distant metastases to the lung.

  4. Phyllodes Tumor of Anogenital Mammary-like Glands with Diffuse Pseudoangiomatous Stromal Hyperplasia.

    Science.gov (United States)

    Elıyatkin, Nuket; Top, Omer Erdinç; Yalçin, Evrim; Zengel, Baha; Ozgür, Hakan; Aykas, Ahmet; Vardar, Enver

    2013-11-07

    Anogenital mammary-like glands may give rise to various pathologic lesions identical to those known in mammary pathology. Tumor occurring in the anogenital region is extremely rare. The histogenetic origin of this tumor is controversial as it is being debated whether such lesions evolve from ectopic breast tissue and most recently, anogenital mammary-like gland. We report a 28-year-old girl who presented with a painless mass in the anogenital region, which was subsequently excised. Microscopic examination revealed morphologic pattern characteristic of benign phyllodes tumor with pseudoangiomatous stromal hyperplasia. We present this case to emphasize the importance of recognizing this uncommon lesion occurring at an extremely unusual site. We also discuss the histogenesis of phyllodes tumor and related lesions occurring in the anogenital region in light of the current literature along with a brief review of the previously reported cases of anogenital mammary-like glands.

  5. Meningeal fibroma: a rare meningioma mimic.

    Science.gov (United States)

    Kakkar, Aanchal; Sharma, Mehar C; Goyal, Nishant; Sarkar, Chitra; Suri, Vaishali; Garg, Ajay; Kale, Shashank S; Suri, Ashish

    2014-08-01

    Meningeal fibromas are rare intracranial tumors that mimic meningiomas radiologically as well as histologically. The authors report 2 cases of meningeal fibroma with detailed clinical, radiological, histopathological, and immunohistochemical features, and discuss the differential diagnosis of this entity. Knowledge of this rare tumor is essential for pathologists to be able distinguish it from more common meningeal tumors, especially in younger patients. This knowledge is also essential for neurosurgeons, as incomplete resection may lead to tumor recurrence, and such patients require close follow-up.

  6. Technical Insights into Highly Sensitive Isolation and Molecular Characterization of Fixed and Live Circulating Tumor Cells for Early Detection of Tumor Invasion

    Science.gov (United States)

    Laget, Sophie; Dhingra, Dalia M.; BenMohamed, Fatima; Capiod, Thierry; Osteras, Magne; Farinelli, Laurent; Jackson, Stephen; Paterlini-Bréchot, Patrizia

    2017-01-01

    Circulating Tumor Cells (CTC) and Circulating Tumor Microemboli (CTM) are Circulating Rare Cells (CRC) which herald tumor invasion and are expected to provide an opportunity to improve the management of cancer patients. An unsolved technical issue in the CTC field is how to obtain highly sensitive and unbiased collection of these fragile and heterogeneous cells, in both live and fixed form, for their molecular study when they are extremely rare, particularly at the beginning of the invasion process. We report on a new protocol to enrich from blood live CTC using ISET® (Isolation by SizE of Tumor/Trophoblastic Cells), an open system originally developed for marker-independent isolation of fixed tumor cells. We have assessed the impact of our new enrichment method on live tumor cells antigen expression, cytoskeleton structure, cell viability and ability to expand in culture. We have also explored the ISET® in vitro performance to collect intact fixed and live cancer cells by using spiking analyses with extremely low number of fluorescent cultured cells. We describe results consistently showing the feasibility of isolating fixed and live tumor cells with a Lower Limit of Detection (LLOD) of one cancer cell per 10 mL of blood and a sensitivity at LLOD ranging from 83 to 100%. This very high sensitivity threshold can be maintained when plasma is collected before tumor cells isolation. Finally, we have performed a comparative next generation sequencing (NGS) analysis of tumor cells before and after isolation from blood and culture. We established the feasibility of NGS analysis of single live and fixed tumor cells enriched from blood by our system. This study provides new protocols for detection and characterization of CTC collected from blood at the very early steps of tumor invasion. PMID:28060956

  7. Not so Rare, Rare Diseases

    Science.gov (United States)

    Waldman, H. Barry; Perlman, Steven P.; Munter, Beverly L.; Chaudhry, Ramiz A.

    2008-01-01

    A rare disease or condition is defined by federal legislation such that it: (1) affects less than 200,000 persons in the U.S.; or (2) affects more than 200,000 persons in the U.S. but for which there is no reasonable expectation that the cost of developing and making available in the U.S. a drug for such disease or condition will be recovered from…

  8. Pediatric Synovial Sarcoma in the Retropharyngeal Space: A Rare and Unusual Presentation

    Directory of Open Access Journals (Sweden)

    Sanjay Vaid

    2015-01-01

    Full Text Available Synovial sarcomas in the head and neck are extremely rare tumors, especially in the pediatric population. 3–5% of synovial sarcomas occur in the head and neck region displaying varied imaging and histopathological features resulting in frequent misdiagnosis. These tumors have a poor prognosis; hence early diagnosis and accurate classification based on imaging, histopathology, and immunohistochemistry are critical for prompt treatment. To the best of our knowledge, imaging findings of pediatric retropharyngeal lipomatous synovial sarcoma have not been reported to date in English medical literature. We report, for the first time, a rare case of retropharyngeal lipomatous synovial sarcoma in a ten-year-old child and discuss the case-specific imaging findings in our patient using magnetic resonance imaging and computed tomography.

  9. 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.)

  10. Limb salvage surgery for malignant bone tumors of the extremities in children and adolescents%儿童及青少年保留骨骺的保肢手术临床研究

    Institute of Scientific and Technical Information of China (English)

    Zhen Wang; Zheng Guo; Xiangdong Li; Luyu Huang; Qiang Ji; Haiqiang Wang; Hongxun Sang; Jun Fu

    2009-01-01

    lost to follow up. 29 cases had complete clinical data. Postoperative follow-up was 12-72 months. Recurrences were seen in 3 cases, with one local recurrence in type Ⅲ cases one year post tumor resection, other 2 recurrences around the femoral vessels in type Ⅱ cases 15 months and 30 months after the tumor resection respectively. The recurrence rate accounted for 10.34%. Amputation was performed for the cases with recurrences. But pulmonary metastasis developed and the patients died. In this cohort, 9 cases died. Five-year survival rate constituted 57.94% demonstrated by Kaplan-Meier sur-vival analysis. The median survival time for male patients was 64.36 months, and that for female patients was 56.00 months (P=0.0403). The sites, stages and types were not associated with the survival time. Four cases reported 5 complications (17.24%). The complications included fracture of the allograft, limb length discrepancy, nerve injury, breakage of nails, and loosening of the screws. No patient reported skin necrosis, hematoma, infection, rejection to the allograft, and nonunion at the end of the follow-up. According to the functional evaluation criteria after surgical treatment of malignant tumors of the extremities introduced by Enneking, excellent functional results were reported in 11 cases, good in 13 cases, fair in 3 cases, and poor in 2 cases. The excellent or good result rate was 83%. The excision interface involved partial epiphysis in 4 cases, involved the epiphyseal plate in 8 cases. Intact epiphysis was preserved in 17 cases. Five cases with poor postoperative functional results were in types Ⅱ and Ⅲ. Average time of bone union was 2.5 months for epiphyseal end and 3.8 months for diaphyseal end. According to the Intemational Society of Limb Salvage (ISOLS) radiological implants evaluation system, excellent reconstruction was observed in 23 cases, good reconstruction in 4 cases, fair reconstruction in 1 case, and poor reconstruction in 1 case. All patients in

  11. Langerhans cell histiocytosis of atlantoaxial joint in a middle-aged man presenting with deafness as first symptom and soft-tissue mass at neck showing excellent response to radiotherapy alone: Report of an extremely rare and unusual clinical condition and review of literature

    Science.gov (United States)

    Mondal, Dodul; Julka, P. K.; Jana, Manisha; Walia, Ritika; Chaudhuri, Tamojit

    2014-01-01

    Langerhans cell histiocytosis (LCH) is a disorder of clonal proliferation of dendritic cell mainly occurring in children. Spine involvement is rare. This usually presents with pain and torticollis when neck is involved. Histopathology with immunohistochemistry is confirmatory. Local curative therapy with excision or curettage is used for localized disease. Radiotherapy is usually reserved for selected cases. Systemic chemotherapy is the treatment of choice for widespread systemic disease. In this article, we present an unusual presentation of atlantoaxial LCH with mastoid involvement resulting in hearing loss as the first symptom and quadruparesis in a middle aged male patient, which was also associated with soft-tissue mass at the nape of the neck and deafness. The patient was treated with radical radiotherapy, which provided excellent response to the disease. Involvement of atlantoaxial joint and temporal bone associated with soft-tissue mass neck and deafness in a middle-aged man is an extremely rare clinical situation. PMID:25506166

  12. Rare primary retroperitoneal teratoma masquerading as adrenal ...

    African Journals Online (AJOL)

    J.M. Ratkal

    investigation was found to have a right adrenal mass were collected. ... in children, but their prevalence in adults is exceedingly rare. The adrenal gland is ... tumors, the abdominal CT and chest X-ray images taken prior to surgery were.

  13. Other Rare Unusual Cancers of Childhood

    Science.gov (United States)

    ... patient depends on the type of gene change. Pheochromocytoma and Paraganglioma Pheochromocytoma and paraganglioma are rare tumors that come from the same type of nerve tissue . Pheochromocytoma forms in the adrenal glands . There are two ...

  14. A rare case report of a primary large-cell neuroendocrine carcinoma of the breast with coexisting Paget disease.

    Science.gov (United States)

    Psoma, Elizabeth; Nikolaidou, Olga; Stavrogianni, Thomai; Mavromati, Areti; Lytras, Konstantinos; Xirou, Persefoni; Koumbanaki, Melanie; Panagiotopoulou, Dimitra

    2012-01-01

    Neuroendocrine carcinomas are malignancies deriving from neuroendocrine cells existing in various sites of the body, most commonly in the lungs and gastrointestinal tract. Primary neuroendocrine carcinomas of the breast are extremely rare, and their diagnosis is confirmed by positive neuroendocrine markers. We describe a case of a 46-year-old woman with a palpable mass in her left breast for the previous 3 months. The tumor was resected and a primary large-cell neuroendocrine tumor of the breast was confirmed by histopathologic examination. Paget disease of the nipple was noted as well.

  15. 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...

  16. French National Registry of Rare Peritoneal Surface Malignancies

    Science.gov (United States)

    2016-07-12

    Rare Peritoneal Surface Malignancies; Pseudomyxoma Peritonei; Peritoneal Mesothelioma; Desmoplastic Small Round Cell Tumor; Psammocarcinoma; Primary Peritoneal Serous Carcinoma; Diffuse Peritoneal Leiomyomatosis; Appendiceal Mucinous Neoplasms

  17. Ovarian tumor-derived ectopic hyperprolactinemia.

    Science.gov (United States)

    Elms, Autumn F; Carlan, S J; Rich, Amy E; Cerezo, Lizardo

    2012-12-01

    To describe extreme hyperprolactinemia originating from a pituitary adenoma in the wall of an ovarian dermoid. This is a description of an unusual case and a review of ectopic prolactin production. Ectopic production of prolactin is a rare condition that has been reported in isolated organ system pathology including ovaries. An ovarian dermoid is a benign neoplasm that has the potential for active unregulated endocrine function. Hyperprolactinemia can result from functioning lactotrophs found in ovarian dermoids and can lead to clinical sequelae. Definitive treatment of the condition requires surgical removal of the functioning endocrine tissue. Extreme hyperprolactinemia caused by a pituitary tumor located within a dermoid has not been reported before. We present a case of profound hyperprolactinemia originating from a pituitary adenoma found in the wall of an ovarian dermoid and give a broad overview of the condition and literature. Ectopic prolactin production should always be considered in symptomatic patients found to have elevated serum levels and no findings on brain imaging.

  18. Dentinogenic ghost cell tumor

    Directory of Open Access Journals (Sweden)

    Singhaniya Shikha

    2009-01-01

    Full Text Available Dentinogenic ghost cell tumor (DGCT is a rare tumorous form of calcifying odontogenic cyst and only a small number of cases have been described. It is a locally invasive neoplasm that is characterized by ameloblastoma-like epithelial islands, ghost cells and dentinoid. The present report describes a case of a 21-year-old male with a tumor in the posterior region of the mandible, showing features of DGCT.

  19. Olfactory ensheathing cell tumor

    Directory of Open Access Journals (Sweden)

    Ippili Kaushal

    2009-01-01

    Full Text Available Olfactory ensheathing cells (OECs are found in the olfactory bulb and olfactory nasal mucosa. They resemble Schwann cells on light and electron microscopy, however, immunohistochemical staining can distinguish between the two. There are less than 30 cases of olfactory groove schwannomas reported in the literature while there is only one reported case of OEC tumor. We report an OEC tumor in a 42-year-old male and discuss the pathology and origin of this rare tumor.

  20. Endometrioid Paraovarian Borderline Cystic Tumor in an Infant with Proteus Syndrome

    Directory of Open Access Journals (Sweden)

    Liliana Vasquez

    2015-01-01

    Full Text Available Ovarian and paraovarian neoplasms are uncommon in children, mainly originating from germ cell tumors and, least frequently, epithelial tumors. There is an association between genital tract tumors and Proteus syndrome, a rare, sporadic, and progressive entity, characterized by a postnatal overgrowth in several tissues caused by a mosaic mutation in the AKT1 gene. We describe a 20-month-old asymptomatic infant with Proteus syndrome who developed an endometrioid paraovarian borderline cystic tumor. This is the youngest patient so far reported in the literature with this rare syndrome and an adnexal tumor of borderline malignancy. A total of nine patients have been described with female tract tumors and associated Proteus syndrome, which includes bilateral ovarian cystadenomas and other benign masses. A paraovarian neoplasm is extremely rare in children and could be considered a criterion for Proteus syndrome. Standardized staging and treatment of these tumors are not well established; however, most authors conclude that these neoplasms must be treated as their ovarian counterparts.