WorldWideScience

Sample records for rare tumor entity

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

    African Journals Online (AJOL)

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

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

    Science.gov (United States)

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

    2017-02-01

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

  3. Hybrid Odontogenic Lesion: A Rare Entity

    Directory of Open Access Journals (Sweden)

    Reza Imani

    2017-03-01

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

  4. 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.

  5. Thanatophoric Dysplasia: A Rare Entity

    Directory of Open Access Journals (Sweden)

    N.S. Naveen

    2011-05-01

    Full Text Available Thanatophoric dysplasia is the lethal skeletal dysplasia characterized by marked underdevelopment of the skeleton and short-limb dwarfism. The child will be having a short neck, narrow thoracic cage and protuberant abdomen. Other anatomical features include a relatively enlarged head with frontal bossing, prominent eyes, hypertelorism and the depressed nasal bridge. The diagnosis is usually made with the ultrasonography in the second trimester. In this study we report a case of this rare entity with emphasis on its anatomical features, abnormalities and clinical profile with relevant review of literature.

  6. Maxillary Osteomyelitis: A Rare Entity

    Directory of Open Access Journals (Sweden)

    Ayaaz Habib

    2016-01-01

    Full Text Available Osteomyelitis of the maxilla is now a rare event with the advent of antibiotics. The two predominant causes are odontogenic infections and sinusitis. Immunocompromised states such as diabetes, HIV, and malnutrition increase the risk of osteomyelitis. It is important to recognize this early as it is a difficult entity to treat with potentially serious consequences. We report an unusual case of right sided maxillary osteomyelitis in a lady with poorly controlled diabetes in rural Lincolnshire. Biopsy of the right maxillary bone showed features of acute osteomyelitis. This responded well to a prolonged course of oral antibiotics.

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

    Directory of Open Access Journals (Sweden)

    Parwani Anil V

    2007-05-01

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

  8. Primary malignancy of seminal vesicle: A rare entity

    Directory of Open Access Journals (Sweden)

    Rajaraman Ramamurthy

    2011-01-01

    Full Text Available We report a rare case of seminal vesicle malignancy (primitive neuro ectodermal tumor in a 40-year-old male patient. He was treated with enbloc resection of the tumor and ureteric reimplantation. In view of the rarity of this entity, management of these tumors should be individualized.

  9. Bednar Tumor: An Uncommon Entity.

    Science.gov (United States)

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

    2016-01-01

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

  10. Malignant struma ovarii: a rare oncologic entity

    International Nuclear Information System (INIS)

    Barbosa, Frederico Paes; Mendonca, Sandro Bichara; Souza, Thiago Ferreira de

    2004-01-01

    Struma ovarii (SO) malignant is a rare ovarian teratoid tumor, consisting predominantly of thyroid tissue. It has low incidence and a few cases described by the literature. Usually appears in the 4th or 5th decade of life. Generally they are diagnosed after surgery, being the diagnostic reserved for the lesions on the ectopic thyroid tissue based on cellular atypia, mitotic activity, capsular invasion and metastases. Due to its rarity,it does not still have a consensus about the best therapeutical management and protocol of accompaniment for patients with this neoplastic entity. This article reports a case of a patient with malignant S O, reviewing the relevant aspects of the literature. (author)

  11. Cardiofaciocutaneous syndrome: A rare entity

    Directory of Open Access Journals (Sweden)

    S Pavithra

    2012-01-01

    Full Text Available The cardiofaciocutaneous (CFC syndrome is a condition of sporadic occurrence, with patients showing multiple congenital anomalies and mental retardation and characteristic dysmorphic features. We, thus, report a rare case of this syndrome in a 1-year-old child who presented with typical features of CFC syndrome.

  12. Intracranial chondroma: a rare entity.

    Science.gov (United States)

    Maheshwari, Veena; Mehdi, Ghazala; Varshney, Manoranjan; Jain, Anshu; Vashishtha, Sonal; Gaur, Kavita; Srivastava, Vinod Kumar

    2011-05-12

    Intracranial chondroma is a rare benign cartilaginous tumour with an incidence of less than 1% of all primary intracranial tumours. The authors are reporting here a case of intracranial chondroma in a 40-year-old man who presented with 5-month history of headache and gradual diminution of vision. A tentative diagnosis of chondroma was made on imprint cytology which was confirmed on histopathological examination.

  13. 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.

  14. Pancreatic pseudopapillary tumour: A rare misdiagnosed entity.

    Science.gov (United States)

    Affirul, C A; Qisti, F N; Zamri, Z; Azlanuddin, A; Hairol, A O; Razman, J

    2014-01-01

    Solid pseudo papillary pancreatic tumour is a rare entity. The atypical presentation causes a delayed or misdiagnosis of these pathology. It commonly affects the female population in the 2nd and 3rd decade of life. The presentation varies from non-specific abdominal pain to incidental findings in asymptomatic patients. It is a low-grade premalignant condition that is curable by excision of the tumour. This paper presents a 17-year-old girl with intra-abdominal mass diagnosed with solid pseudo papillary tumour that underwent Whipple's procedure. We discuss the presentations, diagnosis and pathology findings of this rare pathology. The diagnosis remains an enigma in view of the nature and location of the tumour. Resection is still the best choice remains for this condition. Copyright © 2014 The Authors. Published by Elsevier Ltd.. All rights reserved.

  15. [Isolated splenic metastases from cervical cancer: a rare entity].

    Science.gov (United States)

    Villalón-López, José Sebastián; Souto-del Bosque, Rosalía; Montañez-Lugo, Juan Ignacio; Chávez-González, Bruno

    2014-01-01

    Splenic metastases from solid tumors are a rare event with an incidence of only 2.9% to 9%. Splenic metastases from cervical cancer are a rare entity. Only a few cases have been reported of isolated spleen metastases from cervical cancer. We present the case of a 76-year-old woman with moderately differentiated endocervical adenocarcinoma stromal and endocervical invasion. Clinical stage was Ib1 and Ca-125 values of 150 U. She was managed with hysterectomy and pelvic lymphadenectomy. She received pelvic radiotherapy (45 Gy) followed 24 Gy of brachytherapy. Two years later she presented with abdominal pain. Abdominal computed tomography showed two splenic parenchymal lesions without disease in the remainder of the abdominal cavity and chest with a Ca-125 of 2,733 U. The patient is submitted to splenectomy. Histopathology demonstrates splenic metastases of well-differentiated adenocarcinoma from the endocervix. Immunohistochemical stain showed positivity from carcinoembryonic antigen; estrogen and progesterone receptors are negative. Ca-125 level 8 weeks after surgery was 16 U/ml. The patient received six cycles of adjuvant chemotherapy with paclitaxel and cisplatin. At 12 months follow-up the patient is alive and without evidence of tumor activity. The spleen is an uncommon site of metastasis. Splenectomy is considered the appropriate treatment in order to avoid complications such as splenic rupture and splenic vein thrombosis as well as to improve pain control from splenomegaly. Twelve months after surgery our patient is alive and without evidence of tumor activity.

  16. Teratoid Wilms′ tumor - A rare renal tumor

    Directory of Open Access Journals (Sweden)

    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.

  17. Retroperitoneal Cellular Angiofibroma: A Rare Gynecological Entity

    Directory of Open Access Journals (Sweden)

    Ana Brandão

    2017-12-01

    Full Text Available Cellular angiofibroma is a mesenchymal tumor, described in 1997, without gender preference, that usually appears at age 40. The vulvovaginal area is the most common site in women, mimicking vulvar benign tumors, like Bartholin gland cyst. However, there are a few described cases of a deep or extra-pelvic angiofibroma. Excision is the treatment of choice and the recurrence rate appears to be low. We present the case of a woman with a heterogeneous tumor in the right adnexial region. At the surgery, a retroperitoneal tumor was excised and the histopathological tissue analysis revealed a cellular angiofibroma.

  18. Pseudomyxoma peritonei (PMP) – a rare entity

    African Journals Online (AJOL)

    Department of Radiology, University of Pretoria. Corresponding author: Z Ebrahim (zaeem@iafrica.com). Pseudomyxoma peritonei (PMP) is a rare complication of mucinous tumours of appendiceal or ovarian origin that results in peritoneal and omental implants. In addition to the appendix and the ovary, other rare.

  19. Urethral Leiomyoma: A Rare Clinical Entity

    Directory of Open Access Journals (Sweden)

    Ng Beng Kwang

    2016-01-01

    Full Text Available Extrauterine leiomyomas are encountered occasionally, which can pose a diagnostic dilemma and challenge to the gynaecologist. We report a rare case of urethral leiomyoma. A 31-year-old woman with history of primary subfertility presented with mass at her urethral meatus and lower urinary tract symptoms. She underwent examination under anaesthesia and excision of the urethral mass. Histopathological examination confirmed leiomyoma. Diagnosis and management of this common growth situated at a rare location were reviewed and discussed.

  20. Bilateral cerebellopontine arachnoid cyst: A rare entity.

    Science.gov (United States)

    Sharma, Anand; Sharma, Achal; Mittal, Radhey S; Gandhi, Ashok

    2015-01-01

    Bilateral cerebellopontine angle (CPA) arachnoid cysts (ACs) are very rare: only one case is reported in literature. Pathogenesis of those cysts is unknown; they are thought to be congenital. The presenting symptoms of CPA AC are frequently nonspecific or otological. The management of ACs of the CPA is controversial. We are reporting two cases of bilateral CPA AC with their pathophysiology and review of literature.

  1. Herpes zoster oticus: A rare clinical entity

    Directory of Open Access Journals (Sweden)

    Shailesh Gondivkar

    2010-01-01

    Full Text Available Herpes zoster oticus also known as Ramsay Hunt syndrome is a rare complication of herpes zoster in which reactivation of latent varicella zoster virus infection in the geniculate ganglion causes otalgia, auricular vesicles, and peripheral facial paralysis. Ramsay Hunt syndrome is rare in children and affects both sexes equally. Incidence and clinical severity increases when host immunity is compromised. Because these symptoms do not always present at the onset, this syndrome can be misdiagnosed. Although secondary to Bell′s palsy in terms of the cause of acute atraumatic peripheral facial paralysis, Ramsay Hunt syndrome, with incidence ranged from 0.3 to 18%, has a worse prognosis. Herpes zoster oticus accounts for about 12% cases of facial palsy, which is usually unilateral and complete and full recovery occurs in only about 20% of untreated patients. The most advisable method to treat Ramsay Hunt syndrome is the combination therapy with acyclovir and prednisone but still not promising, and several prerequisites are required for better results. We present a case of 32-year-old man suffering from Ramsay Hunt syndrome with grade V facial palsy treated effectively with rehabilitation program, after the termination of the combination therapy of acyclovir and prednisone.

  2. Urachal remnant carcinoma - a rare entity

    Directory of Open Access Journals (Sweden)

    Vanesha Naidu

    2013-06-01

    Full Text Available Primary malignancy of the urachal remnant is a rare neoplasm that accounts for less than 0.01% of all adult cancers, with an estimated annual incidence of 1:5 million. The tumour carries a grave prognosis that attests to its highly aggressive nature. Owing to its extra-peritoneal location, the tumour runs a relatively silent clinical course until late presentation, when most patients display extensive local invasion and metastatic spread. In this report, we highlight a case of primary malignancy of the urachus that on initial clinical evaluation masqueraded as a Sister Mary Joseph’s nodule. Characteristic imaging features, however, proved decisive in establishing the diagnosis of a urachal carcinoma.

  3. Concomitant hypo-hyperdontia: A rare entity

    Directory of Open Access Journals (Sweden)

    Yin-Lin Wang

    2018-03-01

    Full Text Available Background/purpose: Concomitant hypo-hyperdontia (CHH is a rare numeric dental anomaly characterized by congenital missing teeth and supernumerary teeth occurring in the same individual. Due to its rarity and sporadicity, the causes of CHH have been completely unknown. Detailed characterization and presentation of more CHH cases not only strengthen clinical diagnosis and treatment for the patients but facilitate the search for etiological factors of the disorder. Materials and methods: From a pedodontic patient population, 21 CHH subjects, with a mean age of 6 years 10 months, were identified and characterized. Dental records and radiographs were scrutinized and analyzed for the distribution and frequencies of involved teeth and concurrent dental anomalies. Through further literature review, 59 CHH cases with supernumeraries in the premaxillary region were retrieved for comparative analyses. Results: The boys were affected twice as often as the girls. While most cases were unrelated and sporadic, two sisters and a pair of identical twins from two unrelated families were presented. Of all cases, only one was of syndromic CHH carrying Duchenne muscular dystrophy. Bimaxillay CHH, with anomalies involving two jaws, occurred more than 4 times as often as maxillary CHH. While all supernumeraries were found in premaxillary region, hypodontia frequently involved lateral incisors and premolars of both jaws. Conclusion: As genetic contribution to CHH is strongly suggested by its familial occurrence and syndromic cases, environmental factors seem to play certain roles in modifying disease phenotypes. Judicious use of radiographs during early mixed dentition stage enhances clinical diagnosis and treatment of CHH. Keywords: Tooth agenesis, Supernumerary, Numeric anomaly, Premaxillary

  4. Ossification of the bilateral Achilles tendon: a rare entity

    International Nuclear Information System (INIS)

    Arora, Abhishek J; Arora, Richa

    2015-01-01

    Ossification of the Achilles tendon is a rare clinical entity comprising of one or more segments of variable sized ossified masses in the fibrocartilaginous substance of the tendon. The etiology of ossification of the Achilles tendon is multifactorial with recurrent trauma and surgery comprising major predisposing factors, with others being metabolic, systemic, and infectious diseases. The possibility of a genetic predisposition towards this entity has also been raised, but has not yet been proven. We present a rare case of ossification of the bilateral Achilles tendons without any history of trauma or surgery in a 48-year-old female patient

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

    Science.gov (United States)

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

    2015-01-01

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

  6. Schwannoma of the Recurrent Laryngeal Nerve : A Rare Entity

    NARCIS (Netherlands)

    de Heer, Linda M; Teding van Berkhout, F; Priesterbach, Loudy; Buijsrogge, Marc P

    Neurogenic tumors are the most common posterior mediastinal tumors in adults. Schwannomas originating from the recurrent laryngeal nerve are rare. The present study describes a 46-year-old man with a tumor in the left superior mediastinum. Because of the narrow relationship with the aorta and the

  7. Pediatric Type Follicular Lymphoma: A Rare Entity with Excellent Prognosis

    Science.gov (United States)

    2018-01-19

    YYYY) 12. REPORT TYPE 19/01/2018 Poster 4. TITLE AND SUBTITLE Pediatric -Type Follicular Lymphoma: A Rare Entity with Excellent Prognosis 6. AUTHOR(S...lymphoma is common in older adults but rare in pediatric and young adult patients. Pediatric follicular lymphoma comprises a only 6.5% of childhood... Pediatric follicular lymphoma is defined by a localized high grade appearing lymphoma that lacks these gene rearrangements. Other diagnoses to rule out

  8. Amelogenesis imperfecta and localised aggressive periodontitis: A rare clinical entity

    Directory of Open Access Journals (Sweden)

    Gayatri Gundannavar

    2013-01-01

    Full Text Available This case report presents two female patients whose chief complaint was discoloration of teeth. On careful clinical examination it was found that the patients had features of amelogenesis imperfecta and localised aggressive periodontitis. This article will give an insight of clinical and radiographic features of amelogenesis imperfecta with localised aggressive periodontitis, which is a rare clinical entity.

  9. Lichen planus hypertrophicus of the vulva - a rare entity.

    Science.gov (United States)

    Job, Anupa Mary; Kaimal, Sowmya

    2017-09-01

    Vulvovaginal lichen planus is a type of chronic inflammatory dermatosis of the vulva, usually seen as a part of widespread lichen planus. The common clinical types of vulval lichen planus include papulosquamous, erosive and pigmented types. Hypertrophic lichen planus of the vulva is a rare entity. We report a case of hypertrophic lichen planus of the vulva which clinically simulated genital warts.

  10. Pure primary small cell carcinoma of urinary bladder: A rare diagnostic entity

    Directory of Open Access Journals (Sweden)

    Sonia Gon

    2013-01-01

    Full Text Available Small cell carcinoma of the bladder is a rare, aggressive, poorly differentiated neuroendocrine neoplasm accounting for only 0.3-0.7% of all bladder tumors. Since the tumor is very rare, pathogenesis is uncertain. Small cell carcinomas of the urinary bladder are mixed with classic urothelial carcinomas or adenocarcinomas of the bladder in 68% cases, making pure primary small cell carcinoma even a rarer entity. The unknown etiology and natural history of small cell carcinoma of the urinary bladder represent a challenge both to the pathologist and urologists for its diagnosis and treatment, respectively.

  11. Adenosquamous carcinoma of vesicovaginal fistula: a rare entity.

    Science.gov (United States)

    Tabali, Rudresh; Ramkumar, Aravind

    2014-01-01

    A 56-year-old lady presented with a vesicovaginal fistula (VVF) along with past history of abdominal hysterectomy. Biopsy of the fistulous tract showed squamous cell carcinoma (SCC). Patient underwent radical cystourethrectomy, total vaginectomy, and bilateral pelvic lymph node dissection along with ileal conduit. The final histopathology report of the resected specimen showed adenosquamous carcinoma in VVF. As this is a rare entity, we are reporting this case.

  12. Adenosquamous Carcinoma of Vesicovaginal Fistula: A Rare Entity

    Directory of Open Access Journals (Sweden)

    Rudresh Tabali

    2014-01-01

    Full Text Available A 56-year-old lady presented with a vesicovaginal fistula (VVF along with past history of abdominal hysterectomy. Biopsy of the fistulous tract showed squamous cell carcinoma (SCC. Patient underwent radical cystourethrectomy, total vaginectomy, and bilateral pelvic lymph node dissection along with ileal conduit. The final histopathology report of the resected specimen showed adenosquamous carcinoma in VVF. As this is a rare entity, we are reporting this case.

  13. Reverse (posterior) cloaca with congenital rectal stenosis and pseudohermaphrodism: a rare entity with rare association

    Science.gov (United States)

    Ahmed, Intezar; Kureel, Shiv Narain; Chandra, Naveen

    2010-01-01

    Reverse (posterior) cloaca is itself a rare entity and association of this defect with other congenital syndromes or defects is extremely rare to find. To diagnose this unique anomaly one should be aware about this very infrequently encountered clinical entity. Reverse cloaca is a very rare variant of anorectal malformation in which urethra and vagina fused to form a urogenital sinus that diverts posteriorly and opens in the anterior rectal wall at the anus or immediately anterior to it. We report a neonate with the rare clinical entity of reverse cloaca type C, pseudophallus with rudimentary urethra as a component of female pseudohermaphrodism and congenital rectal stenosis. Surprisingly, there was no associated urological abnormality on ultrasonography and laparotomy. PMID:22797480

  14. Autosomal recessive anhidrotic ectodermal dysplasia: A rare entity

    Directory of Open Access Journals (Sweden)

    Sangita Ghosh

    2014-01-01

    Full Text Available We describe a case of anhidrotic ectodermal dysplasia (AED with an autosomal recessive mode of inheritance, a very rare entity, in a 2-year-old female child of two asymptomatic, consanguineous parents. Their previous child also had a similar condition. Autosomal recessive AED (AR-AED can have its full expression both in males and females and it is clinically indistinguishable from the x-linked recessive AED (XL-AED, which is the most common type of ectodermal dysplasia. Unlike the partially symptomatic carriers of XL-AED, the heterozygotes of AR-AED are phenotypically asymptomatic.

  15. Isolated celiac trunk aneurysm dissection: A rare entity

    Directory of Open Access Journals (Sweden)

    Esteban-Zubero, Eduardo

    2017-04-01

    Full Text Available Spontaneous and isolated celiac artery dissection is a rare clinical disease. It is the fourth cause of abdominal aneurysms behind those that occur in the splenic, hepatic, and superior mesenteric arteries. It is important to suspect the diagnosis of this entity whose clinical symptoms are unspecific. We report the case of a 60 year-old male treated in our hospital because of this illness, and present a review of this pathology, specially focused on the diagnosis and the different treatments available.

  16. Gastric Volvulus: A Rare Entity Case Report and Literature Review

    Science.gov (United States)

    Akhtar, Aisha; Sheikh, Abdul Ahad E; Sheikh, Abu Baker; Perisetti, Abhilash

    2018-01-01

    Gastric volvulus is a rare entity defined as an abnormal rotation of the stomach around itself. It is a diagnosis of exclusion; the clinical index of suspicion is always low and is mostly diagnosed on imaging or on the surgery table. When it occurs, it is an emergency due to the risk of strangulation and consequent gangrene of the stomach. Mesentero-axial (MA) gastric volvuli constitute one-third of all cases. Here, we are present an interesting case of acute MA gastric volvulus diagnosed with imaging and treated subsequently. PMID:29755908

  17. Solitary plasmacytoma of the mandible - a rare entity.

    Science.gov (United States)

    Baad, Rajendra; Kapse, Sonam C; Rathod, Nanita; Sonawane, Kishor; Thete, Sanjay Gangadhar; Kumar, M Naveen

    2013-06-01

    Plasma cell dyscrasias (multiple myeloma, solitary plasmocytoma of bone and extra medullary plasmocytoma) are cha¬racterized by a monoclonal neoplastic proliferation of plasma cells of which Solitary plasmocytoma of bone (SPB) is a localized form. SPB is most frequently seen in vertebrae and secondarily in long bones. Its presence in jaws is extremely rare. The malignant plasma cells express monotypic cytoplasmic immunoglobulins and plasma cell-associated antigens, with an absence of immature B-cell antigens. Here we report a unique case of plasmacytoma in the right side of mandible, a chronology for diagnosis of the lesion is also reviewed along with clinical, radiographic, histopathological and immunohistochemical evidence. How to cite this article: Baad R, Kapse S C, Rathod N, Sonawane K, Thete S G, Naveen M K. Solitary Plasmacytoma of the Mandible - A Rare Entity. J Int Oral Health 2013; 5(3):97-101.

  18. Fibrolamellar Hepatocellular Carcinoma Presenting as Obstructive Jaundice: Uncommon Presentation of a Rare Entity

    International Nuclear Information System (INIS)

    Arora, Richa

    2015-01-01

    Fibrolamellar hepatocellular carcinoma is a rare primary malignant liver tumor, significantly different from generic hepatocellular carcinoma with distinct demographics, risk factors, imaging features, histopathology and prognosis. Unlike conventional hepatocellular carcinoma, it presents in young individuals with no preexisting hepatitis or cirrhosis and does not cause elevation of serum alpha feto proteins in most cases. This paper presents a case report of this rare tumor in a young female with an unusual clinical manifestation of obstructive jaundice (which has not been reported so far) along with a review of its imaging and pathological features, with treatment options. Fibrolamellar HCC is a rare variant of classic HCC with different epidemiology, risk factors, clinical manifestations, radiological, pathological and prognostic features. Therefore, it is important to be familiar with the entity for its early diagnosis and management

  19. Mucoepidermoid carcinoma of the anterior maxilla: A rare entity

    Directory of Open Access Journals (Sweden)

    Smitha Kullaje

    2014-01-01

    Full Text Available Mucoepidermoid carcinoma is the second most common salivary gland tumor primarily involving major salivary glands and intraoral minor salivary glands. Although rare, it can occur in the jaw bones, but it is very rare in the maxillary anterior region. Here, we present a case of mucoepidermoid carcinoma occurring in maxillary anterior region in a 33-year-old male patient presented as painless swelling clinically appearing similar to a periapical or nasopalatine cyst. The final diagnosis of this lesion was made after microscopic examination of biopsy specimen.

  20. 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.

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

    Directory of Open Access Journals (Sweden)

    Dhana Lakshmi Jeyasivanesan

    2018-01-01

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

  2. Primary Adult Renal Ewing's Sarcoma: A Rare Entity

    OpenAIRE

    Ravindra Mukkunda; Ramachandran Venkitaraman; Khin Thway; Toon Min; Cyril Fisher; Alan Horwich; Ian Judson

    2009-01-01

    Background. Ewing's sarcoma of extraskeletal origin is uncommon and that is of primary renal origin in adults are rare. There is no consensus on the optimal management of Ewing's tumors of renal origin. Methods. A retrospective review of the clinical features, treatment, and outcome of adult patients with primary renal extra-skeletal Ewing's sarcoma who were treated at the Royal Marsden hospital from January 1993–December 2007 is reported. Results. Seven adult patien...

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

    Science.gov (United States)

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

    2017-01-01

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

  4. A Rare But Important Entity: Epistaxis in Infants.

    Science.gov (United States)

    DeLaroche, Amy M; Tigchelaar, Helene; Kannikeswaran, Nirupama

    2017-01-01

    Epistaxis is a common emergency department (ED) complaint; however, this entity is rare among children younger than 2 years of age. In this age group, epistaxis may be a presenting sign of a bleeding disorder or nonaccidental trauma. We present a case of a 2-month-old infant who was evaluated in the pediatric ED for epistaxis and discharged home. The infant returned 2 days later with facial swelling and irritability, and was found to have significant head trauma. In this article we review the epidemiology and differential diagnoses for epistaxis among infants, as well as the initial approach to the evaluation of this uncommon clinical entity in this age group. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: Potential etiologies for epistaxis among infants and young children include severe systemic disease and nonaccidental trauma. Given its rarity and possible clinical significance, the approach to epistaxis differs in this age group. Emergency physicians should screen for and exclude a bleeding disorder while also having a very low threshold for a nonaccidental trauma evaluation. Copyright © 2016 Elsevier Inc. All rights reserved.

  5. DPG-plus syndrome: new report of a rare entity.

    Science.gov (United States)

    Azurara, Laura; Marçal, Mónica; Vieira, Filipa; Tuna, Madalena Lopo

    2015-11-12

    Pituitary gland duplication is a particularly rare finding. Different theories have been proposed to explain its pathogenesis, however, this phenomenon is not yet totally understood. Recently, duplication of the pituitary gland (DPG)-plus syndrome has been described, associating DPG with other blastogenic defects. We present the clinical and imaging findings of a newborn girl with DPG, associated with multiple other midline anomalies, including a nasopharyngeal teratoma, palate cleft deformity, bifid nasal bridge, tongue and uvula, hypoplasia of the basis pontis and corpus callosum, duplication of the basilar artery and hypothalamic hamartoma. We describe our patient's multidisciplinary team approach and emphasise the importance of reporting upcoming cases, in order to give more insight into the understanding of this complex entity. 2015 BMJ Publishing Group Ltd.

  6. Large gangliocytic paraganglioma of the duodenum: A rare entity.

    Science.gov (United States)

    Hernández, Alejandra Gordillo; Lanuza, Eduardo Dominguez-Adame; Matias, Auxiliadora Cano; Huertas, Rosario Perez; Rodriguez, Katherine Maria Gallardo; Perez, Purificacion Gallinato; Mompean, Fernando Oliva

    2015-08-27

    Gangliocytic paragangliomas are rare tumors that almost exclusively occur within the second portion of the duodenum. Although these tumors generally have a benign clinical course, they have the potential to recur or metastasize to regional lymph nodes. The case report presented here describes a 57-year-old female patient with melena, progressive asthenia, anemia, and a mass in the second-third portion of the duodenum that was treated by local excision. The patient was diagnosed with a friable bleeding tumor. The histologic analysis showed that the tumor was a 4 cm gangliocytic paraganglioma without a malignant cell pattern. In the absence of local invasion or distant metastasis, endoscopic resection represents a feasible, curative therapy. Although endoscopic polypectomy is currently considered the treatment of choice, it is not recommended if the size of the tumor is > 3 cm and/or there is active or recent bleeding. Patients diagnosed with a gangliocytic paraganglioma should be closely followed-up for possible local recurrence.

  7. Glomus tumor of penis- A rare case

    Directory of Open Access Journals (Sweden)

    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.

  8. Headache associated with airplane travel: a rare entity.

    Science.gov (United States)

    Cherian, Ajith; Mathew, Mini; Iype, Thomas; Sandeep, P; Jabeen, Afshan; Ayyappan, K

    2013-01-01

    Airplane travel headache is rare and has recently been described as a new form of headache associated with a specific situation. Of the 1,208 patients with primary headaches attending a tertiary care neurology hospital, two (0.16%) patients satisfied the criteria for headache related to airplane travel. Both the patients fulfilled the proposed diagnostic criteria for airplane travel headache. This unique headache had a mean duration of 24 minutes, localized to the medial supraorbital region described as having an intense jabbing or stabbing character that occurred exclusively and maximally during aircraft landing or take-off, following which pain intensity subsided . This rare headache felt on aircraft descent is probably due to the squeeze effect on the frontal sinus wall, when air trapped inside it contracts producing a negative pressure leading to mucosal edema, transudation and intense pain. Use of nasal decongestants either alone or in combination with naproxen sodium prior to ascent and descent abated the headache episodes. Awareness about this unique entity is essential to provide proper treatment and avoid patient suffering.

  9. Two rare entities in the same palate lesion: hyalinizing-type clear cell carcinoma and necrotizing sialometaplasia.

    Science.gov (United States)

    Arpaci, Rabia Bozdoğan; Kara, Tuba; Porgali, Canan; Serinsoz, Ebru; Polat, Ayse; Vayisoglu, Yusuf; Ozcan, Cengiz

    2014-05-01

    Hyalinizing clear cell carcinoma is a low-grade malignant epithelial neoplasm of the salivary glands. The tumor has epithelial cells and lacks myoepithelial cells. Necrotizing sialometaplasia is a benign, self-limiting lesion of the salivary glands. The clinical and histologic features mimic those of mucoepidermoid carcinoma or squamous cell carcinoma. The importance of these entities are the rarity of both of them and their potential to be misdiagnosed as other lesions. Pathologists and clinicians should be aware of these entities to prevent misdiagnosis. This is the first clinical report of 2 rare and consecutive different entities of the same location on the hard palate to our knowledge.

  10. Bronchial carcinoid tumors: A rare malignant tumor

    African Journals Online (AJOL)

    2015-02-03

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

  11. Primary Adult Renal Ewing's Sarcoma: A Rare Entity

    Science.gov (United States)

    Mukkunda, Ravindra; Venkitaraman, Ramachandran; Thway, Khin; Min, Toon; Fisher, Cyril; Horwich, Alan; Judson, Ian

    2009-01-01

    Background. Ewing's sarcoma of extraskeletal origin is uncommon and that is of primary renal origin in adults are rare. There is no consensus on the optimal management of Ewing's tumors of renal origin. Methods. A retrospective review of the clinical features, treatment, and outcome of adult patients with primary renal extra-skeletal Ewing's sarcoma who were treated at the Royal Marsden hospital from January 1993–December 2007 is reported. Results. Seven adult patients with primary renal Ewing's sarcoma were identified. All four patients with nonmetastatic disease had radical nephrectomy and received adjuvant chemotherapy +/− radiotherapy. Two developed metastatic disease while on adjuvant chemotherapy, and one patient relapsed after 55 months. The three patients with metastatic disease at presentation did not have nephrectomy and were treated with chemotherapy. All three patients had disease progression with a dismal outcome. Only one patient in the whole group is alive and disease free. The median overall survival was 62.8 months, and the median disease-free survival in patients with nonmetastatic disease after combined modality treatment was 30.3 months. Conclusion. Primary adult renal Ewing's sarcoma is an aggressive tumor with a propensity for early metastasis. Radical nephrectomy with adjuvant combination chemotherapy produced the best results but the outlook remained poor with only one patient experiencing long disease-free survival. PMID:19478963

  12. Primary Adult Renal Ewing's Sarcoma: A Rare Entity

    Directory of Open Access Journals (Sweden)

    Ravindra Mukkunda

    2009-01-01

    Full Text Available Background. Ewing's sarcoma of extraskeletal origin is uncommon and that is of primary renal origin in adults are rare. There is no consensus on the optimal management of Ewing's tumors of renal origin. Methods. A retrospective review of the clinical features, treatment, and outcome of adult patients with primary renal extra-skeletal Ewing's sarcoma who were treated at the Royal Marsden hospital from January 1993–December 2007 is reported. Results. Seven adult patients with primary renal Ewing's sarcoma were identified. All four patients with nonmetastatic disease had radical nephrectomy and received adjuvant chemotherapy +/− radiotherapy. Two developed metastatic disease while on adjuvant chemotherapy, and one patient relapsed after 55 months. The three patients with metastatic disease at presentation did not have nephrectomy and were treated with chemotherapy. All three patients had disease progression with a dismal outcome. Only one patient in the whole group is alive and disease free. The median overall survival was 62.8 months, and the median disease-free survival in patients with nonmetastatic disease after combined modality treatment was 30.3 months. Conclusion. Primary adult renal Ewing's sarcoma is an aggressive tumor with a propensity for early metastasis. Radical nephrectomy with adjuvant combination chemotherapy produced the best results but the outlook remained poor with only one patient experiencing long disease-free survival.

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

    Science.gov (United States)

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

    2014-05-01

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

  14. Persistent Mullerian Duct Syndrome: a rare entity with a rare presentation in need of multidisciplinary management.

    Science.gov (United States)

    Da Aw, Lin; Zain, Murizah M; Esteves, Sandro C; Humaidan, Peter

    2016-01-01

    A typical male looking adolescent with a legal female gender assignment presented with haematuria. Investigations led to the diagnosis of Persistent Mullerian Duct Syndrome. The condition is indeed a rare entity that needs a multidisciplinar team management. Case hypothesis: A case of Persistent Mullerian Duct Syndrome undiagnosed at birth because karyotyping was defaulted, thus resulting in a significant impact on the legal gender assignment and psychosocial aspects. Promising future implications: The reporting of this case is important to create awareness due to its rarity coupled with the rare presentation with hematuria as a possible masquerade to menstruation. There were not only medical implications, but also psychosocial and legal connotations requiring a holistic multidisciplinary management. Copyright® by the International Brazilian Journal of Urology.

  15. Persistent Mullerian Duct Syndrome: a rare entity with a rare presentation in need of multidisciplinary management

    Directory of Open Access Journals (Sweden)

    Lin Da Aw

    Full Text Available ABSTRACT Main findings: A typical male looking adolescent with a legal female gender assignment presented with haematuria. Investigations led to the diagnosis of Persistent Mullerian Duct Syndrome. The condition is indeed a rare entity that needs a multidisciplinary team management. Case hypothesis: A case of Persistent Mullerian Duct Syndrome undiagnosed at birth because karyotyping was defaulted, thus resulting in a significant impact on the legal gender assignment and psychosocial aspects. Promising future implications: The reporting of this case is important to create awareness due to its rarity coupled with the rare presentation with hematuria as a possible masquerade to menstruation. There were not only medical implications, but also psychosocial and legal connotations requiring a holistic multidisciplinary management.

  16. 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.

  17. Rare, but challenging tumors: NET

    International Nuclear Information System (INIS)

    Ivanova, D.; Balev, B.

    2013-01-01

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

  18. Lingual fibrolipoma – a rare clinicopathological entity | Mungul ...

    African Journals Online (AJOL)

    Lipoma is a relatively common benign tumour occurring at sites of the body which are rich in adipose tissue. Due to sparsity of adipose tissue within the oral cavity, lipoma rarely presents in this region. Fibrolipoma of the tongue is a rare lesion. There are only 14 cases reported in the literature. An 85-year-old patient ...

  19. Tubercular myositis of infraspinatus: a rare clinical entity

    OpenAIRE

    Vikas Verma; Yogesh Kumar Yadav; Anuj Rastogi; Farid Mohammed

    2016-01-01

    Tuberculosis of the musculoskeletal system is generally confined to bones and joints. The surrounding soft tissue is secondarily infected. Tuberculous bursitis, tenosynovitis and primary pyomyositis are rarer manifestations of the disease. Of these, primary tuberculouspyomyositis is probably the rarest entity. We report a case of tubercular myositis of infraspinatus in an 8 year-old female who presented with pain, low grade fever, weight loss, anorexia, progressively increasing pain in the sc...

  20. Extrapelvic endometriosis: a rare entity or an under diagnosed condition?

    Science.gov (United States)

    2013-01-01

    Endometriosis is a clinical entity characterized by the presence of normal endometrial mucosa abnormally implanted in locations other than the uterine cavity. Endometriosis can be either endopelvic or extrapelvicdepending on the location of endometrial tissue implantation. Despite the rarity of extrapelvic endometriosis, several cases of endometriosis of the gastrointestinal tract, the urinarytract, the upper and lower respiratory system, the diaphragm, the pleura and the pericardium, as well as abdominal scars loci have been reported in the literature. There are several theories about the pathogenesis and the pathophysiology of endometriosis. Depending on the place of endometrial tissue implantation, endometriosis can be expressed with a wide variety of symptoms. The diagnosis of this entity is neither easy nor routine. Many diagnostic methods clinical and laboratory have been used, but none of them is the golden standard. The multipotent localization of endometriosis in combination with the wide range of its clinical expression should raise the clinical suspicion in every woman with periodic symptoms of extrapelvic organs. Finally, the therapeutic approach of this clinical entity is also correlated with the bulk of endometriosis and the locum that it is found. It varies from simple observation, to surgical treatment and treatment with medication as well as a combination of those. Virtual slides The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1968087883113362. PMID:24294950

  1. Angiomyolipoma of the cervix – report of a rare entity

    African Journals Online (AJOL)

    Arun Kumar Agnihotri

    KEY WORDS: Angiomyolipoma; Uterine cervix; PEComas; Uterine tumor. INTRODUCTIONV .... abdominal mass and dysfunctional uterine bleeding should include the AMLs even ... Tovet TP, Mateva SA, Nikolova MR, Gorchev. GA. Uterine ...

  2. Diffuse corpus callosum infarction - Rare vascular entity with differing etiology.

    Science.gov (United States)

    Mahale, Rohan; Mehta, Anish; Buddaraju, Kiran; John, Aju Abraham; Javali, Mahendra; Srinivasa, Rangasetty

    2016-01-15

    Infarctions of the corpus callosum are rare vascular events. It is relatively immune to vascular insult because of its rich vascular supply from anterior and posterior circulations of brain. Report of 3 patients with largely diffuse acute corpus callosum infarction. 3 patients with largely diffuse acute corpus callosum infarction were studied and each of these 3 patients had 3 different aetiologies. The 3 different aetiologies of largely diffuse acute corpus callosum infarction were cardioembolism, tuberculous arteritis and takayasu arteritis. Diffuse corpus callosum infarcts are rare events. This case series narrates the three different aetiologies of diffuse acute corpus callosum infarction which is a rare vascular event. Copyright © 2015 Elsevier B.V. All rights reserved.

  3. A case report on esophageal tuberculosis – A rare entity

    Directory of Open Access Journals (Sweden)

    Vatsal Khanna

    2017-01-01

    Full Text Available This is a case report of a rare form of tuberculosis in a patient presenting with dysphagia. Patient was subjected to upper gastrointestinal endoscopy, which revealed an ulcerative growth in the distal esophagus. Histopathology revealed esophageal tuberculosis. Patient was managed conservatively with Anti-Tuberculosis Treatment (ATT. Follow up endoscopy after two months revealed resolution of the growth and patient was symptomatically better. In spite of the rare nature of the disease, it can be managed effectively with ATT to avoid complications (fistula, stricture, and esophageal perforation, which might warrant surgery.

  4. Fournier′s gangrene of the penis: A rare entity

    Directory of Open Access Journals (Sweden)

    Ashutosh Talwar

    2010-01-01

    Full Text Available Fournier′s gangrene is a rare, fulminant, but and usually a localized disease of the scrotum and penis, with an occasional extension up to the abdominal wall. The usual organism is an anaerobic streptococcus synergistic with other organisms. A 45-year-old male presented with fever and pain and a brownish-black discolouration of the penis, of four days. Our case was unusual in that the penis was involved, which is very rare. Early therapy is the key, including hospitalization, debridement of the entire shaft of the penis distal to the devastated area, without excising the normal skin, parenteral broad-spectrum antibiotics, and skin grafting.

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

    Directory of Open Access Journals (Sweden)

    E. C. Castro

    2012-01-01

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

  6. The Fourth Branchial Complex Anomaly: A Rare Clinical Entity

    OpenAIRE

    Patel, Alpen B.; Hinni, Michael L.

    2011-01-01

    Fourth branchial pouch anomalies are rare congenital disorders of the neck and are a consequence of abnormal development of the branchial apparatus during embryogenesis. Failure to appropriately recognize these anomalies may result in misdiagnosis, insufficient treatment, and continued recurrence. Here, we present an unique presentation of two cases, describe their diagnosis, clinical course, and management, and review the literature regarding these interesting anomalies.

  7. The fourth branchial complex anomaly: a rare clinical entity.

    Science.gov (United States)

    Patel, Alpen B; Hinni, Michael L

    2011-01-01

    Fourth branchial pouch anomalies are rare congenital disorders of the neck and are a consequence of abnormal development of the branchial apparatus during embryogenesis. Failure to appropriately recognize these anomalies may result in misdiagnosis, insufficient treatment, and continued recurrence. Here, we present an unique presentation of two cases, describe their diagnosis, clinical course, and management, and review the literature regarding these interesting anomalies.

  8. Pseudomyxoma peritonei (PMP) – a rare entity | Ebrahim | SA ...

    African Journals Online (AJOL)

    Pseudomyxoma peritonei (PMP) is a rare complication of mucinous tumours of appendiceal or ovarian origin that results in peritoneal and omental implants. Clinical morbidity and mortality arise from the fact that copious amounts of extracellular and peritoneal mucin result in distortion and loss of function of visceral organs.

  9. Enterobius vermicularis in the nose: A rare entity

    OpenAIRE

    Kaniyur, Vishnu; Chandra Prasad, Kishore H.; Devan, P. P.; Doddamani, S. S.; Balachandran, Bharati; Kulkarni, Vikram

    2005-01-01

    A rare case of enterobius vermicularis pin-worm is reported in the nose. An 11-year-old girl presented with the vague symptoms of crawling sensation in the nose for few weeks, who had received treatment for allergic rhinitis. The nasal secretions were examined and confirmed the diagnosis of pinworm infection and treated by albendazole.

  10. Post-traumatic cervical pneumorrhachis--a rare entity.

    Science.gov (United States)

    Gill, Maneet; Sreenivas, Mukkamala; Beniwal, Rajveer Singh

    2011-02-01

    Pneumorrhachis(PR) is a rare phenomenon and post traumatic PR even more so. Presentation can vary from asymptomatic to significant neurological deficit and so the management has to be individualised. We present a case of post-traumatic cervical PR.

  11. Grade V cherubism: A rare and aggressive entity

    Directory of Open Access Journals (Sweden)

    Karthikeya Patil

    2009-01-01

    Full Text Available Cherubism is a non-neoplastic, rare, hereditary childhood disease of bone characterized by bilateral enlargement of the jaws caused by bone degradation and replacement by fibrous tissue. The affected child is reminiscent of the cherub portrayed in Renaissance art. We report the clinicopathological and radiographic features of cherubism and review of literature.

  12. Spontaneous common bile duct perforation—A rare clinical entity

    Directory of Open Access Journals (Sweden)

    Melissa Amberger

    Full Text Available Introduction: Spontaneous common bile duct perforation is an uncommon clinical entity in both adults and children. Few case reports have been published since the first clinical description in 1882. Our work has been reported in line with SCARE criteria. Presentation of case: Herein, we describe the case of a 28 year-old female who suffered spontaneous common bile duct perforation while admitted for choledocholithiasis. Discussion: The perforation occurred while in-hospital, and extensive imaging and laboratory tests characterized the disease in detail. To our knowledge, this is the first report of spontaneous common bile duct perforation witnessed from pre-perforation through definitive management. Conclusion: Physicians and Surgeons should seek out this uncommon diagnosis in the patient with suspected Choledocholithiasis who suddenly become peritoneal on physical exam so that definitive care can be expedited. Keywords: Common bile duct, Biliary peritonitis, Choledocholithiasis

  13. Giant Interfrontal Encephalocele in an Infant: A Rare Entity.

    Science.gov (United States)

    Faheem, Mohd; Singh, Sunil Kumar; Ojha, Bal Krishna; Chandra, Anil; Srivastava, Chhitij; Jaiswal, Manish; Zeeshan, Qazi

    2016-01-01

    Interfrontal encephalocele is one of the rare varieties of anterior encephalocele, and a giant interfrontal encephalocele is extremely rare. The authors could find only one case report of giant interfrontal encephalocele in the literature. Anterior encephaloceles are more prevalent in South-East Asia and some northern parts of India. Giant encephalocele poses a great challenge to neurosurgeons and neuroanesthetists during surgery, as these infants usually have a low birth weight and a large sac, thus making the infant prone to hypothermia and blood loss among other risks. We encountered a patient with a giant interfrontal encephalocele aged 1 month. The rarity of this case prompted us to this report. © 2016 S. Karger AG, Basel.

  14. Primary tubercular caecal perforation: a rare clinical entity

    Directory of Open Access Journals (Sweden)

    Moses Sonia

    2010-03-01

    Full Text Available Abstract Background Intestinal tuberculosis is a common problem in endemic areas, causing considerable morbidity and mortality. An isolated primary caecal perforation of tubercular origin is exceptionally uncommon. Case presentation We report the case of a 39 year old male who presented with features of perforation peritonitis, which on laparotomy revealed a caecal perforation with a dusky appendix. A standard right hemicolectomy with ileostomy and peritoneal toileting was done. Histopathology revealed multiple transmural caseating granulomas with Langerhans-type giant cells and acid-fast bacilli, consistent with tuberculosis, present only in the caecum. Conclusions We report this extremely rare presentation of primary caecal tuberculosis to sensitize the medical fraternity to its rare occurrence, which will be of paramount importance owing to the increasing incidence of tuberculosis all over the world, especially among the developing countries.

  15. Xanthogranulomatous urethritis and cystitis: a rare clinical and pathological entity

    Directory of Open Access Journals (Sweden)

    Lewkowicz Dorota

    2015-09-01

    Full Text Available Xanthogranulomatous inflammation of the urethra and the urinary bladder is a very rare pathological condition characterized by a chronic inflammatory infiltration composed mainly of foamy macrophages, with the presence of multinucleated giant cells. In a clinical examination, it can mimic urinary bladder carcinoma. This report presents the extremely rare case of a co-existing xanthogranulomatous urethritis and cystitis in a 64-year-old woman with recurrent dysuria, and with the suspicion of malignancy - as indicated on the basis of a cystoscopic examination. The standard treatment of this disorder is surgical resection, but in the presented case, only a diagnostic biopsy was performed. Because of the persistence of clinical symptoms, a cystoscopic examination and biopsy was repeated three times in a two year period. There was no malignancy seen in the repeated biopsies.

  16. The Fourth Branchial Complex Anomaly: A Rare Clinical Entity

    Directory of Open Access Journals (Sweden)

    Alpen B. Patel

    2011-01-01

    Full Text Available Fourth branchial pouch anomalies are rare congenital disorders of the neck and are a consequence of abnormal development of the branchial apparatus during embryogenesis. Failure to appropriately recognize these anomalies may result in misdiagnosis, insufficient treatment, and continued recurrence. Here, we present an unique presentation of two cases, describe their diagnosis, clinical course, and management, and review the literature regarding these interesting anomalies.

  17. Bifid mandibular canal: a rare or underestimated entity?

    Directory of Open Access Journals (Sweden)

    Ibrahim Nasseh

    2016-10-01

    Full Text Available One of the rare anatomical variations that can be of significant importance for the dentist is the bifid mandibular canal. Many complications can occur from this condition such as failure of anesthesia when performing inferior alveolar nerve block, difficulties during the surgical extraction of the third mandibular molar, and during implants placement. Therefore, good knowledge of this condition is essential. In this report, we describe the radiographic finding of a unilateral bifid mandibular canal.

  18. Riedel thyroiditis: Fine needle aspiration findings of a rare entity.

    Science.gov (United States)

    Weidner, Anna-Sophie; Molina, David; DeSimone, Robert A; Cohen, Marc A; Giorgadze, Tamar; Scognamiglio, Theresa; Hoda, Rana S

    2015-09-01

    Riedel thyroiditis is a rare fibrosing disorder characterized by extension of the fibroinflammatory process beyond the thyroid capsule. Due to the nature of this lesion, fine-needle aspiration often yields scant material and may be interpreted as non-diagnostic. In this report, we describe cytologic features that allow the cytopathologist to favor a diagnosis of Riedel thyroiditis, thereby guiding appropriate further work-up and management. © 2015 Wiley Periodicals, Inc.

  19. Graves’ Disease With Unilateral Involvement: A Rare Entity

    OpenAIRE

    Gülsüm Gönülalan; Mehtap Çakır

    2011-01-01

    Graves’ disease usually affects both lobes of the gland, thus, unilateral Graves’ hyperthyroidism has been reported very rarely. Here, we report a case of Graves’ disease presenting with unilateral involvement of the thyroid gland. Thyroid function tests revealed thyrotoxicosis and scintigraphy with technetium-99m showed increased diffuse unilateral radioisotope uptake in the right lobe with suppressed activity in the left lobe. The patient underwent oral antithyroid drug treatment. Graves’ ...

  20. Cavitatory mesenteric lymph node syndrome: A rare entity

    International Nuclear Information System (INIS)

    Vibhuti; Vishal, Kalia; Gill, Anakhvir

    2010-01-01

    Celiac disease is a gluten sensitive enteropathy that involves an abnormal immunological response to glutens in wheat, rye etc. It predominantly involves the small intestinal mucosa, though, extra luminal manifestations can also occur. One rare extraluminal manifestation is cavitatory mesenteric lymph node syndrome. It occurs in refractory celiac disease and is associated with poor prognosis due to various complications. The diagnosis is often made on imaging when cystic mesenteric lymph nodes with fat-fluid levels are seen and this can then be confirmed by histopathological examination. We recently had a typical case where we were able to make this diagnosis

  1. Congenital insensitivity to pain: Case report of a rare entity

    Directory of Open Access Journals (Sweden)

    Swati Dahiya

    2018-01-01

    Full Text Available Hereditary sensory and autonomic neuropathies (HSANs are a group of disorders characterized by insensitivity to noxious stimuli and autonomic dysfunction, associated with pathological abnormalities of the peripheral nerves. Five types of HSAN have been reported in literature, out of which Type V known as congenital insensitivity to pain (CIP is a rare autosomal recessive condition. Self-mutilation is an invariable feature of this disorder, involving the teeth and orofacial structures. This case report describes a case of a 6-year-old girl with CIP brought by her parents for prostheses to replace her self-extracted primary teeth.

  2. Ipsilateral Floating Hip and Floating Knee – A Rare Entity

    Directory of Open Access Journals (Sweden)

    Yashavantha Kumar

    2013-04-01

    Full Text Available Introduction: Ipsilateral floating hip and floating knee are very rare injuries. These injuries so uncommon that only three cases of similar kind have been reported. These injuries are due to high velocity injuries following motor vehicle accidents. Management of such complex injuries is a challenging task even in experienced hands as there are no standard treatment guidelines for such fractures. Case Report: We hereby report a 20 yr old male who sustained ipsilateral floating hip and ipsilateral floating knee injuries following motor vehicle accident. Patient was stabilized initially and later taken up for surgery. Patient was treated with interlocking nail for femur and tibia in the same sitting whereas acetabulam fracture was managed conservatively. At five months all the fractures united well with restoration of good range of motion in both hip and knee. Conclusion: Ipsilateral floating knee and floating hip are very rare injuries seen following high velocity motor vehicle accidents. There are no standard guidelines for treatment of those fractures as only a few cases of similar kind have been reported in literature. Early fixation and aggressive mobilization ensures fracture union and fewer complications. Keywords: Floating hip, Floating Knee, Ipsilateral.

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

    Directory of Open Access Journals (Sweden)

    Theophilus Maksha Dabkana

    2015-01-01

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

  4. Bilateral sertoli-leydig cell tumor of the ovary: A rare case report

    Directory of Open Access Journals (Sweden)

    Alam Kiran

    2009-01-01

    Full Text Available Sertoli leydig cell tumors also known as arrhenoblastoma, are a rare member of the sex cord-stromal tumor group of ovarian and testicular cancers, comprising less than 1% of all ovarian tumors, which occur in young adults and are almost always unilateral. We hereby report a case of a 17-year-old female presenting with a short history of irregular menses and an abdominal lump, which was histologically proven to be a bilateral sertoli leydig cell tumor of the ovary, an exceptionally rare entity in itself.

  5. Hemorrhagic Transformation of Scrub Typhus Encephalitis: A Rare Entity.

    Science.gov (United States)

    Kim, H-C; Yoon, K-W; Yoo, D-S; Cho, C-S

    2015-12-01

    Central nervous system (CNS) involvement of scrub typhus infection is well known. Most CNS involvement of scrub typhus infection present as meningitis or encephalitis. We report on a patient suffering from hemorrhagic transformation of intracranial lesions caused by Orientia tsutsugamushi. A 53-year-old female farmer who was infected by scrub typhus was treated with doxycycline and recovered from the systemic illness. However, headache persisted. Brain radiologic studies revealed acute intracranial hemorrhage and enhancing lesion, which implied a CNS involvement. Hemorrhagic transformation of encephalitis by scrub typhus is very rare complication and to our best knowledge, this is the first report of hemorrhagic transformation of scrub typhus encephalitis. Clinician should consider the possibility of hemorrhagic transformation of encephalitis in cases of scrub typhus infection.

  6. Infrasellar craniopharyngioma of the posterior nasal septum: a rare entity.

    Science.gov (United States)

    Chiun, Kian Chai; Tang, Ing Ping; Vikneswaran, Tharumalingam; Nurshaline Pauline, H Kipli

    2012-02-01

    To report an unusual location of infrasellar craniopharyngioma in a peadiatric patient. A six-year-old boy presented with persistent bilateral nasal obstruction for one year. Clinical examination revealed a posterior choanal mass arising from septum and the finding was confirmed by paranasal sinuses computed tomography scan. He then underwent wide local excision. Histopathological examination confirmed the diagnosis of craniopharyngioma (adamantinomatous type). There were no signs and symptoms of recurrence after a year of followup. Infracranial craniopharyngioma without sellar involvement is extremely rare. Persistent nasal obstruction without endocrine dysfunction is the common presentation. Radiological imaging is important to diagnose and assess the extent. The mainstay of treatment for infrasellar craniopharyngioma is surgery. Regular follow up is mandatory.

  7. Gingival leiomyomatous hamartoma of the maxilla: a rare entity.

    Science.gov (United States)

    Raghunath, Vandana; Manjunatha, Bhari Sharanesha; Al-Thobaiti, Yasser

    2016-05-09

    Hamartoma is a tumour-like malformation appearing as a focal overgrowth of normal cells. Leiomyomatous hamartomas (LHs) are rare in the oral cavity and commonly seen in the Japanese and less than 40 cases have been reported in the Japanese and English literature. The clinical differential diagnoses are irritational (traumatic) fibroma and congenital epulis. It has to be differentiated histopathologically from its neoplastic counterparts and mesenchymomas. Hence, we report such a case of LHs, which presented as a sessile gingival growth occurring in the midline in a 15-year-old girl. The final diagnosis was based on the histopathological appearance which was confirmed by immunohistochemical staining of various markers. A review of the literature of previous cases was also carried out. 2016 BMJ Publishing Group Ltd.

  8. A Rare Tumor of Nasal Cavity: Glomangiopericytoma

    Directory of Open Access Journals (Sweden)

    Aysegul Verim

    2014-01-01

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

  9. Primary epithelial myoepithelial carcinoma of lung, reporting of a rare entity, its molecular histogenesis and review of the literature.

    Science.gov (United States)

    Arif, Farzana; Wu, Susan; Andaz, Shahriyour; Fox, Stewart

    2012-01-01

    Primary epithelial myoepithelial carcinoma of lung is a rare entity and is thought to arise from the submucosal bronchial glands distributed throughout the lower respiratory tract. Because of the rarity of this tumor, we describe one case of epithelial myoepithelial carcinoma arising in the bronchus intermedius and presenting as an endobronchial mass. A 57-year-old male patient presented with an incidental finding of an endobronchial mass located in the lumen of the right lower lobe bronchus and caused near total luminal occlusion of the bronchus. An endobronchial carcinoid tumor was entertained clinically. Subsequently the patient underwent an uneventful videothoracoscopic lobectomy of lower and middle lobes of the right lung. Morphologically and immunohistochemically the tumor was characterized by two cell populations with epithelial and myoepithelial cells forming duct-like structure. The final diagnosis of epithelial myoepithelial carcinoma of lung was rendered.

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

    Directory of Open Access Journals (Sweden)

    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.

  11. A Rare Entity: Bilateral First Rib Fractures Accompanying Bilateral Scapular Fractures

    Directory of Open Access Journals (Sweden)

    Gultekin Gulbahar

    2015-01-01

    Full Text Available First rib fractures are scarce due to their well-protected anatomic locations. Bilateral first rib fractures accompanying bilateral scapular fractures are very rare, although they may be together with scapular and clavicular fractures. According to our knowledge, no case of bilateral first rib fractures accompanying bilateral scapular fractures has been reported, so we herein discussed the diagnosis, treatment, and complications of bone fractures due to thoracic trauma in bias of this rare entity.

  12. A Rare Entity: Bilateral First Rib Fractures Accompanying Bilateral Scapular Fractures.

    Science.gov (United States)

    Gulbahar, Gultekin; Kaplan, Tevfik; Turker, Hasan Bozkurt; Gundogdu, Ahmet Gokhan; Han, Serdar

    2015-01-01

    First rib fractures are scarce due to their well-protected anatomic locations. Bilateral first rib fractures accompanying bilateral scapular fractures are very rare, although they may be together with scapular and clavicular fractures. According to our knowledge, no case of bilateral first rib fractures accompanying bilateral scapular fractures has been reported, so we herein discussed the diagnosis, treatment, and complications of bone fractures due to thoracic trauma in bias of this rare entity.

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

    African Journals Online (AJOL)

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

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

    Directory of Open Access Journals (Sweden)

    Omer Alici

    2015-01-01

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

  15. Compound composite odontome erupting into the oral cavity: A rare entity

    Directory of Open Access Journals (Sweden)

    Sunira Chandra

    2010-01-01

    Full Text Available Odontomas are the most common odontogenic tumors. They are usually asymptomatic and are often discovered during routine radiography. Eruption of an odontome into the oral cavity is rare. Odontomas are the most common odontogenic tumors. They are usually asymptomatic and are often discovered during routine radiography. Eruption of an odontome into the oral cavity is rare. We report an unusual case of erupting compound composite odontoma. we report an unusual case of erupting compound composite odontoma.

  16. Impact of hypothyroidism on primary anal malignant melanoma: a rare entity.

    Science.gov (United States)

    Singh, Siddharth; Verma, Satyajeet; Kala, Sanjay

    2014-01-01

    Primary melanoma of the anal canal is rare and highly malignant condition, which is 1% of all invasive tumors in this site. This condition is often mistaken for benign conditions as either hemorrhoids or rectal polyp. Thyroid-stimulating hormone stimulation causes high proliferation of malignant melanoma. The association of hypothyroidism with primary malignant melanoma of anal canal is very rare. We are reporting such a very rare case.

  17. Impact of hypothyroidism on primary anal malignant melanoma: A rare entity

    Directory of Open Access Journals (Sweden)

    Siddharth Singh

    2014-01-01

    Full Text Available Primary melanoma of the anal canal is rare and highly malignant condition, which is 1% of all invasive tumors in this site. This condition is often mistaken for benign conditions as either hemorrhoids or rectal polyp. Thyroid-stimulating hormone stimulation causes high proliferation of malignant melanoma. The association of hypothyroidism with primary malignant melanoma of anal canal is very rare. We are reporting such a very rare case.

  18. Tuberculous mastitis diagnosed on cytology - case report of a rare entity

    Directory of Open Access Journals (Sweden)

    Riti Tushar Kanti Sinha

    2017-01-01

    Full Text Available Tuberculous mastititis is a rare clinical entity and usually affects women from the Indian subcontinent. It often mimics breast carcinoma and pyogenic breast abscess. Fine needle aspiration cytology (FNAC is a very essential diagnostic tool when other routine laboratory investigations are not helpful in reaching to the conclusion. Tuberculosis (TB of the breast is an uncommon presentation of TB even in countries where the incidence of pulmonary and extrapulmonary TB is high. Radiological imaging is not diagnostic.

  19. Vaginal Endosalpingiosis Case Report: A Rare Entity Presenting as Intermenstrual Bleeding

    Directory of Open Access Journals (Sweden)

    Sara Câmara

    2017-01-01

    Full Text Available Endosalpingiosis is a benign and rare entity whose pathophysiology remains unknown. It has been described in pelvic organs, the abdomen, or axillar lymph nodes. Its underrecognition has occasionally led to its misinterpretation for an adenocarcinoma. This case reports the treatment and follow-up of vaginal endosalpingiosis, presenting as a vaginal polyp in a premenopausal women with intermenstrual bleeding. To our knowledge this is the first reported case of vaginal endosalpingiosis and the second mucosal localization after bladder endosalpingiosis.

  20. Foreign body in the nasopharynx- A rare entity: a Case Report

    Directory of Open Access Journals (Sweden)

    Soumik Saha

    2013-06-01

    Full Text Available Abstract Nasopharynx is an exceptionally rare anatomical location for foreign body impactation . As an otorhinolaryngologist, one may face challenges in diagnosis and removal of foreign body from airways . A metallic foreign body after being inhaled and ultimately being lodged in the nasopharynx is a rare entity. We report a case of nasopharyngeal foreign body(NFB in a 1½ year old male child. The foreign body was diagnosed by diagnostic nasal endoscopy and documented by X-Ray of nasopharynx - lateral view [FIG.-2] and the same was removed under general anaesthesia. While keeping the child in Rose's position. Keywords foreign body nasopharynx

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

    Directory of Open Access Journals (Sweden)

    Senem Yaman Tunç

    2016-12-01

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

  2. An aggressive angiomyxoma of vulva - A rare entity - A case report

    Directory of Open Access Journals (Sweden)

    Bishnu Prasad Das

    2016-01-01

    Full Text Available Aggressive angiomyxoma is a rare, locally invasive mesenchymal tumor predominantly presenting in women of reproductive age and also having a moderate-to-high risk for local relapse. Hence, it needs to be differentiated from other mesenchymal tumors occurring in this region. We present here a case of a 40-year-old female presenting with a large, fleshy, pedunculated mass on the right labia majora.

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

    Directory of Open Access Journals (Sweden)

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

    2015-04-01

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

  4. Low-grade myofibroblastic sarcoma of the larynx: A rare entity with review of literature

    Directory of Open Access Journals (Sweden)

    Divya Khosla

    2013-01-01

    Full Text Available Mesenchymal neoplasms of the larynx are rare and make up approximately 0.3% to 1.0% of all malignancies at this location. Low grade myofibroblastic sarcoma (LGMS of larynx is a rare entity. We describe a rare case of LGMS of larynx who presented with complaint of hoarseness of voice. The patient was treated with total laryngectomy plus partial pharyngectomy followed by post-operative radiotherapy. Histopathologically, the lesion was composed of spindle cells that manifested variable cellular anaplasia and expressed smooth muscle actin (SMA and focally S-100.One of the resection limits was involved so patient was given post-operative radiotherapy. The patient is alive and disease free 14 months after surgery. The characteristic clinical, histopathological features and treatment of this case are described with a literature review.

  5. New Brain Tumor Entities Emerge from Molecular Classification of CNS-PNETs

    DEFF Research Database (Denmark)

    Sturm, Dominik; Orr, Brent A; Toprak, Umut H

    2016-01-01

    with a recurrent genetic alteration and distinct histopathological and clinical features. These new molecular entities, designated "CNS neuroblastoma with FOXR2 activation (CNS NB-FOXR2)," "CNS Ewing sarcoma family tumor with CIC alteration (CNS EFT-CIC)," "CNS high-grade neuroepithelial tumor with MN1 alteration...

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

    Science.gov (United States)

    Rutkowski, Piotr; Przybył, Joanna; Świtaj, Tomasz

    2014-08-01

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

  7. Papillary poroid hidradenoma: a distinct histopathological entity a rare case report.

    Science.gov (United States)

    Nv, Dravid; As, Gadre; Rp, Damle; Ps, Bagale; Kh, Suryawanshi

    2014-03-01

    Papillary poroid hidradenoma is a very rare entity which belongs to poroid neoplasms, which represents 10% of sudoriferous tumours. It can be easily misdiagnosed as a malignant neoplasm. A 34-years-old male presented with an asymptomatic mass over the right chest wall, below the nipple, of 18 months duration. Clinical examination revealed tense cystic swelling which revealed hypoechoic and solid components on imaging. Clinically, a malignancy was suspected and a surgical excision was done. Histology and immunoreactivity to pan cytokeratin, epithelial membrane antigen and carcino- embryonic antigen confirmed the diagnosis. We are reporting this case because of the rarity of its presentation.

  8. Pleuroperitoneal Mesothelioma: A Rare Entity on 18F-FDG PET/CT

    Science.gov (United States)

    Sahoo, Manas Kumar; Mukherjee, Anirban; Girish; Parida, Kumar; Agarwal, Krishan Kant; Bal, Chandrasekhar; Tripathi, Madhavi; Das, Chandan Jyoti; Shamim, Shamim Ahmed

    2017-01-01

    Pleuroperitoneal mesothelioma is an extremely rare entity. Only few cases are reported worldwide. We hereby represent a case of pleural mesothelioma referred for F-18-Fluorodeoxyglucose positron emission tomography/computed tomography for response evaluation. Diffuse F-18-Fluorodeoxyglucose avid peritoneal and omental thickening noted which subsequently turned out to be mesothelial involvement on peritoneal biopsy. This case demonstrates the role of F-18-Fluorodeoxyglucose positron emission tomography/computed tomography in detecting other sites of involvement in case of malignant mesothelioma. PMID:28242997

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

    Directory of Open Access Journals (Sweden)

    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.

  10. New Brain Tumor Entities Emerge from Molecular Classification of CNS-PNETs.

    Science.gov (United States)

    Sturm, Dominik; Orr, Brent A; Toprak, Umut H; Hovestadt, Volker; Jones, David T W; Capper, David; Sill, Martin; Buchhalter, Ivo; Northcott, Paul A; Leis, Irina; Ryzhova, Marina; Koelsche, Christian; Pfaff, Elke; Allen, Sariah J; Balasubramanian, Gnanaprakash; Worst, Barbara C; Pajtler, Kristian W; Brabetz, Sebastian; Johann, Pascal D; Sahm, Felix; Reimand, Jüri; Mackay, Alan; Carvalho, Diana M; Remke, Marc; Phillips, Joanna J; Perry, Arie; Cowdrey, Cynthia; Drissi, Rachid; Fouladi, Maryam; Giangaspero, Felice; Łastowska, Maria; Grajkowska, Wiesława; Scheurlen, Wolfram; Pietsch, Torsten; Hagel, Christian; Gojo, Johannes; Lötsch, Daniela; Berger, Walter; Slavc, Irene; Haberler, Christine; Jouvet, Anne; Holm, Stefan; Hofer, Silvia; Prinz, Marco; Keohane, Catherine; Fried, Iris; Mawrin, Christian; Scheie, David; Mobley, Bret C; Schniederjan, Matthew J; Santi, Mariarita; Buccoliero, Anna M; Dahiya, Sonika; Kramm, Christof M; von Bueren, André O; von Hoff, Katja; Rutkowski, Stefan; Herold-Mende, Christel; Frühwald, Michael C; Milde, Till; Hasselblatt, Martin; Wesseling, Pieter; Rößler, Jochen; Schüller, Ulrich; Ebinger, Martin; Schittenhelm, Jens; Frank, Stephan; Grobholz, Rainer; Vajtai, Istvan; Hans, Volkmar; Schneppenheim, Reinhard; Zitterbart, Karel; Collins, V Peter; Aronica, Eleonora; Varlet, Pascale; Puget, Stephanie; Dufour, Christelle; Grill, Jacques; Figarella-Branger, Dominique; Wolter, Marietta; Schuhmann, Martin U; Shalaby, Tarek; Grotzer, Michael; van Meter, Timothy; Monoranu, Camelia-Maria; Felsberg, Jörg; Reifenberger, Guido; Snuderl, Matija; Forrester, Lynn Ann; Koster, Jan; Versteeg, Rogier; Volckmann, Richard; van Sluis, Peter; Wolf, Stephan; Mikkelsen, Tom; Gajjar, Amar; Aldape, Kenneth; Moore, Andrew S; Taylor, Michael D; Jones, Chris; Jabado, Nada; Karajannis, Matthias A; Eils, Roland; Schlesner, Matthias; Lichter, Peter; von Deimling, Andreas; Pfister, Stefan M; Ellison, David W; Korshunov, Andrey; Kool, Marcel

    2016-02-25

    Primitive neuroectodermal tumors of the central nervous system (CNS-PNETs) are highly aggressive, poorly differentiated embryonal tumors occurring predominantly in young children but also affecting adolescents and adults. Herein, we demonstrate that a significant proportion of institutionally diagnosed CNS-PNETs display molecular profiles indistinguishable from those of various other well-defined CNS tumor entities, facilitating diagnosis and appropriate therapy for patients with these tumors. From the remaining fraction of CNS-PNETs, we identify four new CNS tumor entities, each associated with a recurrent genetic alteration and distinct histopathological and clinical features. These new molecular entities, designated "CNS neuroblastoma with FOXR2 activation (CNS NB-FOXR2)," "CNS Ewing sarcoma family tumor with CIC alteration (CNS EFT-CIC)," "CNS high-grade neuroepithelial tumor with MN1 alteration (CNS HGNET-MN1)," and "CNS high-grade neuroepithelial tumor with BCOR alteration (CNS HGNET-BCOR)," will enable meaningful clinical trials and the development of therapeutic strategies for patients affected by poorly differentiated CNS tumors. Copyright © 2016 Elsevier Inc. All rights reserved.

  11. Pleuroparenchymal Fibroelastosis: A New Entity within the Spectrum of Rare Idiopathic Interstitial Pneumonias

    Directory of Open Access Journals (Sweden)

    Karina Portillo

    2015-01-01

    Full Text Available Pleuroparenchymal fibroelastosis (PPFE is a rare entity that has been recently included in the official American Thoracic Society/European Respiratory Society (ATS/ERS statement in 2013 as a group of rare idiopathic interstitial pneumonias (IIPs. PPFE is characterized by pleural and subpleural parenchymal thickening due to elastic fiber proliferation, mainly in the upper lobes. The etiology of the disease is unclear, although some cases have been associated as a complication after bone marrow transplantation, lung transplantation (LT, chemotherapy, and recurrent respiratory infections. The patients usually report progressive dyspnea and dry cough and are predisposed to develop spontaneous or iatrogenic pneumothoraces after surgical lung biopsy (SLB for its diagnosis. That is why better awareness with the clinical and radiologic features can help optimal management by the multidisciplinary team. Novel invasive techniques such as cryobiopsy may become useful tools in these patients as it could spare SLB. We present the first reported cases in Spain.

  12. Myxedema Coma with Reversible Cardiopulmonary Failure: a Rare Entity in 21St Century.

    Science.gov (United States)

    Dhakal, Prajwal; Pant, Manisha; Acharya, Pranab Sharma; Dahal, Sumit; Bhatt, Vijaya Raj

    2015-09-01

    Myxedema coma, a rare entity in 21st century in developed nations, is a decompensated phase of hypothyroidism with high mortality rates. We describe a young woman with myxedema, who developed respiratory failure, congestive heart failure and significant pericardial effusion, some of the uncommon manifestations. Decreased cardiac contractility can result in cardiomyopathy and heart failure. As illustrated by this case, myxedema can also result in significant pericardial effusion due to increased vascular permeability. Myxedema can further be complicated by alveolar hypoventilation and respiratory failure secondary to the lack of central drive as well as respiratory muscle weakness. Prompt therapy with thyroid hormone replacement, glucocorticoid therapy, aggressive supportive care and management of the precipitating event can save lives and reverse the cardiopulmonary symptoms, as in our patient. Hence, physicians should have a high index of suspicion for myxedema coma in patients with unexplained cardiopulmonary failure. Our report is, therefore, aimed at bringing awareness about the rare but fatal manifestations of myxedema coma.

  13. Extranasopharyngeal angiofibroma of the posterior nasal septum: a rare clinical entity.

    Science.gov (United States)

    Atmaca, Sinan; Bayraktar, Cem; Yıldız, Levent

    2013-01-01

    Angiofibroma of extranasopharyngeal origin is very rare. Although it is usually originated from any mucosal structure in the head and neck region, maxilla is the most common involvement site. The nasal septum is an exceptional anatomic site of an angiofibroma. Surgery is the best treatment modality and recurrence is very rare. Nasal septal angiofibromas must be considered in the differential diagnosis of nasal vascular masses arising from the nasal septum. In this article, we report a 37-year-old male case with nasal septal angiofibroma who underwent surgical resection of the tumor. This is the 16th case in the literature.

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

    Directory of Open Access Journals (Sweden)

    Binesh Fariba

    2017-03-01

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

  15. 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.

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

    Directory of Open Access Journals (Sweden)

    Anirban Ghosh

    2017-12-01

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

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

    African Journals Online (AJOL)

    Arun Kumar Agnihotri

    2014-02-26

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

  18. Low-grade central osteosarcoma in proximal humerus: a rare entity

    Directory of Open Access Journals (Sweden)

    Tang F

    2017-10-01

    Full Text Available Fan Tang,1,2 Li Min,1,2 Yong Zhou,1 Yi Luo,1 Chongqi Tu1 1Department of Orthopedics, West China Hospital, Sichuan University, Chengdu, People’s Republic of China; 2Sarcoma Biology Laboratory, Center for Sarcoma and Connective Tissue Oncology, Massachusetts General Hospital, Boston, MA, USA Abstract: Low-grade central osteosarcoma is a rare subtype of tumor with low-grade malignancy. Currently, wide resection with negative resection margin is the standard treatment for this disease. The role of neoadjuvant chemotherapy in low-grade central osteosarcoma was controversial and was mostly considered for tumors containing high-grade focal areas. Local tumor recurrences often exhibited a tumor with higher histologic grade or differentiation with the potential for metastases. In low-grade central osteosarcoma, timely wide resection after definite diagnosis can result in 5-year survival for almost 90%. However, the relatively nonspecific radiological and pathological findings make diagnosis very difficult. MDM2 and CDK4 are specific and provide sensitive markers for the diagnosis of low-grade central osteosarcoma, helping to differentiate low-grade central osteosarcoma from some benign lesions, including fibrous dysplasia, bone giant cell tumor, and chondrosarcoma. Here, we report the case of a 19-year-old woman with low-grade central osteosarcoma located at the proximal humerus. The affected site was rare, but the sensitive biomarkers CDK4 and MDM2 were positive. The patient recovered well after wide tumor resection following a proximal humerus endoprosthesis replacement. Our case highlighted the management strategies in low-grade central osteosarcoma. Being familiar with radiographic features, understanding the biological characteristics, and mastering diagnostic biomarkers can help oncologists avoid embarrassing situations in treatment when this rare tumor is highly suspected, even when located at an uncommon site. The discussion in this report

  19. Simultaneous or Early Sequential Rupture of Multiple Intracranial Aneurysms: A Rare and Insufficiently Understood Entity.

    Science.gov (United States)

    Hou, Kun; Zhao, Jinchuan; Zhang, Yang; Zhu, Xiaobo; Zhao, Yan; Li, Guichen

    2016-05-01

    Simultaneous or early sequential rupture of multiple intracranial aneurysms (MIAs) is encountered rarely, with no more than 10 cases having been reported. As a result of its rarity, there are a lot of questions concerning this entity need to be answered. A 67-year-old woman was admitted to the First Hospital of Jilin University (Eastern Division) from a local hospital after a sudden onset of severe headache, nausea, and vomiting. Head computed tomography (CT) at the local hospital revealed diffuse subarachnoid hemorrhage (SAH) that was concentrated predominately in the suprasellar cistern and interhemispheric fissure. During her transfer to our hospital, she experienced another episode of sudden headache. CT on admission to our hospital revealed that the SAH was increased with 2 isolated hematomas both in the interhemispheric fissure and the left paramedian frontal lobe. Further CT angiography and intraoperative findings were in favor of early sequential rupture of 2 intracranial aneurysms. To further elucidate the characteristics, mechanism, management, and prognosis of this specific entity, we conducted a comprehensive review of the literature. The mechanism of simultaneous or early sequential rupture of MIAs is still obscure. Transient elevation of blood pressure might play a role in the process, and preventing the sudden elevation of blood pressure might be beneficial for patients with aneurysmal SAH and MIAs. The management of simultaneously or early sequentially ruptured aneurysms is more complex for its difficulty in responsible aneurysm determination, urgency in treatment, toughness in intraoperative manipulation and poorness in prognosis. Copyright © 2016 Elsevier Inc. All rights reserved.

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

    African Journals Online (AJOL)

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

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

    Directory of Open Access Journals (Sweden)

    Fatma Dursun

    2015-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Douglas Kwazneski II

    2016-01-01

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

  3. Intraosseous polymorphous low-grade adenocarcinoma of mandible: A rare entity

    Directory of Open Access Journals (Sweden)

    Gayathri Ramesh

    2012-01-01

    Full Text Available Polymorphous low-grade adenocarcinoma is a rare type of minor salivary gland malignancy. The characteristic features of these tumors are the varied histomorphology and the malignant, albeit indolent behavior. It occurs commonly in the minor salivary glands, with the palate (58.5% being the most common intra oral site. Maxillary area (2%, mandibular mucosal area (1.5% and posterior trigone region (0.5% are the least affected areas. An occasional case has been reported arising from an intraosseous location i.e the maxilla, and only two cases have been reported in the English literature originating from the mandible. One such very rare case of polymorphous low-grade adenocarcinoma of the mandible, which radiographically has a soap bubble appearance, is reported here.

  4. Krukenberg Tumor: A Rare Cause of Ovarian Torsion

    Directory of Open Access Journals (Sweden)

    Sameer Sandhu

    2012-01-01

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

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    Maithili M Kulkarni

    2016-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Niharika Bisht

    2017-12-01

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

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

    Directory of Open Access Journals (Sweden)

    Wolfgang Hohenforst-Schmidt

    2017-01-01

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

  9. Rare primary malignant tumors of the liver

    International Nuclear Information System (INIS)

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

    1991-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Asuman ARGON

    2017-05-01

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

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

    Directory of Open Access Journals (Sweden)

    Mohsen Mirmohammadsadeghi

    2015-03-01

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

  12. 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.

  13. Intraepidermal Merkel cell carcinoma: A case series of a rare entity with clinical follow up.

    Science.gov (United States)

    Jour, George; Aung, Phyu P; Rozas-Muñoz, Eduardo; Curry, Johnathan L; Prieto, Victor; Ivan, Doina

    2017-08-01

    Merkel cell carcinoma (MCC) is a rare but aggressive cutaneous carcinoma. MCC typically involves dermis and although epidermotropism has been reported, MCC strictly intraepidermal or in situ (MCCIS) is exceedingly rare. Most of the cases of MCCIS described so far have other associated lesions, such as squamous or basal cell carcinoma, actinic keratosis and so on. Herein, we describe 3 patients with MCC strictly in situ, without a dermal component. Our patients were elderly. 2 of the lesions involved the head and neck area and 1 was on a finger. All tumors were strictly intraepidermal in the diagnostic biopsies, and had histomorphologic features and an immunohistochemical profile supporting the diagnosis of MCC. Excisional biopsies were performed in 2 cases and failed to reveal dermal involvement by MCC or other associated malignancies. Our findings raise the awareness that MCC strictly in situ does exist and it should be included in the differential diagnosis of Paget's or extramammary Paget's disease, pagetoid squamous cell carcinoma, melanoma and other neoplasms that typically show histologically pagetoid extension of neoplastic cells. Considering the limited number of cases reported to date, the diagnosis of isolated MCCIS should not warrant a change in management from the typical MCC. © 2017 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.

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

    Directory of Open Access Journals (Sweden)

    Deepa Hatwal

    2014-01-01

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

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

    Directory of Open Access Journals (Sweden)

    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.

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

    Directory of Open Access Journals (Sweden)

    Liu Yang

    2012-09-01

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

  17. Melanotic MiT family translocation neoplasms: Expanding the clinical and molecular spectrum of this unique entity of tumors.

    Science.gov (United States)

    Saleeb, Rola M; Srigley, John R; Sweet, Joan; Doucet, Cedric; Royal, Virginie; Chen, Ying-Bei; Brimo, Fadi; Evans, Andrew

    2017-11-01

    MiT family translocation tumors are a group of neoplasms characterized by translocations involving MiT family transcription factors. The translocation renal cell carcinomas, TFE3 (Xp11.2) and TFEB (t6;11) are known members of this family. Melanotic Xp11 translocation renal cancer is a more recently described entity. To date only 14 cases have been described. It is characterized by a distinct set of features including a nested epithelioid morphology, melanin pigmentation, labeling for markers of melanocytic differentiation, lack of labeling for markers of renal tubular differentiation, predominance in a younger age population and association with aggressive clinical behavior. There are noted similarities between that entity and TFE3 associated PEComas. There are no cases reported of equivalent melanotic TFEB translocation renal cancer. We report 2 rare cases of melanotic translocation renal neoplasms. The first is a melanotic TFE3 translocation renal cancer with an indolent clinical course, occurring in a patient more than 3-decades older than the usual average age in which such tumors have been described. The other case is, to our knowledge, the first reported melanotic TFEB translocation cancer of the kidney. Both cases exhibit the same H&E morphology as previously reported in melanotic translocation renal cancers and label accordingly with HMB45 and Melan-A. While the TFE3 melanotic tumor lacked any evidence of renal tubular differentiation, the TFEB melanotic cancer exhibited some staining for renal tubular markers. Based on the unique features noted above, these two cases expand the clinical and molecular spectrum of the melanotic translocation renal cancers. Copyright © 2017 Elsevier GmbH. All rights reserved.

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

    Science.gov (United States)

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

    2017-01-01

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

  19. Primary angiosarcoma of the testis: report of a rare entity and review of the literature

    Directory of Open Access Journals (Sweden)

    Rao Uma NM

    2007-07-01

    Full Text Available Abstract Background Primary testicular angiosarcomas are extremely rare, and their clinicopathologic features are not well described. Our objective was to further define the clinical features and pathologic spectra of primary testicular angiosarcomas. Methods Six previously reported case reports were identified in the English language medical literature using MEDLINE and a subsequent bibliographic search of all pertinent reports and reviews was performed. After excluding 2 cases because they did not involve the testis, we identified 4 previously reported cases of true primary testicular angiosarcoma. We also searched the electronic medical archival records of our institution and identified one additional unreported case of true primary testicular angiosarcomas. Data were extracted on the demographics, predisposing factors, clinical presentation, gross pathology, microscopic pathology, immunophenotype, therapy, and outcomes of each of these 5 cases of true primary testicular angiosarcomas. Results Primary testicular angiosarcomas were found at a mean age of 43.4 years. None of the cases was associated with exposure to radiation, arsenic, thorium dioxide, or vinyl chloride. However, 1 case was associated with hydrocele. It typically presented with painless mass (mean size, 6.3 cm. Histologically, all showed classic anastomosing channels lined by plump hyperchromatic cells, though most showed epithelioid cytology and some showed solid architectural pattern. One patient had multiple metastatic recurrences but eventual outcome was not available, and 1 patient died a month after diagnosis from stroke but no autopsy was performed. The remaining 3 patients were alive at the time of publication of their respective cases (mean, 17 months. Conclusion Primary testicular angiosarcomas are typically rare tumors of men of all ages that appear to segregate into 2 groups; one associated with teratoma and occurring in young people, and the other occurring in the

  20. Synovial Sarcoma-A Rare Tumor of the Larynx

    Directory of Open Access Journals (Sweden)

    Ghodrat Mohammadi

    2016-05-01

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

  1. Role of Imaging and Cytogenetics in Evaluation of DiGeorge Syndrome - A Rare Entity in Clinical Practice.

    Science.gov (United States)

    Ramachandran, Rajoo; Babu, Sellappan Rajamanickam; Ilanchezhian, Subramanian; Radhakrishnan, Prabhu Radhan

    2015-01-01

    DiGeorge syndrome is a congenital genetic disorder that affects the endocrine system, mainly the thymus and parathyroid glands. The syndrome produces different symptoms, which vary in severity and character between patients. It manifests with craniofacial dysmorphism and defects in the heart, parathyroid, and thymus. Patients can present with a palatal deformity and nasal speech. This rare entity is caused mainly due to deletion of chromosome 22q11.2. Radiographic evaluation of DiGeorge syndrome is necessary to define aberrant anatomy, evaluate central nervous system, craniofacial abnormalities, musculoskeletal system, and cardiothoracic contents. It also helps in planning surgical procedures and surgical reconstructions. We report a case of DiGeorge syndrome in a 4-month-old neonate and discuss the clinical, imaging, and cytogenetic findings that helped in the diagnosis of this rare entity.

  2. Role of Imaging and Cytogenetics in Evaluation of DiGeorge Syndrome - A Rare Entity in Clinical Practice

    Directory of Open Access Journals (Sweden)

    Rajoo Ramachandran

    2015-01-01

    Full Text Available DiGeorge syndrome is a congenital genetic disorder that affects the endocrine system, mainly the thymus and parathyroid glands. The syndrome produces different symptoms, which vary in severity and character between patients. It manifests with craniofacial dysmorphism and defects in the heart, parathyroid, and thymus. Patients can present with a palatal deformity and nasal speech. This rare entity is caused mainly due to deletion of chromosome 22q11.2. Radiographic evaluation of DiGeorge syndrome is necessary to define aberrant anatomy, evaluate central nervous system, craniofacial abnormalities, musculoskeletal system, and cardiothoracic contents. It also helps in planning surgical procedures and surgical reconstructions. We report a case of DiGeorge syndrome in a 4-month-old neonate and discuss the clinical, imaging, and cytogenetic findings that helped in the diagnosis of this rare entity.

  3. Spinal angiolipomas: A puzzling case and review of a rare entity

    Directory of Open Access Journals (Sweden)

    Faris Shweikeh

    2017-01-01

    Full Text Available Patients with spinal epidural abscesses (SEAs may have a variable presentation. Such an infection has a typical appearance on magnetic resonance imaging (MRI and enhances with gadolinium. We present a case that was a diagnostic challenge where pre- and intra-operative findings resulted in conflicting impressions. The mimicker was a spinal angiolipoma (SAL. The authors then provide a thorough review of this rare spinal neoplasm. A 55-year-old man presented with back pain, paresis, paresthesia, and urinary retention. MRI was indicative of a longitudinal epidural thoracic mass with a signal homogeneous to nearby fat, curvilinear vessels, and lack of enhancement. Although at emergent surgery, the lesion was found to contain abundant purulent material. Microbiology was positive for methicillin-resistant Staphylococcus aureus and consistent with SEA without evidence of neoplasia. While the imaging features were suggestive of an angiolipoma, the findings at surgery made SEA more likely, which were validated histopathologically. The diagnosis of SEA is often clear-cut, and the literature has reported only a few instances in which it masqueraded as another process such as lymphoma or myelitis. The case highlights SEA masquerading as an angiolipoma, and further demonstrates to clinicians that obtaining tissue diagnosis plays a crucial role diagnostically and therapeutically. SALs, on the other hand, are slow-growing tumors that can be infiltrating or noninfiltrating. They typically present with chronic symptoms and T1-MRI shows an inhomogeneous picture. Complete surgical excision is standard of care and patients tend to do well afterward.

  4. Metastasis of the epididymis and spermatic cord from pancreatic adenocarcinoma: A rare entity. Description of a case and revision of literature.

    Science.gov (United States)

    Di Franco, Carmelo Agostino; Rovereto, Bruno; Porru, Daniele; Zoccarato, Valeria; Regina, Cesare; Cebrelli, Tiziano; Fiorello, Nicolò; Viglio, Alessandra; Galvagno, Lavinia; Marchetti, Carlo; Ringressi, Andrea; Barletta, Davide; Giliberto, Giovanni

    2018-03-31

    Metastatic epididymal and spermatic cord adenocarcinoma from epithelial tumors are a rare condition. The most frequent primary cancers are prostate, lung, kidney, gastrointestinal tumors and breast. In literature, there are very low number of cases reporting metastasis from pancreatic cancer to epididymis and spermatic cord. We report a case of 70-years old man with history of left orchiectomy for undescended testicle, who presented to our department with a palpable nodule in the right scrotum. Scrotal ultrasound revealed an inhomogeneous hypoechoic nodule of epididymis and/or spermatic cord. Neoplastic markers showed high levels of CEA (carcinoembryonic antigen) and bHCG (beta Human Chorionic Gonadotropin). The patient underwent right surgical scrotal exploration with orchifunicolectomy. Pathologic examination revealed pathologic tissue showing rare glandular structures. Immunohistochemistry profile was compatible with malign epithelial neoplasm with glandular differentiation. Total body CT-scan revealed pathologic tissue in pancreas between head and body and a suspect pathologic lesion in liver and 18-FDG PET-scan confirmed the pancreatic neoplastic mass and a suspect secondary hepatic lesion. Biopsy of pancreatic pathologic area was positive for ductal pancreatic adenocarcinoma. The patient was sent to oncologic evaluation and started chemotherapy. Malignancies of epididymis and spermatic cord are rare entities and, in literature, very low number of cases of metastasis from pancreatic carcinoma to epididymis and spermatic cord are described. Early differential diagnosis is fundamental mostly in those patients with age range unusual for testis cancers.

  5. Orbital Chondroma: A rare mesenchymal tumor of orbit

    Directory of Open Access Journals (Sweden)

    Ruchi S Kabra

    2015-01-01

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

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

    Science.gov (United States)

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

    2014-05-01

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

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

    Directory of Open Access Journals (Sweden)

    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

  8. Rare giant cell tumor involvement of the olecranon bone

    Directory of Open Access Journals (Sweden)

    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.

  9. Nivolumab and Ipilimumab in Treating Patients With Rare Tumors

    Science.gov (United States)

    2018-05-14

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

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

    Science.gov (United States)

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

    2012-10-01

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

  11. Angiofibroma on cheek mucosa: A rare entity and its management with laser

    Directory of Open Access Journals (Sweden)

    Rajesh Kumar Thakur

    2014-01-01

    Full Text Available A rare presentation of an angiofibroma in the oral cavity is reported, which was treated with a diode laser. The benefits of laser treatment include bloodless procedure with instant precise coagulation of vessels. Although rare and unusual, it is suggested that angiofibroma should be included as one of the differential diagnoses of soft tissue swellings in the oral cavity.

  12. A case of Occupational Methemoglobinemia (MetHb: A Rare Entity and Unique Treatment

    Directory of Open Access Journals (Sweden)

    Suvarna Patil

    2017-11-01

    Full Text Available Methemoglobinemia is acute emergency which have precise and effective treatment if instituted in time. Methemoglobinemia due to chemical exposure is a known entity. But it required a high index of suspicion to look for it in busy casualty. Treatment with methylene blue is safe and truly lifesaving if instituted in time. Here we are presenting a case of Occupational methemoglobinemia who was treated successfully.

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

    Directory of Open Access Journals (Sweden)

    Dibakar Podder

    2015-04-01

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

  14. A Rare Entity: Bilateral First Rib Fractures Accompanying Bilateral Scapular Fractures

    OpenAIRE

    Gulbahar, Gultekin; Kaplan, Tevfik; Turker, Hasan Bozkurt; Gundogdu, Ahmet Gokhan; Han, Serdar

    2015-01-01

    First rib fractures are scarce due to their well-protected anatomic locations. Bilateral first rib fractures accompanying bilateral scapular fractures are very rare, although they may be together with scapular and clavicular fractures. According to our knowledge, no case of bilateral first rib fractures accompanying bilateral scapular fractures has been reported, so we herein discussed the diagnosis, treatment, and complications of bone fractures due to thoracic trauma in bias of this rare en...

  15. Tentorial and dural calcification with tertiary hyperparathyroidism: a rare entity in chronic renal failure

    International Nuclear Information System (INIS)

    Dorenbeck, U.; Bretschneider, T.; Feuerbach, S.; Leingaertner, T.; Kraemer, B.K.

    2002-01-01

    A case of rare calcification of the tentorium cerebelli, the thoracal dura mater of the spine, and the sclera in an adult male patient with tertiary hyperparathyroidism is presented. The often reported feature in the skull is that of a combination of osteopenia and osteosclerosis giving a granular bone texture. Extensive dura calcification with this condition has very rarely been reported. It is the aim of this paper to document the latter in a patient with chronic renal failure and tertiary hyperparathyroidism. (orig.)

  16. Astroblastoma: beside being a tumor entity, an occasional phenotype of astrocytic gliomas?

    Directory of Open Access Journals (Sweden)

    Mellai M

    2015-02-01

    diagnostic assessment as astroblastoma, and it is hypothesized that the typical perivascular distribution of cells may not indicate a separate and unique tumor entity, but may be a peculiarity that can be acquired by astrocytic gliomas when an unknown cause from the tumor microenvironment influences the relationship between vessels and tumor cells. Keywords: gliomas, cell lines, histology, genetics

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

    Science.gov (United States)

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

    2016-11-15

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

  18. Nasal Septal Angiofibroma in a Post-Menopausal Woman: A Rare Entity

    Science.gov (United States)

    Dayana, Farah; Fadzilah, Fazalina Mohd; Gendeh, Balwant Singh

    2015-01-01

    Juvenile angiofibromas (JAs) are well-characterised in literature, arising typically in the posterolateral wall of the nasal cavity of young males. Numerous theories have been proposed to explain the occurrence of this unique and rare tumour. Angiofibromas originating in other sites within the head and neck have been described but this is exceedingly rare, constituting less than 2% of all diagnosed cases. Extranasopharyngeal angiofibroma is a rare lesion, and more importantly, controversial. It is not known whether it is actually a relative of the well-known JA that is seen exclusively in adolescent males. We present the case of a post-menopausal woman with unilateral nasal obstruction who was unexpectedly diagnosed as nasal septal angiofibroma. PMID:26816925

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

    Directory of Open Access Journals (Sweden)

    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

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

    Directory of Open Access Journals (Sweden)

    Soheila Aminimoghaddam

    2012-11-01

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

  1. Vulvar Lobular Capillary Hemangioma: A Rare Location for a Frequent Entity

    Directory of Open Access Journals (Sweden)

    F. Abreu-dos-Santos

    2016-01-01

    Full Text Available Lobular capillary hemangioma, or pyogenic granuloma, is an acquired hemorrhagic benign vascular lesion of the skin and mucous membranes. The pyogenic granuloma of the vulva is a rare finding and a limited number of case reports are available in the literature. To the best of our knowledge this is the first case described as a single pyogenic granuloma on the vulva.

  2. Pleomorphic lipoma of the neck in an infant: A rare clinical entity

    Directory of Open Access Journals (Sweden)

    Ram Samujh

    2017-01-01

    Full Text Available Lipomas are rare in the pediatric age group. A 10-month-old male child presented with an asymptomatic neck mass which was evaluated and excised completely. Histopathology was consistent with pleomorphic lipoma, not previously reported in children.

  3. The Amyand's Hernia: A Rare Clinical Entity Diagnosed by Computed Tomography.

    Science.gov (United States)

    Keskin, Suat; Simşek, Cihan; Keskin, Zeynep

    2013-01-01

    Amyand's hernia, named for the first person to describe an inguinal hernia containing the vermiform appendix, is an uncommon variant of an inguinal hernia. Amyand's hernia is an extremely rare condition and is often misdiagnosed. Traditionally, these hernias have been diagnosed at surgery but are increasingly diagnosed by abdominal computed tomography (CT) scans. CT of the abdomen may help in guiding the diagnosis.

  4. Infratemporal fossa extension of primary parotid squamous cell carcinoma: a rare entity with diagnosis dilemma

    International Nuclear Information System (INIS)

    Karaf, J.H.; Gendeh, B.S.; Yunus, M.R.M.

    2015-01-01

    Primary squamous cell carcinoma of parotid gland is rare but it is an aggressive neoplasm that can have extension into the infratemporal fossa. We report a 60-year-old female presenting with toothache over left lower molar causing a diagnostic dilemma. (author)

  5. Post appendectomy acalculus bilateral ureteric obstruction: A rare entity in children

    Directory of Open Access Journals (Sweden)

    Vipul Gupta

    2013-01-01

    Full Text Available Bilateral acalculus ureteric obstruction is described as rare sequelae of acute appendicitis in two paediatric patients aged 6 and 11 years presented with features of anuria. Imaging and endoscopic evaluation confirmed bilateral ureteric obstruction secondary to bladder wall oedema as an inflammatory reaction to appendix. Both cases recovered following bilateral ureteric stenting and are doing well.

  6. Adult-onset cystic hygroma: A case report of rare entity.

    Science.gov (United States)

    Bahl, Sumit; Shah, Vandana; Anchlia, Sonal; Vyas, Siddharth

    2016-01-01

    Cystic hygroma is a benign congenital malformation of the lymphatic system that occurs in infant or children younger than 2 years of age. Although cystic hygroma is well recognized in pediatric practice, it seldom presents de novo in adulthood. These are commonly present in head and neck but can be present anywhere. Cystic hygroma is very rare in adults, but it should be considered in the differential diagnosis of adult neck swellings. Patients presenting with a painless, soft, fluctuant, and enlarging neck mass should have a careful history and physical examination along with radiological imaging to assist with diagnosis. Surgical intervention is the treatment of choice for this rare condition. Here, we are reporting a case of cystic hygroma in a 32-year-old male patient in the neck region. The objectives of this case report are to discuss the clinical presentation, diagnosis, histopathological findings and management of this malformation.

  7. Intrinsic brainstem schwannoma – A rare clinical entity and a histological enigma

    Directory of Open Access Journals (Sweden)

    Anil Kumar Sharma

    2016-01-01

    Full Text Available Intraparenchymal schwannomas arising in the brainstem are very rare, and only eight cases have been reported in literature till now. We report an intraparenchymal brainstem schwannoma presenting with the classical clinical presentation of an intrinsic brainstem lesion, and discuss its clinicoradiological characteristics and histological origins. We highlight the importance of an intraoperative frozen section diagnosis in such cases. Intraoperative tissue diagnosis significantly may alter the surgical strategy, which should be aimed at near total intracapsular decompression of the schwannoma.

  8. Idiopathic pleuroparenchymal fibroelastosis, a rare entity of interstitial pneumonia: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Park, Jong Chun; Chae, Eun Jin; Song, Joon Seon [University of Ulsan College of Medicine, Asan Medical Center, Seou (Korea, Republic of)

    2014-04-15

    Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently described, very rare type of fibrotic interstitial lung disease predominantly involving subpleural areas of both upper lungs. IPPFE has distinctive radiologic and pathologic features: progressive subpleural opacity with fibrotic changes, predominantly in upper lungs, and dense elastic component on histology. We experienced one case of surgically confirmed IPPFE, with progression of radiologic findings on the serial CT examinations. We herein report the characteristic radiologic features of IPPFE with pathologic and clinical manifestations.

  9. The Amyand’s Hernia: A Rare Clinical Entity Diagnosed by Computed Tomography

    Directory of Open Access Journals (Sweden)

    Suat Keskin

    2013-01-01

    Full Text Available Amyand’s hernia, named for the first person to describe an inguinal hernia containing the vermiform appendix, is an uncommon variant of an inguinal hernia. Amyand’s hernia is an extremely rare condition and is often misdiagnosed. Traditionally, these hernias have been diagnosed at surgery but are increasingly diagnosed by abdominal computed tomography (CT scans. CT of the abdomen may help in guiding the diagnosis.

  10. Basaloid carcinoma of the pancreas--clinicopathological presentation and oncogenetic snapshot of a rare entity.

    Science.gov (United States)

    Szasz, A Marcell; Szirtes, Ildiko; Tihanyi, Balazs; Barkaszi, Bernadett; Baranyai, Zsolt; Tihanyi, Tibor; Harsanyi, Laszlo; Timar, Jozsef; Kulka, Janina

    2015-02-01

    We report a case of basaloid pancreatic carcinoma with clinical, pathological, and genomic data. The 73-year-old male patient had jaundice, acholic stool, diarrhea, weight loss, and a large, painless gall bladder. His GGT was highly elevated. The pancreatic head contained a tumor, which was resected by partial pancreatoduodenectomy with pancreato-gastric anastomosis, cholecystectomy, and lymphadenectomy. On gross examination, a 3.8-cm white firm nodule was found, which microscopically was composed of basaloid cell nests with a less than usual desmoplastic stromal background and focally PANIN. Immunohistochemical profile displayed strong CK5/6, CK19, p63, EGFR, vimentin, and evident CK14 expression and absence of expression of CK7, chromogranin, synaptophysin, and BRCA1. A high Ki-67 index and p53 expression were noted. Sequencing of the most frequent 46 oncogenes with ionTorrent (AmpliSeq PCR) method identified PIK3CA, KRAS, and TP53 genes as drivers and variants of the FGFR3, PDGFRA, KIT, KDR, EGFR, RET, and ATM genes. The tumor we report displays histopathological appearances similar to the previously described case and a genomic landscape fitting to the general population of pancreatic carcinomas. We hypothesize that this tumor may belong to the group of DNA damage repair-deficient pancreatic carcinoma subgroup.

  11. Lipoleiomyoma of the Uterus and Primary Ovarian Leiomyoma in a Postmenopausal Woman: Two Rare Entities in the Same Individual

    Directory of Open Access Journals (Sweden)

    Sefa Kelekci

    2015-01-01

    Full Text Available Uterine lipoleiomyomas are rare benign tumours that are composed of various mixtures of smooth muscle and mature fat tissue. Leiomyomas, which arise primarily in the ovary, are extremely rare tumours that account for 0.5–1% of all benign ovarian tumours. To the best of our knowledge, we present the first case of an ovarian leiomyoma coexisting with a uterine lipoleiomyoma in the postmenopausal period. A 59-year-old, gravida 4, para 3, postmenopausal woman exhibited pelvic discomfort and increased frequency of micturition. A pelvic examination revealed a solid, tender mass on the left side that could not be clearly separated from the uterus. She underwent a laparotomy with an initial diagnosis of a left ovarian mass. She had previously undergone a total abdominal hysterectomy and bilateral salpingo-oophorectomy. A histopathological examination revealed a uterine lipoleiomyoma, composed of variable amounts of smooth muscle cells and mature adipocytes and a right ovarian leiomyoma composed of interlacing bundles and fascicles of spindle cells. The coexistence of these two rare entities in the same individual may represent a common pathway as a stimulating agent. This case may help to clarify the pathogenesis of these lesions.

  12. Salivary duct cyst on lower lip: A rare entity and literature review

    OpenAIRE

    Tandon, Ankita; Sircar, Keya; Chowdhry, Aman; Bablani, Deepika

    2014-01-01

    Mucocele forms because of salivary gland mucous extravasation or retention and is usually related to trauma in the area of the lower lips. Salivary duct cyst, however, is a type of mucous retention cyst which is almost never located on the lower lip. The aim of this paper is to report this extremely rare salivary duct cyst present on the lower lip and to critically review the literature to build important concepts that would help clinicians in the diagnosis and treatment of this pathology.

  13. Salivary duct cyst on lower lip: A rare entity and literature review.

    Science.gov (United States)

    Tandon, Ankita; Sircar, Keya; Chowdhry, Aman; Bablani, Deepika

    2014-09-01

    Mucocele forms because of salivary gland mucous extravasation or retention and is usually related to trauma in the area of the lower lips. Salivary duct cyst, however, is a type of mucous retention cyst which is almost never located on the lower lip. The aim of this paper is to report this extremely rare salivary duct cyst present on the lower lip and to critically review the literature to build important concepts that would help clinicians in the diagnosis and treatment of this pathology.

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

    Directory of Open Access Journals (Sweden)

    Mehmet Akif Özgül

    2014-01-01

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

  15. Cavernous sinus thrombosis following dental extraction: a rare case report and forgotten entity.

    Science.gov (United States)

    Aggarwal, Karun; Rastogi, Sanjay; Joshi, Atul; Kumar, Ashish; Chaurasia, Archana; Prakash, Rajat

    2017-10-01

    Prior to the advent of efficacious antimicrobial agents, the mortality rate from cavernous sinus thrombosis (CST) was effectively 100%. There have been very few reports of CST associated with tooth extraction. A 40-year-old female presented to the emergency room with swelling over the right side of the face and history of extraction in the upper right region by an unregistered dental practitioner. The patient presented with diplopia, periorbital ecchymosis, and chemosis of the right eye. A computed tomography scan revealed venous dilatation of the right superior ophthalmic vein. The patient was immediately treated with incision and drainage, intravenous antibiotics, and heparin (low molecular weight). Unfortunately, the patient died two days after surgery due to complications from the disease. CST is a rare disease with a high mortality rate. Therefore, dental health education in rural areas, legal action against unregistered dental practitioners, early diagnosis, and aggressive antibiotic treatment can prevent future mortality resulting from CST.

  16. Aggressive NK-cell leukemia: A rare entity with diagnostic and therapeutic challenge

    Directory of Open Access Journals (Sweden)

    Alia Nazarullah

    2016-06-01

    Full Text Available Aggressive natural killer cell leukemia (ANKL is a rare neoplasm of mature natural killer cells, with an extremely poor overall survival, which is almost always EBV related, with majority of cases reported in East Asia. Here we report the case of an ANKL presenting in a young Hispanic male with secondary hemophagocytosis. Aggressive clinical course, high EBV DNA levels and leukemic presentation, often with associated hemophagocytosis, should raise suspicion of an NK/T-cell neoplasm like ANKL. Due to significant diagnostic overlap with extranodal NK/T-cell lymphoma, nasal type (ENKL, accurate diagnostic classification is crucial due to differing treatment and prognosis. L-asparaginase including chemotherapy followed by allogeneic stem cell transplantation appears to slightly prolong overall survival, but relapse is almost inevitable. Clinical monitoring of EBV DNA levels shows good correlation with disease activity.

  17. Toxoplasmosis of spinal cord in acquired immunodeficiency syndrome patient presenting as paraparesis: A rare entity

    Directory of Open Access Journals (Sweden)

    Sachin R Agrawal

    2014-01-01

    Full Text Available Although brain has been the most common site for toxoplasma infection in acquired immunodeficiency syndrome patients, involvement of spinal cord by toxoplasma has been rarely found. Spinal cord toxoplasmosis can present as acute onset weakness in both lower limbs associated with sensory and bladder dysfunction. A presumptive diagnosis can be made in patients with CD4 count <100/mm 3 based on a positive serum Toxoplasma gondii IgG antibodies, no recent prophylaxis against toxoplasmosis, intramedullary ring enhancing lesion in spinal cord supported by similar lesions in brain parenchyma. Institutions of antitoxoplasma treatment in such patients result in prompt clinical response and therefore avoiding the need of unnecessary invasive diagnostic tests. Here, we report a case of toxoplasmic myelitis in immunocompromised patient presenting as myelopathy who showed significant clinical improvement after starting antitoxoplasma treatment. Hence toxoplasmic myelitis should be considered in toxoplasma seropositive immunocompromised patients presenting as myelopathy and imaging studies showing ring enhancing intramedullary lesion.

  18. Neuroendocrine Merkel cell nodal carcinoma of unknown primary site: management and outcomes of a rare entity.

    Science.gov (United States)

    Kotteas, E A; Pavlidis, N

    2015-04-01

    Merkel cell nodal carcinoma of unknown primary (MCCUP) is a rare neuroendocrine tumour with distinct clinical and biological behaviour. We conducted a review of retrospective data extracted from 90 patients focusing on the management and outcome of this disease. We also compared life expectancy of these patients with the outcome of patients with known Merkel primaries and with neuroendocrine cancers of unidentifiable primary. There is a limited body of data for this type of malignancy, however, patients with Merkel cell nodal carcinoma of unknown primary site, seem to have better survival when treated aggressively than patients with cutaneous Merkel tumours of the same stage and equal survival with patients with low-grade neuroendocrine tumour of unknown origin. The lack of prospective trials, and the inadequate data, hamper the management of these tumours. Establishment of treatment guidelines is urgently needed. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  19. Dental Management of a Pediatric Patient with Moyamoya Syndrome: A Rare Clinical Entity.

    Science.gov (United States)

    Ko, Brittany L; Unkel, John H

    2018-01-01

    Moyamoya disease (MMD) is a rare cerebrovascular disorder involving progressive constriction of the internal carotid artery and its branches. The disease has a particularly aggressive course in very young patients, and early surgical intervention is often necessary to prevent permanent neurological damage. MMD patients have an increased risk of stroke development, which may be provoked by pain or anxiety. Currently, no reports of pediatric patients with MMD exist in the dental literature. The purpose of this paper was to discuss the dental management of a two-year-old with moyamoya disease who presented with early childhood caries and dental fear, offering recommendations for dental providers with emphasis on stroke prevention, collaboration with the medical team, anesthesia considerations for patients with increased stroke risk, and the challenges to maintain the oral health of a patient undergoing complex medical treatment.

  20. A Rare Entity: Traumatic Thoracic Aortic Injury in a Patient with Aberrant Right Subclavian Artery.

    Science.gov (United States)

    Patel, Hiten Mohanbhai; Banerjee, Shubhabrata; Bulsara, Shahzad; Sahu, Tapish; Sheorain, Virender K; Grover, Tarun; Parakh, Rajiv

    2017-05-01

    Aberrant right subclavian artery is an uncommon entity incidence ranging from 0.5 to 2.5%. Management of thoracic aortic injury in the presence of such anomalies can be a challenge. We present here a case of traumatic aortic injury, which was incidentally found to have an asymptomatic aberrant right subclavian artery. The patient was managed by an endovascular repair of thoracic aortic injury with an endograft and a right carotid to subclavian artery bypass as a hybrid procedure. A 40-year male patient was brought to the emergency in shock with an alleged history of road traffic accident an hour back. After initial resuscitation as per advance trauma life support protocol, imaging revealed thoracic aortic injury with aberrant right subclavian artery with multiple rib and bilateral humerus fracture. After primary stabilization of arm fractures, the patient was shifted to a hybrid operation room. As the aortic injury was within 10 mm of the origin of both subclavian arteries, it was decided to cover the origin of both subclavian arteries and land the endograft distal to the left carotid artery origin. Since there was a right dominant vertebral artery on imaging, right carotid to right subclavian artery bypass was done with expanded polytetrafluoroethylene graft to prevent posterior circulatory stroke along with thoracic endovascular aortic repair to seal the thoracic aortic injury. After endovascular repair of thoracic aortic injury, left subclavian artery perfusion was maintained through left vertebral artery; and hence, revascularization of left subclavian artery was deferred. After management of all fractures, the patient was discharged 3 weeks after the date of admission without any complications. At 6 months follow-up, patient was stable and images showed patent bypass graft and sealed aortic injury. In a trauma setting with multiple injuries, hybrid procedure with a thoracic endograft is associated with low mortality and morbidity; hence, it is the treatment

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

    Science.gov (United States)

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

    2018-01-23

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

  2. Bone Involvement by Adenocarcinoma of the Uterine Cervix: A Rare Entity

    International Nuclear Information System (INIS)

    Crisci, Vincenzina; Mainenti, Pier Paolo; Corvino, Fabio; Lauria, Rossella; Maurea, Simone

    2015-01-01

    Adenocarcinoma is the second most frequent cancer of the uterine cervix after squamous carcinoma, and the most frequent histotype is the mucinous one. Endo-cervical adenocarcinoma accounts for about 10–30% of all cervical cancers and clinically the lesion can be asymptomatic or, more frequently, presenting with anomalous bleeding and/or vaginal discharge. A 41-year-old woman with a diagnosis of adenocarcinoma of the uterine cervix was subjected to chemotherapy after radical surgery. During the follow-up, the patient underwent a Positron Emission Tomography integrated with Computed Tomography and pelvic Magnetic Resonance, which showed rapid and diffuse disease progression from the site of the lesion to the pelvic bones. Bone involvement in patients with cervical cancer, being a rare event, is significant since it greatly reduces life expectancy. The majority of metastatic bone lesions in cervical cancer seem to be of osteolytic nature. In our patient, Positron Emission Tomography integrated with Computed Tomography and Magnetic Resonance were the imaging methods used during the follow-up and both techniques clearly showed diffuse and rapid tumour spread to the bones

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

    DEFF Research Database (Denmark)

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

    2017-01-01

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

  4. ADC histogram analysis of muscle lymphoma - Correlation with histopathology in a rare entity.

    Science.gov (United States)

    Meyer, Hans-Jonas; Pazaitis, Nikolaos; Surov, Alexey

    2018-06-21

    Diffusion weighted imaging (DWI) is able to reflect histopathology architecture. A novel imaging approach, namely histogram analysis, is used to further characterize lesion on MRI. The purpose of this study is to correlate histogram parameters derived from apparent diffusion coefficient- (ADC) maps with histopathology parameters in muscle lymphoma. Eight patients (mean age 64.8 years, range 45-72 years) with histopathologically confirmed muscle lymphoma were retrospectively identified. Cell count, total nucleic and average nucleic areas were estimated using ImageJ. Additionally, Ki67-index was calculated. DWI was obtained on a 1.5T scanner by using the b values of 0 and 1000 s/mm2. Histogram analysis was performed as a whole lesion measurement by using a custom-made Matlabbased application. The correlation analysis revealed statistically significant correlation between cell count and ADCmean (p=-0.76, P=0.03) as well with ADCp75 (p=-0.79, P=0.02). Kurtosis and entropy correlated with average nucleic area (p=-0.81, P=0.02, p=0.88, P=0.007, respectively). None of the analyzed ADC parameters correlated with total nucleic area and with Ki67-index. This study identified significant correlations between cellularity and histogram parameters derived from ADC maps in muscle lymphoma. Thus, histogram analysis parameters reflect histopathology in muscle tumors. Advances in knowledge: Whole lesion ADC histogram analysis is able to reflect histopathology parameters in muscle lymphomas.

  5. Analysis of primary intrathoracic extrapulmonary hydatid cysts a rare clinical entity

    International Nuclear Information System (INIS)

    Ahmad, M.; Ullah, M.K.; Imran, M.; Bilal, A.; Khan, M.A.; Ali, S.Z.

    2015-01-01

    prescribed to all patients for as long as 3 months postoperatively. No complication, recurrence, or death occurred during the follow up period of 13 ± 15.4 months (range, 2-36 months). Conclusion: The extrapulmonary location of Hydatid cysts within the thorax is very rare. This rarity may cause difficulties in diagnosis. To avoid recurrence, it is necessary to resect the affected tissues completely and an anthelmintic medical regimen post operatively. (author)

  6. RARE CASE OF DESMOID TUMOR OF URINARY BLADDER

    Directory of Open Access Journals (Sweden)

    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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2008-08-15

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

  8. Epithelioid hemangioendothelioma and multiple thoraco-lumbar lateral meningoceles: two rare pathological entities in a patient with NF-1

    International Nuclear Information System (INIS)

    Reis, C.; Carneiro, E.; Fonseca, J.; Salgado, A.; Pereira, P.; Vaz, R.; Pinto, R.; Capelinha, A.F.; Lopes, J.M.

    2005-01-01

    Epithelioid hemangioendothelioma (EHE) is a rare vascular soft-tissue tumour of intermediate malignancy. Neurofibromatosis type I (NF-1) is a genetic syndrome associated with soft tissue sarcoma and higher risk of developing neoplasia. Lateral meningoceles are uncommon entities, being mostly associated with NF-1. We report a case of a 31-year-old woman, with NF-1 and past history of right thalamic/peduncular astrocytoma WHO grade II, admitted to the Neurosurgery Department in December 2003 due to severe low back pain, irradiating to the left leg without a radicular pattern. Thoraco-lumbar magnetic resonance imaging (MRI) showed a large left posterior paravertebral expansive lesion, bilateral and multiple thoraco-lumbar lateral meningoceles and dural ectasias with scalloping of the vertebral bodies. Biopsy of the paravertebral mass lesion disclosed EHE. We present this case because of the novel association between NF-1 and EHE, and the unusual aggressiveness of the neoplasia. Additionally, we highlight the co-existence of bilateral and multiple lateral meningoceles. (orig.)

  9. New Brain Tumor Entities Emerge from Molecular Classification of CNS-PNETs

    NARCIS (Netherlands)

    Sturm, Dominik; Orr, Brent A.; Toprak, Umut H.; Hovestadt, Volker; Jones, David T. W.; Capper, David; Sill, Martin; Buchhalter, Ivo; Northcott, Paul A.; Leis, Irina; Ryzhova, Marina; Koelsche, Christian; Pfaff, Elke; Allen, Sariah J.; Balasubramanian, Gnanaprakash; Worst, Barbara C.; Pajtler, Kristian W.; Brabetz, Sebastian; Johann, Pascal D.; Sahm, Felix; Reimand, Jüri; Mackay, Alan; Carvalho, Diana M.; Remke, Marc; Phillips, Joanna J.; Perry, Arie; Cowdrey, Cynthia; Drissi, Rachid; Fouladi, Maryam; Giangaspero, Felice; Łastowska, Maria; Grajkowska, Wiesława; Scheurlen, Wolfram; Pietsch, Torsten; Hagel, Christian; Gojo, Johannes; Lötsch, Daniela; Berger, Walter; Slavc, Irene; Haberler, Christine; Jouvet, Anne; Holm, Stefan; Hofer, Silvia; Prinz, Marco; Keohane, Catherine; Fried, Iris; Mawrin, Christian; Scheie, David; Mobley, Bret C.; Schniederjan, Matthew J.; Santi, Mariarita; Buccoliero, Anna M.; Dahiya, Sonika; Kramm, Christof M.; von Bueren, André O.; von Hoff, Katja; Rutkowski, Stefan; Herold-Mende, Christel; Frühwald, Michael C.; Milde, Till; Hasselblatt, Martin; Wesseling, Pieter; Rößler, Jochen; Schüller, Ulrich; Ebinger, Martin; Schittenhelm, Jens; Frank, Stephan; Grobholz, Rainer; Vajtai, Istvan; Hans, Volkmar; Schneppenheim, Reinhard; Zitterbart, Karel; Collins, V. Peter; Aronica, Eleonora; Varlet, Pascale; Puget, Stephanie; Dufour, Christelle; Grill, Jacques; Figarella-Branger, Dominique; Wolter, Marietta; Schuhmann, Martin U.; Shalaby, Tarek; Grotzer, Michael; van Meter, Timothy; Monoranu, Camelia-Maria; Felsberg, Jörg; Reifenberger, Guido; Snuderl, Matija; Forrester, Lynn Ann; Koster, Jan; Versteeg, Rogier; Volckmann, Richard; van Sluis, Peter; Wolf, Stephan; Mikkelsen, Tom; Gajjar, Amar; Aldape, Kenneth; Moore, Andrew S.; Taylor, Michael D.; Jones, Chris; Jabado, Nada; Karajannis, Matthias A.; Eils, Roland; Schlesner, Matthias; Lichter, Peter; von Deimling, Andreas; Pfister, Stefan M.; Ellison, David W.; Korshunov, Andrey; Kool, Marcel

    2016-01-01

    Primitive neuroectodermal tumors of the central nervous system (CNS-PNETs) are highly aggressive, poorly differentiated embryonal tumors occurring predominantly in young children but also affecting adolescents and adults. Herein, we demonstrate that a significant proportion of institutionally

  10. New Brain Tumor Entities Emerge from Molecular Classification of CNS-PNETs

    NARCIS (Netherlands)

    Sturm, Dominik; Orr, Brent A.; Toprak, Umut H.; Hovestadt, Volker; Jones, David T W; Capper, David; Sill, Martin; Buchhalter, Ivo; Northcott, Paul A.; Leis, Irina; Ryzhova, Marina; Koelsche, Christian; Pfaff, Elke; Allen, Sariah J.; Balasubramanian, Gnanaprakash; Worst, Barbara C.; Pajtler, Kristian W.; Brabetz, Sebastian; Johann, Pascal D.; Sahm, Felix; Reimand, Jüri; Mackay, Alan; Carvalho, Diana M.; Remke, Marc; Phillips, Joanna J.; Perry, Arie; Cowdrey, Cynthia; Drissi, Rachid; Fouladi, Maryam; Giangaspero, Felice; Łastowska, Maria; Grajkowska, Wiesława; Scheurlen, Wolfram; Pietsch, Torsten; Hagel, Christian; Gojo, Johannes; Lötsch, Daniela; Berger, Walter; Slavc, Irene; Haberler, Christine; Jouvet, Anne; Holm, Stefan; Hofer, Silvia; Prinz, Marco; Keohane, Catherine; Fried, Iris; Mawrin, Christian; Scheie, David; Mobley, Bret C.; Schniederjan, Matthew J.; Santi, Mariarita; Buccoliero, Anna M.; Dahiya, Sonika; Kramm, Christof M.; Von Bueren, André O.; Von Hoff, Katja; Rutkowski, Stefan; Herold-Mende, Christel; Frühwald, Michael C.; Milde, Till; Hasselblatt, Martin; Wesseling, Pieter; Rößler, Jochen; Schüller, Ulrich; Ebinger, Martin; Schittenhelm, Jens; Frank, Stephan; Grobholz, Rainer; Vajtai, Istvan; Hans, Volkmar; Schneppenheim, Reinhard; Zitterbart, Karel; Collins, V. Peter; Aronica, Eleonora; Varlet, Pascale; Puget, Stephanie; Dufour, Christelle; Grill, Jacques; Figarella-Branger, Dominique; Wolter, Marietta; Schuhmann, Martin U.; Shalaby, Tarek; Grotzer, Michael; Van Meter, Timothy; Monoranu, Camelia Maria; Felsberg, Jörg; Reifenberger, Guido; Snuderl, Matija; Forrester, Lynn Ann; Koster, Jan; Versteeg, Rogier; Volckmann, Richard; Van Sluis, Peter; Wolf, Stephan; Mikkelsen, Tom; Gajjar, Amar; Aldape, Kenneth; Moore, Andrew S.; Taylor, Michael D.; Jones, Chris; Jabado, Nada; Karajannis, Matthias A.; Eils, Roland; Schlesner, Matthias; Lichter, Peter; Von Deimling, Andreas; Pfister, Stefan M.; Ellison, David W.; Korshunov, Andrey; Kool, Marcel

    2016-01-01

    Summary Primitive neuroectodermal tumors of the central nervous system (CNS-PNETs) are highly aggressive, poorly differentiated embryonal tumors occurring predominantly in young children but also affecting adolescents and adults. Herein, we demonstrate that a significant proportion of

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

    Directory of Open Access Journals (Sweden)

    Yoshinori Handa

    2015-01-01

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

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

    Science.gov (United States)

    Bhardwaj, Sharonlin; Varma, Seema

    2018-03-01

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

  13. Bilateral sertoli-leydig cell tumor of the ovary: A rare case report

    OpenAIRE

    Alam Kiran; Maheshwari Veena; Rashid Seema; Bhargava Shruti

    2009-01-01

    Sertoli leydig cell tumors also known as arrhenoblastoma, are a rare member of the sex cord-stromal tumor group of ovarian and testicular cancers, comprising less than 1% of all ovarian tumors, which occur in young adults and are almost always unilateral. We hereby report a case of a 17-year-old female presenting with a short history of irregular menses and an abdominal lump, which was histologically proven to be a bilateral sertoli leydig cell tumor of the ovary, an exceptionally rare...

  14. Bronchoscopic management of a rare benign endobronchial tumor

    Directory of Open Access Journals (Sweden)

    K. Madan

    2012-09-01

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

  15. Co-occurrence of Calcifying Odontogenic Cyst, Aggressive Central Giant Cell Granuloma and Central Odontogenic Fibroma: Report of a Very Rare Entity and Its Surgical Management

    Directory of Open Access Journals (Sweden)

    Touraj Vaezi

    2016-09-01

    Full Text Available Calcifying odontogenic cyst (COC, Central odontogenic fibroma (COF and aggressive central giant cell granuloma (CGCG are rare pathologic diseases affecting the jaws. While the Co-existence of two of them is reported in the literature, existence of all three conditions in one patient is an extremely rare entity. In the present report, initial biopsy revealed fibrosarcoma, therefore mandibular resection was performed for the subject. Sectional Histopathologic evaluation revealed the co-existence of three conditions through histopathologic evaluation. This report emphasizes the importance of precise microscopical evaluation of jaw lesions and thorough sectional examination of the lesions to reach the precise diagnosis. Treatment modalities and follow-up radiographs are also provided to help clinicians manage these entities.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1975-02-01

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

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

    Directory of Open Access Journals (Sweden)

    E Rajendra Reddy

    2013-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Susant Mohanty

    2014-01-01

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

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

    Science.gov (United States)

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

    2012-08-01

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

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

    Directory of Open Access Journals (Sweden)

    C Anand Kumar

    2010-01-01

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

  1. 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

  2. Myxoid variant of adrenocortical tumors. Report of two cases with unique entity

    International Nuclear Information System (INIS)

    Suzuki, Rumi; Ito, Yukio; Okamoto, Takahiro; Iihara, Masatoshi; Obara, Takao; Tsuiki, Mika; Takano, Kazue; Nishikawa, Toshio; Aiba, Motohiko

    2007-01-01

    Cases in the title are reported. Case 1, a 57-year old female, had fracture and reduced limbic muscular force due to Cushing syndrome. CT image gave a big tumor in the left adrenal and 131 I-adosterol scintigraphy revealed the increased and decreased 131 I accumulation in the left and right adrenal, respectively: the carcinoma suspected. Only tumor portion was dissected out and was found to be an adenoma in histology. Case 2, a 50-year old male, had Cushing syndrome and extreme obesity. Previous CT presented a big tumor in the left adrenal; biopsy was performed under CT; and before surgery, the tumor was partly calcified in CT, which was found to be a carcinoma. In both cases, histology gave findings of the interstitial and extracellular disposition of myxoid material and of pseudoglandular pattern. (R.T.)

  3. A rare occurrence of a steroid cell tumor of the pelvic mesentery: a case report

    OpenAIRE

    Louis Robert; Murhekar Kanchan; Majhi Urmila

    2011-01-01

    Abstract Introduction Steroid cell tumors are microscopically characterized by abundant eosinophilic or vacuolated cytoplasm that is often positive for fat stains. These tumors could be of ovarian or ectopic adrenal origin. We present a rare case of a steroid cell tumor arising from the pelvic mesentery. Case presentation A 31-year-old Asian woman was undergoing treatment for infertility and virilizing symptoms. She underwent laparoscopy followed by laprotomy for a suspected ovarian cyst/pelv...

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

    Directory of Open Access Journals (Sweden)

    Nicole Kapral, MD

    2018-06-01

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

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

    Directory of Open Access Journals (Sweden)

    Harish S Permi

    2013-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Abdulkadir Tepeler

    2011-01-01

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

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

    International Nuclear Information System (INIS)

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

    2003-01-01

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

  8. Pancreatic non-functioning neuroendocrine tumor: a new entity genetically related to Lynch syndrome

    OpenAIRE

    Serracant Barrera, Anna; Serra Pla, Sheila; Blázquez Maña, Carmen María; Salas, Rubén Carrera; García Monforte, Neus; Bejarano González, Natalia; Romaguera Monzonis, Andreu; Andreu Navarro, Francisco Javier; Bella Cueto, Maria Rosa; Borobia, Francisco G.

    2017-01-01

    Some pancreatic neuroendocrine tumors (P-NETs) are associated with hereditary syndromes. An association between Lynch syndrome (LS) and P-NETs has been suggested, however it has not been confirmed to date. We describe the first case associating LS and P-NETs. Here we report a 65-year-old woman who in the past 20 years presented two colorectal carcinomas (CRC) endometrial carcinoma (EC), infiltrating ductal breast carcinoma, small intestine adenocarcinoma, two non-functioning P-NETs and seboma...

  9. Esophageal Granular Cell Tumor and Eosinophilic Esophagitis: Two Interesting Entities Identified in the Same Patient

    Directory of Open Access Journals (Sweden)

    Alfredo J. Lucendo

    2008-02-01

    Full Text Available We illustrate the case of a 41-year-old male with allergic manifestations since childhood. He sought medical attention for intermittent, progressive dysphagia from which he had been suffering for a number of years, having felt the sensation of a retrosternal lump and a self-limited obstruction to the passage of food. Endoscopy detected a submucosal tumor in the upper third of the esophagus, which was typified, via biopsy, as a granular cell tumor with benign characteristics and probably responsible for the symptoms. Two years later, the patient sought medical attention once again as these symptoms had not abated, hence digestive endoscopy was repeated. This revealed stenosis of the junction between the middle and lower thirds of the organ which had not been detected previously but was passable under gentle pressure. Eosinophilic esophagitis was detected after biopsies were taken. Esophageal manometry identified a motor disorder affecting the esophageal body. Following three months of treatment using fluticasone propionate applied topically, the symptoms went into remission, esophageal stenosis disappeared and the esophageal biopsies returned to normal. This is the first documented case of the link between granular cell tumors and Eosinophilic esophagitis, two different disorders which could cause dysphagia in young patients.

  10. 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

  11. Vitamin K deficiency bleeding presenting as nodular purpura in infancy: A rare and life-threatening entity

    Directory of Open Access Journals (Sweden)

    Pratik Gahalaut

    2013-01-01

    Full Text Available Vitamin K deficiency bleeding (VKDB disorder is an uncommon entity, which occurs due to inadequate activity of vitamin K-dependant coagulation factors. An 8-months-old exclusively breast-fed male infant presented with multiple, purpuric and nodular non-collapsible swellings on trunk of 4 days duration. Investigations revealed raised activated partial thromboplastin time and prothrombintime. Fibrinogen level and platelet counts were normal. Late VKDB usually presents as intra-cranial or mucosal hemorrhages. [1] Though skin and mucosal bleeding may occur in 1/3 rd of infants with VKDB, ′nodular purpura′ is not the common presenting feature. Earlier recognition of VKDB and immediate investigation/treatment helps prevent the potentially fatal outcome of the disease. Very little is mentioned about this entity in dermatology literature.

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

    Science.gov (United States)

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

    2017-06-01

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

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

    International Nuclear Information System (INIS)

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

    2004-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Gong Li

    2012-04-01

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

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

    Science.gov (United States)

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

    2012-04-19

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

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

    Directory of Open Access Journals (Sweden)

    Karthik Kasireddy

    2016-01-01

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

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

    OpenAIRE

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

    2015-01-01

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

  18. Basaloid squamous carcinoma of skin associated with xeroderma pigmentosum in an 8-year-old child: A rare entity

    Directory of Open Access Journals (Sweden)

    Tashnin Rahman

    2014-01-01

    Full Text Available Xeroderma pigmentosum (XP is a rare autosomal recessive genodermatosis associated with hypersensitivity to ultraviolet (UV light, due to defects in deoxyribonucleic acid (DNA repair. Basaloid squamous cell carcinoma is a rare aggressive variant of squamous cell carcinoma. Patients with XP are at increased risk of developing cutaneous malignancy and are commonly associated with squamous carcinoma. We report an extremely rare case of 8-year-old child with XP along with basaloidsquamous carcinoma of skin; and review of literature related to it.

  19. Epithelioid hemangioendothelioma – A rare pulmonary tumor

    Directory of Open Access Journals (Sweden)

    Sandra Saleiro

    2008-05-01

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

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

    Science.gov (United States)

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

    1984-06-01

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

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

    African Journals Online (AJOL)

    2013-07-09

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

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

    Directory of Open Access Journals (Sweden)

    Braunstein Stefan

    2005-08-01

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

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

    Science.gov (United States)

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

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

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

    Directory of Open Access Journals (Sweden)

    Mijović Žaklina

    2013-01-01

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

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

    Science.gov (United States)

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

    2012-01-01

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

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

    Science.gov (United States)

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

    2015-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Dinesh P Asati

    2015-01-01

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

  9. Pigmented epidermal cyst with dense collection of melanin: A rare entity - Report of a case with review of the literature.

    Science.gov (United States)

    Jayalakshmy, P S; Subitha, K; Priya, P V; Johnson, Gerald

    2012-05-01

    Epidermal cyst is a very common benign cystic lesion of the skin. It is usual to find ulceration of the lining epithelium, rupture of the cyst wall with chronic inflammation and foreign body giant cell reaction. But, it is very rare to see an epidermal cyst with marked accumulation of melanin pigment. Only a few cases of pigmented epidermal cyst with dense collection of melanin pigment have been published in the literature. Here, we are reporting a case of ruptured epidermal cyst with keratin granuloma formation and showing dense collection of melanin pigment.

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

    Directory of Open Access Journals (Sweden)

    Pradnya S Bhadarge

    2017-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Michael L. Wang

    2017-07-01

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

  12. Ischemic Gastritis: A Multicenter Case Series of a Rare Clinical Entity and a Review of the Literature.

    Science.gov (United States)

    Elwir, Saleh; Shaukat, Aasma; Mesa, Hector; Colbach, Christine; Dambowy, Paul; Shaw, Michael

    2016-10-01

    To report a case series of ischemic gastritis and discuss its etiology, management, and associated mortality according to our results and the published English literature. Ischemic gastritis is rare, given the rich blood supply of the stomach. It has been reported in isolated case reports and small case series. Most cases are vascular in origin and associated with a high mortality. Pathology databases from 3 hospitals affiliated with the University of Minnesota Medical School were searched for cases of ischemic gastritis in the last 10 years. Patients' demographics, clinical course, and 1-month and 1-year mortalities were collected from electronic medical records. A total of 12 patients were identified (age range, 32.1 to 83.2), the largest series reported to date. The presenting symptom was gastrointestinal bleeding (8), abdominal pain (2), nausea (1), and symptomatic anemia (1). The etiology included postinterventional radiology embolization (2), hemodynamic changes in the setting of celiac axis stenosis (2), vasculitis (1), systemic hypotension (1), and unknown (6). Treatment included steroid therapy, revascularization by interventional radiology, surgery, or supportive treatment. Thirty-day and 1-year mortalities were 33% and 41%, respectively. Ischemic gastritis is rare, but associated with a high mortality. Evaluation for treatable etiologies should be sought and corrected if present.

  13. Vogt-Koyanagi-Harada disease, a rare entity in Spain: the challenge of worldwide immigration and globalization

    Directory of Open Access Journals (Sweden)

    Alberto Benavente Fernández

    2018-05-01

    Full Text Available Vogt–Koyanagi–Harada disease is rare, mediated by autoimmune melanocyte inflammation and facilitated by genetic predisposition[1-3]. The main clinical features include uveitis, meningitis, tinnitus and sensorineural deafness, and skin and hair depigmentation. It usually develops in four consecutive stages: prodromal, acute uveitic, convalescent, and chronic or recurrent[4]. In view of the first two stages, the differential diagnosis takes into account uveo-meningeal syndromes. Treatment is based on high dose corticosteroids. We present the case of a 14-year-old girl admitted to hospital with fever, progressive uveo-meningeal symptoms, and sensorineural hearing loss. After work-up, the final diagnosis of Vogt–Koyanagi–Harada disease was made.

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

    Directory of Open Access Journals (Sweden)

    Bhagyalakshmi Atla

    2016-01-01

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

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

    Science.gov (United States)

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

    2016-01-01

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

  16. A case of bone marrow necrosis of an idiopathic aetiology: the report of a rare entity with review of the literature.

    Science.gov (United States)

    Bhasin, Tejinder Singh; Sharma, Sonam; Chandey, Manish; Bhatia, Puneet Kaur; Mannan, Rahul

    2013-03-01

    Bone Marrow Necrosis (BMN) is regarded as a rare entity in the aspirates and the trephine biopsies which are obtained from living patients and it is a rare antemortem diagnosis. Usually, BMN is associated with a poor prognosis and it has a 90% association with malignancies (mainly haematological). Its other causes include infections, drugs and other non malignant haematological causes. BMN which occurs due to unknown causes (idiopathic), is very rare. The present case report describes a case of BMN in a 14 year old male child from rural background, who came to the medicine outpatient department of a tertiary care centre in north India, with the complaints of generalized weakness, severe bone pains, especially in the ribcage area and fatigue. His peripheral blood film revealed pancytopaenia. His bone marrow aspiration yielded a watery, sero-sanguinous fluid from 2 different sites. Microscopy revealed "ghost like" haematopoietic cells lying in eosinophilic, proteinaceous material. Bone marrow biopsies from both the sites revealed extensive foci of gelatinous transformation and necrosis. A diagnosis of BMN was rendered. No underlying neoplastic / non neoplastic cause was identified, even after a thorough systematic investigation (haematological/biochemical/ radiological and immunological). Thus, a final diagnosis of BMN due to an idiopathic cause, was finally given. The patient was given supportive treatment, after which he was referred to a higher centre. BMN which arises idiopathically is usually very rare and often precedes the aetiological diagnosis and obscures the diagnosis. Traditionally, the prognoses of the patients with BMN were considered as poor, but with better treatment aids (chemotherapy and supportive treatment), the prognoses of these patients have been found to improve. Vigorous supportive care, along with a disease specific treatment, is the key to its management. This case was worth reporting, as no cause of BMN was elicited in it and we have

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

    Directory of Open Access Journals (Sweden)

    A. A. Ryabtseva

    2015-01-01

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

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

    Science.gov (United States)

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

    2015-01-01

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

  19. 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.

  20. Carcinosarcoma of the Ureter with a Small Cell Component: Report of a Rare Pathologic Entity and Potential for Diagnostic Error on Biopsy

    Directory of Open Access Journals (Sweden)

    Kent Newsom

    2014-01-01

    Full Text Available Carcinosarcomas of the ureter are rare biphasic neoplasms, composed of both malignant epithelial (carcinomatous and malignant mesenchymal (sarcomatous components. Carcinosarcomas of the urinary tract are exceedingly rare. We report a unique case of a carcinosarcoma of the ureter with a chondrosarcoma and small cell tumor component arising in a 68-year-old male who presented with microscopic hematuria. CT intravenous pyelogram revealed right-sided hydroureter and hydronephrosis with thickening and narrowing of the right ureter. The patient underwent robot-assisted ureterectomy with bladder cuff excision and subsequent adjuvant chemotherapy. The patient is disease-free at 32 months after treatment. We provide a brief synoptic review of carcinosarcoma of the ureter and bladder with utilization of immunohistochemical (IHC stains and potential diagnostic pitfalls.

  1. Effect of compositional variations on charge compensation of AlO4 and BO4 entities and on crystallization tendency of a rare-earth-rich aluminoborosilicate glass

    International Nuclear Information System (INIS)

    Quintas, A.; Caurant, D.; Majerus, O.; Charpentier, T.; Dussossoy, J.-L.

    2009-01-01

    This paper presents the structural and crystallization study of a rare-earth-rich aluminoborosilicate glass that is a simplified version of a new nuclear glass proven to be a potential candidate for the immobilization of highly concentrated radioactive wastes that will be produced in the future. In this work, we studied the impact of changing the nature of alkali (Li + , Na + , K + , Rb + , Cs + ) or alkaline-earth (Mg 2+ , Ca 2+ , Sr 2+ , Ba 2+ ) cations present in glass composition on glass structure (by 27 Al and 11 B nuclear magnetic resonance spectroscopy) and on its crystallization tendency during melt cooling at 1 K/min (average cooling rate during industrial process). From these composition changes, it was established that alkali cations were preferentially involved in charge compensation of (AlO 4 ) - and (BO 4 ) - entities in the glassy network comparatively to alkaline-earth cations. Whatever the nature of alkali cations, glass compositions containing calcium gave way to the crystallization of an apatite silicate phase bearing calcium and rare-earth (RE) cations (Ca 2 RE 8 (SiO 4 ) 6 O 2 , RE = Nd or La) but melt crystallization tendency during cooling strongly varied with the nature of alkaline-earth cations.

  2. 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

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

    Directory of Open Access Journals (Sweden)

    Rowland Randall G

    2008-04-01

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

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

    Science.gov (United States)

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

    2015-08-01

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

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

    Science.gov (United States)

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

    2017-11-06

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

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

    Directory of Open Access Journals (Sweden)

    Pradhan M Pagaro

    2014-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Petra Krajnc

    2018-02-01

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

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

    Science.gov (United States)

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

    2016-03-01

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

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

    Directory of Open Access Journals (Sweden)

    Abhijeet Alok

    2015-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Deniz Arslan

    2013-01-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2015-12-15

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

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

    Directory of Open Access Journals (Sweden)

    Radivojcevic Uros

    2016-07-01

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

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

    Directory of Open Access Journals (Sweden)

    Foroulis Christophoros N

    2008-12-01

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

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

    Directory of Open Access Journals (Sweden)

    Angela Sardaro

    2014-10-01

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

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

    International Nuclear Information System (INIS)

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

    1997-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Inez Wyn

    2014-01-01

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

  17. A rare case of primary clear cell sarcoma of the pubic bone resembling small round cell tumor: an unusual morphological variant

    International Nuclear Information System (INIS)

    Nakayama, Shoko; Tsuji, Motomu; Hanafusa, Toshiaki; Yokote, Taiji; Iwaki, Kazuki; Akioka, Toshikazu; Miyoshi, Takuji; Hirata, Yuji; Takayama, Ayami; Nishiwaki, Uta; Masuda, Yuki

    2012-01-01

    Clear cell sarcoma (CCS) and malignant melanoma share overlapping immunohistochemistry with regard to the melanocytic markers HMB45, S100, and Melan-A. However, the translocation t(12; 22)(q13; q12) is specific to CCS. Therefore, although these neoplasms are closely related, they are now considered to be distinct entities. However, the translocation is apparently detectable only in 50%–70% of CCS cases. Therefore, the absence of a detectable EWS/AFT1 rearrangement may occasionally lead to erroneous exclusion of a translocation-negative CCS. Therefore, histological assessment is essential for the correct diagnosis of CCS. Primary CCS of the bone is exceedingly rare. Only a few cases of primary CCS arising in the ulna, metatarsals, ribs, radius, sacrum, and humerus have been reported, and primary CCS arising in the pubic bone has not been reported till date. We present the case of an 81-year-old man with primary CCS of the pubic bone. Histological examination of the pubic bone revealed monomorphic small-sized cells arranged predominantly as a diffuse sheet with round, hyperchromatic nuclei and inconspicuous nucleoli. The cells had scant cytoplasm, and the biopsy findings indicated small round cell tumor (SRCT). Immunohistochemical staining revealed the tumor cells to be positive for HMB45, S100, and Melan-A but negative for cytokeratin (AE1/AE3) and epithelial membrane antigen. To the best of our knowledge, this is the first case report of primary CCS of the pubic bone resembling SRCT. This ambiguous appearance underscores the difficulties encountered during the histological diagnosis of this rare variant of CCS. Awareness of primary CCS of the bone is clinically important for accurate diagnosis and management when the tumor is located in unusual locations such as the pubic bone and when the translocation t(12; 22)(q13; q12) is absent

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

    Science.gov (United States)

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

    2017-01-01

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

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

    Science.gov (United States)

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

    2017-04-04

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

  20. 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

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

    Science.gov (United States)

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

    2018-02-01

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

  2. 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.

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

    Science.gov (United States)

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

    2001-10-01

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

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

    Directory of Open Access Journals (Sweden)

    Amany A. Fathaddin

    2014-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Yan ZhongShu

    2011-11-01

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

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

    Directory of Open Access Journals (Sweden)

    Itziar González

    2018-03-01

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

  7. Combined malignant testicular tumor and splenogonadal fusion. A case story

    DEFF Research Database (Denmark)

    Thomsen, B M; Wierød, F S; Rasmussen, K C

    1997-01-01

    Splenogonadal fusion may be misinterpretated as a primary malignant testicular tumor or as an adenomatoid tumor. Knowledge of this entity is important in order to preserve the testis at surgery. A rare case of simultaneous occurrence of splenogonadal fusion and mixed malignant tumor of the testis...

  8. The inflammatory cytokine TNFα cooperates with Ras in elevating metastasis and turns WT-Ras to a tumor-promoting entity in MCF-7 cells

    International Nuclear Information System (INIS)

    Leibovich-Rivkin, Tal; Liubomirski, Yulia; Meshel, Tsipi; Abashidze, Anastasia; Brisker, Daphna; Solomon, Hilla; Rotter, Varda; Weil, Miguel; Ben-Baruch, Adit

    2014-01-01

    In the present study we determined the relative contribution of two processes to breast cancer progression: (1) Intrinsic events, such as activation of the Ras pathway and down-regulation of p53; (2) The inflammatory cytokines TNFα and IL-1β, shown in our published studies to be highly expressed in tumors of >80% of breast cancer patients with recurrent disease. Using MCF-7 human breast tumor cells originally expressing WT-Ras and WT-p53, we determined the impact of the above-mentioned elements and cooperativity between them on the expression of CXCL8 (ELISA, qRT-PCR), a member of a “cancer-related chemokine cluster” that we have previously identified. Then, we determined the mechanisms involved (Ras-binding-domain assays, Western blot, luciferase), and tested the impact of Ras + TNFα on angiogenicity (chorioallantoic membrane assays) and on tumor growth at the mammary fat pad of mice and on metastasis, in vivo. Using Ras G12V that recapitulates multiple stimulations induced by receptor tyrosine kinases, we found that Ras G12V alone induced CXCL8 expression at the mRNA and protein levels, whereas down-regulation of p53 did not. TNFα and IL-1β potently induced CXCL8 expression and synergized with Ras G12V , together leading to amplified CXCL8 expression. Testing the impact of WT-Ras, which is the common form in breast cancer patients, we found that WT-Ras was not active in promoting CXCL8; however, TNFα has induced the activation of WT-Ras: joining these two elements has led to cooperative induction of CXCL8 expression, via the activation of MEK, NF-κB and AP-1. Importantly, TNFα has led to increased expression of WT-Ras in an active GTP-bound form, with properties similar to those of Ras G12V . Jointly, TNFα + Ras activities have given rise to increased angiogenesis and to elevated tumor cell dissemination to lymph nodes. TNFα cooperates with Ras in promoting the metastatic phenotype of MCF-7 breast tumor cells, and turns WT-Ras into a tumor

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

    Directory of Open Access Journals (Sweden)

    Agnes Assao

    2017-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Simone Albisinni

    2017-07-01

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

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

    Science.gov (United States)

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

    2018-05-01

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

  12. 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.

  13. 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.

  14. 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.

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

    African Journals Online (AJOL)

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

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

    Directory of Open Access Journals (Sweden)

    Kamila Woźniak

    2016-08-01

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

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

    Directory of Open Access Journals (Sweden)

    Jean Nunes dos Santos

    2008-06-01

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

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

    African Journals Online (AJOL)

    Arun Kumar Agnihotri

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

  19. Isolated single coronary artery (RII-B type presenting as an inferior wall myocardial infarction: A rare clinical entity

    Directory of Open Access Journals (Sweden)

    Ankur C. Thummar

    2014-09-01

    Full Text Available Isolated single coronary artery without other congenital cardiac anomalies is very rare among the different variations of anomalous coronary patterns. The prognosis in patients with single coronary varies according to the anatomic distribution and associated coronary atherosclerosis. If the left main coronary artery travels between the aorta and pulmonary arteries, it may be a cause of sudden cardiac death. We present multimodality images of a single coronary artery, in which the whole coronary system originated by a single trunk from the right sinus of Valsalva with inter-arterial course of left main coronary artery. This rare type of single coronary artery was classified as RII-B type according to Lipton's scheme of classification. A significant flow-limiting lesions were found in the right coronary artery that was successfully treated with percutaneous coronary intervention.

  20. 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.

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

    International Nuclear Information System (INIS)

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

    2016-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Kamala Rawson

    2014-01-01

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

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

    Science.gov (United States)

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

    2007-01-01

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

  4. Malignant Peripheral Nerve Sheath Tumor: MRI and CT Findings

    Directory of Open Access Journals (Sweden)

    K. O. Kragha

    2015-01-01

    important in its diagnosis. A rare case of MPNST that produced urinary retention and bowel incontinence is presented that may aid clinicians in the diagnosis of this rare clinical entity. Motor weakness, central enhancement, and immunohistochemistry may assist in the diagnosis of MPNST and differentiation between benign peripheral nerve sheath tumor (BPNST and MPNST.

  5. The prune belly syndrome in a female foetus with urorectal septum malformation sequence: a case report on a rare entity with an unusual association.

    Science.gov (United States)

    Goswami, Dibyajyoti; Kusre, Giriraj; Dutta, Hemonta Kumar; Sarma, Adity

    2013-08-01

    The prune belly syndrome is a rare congenital anomaly which is characterized by the triad of an absent or a deficient development of the abdominal muscle, bilateral cryptorchidism and an anomalous urinary tract. In its full form, this condition occurs only in males. However, a similar condition occurs in females in the absence of cryptorchidism. On the other hand, the urorectal septum malformation sequence is a lethal congenital malformation which is characterized by the development of a phallus like structure, a smooth perineum and the absence of urethral, vaginal and anal openings. We are reporting a case of a female foetus with the prune belly syndrome, which was associated with a urorectal septum malformation sequence. A dead foetus with a protruded abdomen and ambiguous genitalia, was born at 32 weeks of pregnancy. On autopsy, it was found to have female internal genital organs. The left kidney, the urinary bladder and the rectum were absent. The sigmoid colon, the ureters and the fallopian tubes opened into a common cloacal sac. The histopathological examination of the ovary showed the presence of Leydig's cells. The occurrence of the female counterpart of the prune belly syndrome is extremely rare and only few of such cases were found to be discussed in the details in the indexed English literature so far. Hence, we hope that this case report will contribute to the existing knowledge on the prune belly syndrome.

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

    NARCIS (Netherlands)

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

    2012-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Gupta Kirti

    2008-04-01

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

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

    International Nuclear Information System (INIS)

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

    2005-01-01

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

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

    International Nuclear Information System (INIS)

    Ehrenberg, C.; Helmberger, H.

    1998-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Askin Esen Hasturk

    2017-01-01

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

  11. Dynamic collective entity representations for entity ranking

    NARCIS (Netherlands)

    Graus, D.; Tsagkias, M.; Weerkamp, W.; Meij, E.; de Rijke, M.

    2016-01-01

    Entity ranking, i.e., successfully positioning a relevant entity at the top of the ranking for a given query, is inherently difficult due to the potential mismatch between the entity's description in a knowledge base, and the way people refer to the entity when searching for it. To counter this

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

    Directory of Open Access Journals (Sweden)

    Regina Streuli

    2017-06-01

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

  13. Entity associations for search

    NARCIS (Netherlands)

    Reinanda, R.

    2017-01-01

    In this thesis, we investigate the broad problem of computing entity associations for search. Specifically, we consider three types of entity association: entity-entity, entity-document, and entity-aspect associations. We touch upon various domains, starting with specific domains such as the

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

    Directory of Open Access Journals (Sweden)

    Sofia Santos Lopes

    2016-01-01

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

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

    International Nuclear Information System (INIS)

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

    2011-01-01

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

  16. 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.

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

    Directory of Open Access Journals (Sweden)

    Grace Kim

    2015-08-01

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

  18. 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)

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2016-09-15

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

  20. Doença de Marchiafava-Bignami: uma rara entidade com prognóstico sombrio Marchiafava-Bignami disease: a rare entity with a poor outcome

    Directory of Open Access Journals (Sweden)

    Paulo Eduardo Mestrinelli Carrilho

    2013-03-01

    Full Text Available A doença de Marchiafava-Bignami é uma entidade rara, caracterizada por uma degeneração primária do corpo caloso, associada com o consumo crônico do etanol. A doença pode, ocasionalmente, ocorrer em pacientes não etilistas cronicamente desnutridos. Uma deficiência de vitaminas do complexo B é considerada como a hipótese etiopatogênica principal, uma vez que muitos pacientes obtiveram uma melhora após a administração desses compostos. Algumas vezes, entretanto, tal resposta terapêutica não foi observada. O diagnóstico definitivo da doença de Marchiafava-Bignami pode ser problemático e depende das características de estudos de neuroimagem, especialmente a ressonância magnética. Seu tratamento, dessa forma, é ainda controverso, com resultados variáveis. Como estão implicados fatores nutricionais, analogamente à encefalopatia de Wernicke, alguns autores recomendam a reposição de vitaminas do complexo B, particularmente da B1. O presente artigo relata a forma aguda da doença de Marchiafava-Bignami em um paciente masculino dependente do álcool, que apresentou discreta melhora após a administração parenteral das vitaminas do complexo B. Como consequência de suas más condições neurológicas e imunológicas, ele desenvolveu infecções pulmonares múltiplas e permaneceu, por longo tempo, na unidade de terapia intensiva. Seu óbito ocorreu por sepse causada por um fungo raro, o Rhodotorula mucilaginosa. O artigo é um relato clínico da evolução desse paciente, com a apresentação de seus dados de neuroimagem, acompanhada por uma revisão sobre doença de Marchiafava-Bignami e sobre as infecções por Rhodotorula dentro da perspectiva da unidade de cuidado intensivo.Marchiafava-Bignami disease is a rare affliction characterized by primary degeneration of the corpus callosum associated with chronic consumption of ethanol. The disease may occasionally occur in patients who are not alcoholics but are chronically

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

    International Nuclear Information System (INIS)

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

    2016-01-01

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

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

    Science.gov (United States)

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

    2002-01-01

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

  3. A Rare Entity: A Case Report

    African Journals Online (AJOL)

    abp

    2014-07-26

    Jul 26, 2014 ... Key words: Melorheostosis, sclerotic bone dysplasias, CT, MRI ... imperfectly known as clinical presentations are highly variable, and the etiological diagnosis is not fully elucidated. ... Scintigraphy can detect subclinical lesions ... standard radiographs every three months, and an annual bone scan.

  4. Paraneoplastic Cushing Syndrome Due To Wilm's Tumor.

    Science.gov (United States)

    Faizan, Mahwish; Manzoor, Jaida; Saleem, Muhammad; Anwar, Saadia; Mehmood, Qaiser; Hameed, Ambreen; Ali, Agha Shabbir

    2017-05-01

    Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to neoplasm. Paraneoplastic syndromes may be the first or the most prominent manifestations of cancer. Wilm's tumor is the most frequent pediatric renal malignancy and usually presents with abdominal mass. Unusual presentations like acquired von Willebrand disease, sudden death due to pulmonary embolism and Cushing syndrome have been described in the literature. Cushing syndrome, as the presenting symptom of a malignant renal tumor in children, is a very rare entity. Few case reports are available in the literature exploring the option of preoperative chemotherapy as well as upfront nephrectomy. We report a rare case of paraneoplastic Cushing syndrome due to a Wilm's tumor. Based on gradual decrease of postoperative weight, blood pressure, serum adrenocorticotropic hormone, and plasma cortisol levels, along with histological confirmation of Wilm's tumor, paraneoplastic Cushing syndrome due to Wilm's tumor was confirmed.

  5. Paraneoplastic cushing syndrome due to wilm's tumor

    International Nuclear Information System (INIS)

    Faizan, M.; Anwar, S.; Hameed, A.; Manzoor, J.; Saleem, M.; Mehmood, Q.; Ali, A. S.

    2017-01-01

    Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to neoplasm. Paraneoplastic syndromes may be the first or the most prominent manifestations of cancer. Wilm's tumor is the most frequent pediatric renal malignancy and usually presents with abdominal mass. Unusual presentations like acquired von Willebrand disease, sudden death due to pulmonary embolism and Cushing syndrome have been described in the literature. Cushing syndrome, as the presenting symptom of a malignant renal tumor in children, is a very rare entity. Few case reports are available in the literature exploring the option of preoperative chemotherapy as well as upfront nephrectomy. We report a rare case of paraneoplastic Cushing syndrome due to a Wilm's tumor. Based on gradual decrease of postoperative weight, blood pressure, serum adrenocorticotropic hormone, and plasma cortisol levels, alongwith histological confirmation of Wilm's tumor, paraneoplastic Cushing syndrome due to Wilm's tumor was confirmed. (author)

  6. 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.

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

    Science.gov (United States)

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

    2016-12-01

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

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

    Directory of Open Access Journals (Sweden)

    Richard A. Hickman

    2016-12-01

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

  9. Rasmussen's Aneurysm: A Forgotten Entity?

    International Nuclear Information System (INIS)

    Keeling, A. N.; Costello, R.; Lee, M. J.

    2008-01-01

    We present the case of a rare entity which is a complication of a disease process that had almost disappeared from the Western World. With the recent resurgence in reported cases of Mycobacterium tuberculosis (TB) in Western communities, it is important to recognize complications and sequelae. A young alcoholic male with confirmed active TB suffered a cardiac arrest following massive haemoptysis. Multidetector computed tomography angiography diagnosed a Rasmussen's aneurysm, confirmed by digital subtraction angiography and then successfully embolized with glue. We outline this rare case and the embolization technique and review previously documented reports

  10. Liposarcoma of Hypopharynx and Esophagus: a Unique Entity?

    Science.gov (United States)

    Riva, Giuseppe; Sensini, Matteo; Corvino, Andrea; Garzaro, Massimiliano; Pecorari, Giancarlo

    2016-06-01

    Liposarcoma is the most common soft tissue sarcoma in adults. It represents approximately 20 % of all mesenchymal malignancies. It most frequently involves retroperitoneum, trunk, and extremities. Hypopharyngeal and esophageal localization of liposarcoma is extremely rare. We performed a systematic review of literature and reported 26 and 33 cases of hypopharyngeal and esophageal liposarcoma. We analyzed natural history, imaging features, histology, treatment, and prognosis, with a specific focus to similarities and differences between tumors of hypopharynx and esophagus. Hypopharyngeal and esophageal liposarcomas have more similarities than differences. Incidence has a peak at 6th and 7th decades. The diagnostic procedures are barium swallow, endoscopic examination, and CT/MR imaging. Well-differentiated liposarcoma represents the most frequent histological subtype. Surgical excision is the main treatment. Endoscopic resection can be useful for pedunculated tumors of hypopharynx and cervical esophagus. Differences between hypopharyngeal and esophageal liposarcoma are represented by local recurrence rate (greater for hypopharyngeal tumors), number of giant tumors, and time to recurrence (greater for esophageal tumors). Finally, liposarcomas of distal esophagus need more extended approaches. Liposarcomas of hypopharynx and cervical esophagus could be considered a unique pathological entity, with similar features and treatment options. Survival rate is dependent on histological type and location. Local recurrence is common, especially for hypopharyngeal liposarcoma, while the risk of lymph node or distant metastasis is very low. Patients should undergo regular examinations to rule out local recurrence, also for a long time, especially for esophageal tumors.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2017-02-15

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

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

    Directory of Open Access Journals (Sweden)

    Lipika Lipi

    2014-01-01

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

  13. Intraosseous Polymorphous Low Grade Adenocarcinoma of the Mandible: Report of a Rare Case

    Directory of Open Access Journals (Sweden)

    SM R Prakash

    2010-01-01

    Full Text Available Polymorphous low-grade adenocarcinoma (PLGA is an entity under the subclassification of adenocarcinoma from salivary glands. PLGA is an unusual tumor that occur: in the minor salivary gland, particularity in the palate followed by buccal mucosa, upper lip, retromolar area and base of the tongue- The occurance of PLGA in locations other than these is exceedingly rare. We report a rare case of PLGA occuring in the anterior mandible. The clinical, radiographic, computed tomographic and histopathologic findings are discussed.

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

    Directory of Open Access Journals (Sweden)

    Christina Brzezniak

    2017-03-01

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

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

    Directory of Open Access Journals (Sweden)

    Amita Radhakrishnan Nair

    2014-01-01

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

  16. Programming Entity Framework

    CERN Document Server

    Lerman, Julia

    2009-01-01

    Programming Entity Framework is a thorough introduction to Microsoft's new core framework for modeling and interacting with data in .NET applications. This highly-acclaimed book not only gives experienced developers a hands-on tour of the Entity Framework and explains its use in a variety of applications, it also provides a deep understanding of its architecture and APIs -- knowledge that will be extremely valuable as you shift to the Entity Framework version in .NET Framework 4.0 and Visual Studio 2010. From the Entity Data Model (EDM) and Object Services to EntityClient and the Metadata Work

  17. Rare presentation of pancreatic schwannoma: a case report

    Directory of Open Access Journals (Sweden)

    Tofigh Arash

    2008-08-01

    Full Text Available Abstract Introduction Schwannoma is a rare tumor among pancreatic neoplasms. Schwannomas vary in size, and most of them are cystic, mimicking pancreatic cystic lesions. Generally, a definitive diagnosis is made at the time of histological analysis. The mainstay treatment is surgical resection. Case presentation We report an unusual presentation of pancreatic schwannoma with abdominal pain and several episodes of cholangitis in a 54-year-old Caucasian (Iranian man. The condition was not diagnosed pre-operatively and Whipple's procedure was performed. Conclusion Pancreatic schwannoma is an important clinical entity to include in the differential diagnosis of pancreatic lesions. Pre-operative diagnosis is difficult but computed tomographic findings may be helpful. The tumor may also have atypical and rare presentations, such as cholangitis and weight loss. For benign tumors, simple enucleation is usually adequate, whereas malignant tumors require standard oncological resection.

  18. A rare case of extremely high counts of circulating tumor cells detected in a patient with an oral squamous cell carcinoma

    International Nuclear Information System (INIS)

    Wu, Xianglei; Mastronicola, Romina; Tu, Qian; Faure, Gilbert Charles; De Carvalho Bittencourt, Marcelo; Dolivet, Gilles

    2016-01-01

    Despite aggressive regimens, the clinical outcome of head and neck squamous cell carcinoma remains poor. The detection of circulating tumor cells could potentially improve the management of patients with disseminated cancer, including diagnosis, treatment strategies, and surveillance. Currently, CellSearch ® is the most widely used and the only Food and Drug Administration-cleared system for circulating tumor cells detection in patients with metastatic breast, colorectal, or prostate cancer. In most cases of head and neck squamous cell carcinoma, only low counts of circulating tumor cells have been reported. A 56-year-old white male with no particular medical history, was diagnosed with a squamous cell carcinoma of oral cavity. According to the imaging results (computed tomography and 18 F-fluorodeoxyglucose positron emission tomography / computed tomography) and panendoscopy, the TNM staging was classified as T4N2M0. A non-interruptive pelvimandibulectomy was conducted according to the multidisciplinary meeting advices and the postoperative observations were normal. The patient complained of a painful cervical edema and a trismus 6 weeks after the surgery. A relapse was found by computed tomography and the patient died two weeks later. The search for circulating tumor cells in peripheral venous blood by using the CellSearch ® system revealed a very high count compared with published reports at three time points (pre-operative: 400; intra-operative: 150 and post-operative day 7: 1400 circulating tumor cells). Of note, all detected circulating tumor cells were epidermal growth factor receptor negative. We report here for the first time a rare case of oral squamous cell carcinoma with extremely high circulating tumor cells counts using the CellSearch ® system. The absolute number of circulating tumor cells might predict a particular phase of cancer development as well as a poor survival, potentially contributing to a personalized healthcare

  19. [Epithelioid hemangioendothelioma: an uncommon liver tumor].

    Science.gov (United States)

    Pareja, Eugenia; Cortés, Miriam; Rayon, Miguel; Moya, Angel; Mir, Jose

    2010-01-01

    We report the case of a female patient who was referred to our unit because of a solid liver tumor, suggestive of metastasis. After biopsy, the patient was diagnosed with epithelioid hemangioendothelioma of the liver. Epithelioid hemangioendothelioma is a rare entity with an unpredictable, potentially fatal, clinical course and outcome. Due to its rarity, this entity should be considered when a solitary hepatic lesion is detected and should be included in the differential diagnosis with liver metastases. We highlight the infrequency of this tumor, its presentation as a solitary hepatic lesion and the indication of surgical treatment. We describe the clinical and pathological characteristics of epithelioid hemangioendothelioma of the liver and report a new case of this entity. The distinct therapeutic options are discussed. Copyright 2010 Elsevier España, S.L. All rights reserved.

  20. Wilms tumor arising in extracoelomic paravertebral soft tissues.

    LENUS (Irish Health Repository)

    Mulligan, Linda

    2012-02-01

    Extrarenal Wilms tumor (ERWT) is a well-established entity which most commonly arises within the genitourinary tract, including intracoelomic paranephric soft tissue. Rarely, ERWT arises within teratoma, and it tends to occur predominantly in distinct settings, such as females with spinal defects and males with testicular teratomas. We report a unique ERWT arising within an extracoelomic teratoma of the paraspinal musculature, thereby expanding the range of reported locations for this unusual tumor.

  1. Small round blue cell tumor of seminal vesicle in a young patient

    Directory of Open Access Journals (Sweden)

    Adriano A. De Paula

    2006-10-01

    Full Text Available Seminal vesicle tumor is a rare disease with unclear origin. Generally, it is presented as a pelvic mass that can be detected by sonography and digital rectal exam. The authors report a 25-year-old patient with a pelvic mass which the magnetic resonance and surgical specimen reveal a seminal vesicle tumor. Immunohistochemical findings favored a primitive neuroectodermal tumor of the seminal vesicle. Herein, the treatment, histological and histochemical findings of this entity are discussed.

  2. Can vascular risk factors influence number and size of cerebral metastases? A 3D-MRI study in patients with different tumor entities.

    Science.gov (United States)

    Nagel, Sandra; Berk, Benjamin-Andreas; Kortmann, Rolf-Dieter; Hoffmann, Karl-Titus; Seidel, Clemens

    2018-02-01

    There is increasing evidence that cerebral microangiopathy reduces number of brain metastases. Aim of this study was to analyse if vascular risk factors (arterial hypertension, diabetes mellitus, smoking, and hypercholesterolemia) or the presence of peripheral arterial occlusive disease (PAOD) can have an impact on number or size of brain metastases. 200 patients with pre-therapeutic 3D-brain MRI and available clinical data were analyzed retrospectively. Mean number of metastases (NoM) and mean diameter of metastases (mDM) were compared between patients with/without vascular risk factors (vasRF). No general correlation of vascular risk factors with brain metastases was found in this monocentric analysis of a patient cohort with several tumor types. Arterial hypertension, diabetes mellitus, hypercholesterolemia and smoking did not show an effect in uni- and multivariate analysis. In patients with PAOD the number of BM was lower than without PAOD. This was the case independent from cerebral microangiopathy but did not persist in multivariate analysis. From this first screening approach vascular risk factors do not appear to strongly influence brain metastasation. However, larger prospective multi-centric studies with better characterized severity of vascular risk are needed to more accurately detect effects of individual factors. Copyright © 2018 Elsevier B.V. All rights reserved.

  3. Programming Drupal 7 entities

    CERN Document Server

    Michael, Sammy

    2013-01-01

    The book follows a standard tutorial-based approach to create, retrieve, update, and delete Drupal 7 entities, their properties and fields.Programming Drupal 7 Entities is perfect for intermediate or advanced developers new to Drupal entity development who are looking to get a good grounding in how to code using the new paradigm. It's assumed that you will have some experience in PHP development already, and being vaguely familiar with Drupal, GIT, and Drush will also help.

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

    Science.gov (United States)

    Efthimiadis, Christoforos; Ioannidis, Aristeidis; Grigoriou, Marios

    2017-01-01

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

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

    Science.gov (United States)

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

    2017-06-01

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

  6. Primary Orbital Chondromyxoid Fibroma: A Rare Case.

    Science.gov (United States)

    Mullen, Martin G; Somogyi, Marie; Maxwell, Sean P; Prabhu, Vikram; Yoo, David K

    A 56-year-old male with history of chronic sinusitis was found to have a 3 cm left orbital lesion on CT. Subsequent MRI demonstrated a multilobulated enhancing soft tissue lesion at the superotemporal region of the left orbit. Initial biopsy was reported as a low-grade sarcoma. On further evaluation, a consensus was made that the lesion was likely a benign mixed mesenchymal type tumor but should nonetheless be surgically removed. Left lateral orbitotomy was performed which revealed a tumor originating in the lateral orbital bone with segments eroding through the wall of the orbit. Intraoperative frozen sections revealed myoepitheliod tissue with locally aggressive features and the tumor was completely removed. The final histopathologic analysis of the tissue was consistent with a chondromyxoid fibroma. Chondomyxoid fibroma is a rare entity in the orbital bones and is more commonly seen in long bones.

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

    OpenAIRE

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

    2017-01-01

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

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

    Science.gov (United States)

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

    2013-01-01

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

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

    Science.gov (United States)

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

    2017-01-01

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

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

    Science.gov (United States)

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

    2018-01-29

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

  11. Solid pseudopapillary pancreas tumors. Often neglected

    International Nuclear Information System (INIS)

    Herrmann, K.A.; Reiser, M.F.; Zech, C.J.; Helmberger, T.; Bruns, C.

    2008-01-01

    Solid pseudopapillary tumors of the pancreas (SPTP) are rare tumors of the pancreas with low malignancy potential and a very good prognostic outcome after surgery. They typically occur in young women or adolescents and consist of solid, cystic and cystic-hemorrhagic components. Imaging findings in these tumors are characteristic and include a fibrotic capsule with a clear delineation and exhibit solid and cystic-hemorrhagic signal and density characteristics. Calcifications may be present in the periphery of the tumor. The tumor capsule shows contrast enhancement, the solid components in the periphery enhance in the early phase and gradually and inhomogeneously in late phases. MRI is superior to CT and other imaging modalities for characterization of SPTP. Awareness and knowledge of this tumor entity with an excellent prognosis is crucial to guide the patient towards effective, predominantly organ-sparing surgical treatment. (orig.) [de

  12. Neuroendocrine tumors of the pancreas

    International Nuclear Information System (INIS)

    Holzapfel, Konstantin; Rummeny, Ernst J.; Gaertner, Florian C.

    2011-01-01

    Neuroendocrine tumors (NET) of the pancreas are rare entities. Functioning tumors tend to present early with specific symptoms and typical abnormalities in laboratory values. In contrast, non-functioning NET are often diagnosed with delay and become evident by tumor-related symptoms like pain, weight-loss or jaundice. The role of imaging is to localize and delineate the primary tumor and to detect metastases. In the diagnosis of NET radiologic techniques like computed tomography (CT) and magnetic resonance imaging (MRI) are applied. In certain cases nuclear medicine techniques like somatostatin receptor scintigraphy (SRS) and positron emission tomography (PET) using radioactively labelled somatostatin analogues are used. The present article reviews characteristic imaging findings of both functioning and non-functioning NET of the pancreas. (orig.)

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

    Directory of Open Access Journals (Sweden)

    Yamada Masayoshi

    2012-03-01

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

  14. Imaging Biomarkers for Adult Medulloblastomas: Genetic Entities May Be Identified by Their MR Imaging Radiophenotype.

    Science.gov (United States)

    Keil, V C; Warmuth-Metz, M; Reh, C; Enkirch, S J; Reinert, C; Beier, D; Jones, D T W; Pietsch, T; Schild, H H; Hattingen, E; Hau, P

    2017-10-01

    The occurrence of medulloblastomas in adults is rare; nevertheless, these tumors can be subdivided into genetic and histologic entities each having distinct prognoses. This study aimed to identify MR imaging biomarkers to classify these entities and to uncover differences in MR imaging biomarkers identified in pediatric medulloblastomas. Eligible preoperative MRIs from 28 patients (11 women; 22-53 years of age) of the Multicenter Pilot-study for the Therapy of Medulloblastoma of Adults (NOA-7) cohort were assessed by 3 experienced neuroradiologists. Lesions and perifocal edema were volumetrized and multiparametrically evaluated for classic morphologic characteristics, location, hydrocephalus, and Chang criteria. To identify MR imaging biomarkers, we correlated genetic entities sonic hedgehog ( SHH ) TP53 wild type, wingless ( WNT ), and non -WNT/ non -SHH medulloblastomas (in adults, Group 4), and histologic entities were correlated with the imaging criteria. These MR imaging biomarkers were compared with corresponding data from a pediatric study. There were 19 SHH TP53 wild type (69%), 4 WNT -activated (14%), and 5 Group 4 (17%) medulloblastomas. Six potential MR imaging biomarkers were identified, 3 of which, hydrocephalus ( P = .03), intraventricular macrometastases ( P = .02), and hemorrhage ( P = .04), when combined, could identify WNT medulloblastoma with 100% sensitivity and 88.3% specificity (95% CI, 39.8%-100.0% and 62.6%-95.3%). WNT -activated nuclear β-catenin accumulating medulloblastomas were smaller than the other entities (95% CI, 5.2-22.3 cm 3 versus 35.1-47.6 cm 3 ; P = .03). Hemorrhage was exclusively present in non -WNT/ non -SHH medulloblastomas ( P = .04; n = 2/5). MR imaging biomarkers were all discordant from those identified in the pediatric cohort. Desmoplastic/nodular medulloblastomas were more rarely in contact with the fourth ventricle (4/15 versus 7/13; P = .04). MR imaging biomarkers can help distinguish histologic and genetic

  15. 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.

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

    Directory of Open Access Journals (Sweden)

    Hall MJ

    2015-02-01

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

  17. Mucoepidermoid carcinoma ex pleomorphic adenoma of the lacrimal gland: A rare presentation

    Directory of Open Access Journals (Sweden)

    Lily Daniel

    2014-01-01

    Full Text Available Carcinoma ex pleomorphic adenoma in lacrimal gland is a rare entity unlike its salivary gland counterpart. This rare tumor poses a diagnostic challenge to clinicians as pre-operative diagnosis is difficult and diagnosis is only by careful pathological assessment. We report this uncommon lesion in a 62-year-old lady, wherein the malignant component was mucoepidermoid carcinoma. The elderly patient remained clinically and radiologically free of the tumor for two years after complete excision of the tumor but computed tomography at the end of two and a half years showed a recurrent lesion in the region of the lacrimal gland. This makes long term follow up of patients with these rare lacrimal tumors imperative with a minimum period of at least five years.

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

    International Nuclear Information System (INIS)

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

    2014-01-01

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

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

    DEFF Research Database (Denmark)

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

    2017-01-01

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

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

    Directory of Open Access Journals (Sweden)

    Hilal Erinanc

    2018-01-01

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

  1. Programming Entity Framework

    CERN Document Server

    Lerman, Julia

    2010-01-01

    Get a thorough introduction to ADO.NET Entity Framework 4 -- Microsoft's core framework for modeling and interacting with data in .NET applications. The second edition of this acclaimed guide provides a hands-on tour of the framework latest version in Visual Studio 2010 and .NET Framework 4. Not only will you learn how to use EF4 in a variety of applications, you'll also gain a deep understanding of its architecture and APIs. Written by Julia Lerman, the leading independent authority on the framework, Programming Entity Framework covers it all -- from the Entity Data Model and Object Service

  2. 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.

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

    Directory of Open Access Journals (Sweden)

    Caroline J Voskens

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

  4. Usual Tumor at an Unusual Site - A Leiomyoma Masquerading as a Urethral Polyp.

    Directory of Open Access Journals (Sweden)

    Shilpa MN

    2013-01-01

    Full Text Available Polyps and papillomas encompass the most common benign tumors of the urethra.Leiomyoma of urethra is a rare clinical entity which can be diagnosed only with histopathological examination and only around 35 such cases have been reported worldwide.

  5. 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.

  6. CNN-based ranking for biomedical entity normalization.

    Science.gov (United States)

    Li, Haodi; Chen, Qingcai; Tang, Buzhou; Wang, Xiaolong; Xu, Hua; Wang, Baohua; Huang, Dong

    2017-10-03

    Most state-of-the-art biomedical entity normalization systems, such as rule-based systems, merely rely on morphological information of entity mentions, but rarely consider their semantic information. In this paper, we introduce a novel convolutional neural network (CNN) architecture that regards biomedical entity normalization as a ranking problem and benefits from semantic information of biomedical entities. The CNN-based ranking method first generates candidates using handcrafted rules, and then ranks the candidates according to their semantic information modeled by CNN as well as their morphological information. Experiments on two benchmark datasets for biomedical entity normalization show that our proposed CNN-based ranking method outperforms traditional rule-based method with state-of-the-art performance. We propose a CNN architecture that regards biomedical entity normalization as a ranking problem. Comparison results show that semantic information is beneficial to biomedical entity normalization and can be well combined with morphological information in our CNN architecture for further improvement.

  7. Primary Ovarian Leiomyoma: An Uncommon Entity ‑ Case Report ...

    African Journals Online (AJOL)

    Journal of Basic and Clinical Reproductive Sciences · January - June 2013 · Vol 2 · Issue 1. 54 ... case report of primary ovarian leiomyoma, which is a rare entity, in a postmenopausal female with ... There were extensive areas of edema.

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

    Science.gov (United States)

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

    2015-05-01

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

  9. Named Entity Linking Algorithm

    Directory of Open Access Journals (Sweden)

    M. F. Panteleev

    2017-01-01

    Full Text Available In the tasks of processing text in natural language, Named Entity Linking (NEL represents the task to define and link some entity, which is found in the text, with some entity in the knowledge base (for example, Dbpedia. Currently, there is a diversity of approaches to solve this problem, but two main classes can be identified: graph-based approaches and machine learning-based ones. Graph and Machine Learning approaches-based algorithm is proposed accordingly to the stated assumptions about the interrelations of named entities in a sentence and in general.In the case of graph-based approaches, it is necessary to solve the problem of identifying an optimal set of the related entities according to some metric that characterizes the distance between these entities in a graph built on some knowledge base. Due to limitations in processing power, to solve this task directly is impossible. Therefore, its modification is proposed. Based on the algorithms of machine learning, an independent solution cannot be built due to small volumes of training datasets relevant to NEL task. However, their use can contribute to improving the quality of the algorithm. The adaptation of the Latent Dirichlet Allocation model is proposed in order to obtain a measure of the compatibility of attributes of various entities encountered in one context.The efficiency of the proposed algorithm was experimentally tested. A test dataset was independently generated. On its basis the performance of the model was compared using the proposed algorithm with the open source product DBpedia Spotlight, which solves the NEL problem.The mockup, based on the proposed algorithm, showed a low speed as compared to DBpedia Spotlight. However, the fact that it has shown higher accuracy, stipulates the prospects for work in this direction.The main directions of development were proposed in order to increase the accuracy of the system and its productivity.

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

    Directory of Open Access Journals (Sweden)

    Kelven Weijing Chen

    2015-01-01

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

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

    Science.gov (United States)

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

    2015-01-01

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

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

    Science.gov (United States)

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

    2011-10-01

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

  13. Mastering entity framework

    CERN Document Server

    Singh, Rahul Rajat

    2015-01-01

    This book is for .NET developers who are developing data-driven applications using ADO.NET or other data access technologies. This book is going to give you everything you need to effectively develop and manage data-driven applications using Entity Framework.

  14. Myofibroblastic tumor associated to superior vena cava syndrome

    International Nuclear Information System (INIS)

    Ortega Jaramillo, Hector; Durango Gutierrez, Luisa Fernanda; Perez Figueroa, Maria del Pilar

    2005-01-01

    Inflammatory myofibroblastic tumor (IMT) is an uncommon pathological entity of unknown cause, composed of differentiated myofibroblastic cells accompanied by plasma cells, lymphocytes and eosinophils, which involve extrapulmonary and pulmonary tissues. IMT has an unpredictable clinical course, rarely undergoes malignant transformation. Local invasion and involvement of the mediastinum and hiliar structures are unusual manifestations; however; we reports a case of superior vena cava syndrome and IMT

  15. Carotid body tumor imitator: An interesting case of Castleman's disease.

    Science.gov (United States)

    Shakir, Hakeem J; Diletti, Sara M; Hart, Alexandra M; Meyers, Joshua E; Dumont, Travis M; Siddiqui, Adnan H

    2015-01-01

    There are very few reports in the literature of Castleman's disease affecting the carotid artery and a single previous report of a case of Castleman's disease of the neck originally mistaken as a carotid body tumor. We describe a rare case of Castleman's disease, manifesting with classic radiographic hallmarks of a carotid body tumor. The postoperative pathologic examination identified the resected mass as Castleman's lymphadenopathy. The management of this particular case is discussed, and the findings are highlighted. We present a unique case of a tumor initially and incorrectly diagnosed as a carotid body tumor. However, after comprehensive treatment with endovascular and surgical modalities and subsequent pathologic examination, the diagnosis of this rare entity was made.

  16. 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. ...

  17. Periocular xanthogranuloma: A forgotten entity?

    Directory of Open Access Journals (Sweden)

    Charalampos Papagoras

    2010-03-01

    Full Text Available Charalampos Papagoras1, George Kitsos2, Paraskevi V Voulgari1, Anastasia K Zikou3, Maria I Argyropoulou3, Aikaterini Zioga4, Alexandros A Drosos11Rheumatology Clinic, Department of Internal Medicine; 2Department of Ophthalmology; 3Department of Clinical Imaging and Radiology, 4Department of Pathology, Medical School, University of Ioannina, Ioannina, GreeceAbstract: Periocular xanthogranulomatous diseases are a rare group of disorders which are characterized by a predilection to affect the orbit and ocular adnexa and special histopathological features, in particular infiltrates comprising non-Langerhans-derived foamy histiocytes and Touton giant cells. The differential diagnosis is difficult and occasionally definite diagnosis cannot be established even after clinical and histopathological findings are taken together. We describe a case of a middle-aged man who presented with a 10-year history of voluminous eyelid swelling with concomitant late-onset atopic manifestations, namely bronchial asthma and allergic rhinitis with nasal polyps. After thorough clinical and laboratory investigation, including a biopsy of the eyelid, we classified the patient’s disease to a rare entity that has been relatively recently described: periocular xanthogranuloma associated with adult-onset asthma. In a review of the literature, no prospective trials concerning the treatment of this disease were found. The literature mainly contained case reports and case series in which corticosteroids and chemotherapy with alkylating agents have been reported to be beneficial. We treated our patient with a combination of oral corticosteroids and cyclophosphamide pulses and we observed substantial regression of the eyelid masses together with a normalization of systemic immunologic abnormalities.Keywords: periocular xanthogranuloma, adult-onset asthma, non-Langerhans histiocytoses, cyclophosphamide, methylprednisolone

  18. The single entity option

    International Nuclear Information System (INIS)

    Friedlander, M.C.; Roberts, K.M.

    1997-01-01

    Traditionally, an owner hires an engineer to design a power facility or other project and then circulates the completed plans to several contractors for competitive bidding. Although there are many variations on this theme, there is an alternative method which is growing in popularity--the design-build concept. In this construction method, the same entity designs and constructs the facility. The design builder may be a single firm with both design and construction capacity in-house, or it may be a combination of two or more firms with complementary abilities. If there are multiple firms, they may be structured as a joint venture or with one of the firms prime and the others in a subcontracting role. The critical aspect is that the owner contracts with one entity which has the responsibility for both designing and constructing the facility. According to statistics compiled by the Design-Build Institute of America and F.W. Dodge DATALINE2, a national reporter of construction statistics and information, from April 1995 to April 1996 the number of design-build contracts increased 103 percent over the previous year. Of a total $212 billion construction market, about $37.2 billion--18 percent--was design build. The strongest growth was in the category of industrial--plants, refineries, factories and warehouses--in which the concept use was up more than 300 percent from the previous year

  19. Inflammatory Myofibroblastic Tumor of the Right Atrium

    Directory of Open Access Journals (Sweden)

    Neerod K. Jha

    2010-01-01

    Full Text Available Cardiac inflammatory myofibroblastic tumor (IMT is a rare entity and is associated with distinct clinical, pathological and molecular features. The clinical behavior, natural history, biological potential, management and prognosis of such tumors are unclear. We present herewith an adolescent girl who presented with similar entity involving the junction of the right atrium and the inferior vena cava (IVC in association with thrombocytosis and IVC thrombosis leading to obstruction of blood flow. Diagnostic tools included imaging and immuno-histopathology studies. Surgical management included resection of the tumor and thrombo-embolectomy of the IVC under cardiopulmonary bypass. This case is unique due to association of complete obstruction of IVC caused by the strategic location of the tumor, thrombosis of vena cava and association of thrombocytosis. These features have not been reported yet in relation to the cardiac IMT. This report will help in better understanding and management of similar cases in terms of planning cannulation of femoral veins or application of total hypothermic circulatory arrest during cardiopulmonary bypass and prompt us to look for recurrence or metastasis during follow up using echocardiography and laboratory investigations. The possibility of IMT should be kept in the differential diagnosis of cardiac tumors especially in children and adolescents.

  20. Solitary fibrous tumor arising in an intrathoracic goiter

    DEFF Research Database (Denmark)

    Larsen, Stine Rosenkilde; Godballe, Christian; Krogdahl, Annelise

    2010-01-01

    . CONCLUSION: The histological appearance and immunohistochemical reaction pattern of SFT is characteristic. The entity should be considered when dealing with a spindle cell lesion in the thyroid gland. All cases of this site of origin reported have had a benign clinical course. As only a small number of cases......BACKGROUND: Solitary fibrous tumor (SFT) is a rare spindle cell tumor most often found in the mediastinal pleura. Nineteen cases of SFT arising in the thyroid gland have been reported. We report a case of SFT of the thyroid gland with immunohistochemical and cytogenetic investigation. SUMMARY: A 58...

  1. Primary dorsal spine primitive neuroectodermal tumor in an adult patient: Case report and literature review

    Directory of Open Access Journals (Sweden)

    Satyashiva Munjal

    2017-01-01

    Full Text Available Primary spinal primitive neuroectodermal tumor (psPNET is a rare entity with few cases reported in literature. We report a case of a 50-year-old female who presented to us with paraplegia and was diagnosed with extradural dorsal spine psPNET. The diagnosis was not suspected at presentation or on radiology but was established on histopathological examination. It is important to distinguish it from central nervous system primitive neuroectodermal tumors and from other spinal tumors since it follows a different clinical course and therapeutic outcome.

  2. Intrarenal neuroblastoma mimics Wilms' tumor

    International Nuclear Information System (INIS)

    Muniz, Maria T. Cartaxo; Soares, Andrezza B.; Freitas, Elizabete M.; Araujo, Marcela; Pureza, Leda M.M.; Morais, Adriana; Antunes, Consuelo; Salles, Terezinha de J. Marques; Borges, Josenilda C.; Morais, Vera L.L. de; Romualdo Filho, Jose; Magalhaes, Mario H.

    2005-01-01

    This work reports the case history of a child with intrarenal neuroblastoma, initially diagnosed as Wilms' tumor. The patient, a one year and three months old girl, presented a hard abdominal mass on the left flank that extended to the meso gastric region, plus fever and paleness. The ultrasound of the entire abdomen revealed an intrarenal mass. Biopsy with fine needle in many points of the tumor revealed Wilms' tumor. The scarcely of the material, however, made immunohistoquemistry impossible at that moment. Because of the child's severe condition the SIOP protocol was started. As no clinical response was observed, an exploratory laparotomy was indicated with partial resection of the tumor and bone marrow aspiration (MO). The histopathologic study revealed a malignant neoplasia of small cells, poorly differentiated. IHQ was negative for WT-1 and positive for NB-84, synaptofisin, cromogranine. N-myc amplification was observed by molecular biology. The bone marrow aspiration identified metastatic small round cells infiltration. Intrarenal neuroblastoma is a rare entity that clinically and radiographically resembles Wilms' tumor. The objective of this case report is to show the importance of immunohistochemical and molecular analysis in the diagnosis of intrarenal neuroblastoma. (author)

  3. Query containment in entity SQL

    OpenAIRE

    Rull Fort, Guillem; Bernstein, Philip A.; Garcia dos Santos, Ivo; Katsis, Yannis; Melnik, Sergey; Teniente López, Ernest

    2013-01-01

    We describe a software architecture we have developed for a constructive containment checker of Entity SQL queries defined over extended ER schemas expressed in Microsoft's Entity Data Model. Our application of interest is compilation of object-to-relational mappings for Microsoft's ADO.NET Entity Framework, which has been shipping since 2007. The supported language includes several features which have been individually addressed in the past but, to the best of our knowledge, they have not be...

  4. Incidental intraoperative discovery of a pancreatic neuroendocrine tumor associated with chronic pancreatitis

    Directory of Open Access Journals (Sweden)

    Surlin Valeriu

    2012-09-01

    Full Text Available Abstract Pancreatic neuroendocrine tumors are a rare entity with an incidence between 2 per million to 5 per 100 000. Association with pancreatitis (acute or chronic is rare and is considered to be determined by the tumoral obstruction of pancreatic ducts, but sometimes occurs without any apparent relationship between them. Non-functional neuroendocrine pancreatic tumors are usually diagnosed when either very large or metastatic. Small ones are occasionally diagnosed when imagery is performed for other diagnostic reasons. Intraoperative discovery is even rarer and poses problems of differential diagnosis with other pancreatic tumors. Association with chronic pancreatitis is rare and usually due to pancreatic duct obstruction by the tumor. We describe the case of a patient with a small non-functioning neuroendocrine tumor in the pancreatic tail accidentally discovered during surgery for delayed traumatic splenic rupture associated with chronic alcoholic pancreatitis. The tumor of 1.5cm size was well differentiated and confined to the pancreas, and was resected by a distal splenopancreatectomy. Conclusions Surgeons should be well aware of the rare possibility of a non-functional neuroendocrine tumor in the pancreas, associated with chronic pancreatitis, surgical resection being the optimal treatment for cure. Histopathology is of utmost importance to establish the correct diagnosis, grade of differentiation, malignancy and prognosis. Virtual slides The virtual slide(s for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2114470176676003.

  5. Aspergillus Salpingitis: A Rare Case Report

    African Journals Online (AJOL)

    rare entity, the correct diagnosis is of great importance for the indication of proper therapy. KEY WORDS: America ... Salpingitis is the most common serious infection in women .... immune systems, especially those recipients of stem-cell and.

  6. Primary Squamous Cell Carcinoma of Stomach: A Rare Entity ...

    African Journals Online (AJOL)

    Schmidt C, Schmid A, Lüttges JE, Kremer B, Henne-Bruns D. Primary squamous cell carcinoma of the stomach. Report of a case and review of literature. Hepatogastroenterology 2001;48:1033-6. 5. Muto M, Hasebe T, Muro K, Boku N, Ohtsu A, Fujii T, et al. Primary squamous cell carcinoma of the stomach: A case report with ...

  7. Aneurysms in pediatric age: A challenging and rare disease entity ...

    African Journals Online (AJOL)

    Ligation was allowed only after ensuring that the distal collateral circulation was adequate. Results Eight aneurysms were reconstructed and one carotid aneurysm was ligated. No neurologic events occurred after the carotid ligation. Follow-up ranged between 4 months and 4 years and showed no recurrences or occlusion ...

  8. Ichthyosiform large plaque parapsoriasis: report of a rare entity.

    Science.gov (United States)

    Nag, Falguni; Ghosh, Arghyaprasun; Biswas, Projna; Chatterjee, Gobinda; Biswas, Saugato

    2013-09-01

    Large plaque parapsoriasis (LPP) is an idiopathic, chronic scaly dermatosis classified within parapsoriasis group of diseases, occurring commonly in middle aged patients of all races and geographic regions. LPP and its variants are closely related to the patch stage of mycosis fungoides. The two types of LPP mostly described are the poikilodermatous and retiform parapsoriasis. We are reporting an ichthyosiform LPP for its rarity.

  9. Ichthyosiform large plaque parapsoriasis: Report of a rare entity

    Directory of Open Access Journals (Sweden)

    Falguni Nag

    2013-01-01

    Full Text Available Large plaque parapsoriasis (LPP is an idiopathic, chronic scaly dermatosis classified within parapsoriasis group of diseases, occurring commonly in middle aged patients of all races and geographic regions. LPP and its variants are closely related to the patch stage of mycosis fungoides. The two types of LPP mostly described are the poikilodermatous and retiform parapsoriasis. We are reporting an ichthyosiform LPP for its rarity.

  10. Clinical outcomes in traumatic pseudophacocele: A rare clinical entity.

    Science.gov (United States)

    Narang, Priya; Agarwal, Amar

    2017-12-01

    The purpose of this study is to evaluate the clinical outcomes in patients with traumatic pseudophacocele. In this retrospective, interventional case series, scleral wound repair with pars plana vitrectomy and glued intrascleral fixation of an intraocular lens (glued IOL) was performed in 5 eyes of 5 patients. Pupilloplasty was performed in 3 cases whereas aniridia glued IOL fixation was done in 1 case that had total avulsion and loss of iris tissue. The main outcome measures were best-corrected visual acuity (BCVA), intraoperative and postoperative complications during the entire follow-up period. The preoperative vision ranged from hand movement to perception of light in all the patients. The mean postoperative BCVA was 0.42 ± 0.21 Snellen's decimal equivalent (SDE) at final follow-up. Postoperatively, all the cases retained good visual acuity with case 1 and case 2 reporting 0.5 SDE, case 3 had 0.33 SDE, case 5 had 0.67 SDE whereas case 4 had a final visual acuity limited to 0.1 SDE due to associated corneal opacity. The mean follow-up period was 20.2 ± 11.7 months (range from 9 months to 36 months). The IOL was well centered, all the wounds were well apposed and the mean postoperative intraocular pressure was 14.6 ± 1.95 mm Hg. No complications were reported in the entire follow-up period. The clinical outcomes of management of pseudophacocele were encouraging with retention of reasonably good visual potential in all cases.

  11. Retrocaval ureter with vesicoureteric reflux, a very rare entity

    African Journals Online (AJOL)

    M.C. Arya

    2016-12-26

    Dec 26, 2016 ... side vesicoureteric reflux). Intravenous urogram (IVU) showed diagnostic dilemma between pelviureteric junction obstruction and retrocaval ureter (Fig. 2 – IVU suggestive of pelviureteric junction obstruction). For confirmation of the diagnosis magnetic resonance urography (MRU) (T2 weighted) was done ...

  12. Orthostatic Tremor: An Update on a Rare Entity

    Science.gov (United States)

    Benito-León, Julián; Domingo-Santos, Ángela

    2016-01-01

    Background Orthostatic tremor (OT) remains among the most intriguing and poorly understood of movement disorders. Compared to Parkinson’s disease or even essential tremor, there are very few articles addressing more basic science issues. In this review, we will discuss the findings of main case series on OT, including data on etiology, pathophysiology, diagnostic approach, treatment strategies, and outcome. Methods Data for this review were identified by searching PUBMED (January 1966 to August 2016) for the terms “orthostatic tremor” or “shaky leg syndrome,” which yielded 219 entries. We did not exclude papers on the basis of language, country, or publication date. The electronic database searches were supplemented by articles in the authors’ files that pertained to this topic. Results Owing to its rarity, the current understanding of OT is limited and is mostly based on small case series or case reports. Despite this, a growing body of evidence indicates that OT might be a progressive condition that is clinically heterogeneous (primary vs. secondary cases) with a broader spectrum of clinical features, mainly cerebellar signs, and possible cognitive impairment and personality disturbances. Along with this, advanced neuroimaging techniques are now demonstrating distinct anatomical and functional changes, some of which are consistent with neuronal loss. Discussion OT might be a family of diseases, unified by the presence of leg tremor, but further characterized by etiological and clinical heterogeneity. More work is needed to understand the pathogenesis of this condition. PMID:27713855

  13. CASE REPORT Urachal remnant carcinoma - a rare entity

    African Journals Online (AJOL)

    Magnetic resonance imaging (MRI) was not undertaken as it was thought to confer little benefit in the setting of advanced disease. Following histological confirmation of moderately differentiated invasive adenocarcinoma of the urachal remnant, and administration of chemotherapy, the patient developed cerebral metastasis ...

  14. Glial heterotopia of the orbit: a rare cause of proptosis.

    Science.gov (United States)

    Bakhti, Souad; Terkmani, Fella; Tighilt, Nabila; Benmouma, Youcef; Boumehdi, Nazim; Djennas, Mohamed

    2016-11-01

    Glial heterotopia is defined as presence of normal glial tissue in an unusual location without connection with the brain. It is a very rare clinical entity occuring mostly in the head and neck region which is generally present at birth. Orbital location is very rare. We report a case of a 4-month-old girl presenting congenital proptosis with progressive increase. CT scan revealed an intraorbital mass without bony defect. The patient was operated, and resection was subtotal. Histologically, the tumor was composed of glial tissue with plexus choroid and pathologist concluded glial heterotopia. The child is under constant medical supervision because recurrences can be observed after incomplete resection; she had no new clinical signs at 18 months follow-up.

  15. Ovarian Sertoli-Leydig cell tumor with heterologous elements of gastrointestinal type associated with elevated serum alpha-fetoprotein level: an unusual case and literature review.

    Science.gov (United States)

    Horta, Mariana; Cunha, Teresa Margarida; Marques, Rita Canas; Félix, Ana

    2014-11-01

    Here we describe the case of a 19-year-old woman with a poorly differentiated ovarian Sertoli-Leydig cell tumor and an elevated serum alpha-fetoprotein level. The patient presented with diffuse abdominal pain and bloating. Physical examination, ultrasound, and magnetic resonance imaging revealed a right ovarian tumor that was histopathologically diagnosed as a poorly differentiated Sertoli-Leydig cell tumor with heterologous elements. Her alpha-fetoprotein serum level was undetectable after tumor resection. Sertoli-Leydig cell tumors are rare sex cord-stromal tumors that account for 0.5% of all ovarian neoplasms. Sertoli-Leydig cell tumors tend to be unilateral and occur in women under 30 years of age. Although they are the most common virilizing tumor of the ovary, about 60% are endocrine-inactive tumors. Elevated serum levels of alpha-fetoprotein are rarely associated with Sertoli-Leydig cell tumors, with only approximately 30 such cases previously reported in the literature. The differential diagnosis should include common alpha-fetoprotein-producing ovarian entities such as germ cell tumors, as well as other non-germ cell tumors that have been rarely reported to produce this tumor marker.

  16. Entity resolution for uncertain data

    NARCIS (Netherlands)

    Ayat, N.; Akbarinia, R.; Afsarmanesh, H.; Valduriez, P.

    2012-01-01

    Entity resolution (ER), also known as duplicate detection or record matching, is the problem of identifying the tuples that represent the same real world entity. In this paper, we address the problem of ER for uncertain data, which we call ERUD. We propose two different approaches for the ERUD

  17. Multiple Ewing Sarcoma/Primitive Neuroectodermal Tumors in the Mediastinum

    Science.gov (United States)

    Bae, Sung Hwan; Hwang, Jung Hwa; Da Nam, Bo; Kim, Hyun Jo; Kim, Ki-Up; Kim, Dong Won; Choi, In Ho

    2016-01-01

    Abstract Ewing sarcoma/primitive neuroectodermal tumors (ES/PNET) are high-grade malignant neoplasms. These malignancies present very rare tumors of thoracopulmonary area and even rarer in the mediastinum. In our knowledge, ES/PNET presented with multiple mediastinal masses has not been reported previously. We experienced a case of a 42-year-old man presented with gradual onset of left-side pleuritic chest pain. A contrast-enhanced chest computed tomography (CT) scan showed separate 2 large heterogeneously enhancing masses in each anterior and middle mediastinum of the left hemithorax. Positron emission tomography-computed tomography (PET-CT) scan revealed high fluorodeoxyglucose (FDG) uptake in the mediastinal masses. After surgical excision for the mediastinal masses, both of the masses were diagnosed as the ES/PNET group of tumors on the histopathologic examination. The patient refused postoperative adjuvant chemotherapy and came back with local tumor recurrence and distant metastasis on 4-month follow-up after surgical resection. We report this uncommon form of ES/PNET. We are to raise awareness that this rare malignancy should be considered as a differential diagnosis of the malignant mediastinal tumors and which can be manifested as multiple masses in a patient. Understanding this rare entity of extra-skeletal ES/PNET and characteristic imaging findings can help radiologists and clinicians to approach proper diagnosis and better management for this highly malignant tumor. PMID:26886614

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

    Directory of Open Access Journals (Sweden)

    Nicolas Ugolin

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

  19. Primary Leiomyosarcoma of the Breast: A Rare Case Report

    Directory of Open Access Journals (Sweden)

    Pallavi Agrawal

    2015-08-01

    Full Text Available Background: Primary leiomyosarcoma (LMS of the breast is a very rare neoplasm of the breast arising from the mesenchymal tissue. The clinical presentation of this entity simulates other benign and malignant lesions of mesenchymal tissue of the breast.Case presentation: Case 1: A 40-year-old female presented with a right-sided breast lump, which was suspected to be a malignant spindle cell tumor on needle core biopsy (NCB. A multi-disciplinary team performed modified radical mastectomy (MRM with axillary node dissection on the patient with no post-operative chemo-radiation. Case 2: A 70-year-old female presented with a left sided breast lump and a palpable axillary node. Needle core biopsy diagnosed it as malignant spindle cell tumor. The patient underwent MRM with axillary node dissection. It was confirmed to be a case of breast LMS with axillary nodal metastasis. Both patients were followed up for one year with no evidence of recurrence.Conclusion: Both cases underwent MRM with axillary node dissection in our study. However, the role of axillary dissection in the prognosis and disease-free survival of the patients with primary LMS of the breast with axillary metastasis has not been studied yet. The optimal management of this entity remains to be tumor excision with clear margins.

  20. Keratocystic odontogenic tumor: A case report and review of literature

    Directory of Open Access Journals (Sweden)

    Keerthi K Nair

    2015-01-01

    Full Text Available Keratocystic odontogenic tumor (KCOT has been identified as a "tumor" after observation of its biological behavior and genetic abnormalities consistent with neoplastic progression. In 2005, the World Health Organization (WHO working group considered odontogenic keratocyst (OKC to be a tumor and recommended the term KCOT, distinguishing the lesion from the orthokeratinizing variant, which is now considered an OKC or orthokeratinized odontogenic cyst. Very rarely, KCOTs can transform into more aggressive lesions such as ameloblastoma and primary intraosseous carcinoma (PIOSCC. In this paper, we present a case of KCOT involving the angle and ramus of the mandible, with histopathologic evidence of ameloblastomatous changes. We also discuss about the evolution of this lesion from a cyst to a tumor along with the latest updates of the entity.

  1. Spinal tumors

    International Nuclear Information System (INIS)

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

    2004-01-01

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

  2. Report of Two Cases of Combined Odontogenic Tumors: Ameloblastoma with Odontogenic Keratocyst and Ameloblastic Fibroma with Calcifying Odontogenic Cyst.

    Science.gov (United States)

    Neuman, Ashley Nicole; Montague, Lindsay; Cohen, Donald; Islam, Nadim; Bhattacharyya, Indraneel

    2015-09-01

    Combined odontogenic neoplasms have rarely been documented. Such tumors have also been described by other researchers as "hybrid" lesions. The histologic features are often identical to other individually well-established odontogenic neoplasms such as ameloblastoma, adenomatoid odontogenic tumor, ameloblastic fibroma (AF), and ameloblastic fibro-odontoma. Their clinical presentation is variable, ranging from cysts to neoplasms showing varying degrees of aggressive behavior. Most combined tumors contain features of one of the odontogenic tumors in combination with either a calcifying odontogenic cyst (COC) or a calcifying epithelial odontogenic tumor. We present two new cases of combined odontogenic tumors: an ameloblastoma with an odontogenic keratocyst and an AF with COC. Predicting clinical outcome is challenging when a combination tumor is encountered due to the paucity of such lesions. One must understand salient features of these entities and differentiate them from the more common conventional neoplasms to expand classification and provide prognostic criteria.

  3. Metaphyseal giant cell tumor

    International Nuclear Information System (INIS)

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

    1986-01-01

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

  4. Glial tumors with neuronal differentiation.

    Science.gov (United States)

    Park, Chul-Kee; Phi, Ji Hoon; Park, Sung-Hye

    2015-01-01

    Immunohistochemical studies for neuronal differentiation in glial tumors revealed subsets of tumors having both characteristics of glial and neuronal lineages. Glial tumors with neuronal differentiation can be observed with diverse phenotypes and histologic grades. The rosette-forming glioneuronal tumor of the fourth ventricle and papillary glioneuronal tumor have been newly classified as distinct disease entities. There are other candidates for classification, such as the glioneuronal tumor without pseudopapillary architecture, glioneuronal tumor with neuropil-like islands, and the malignant glioneuronal tumor. The clinical significance of these previously unclassified tumors should be confirmed. Copyright © 2015 Elsevier Inc. All rights reserved.

  5. 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

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

    Directory of Open Access Journals (Sweden)

    Sankappa P. Sinhasan

    2014-01-01

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

  7. A Case of Malignant Peripheral Nerve Sheath Tumor with Rhabdomyoblastic Differentiation: Malignant Triton Tumor

    Directory of Open Access Journals (Sweden)

    Kenichiro Mae

    2013-12-01

    Full Text Available Malignant peripheral nerve sheath tumors (MPNST constitute a rare variety of soft tissue sarcomas thought to originate from Schwann cells or pluripotent cells of the neural crest. Malignant triton tumor (MTT, a very rare, highly aggressive soft tissue tumor, is a subgroup of MPNST and is comprised of malignant Schwann cells coexisting with malignant rhabdomyoblasts. We herein report the case of a 24-year-old man who presented a subcutaneous mass in his right thigh. The mass was removed surgically in its entirety and radiation therapy was applied locally to prevent tumor regrowth. Nonetheless, the patient died 10 months after surgery from metastases to the lung and brain. He presented neither cafe-au-lait spots nor cutaneous neurofibromas. The histopathology showed a transition from a neurofibroma to an MTT, making this the second report of an MTT arising from a neurofibroma without neurofibromatosis type 1, an autosomal dominant disorder with which 50-70% of tumors reported in previous studies were associated. A histopathological examination using immunostaining with desmin confirmed this diagnosis. MTT has a poorer prognosis than MPNST and should therefore be regarded as a distinct clinical entity.

  8. Advances in the Genetic Characterization of Cutaneous Mesenchymal Neoplasms: Implications for Tumor Classification and Novel Diagnostic Markers.

    Science.gov (United States)

    Compton, Leigh A; Doyle, Leona A

    2017-06-01

    Cutaneous mesenchymal neoplasms often pose significant diagnostic challenges; many such entities are rare or show clinical and histologic overlap with both other mesenchymal and non-mesenchymal lesions. Recent advances in the genetic classification of many cutaneous mesenchymal neoplasms have not only helped define unique pathologic entities and increase our understanding of their biology, but have also provided new diagnostic markers. This review details these recent discoveries, with a focus on their implications for tumor classification and diagnosis. Copyright © 2017 Elsevier Inc. All rights reserved.

  9. 7 CFR 1738.16 - Eligible entities.

    Science.gov (United States)

    2010-01-01

    ... cooperative, nonprofit, limited dividend or mutual associations, limited liability companies, commercial... or partnerships of individuals are not eligible entities. (2) An entity is not eligible if it serves...

  10. Gastrointestinal stromal tumor masquerading as a lung neoplasm. A case presentation and literature review

    Directory of Open Access Journals (Sweden)

    Papagiannopoulos K

    2008-05-01

    Full Text Available Abstract Gastrointestinal stromal tumors (GISTs are rare neoplasms of the gastrointestinal tract. Their incidence in the esophagus is 1%–3%. Never has a GIST been documented to directly invade the lung. We report a primary esophageal GIST with direct invasion into the lung parenchyma, presenting predominantly with respiratory symptoms. We include a retrospective literature review. Although the principle 'common things are common' usually guides our everyday clinical practice, this case emphasizes that rare entities can mimic common pathologies and underlines the importance of having a clearly defined differential diagnostic list which should be meticulously scrutinized.

  11. Primary peripheral primitive neuroectodermal tumor/Ewing's tumor of the testis in a 46-year-old man-differential diagnosis and review of the literature.

    Science.gov (United States)

    Heikaus, Sebastian; Schaefer, Karl-Ludwig; Eucker, Jan; Hogrebe, Esther; Danebrock, Raihanatou; Wai, Daniel H; Krenn, Veit; Gabbert, Helmut E; Poremba, Christopher

    2009-06-01

    Peripheral primitive neuroectodermal tumor/Ewing's tumors are rare bone and soft tissue malignancies with a highly aggressive clinical course and early metastases occurring at multiple peripheral sites. Here, we present for the first time a case of a 46-year-old man with a primary peripheral primitive neuroectodermal tumor/Ewing's tumor of the testis. The diagnosis of peripheral primitive neuroectodermal tumor/Ewing's tumor was established by histology, immunohistochemistry, and molecular pathology. The tumor revealed a rapid progress in 2 months' time. Therefore, the patient was included in the EURO-E.W.I.N.G.99 study and was placed on chemotherapy. However, the tumor progressed during ongoing therapy, and the patient died in March 2008. In conclusion, though being reported here for the first time, peripheral primitive neuroectodermal tumor/Ewing's tumors should be considered in the differential diagnosis of blue round cell tumors of the testis. A rapid and correct diagnosis of this entity is crucial for fast and accurate therapy, which is stressed by the fatal case presented here.

  12. Acute steroid myopathy: a highly overlooked entity.

    Science.gov (United States)

    Haran, Michal; Schattner, Ami; Kozak, Natasha; Mate, Andras; Berrebi, Alain; Shvidel, Lev

    2018-02-15

    Myopathy in patients being treated with corticosteroids is known primarily among chronically-treated patients or in critically ill and mechanically-ventilated patients receiving corticosteroids, often in high doses. To highlight the entity of acute, early-onset corticosteroid-treatment-associated myopathy and its characteristics. Reporting our experience with four patients and reviewing all published reports of myopathy developing ≤14 days of initiating corticosteroid-treatment. Acute corticosteroid myopathy (ASM) exists, though the syndrome appears to be rare. It is characterized by unpredictability and heterogeneity, sometimes developing within 1-3 days, after a single dose, which may not be high and administered by varied routes. Proximal limb muscle weakness is the most common form, but distal limb, bulbar and respiratory muscles may be involved. Steroid cessation often leads to improvement/resolution, but irreversibility may occur. A high index of suspicion for the possibility of ASM is necessary, to ensure drug discontinuation and recovery. This is particularly true since the entity is not widely recognized and its symptoms are often erroneously interpreted as due to the patient's underlying disease.

  13. Neuroendocrine tumor of vulva: A case report and review of literature

    Directory of Open Access Journals (Sweden)

    Sheikh Zahoor

    2010-01-01

    Full Text Available Neuroendocrine tumor (Merkel cell carcinoma-MCC of the vulva is a very rare entity with less than 15 cases reported in the English literature. It is known for its aggressive behaviour and propensity for early dissemination. The actual cell of origin and etiology of this disease is controversial. In absence of any definite guidelines for management (due to its rarity, extrapolation of data from extra-vulvar MCC seems logical. We present a case of vulvar neuroendocrine tumor who presented at a locally advanced stage.

  14. Primary hyperparathyroidism associated with a giant cell tumor: One case in the distal radius.

    Science.gov (United States)

    Ouzaa, M R; Bennis, A; Iken, M; Abouzzahir, A; Boussouga, M; Jaafar, A

    2015-10-01

    Hyperparathyroidism can present itself as brown tumors (or osteolytic expansive lesions) that usually disappear after normalization of calcium and phosphate levels. It rarely occurs simultaneously with a giant cell tumor. The authors report one case of a localized form at the distal radius in a patient being followed for primary hyperparathyroidism. The diagnostic challenges related to the clinical and radiological similarities of these two pathological entities are discussed, as they can lead to delays in therapeutic management. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  15. Bone tumors

    International Nuclear Information System (INIS)

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

    1991-01-01

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

  16. Programming Entity Framework Code First

    CERN Document Server

    Lerman, Julia

    2011-01-01

    Take advantage of the Code First data modeling approach in ADO.NET Entity Framework, and learn how to build and configure a model based on existing classes in your business domain. With this concise book, you'll work hands-on with examples to learn how Code First can create an in-memory model and database by default, and how you can exert more control over the model through further configuration. Code First provides an alternative to the database first and model first approaches to the Entity Data Model. Learn the benefits of defining your model with code, whether you're working with an exis

  17. Entity ranking using Wikipedia as a pivot

    NARCIS (Netherlands)

    Kaptein, R.; Serdyukov, P.; de Vries, A.; Kamps, J.; Huang, X.J.; Jones, G.; Koudas, N.; Wu, X.; Collins-Thompson, K.

    2010-01-01

    In this paper we investigate the task of Entity Ranking on the Web. Searchers looking for entities are arguably better served by presenting a ranked list of entities directly, rather than a list of web pages with relevant but also potentially redundant information about these entities. Since

  18. Named entity normalization in user generated content

    NARCIS (Netherlands)

    Jijkoun, V.; Khalid, M.A.; Marx, M.; de Rijke, M.

    2008-01-01

    Named entity recognition is important for semantically oriented retrieval tasks, such as question answering, entity retrieval, biomedical retrieval, trend detection, and event and entity tracking. In many of these tasks it is important to be able to accurately normalize the recognized entities,

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

    Science.gov (United States)

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

    2018-02-01

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

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

    Energy Technology Data Exchange (ETDEWEB)

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

    1998-09-01

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

  1. Ameloblastomatous calcifying odontogenic cyst: A rare histologic variant

    Directory of Open Access Journals (Sweden)

    Basavaraj N Kallalli

    2015-01-01

    Full Text Available Ameloblastoma is a well-known odontogenic tumor that can be associated with calcifying odontogenic cysts (COCs, but only a few reports give its clinical and radiographic features. Calcifying odontogenic cyst was first categorized as a distinct entity by Gorlin et al., and has been named after him since then. Calcifying odontogenic cyst is an uncommon developmental odontogenic lesion that demonstrates histopathologic diversity. It is well known that this lesion can occur in association with odontogenic tumors such as complex odontoma and ameloblastoma. The term COC was not included by the World Health Organization (WHO in its report of 2005 and is called calcifying cystic odontogenic tumor (CCOT. Histopathologic examination of ameloblastomatous CCOT reveals ameloblastic islands containing ghost cells. Although association of ameloblastoma with this lesion is important, only a few cases have been reported in literature. The present case report is of ameloblastomatous calcifying cystic odontogenic tumor, a rare histologic variant, in a 20-year-old male patient in the left mandibular posterior region.

  2. Rare earths

    Energy Technology Data Exchange (ETDEWEB)

    Cranstone, D A

    1979-01-01

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

  3. Testing the Application for Analyzing Structured Entities

    OpenAIRE

    Ion IVAN; Bogdan VINTILA

    2011-01-01

    The paper presents the testing process of the application for the analysis of structured text entities. The structured entities are presented. Quality characteristics of structured entities are identified and analyzed. The design and building processes are presented. Rules for building structured entities are described. The steps of building the application for the analysis of structured text entities are presented. The objective of the testing process is defined. Ways of testing the applicat...

  4. Logical Entity Level Sentiment Analysis

    DEFF Research Database (Denmark)

    Petersen, Niklas Christoffer; Villadsen, Jørgen

    2017-01-01

    We present a formal logical approach using a combinatory categorial grammar for entity level sentiment analysis that utilizes machine learning techniques for efficient syntactical tagging and performs a deep structural analysis of the syntactical properties of texts in order to yield precise resu...

  5. A Calculus of Located Entities

    Directory of Open Access Journals (Sweden)

    Adriana Compagnoni

    2014-03-01

    Full Text Available We define BioScapeL, a stochastic pi-calculus in 3D-space. A novel aspect of BioScapeL is that entities have programmable locations. The programmer can specify a particular location where to place an entity, or a location relative to the current location of the entity. The motivation for the extension comes from the need to describe the evolution of populations of biochemical species in space, while keeping a sufficiently high level description, so that phenomena like diffusion, collision, and confinement can remain part of the semantics of the calculus. Combined with the random diffusion movement inherited from BioScape, programmable locations allow us to capture the assemblies of configurations of polymers, oligomers, and complexes such as microtubules or actin filaments. Further new aspects of BioScapeL include random translation and scaling. Random translation is instrumental in describing the location of new entities relative to the old ones. For example, when a cell secretes a hydronium ion, the ion should be placed at a given distance from the originating cell, but in a random direction. Additionally, scaling allows us to capture at a high level events such as division and growth; for example, daughter cells after mitosis have half the size of the mother cell.

  6. SOCIAL EFFECTIVENESS OF BUSINESS ENTITIES

    Directory of Open Access Journals (Sweden)

    Iryna Perevozova

    2016-06-01

    Full Text Available The article is aimed at investigation of social effectiveness of business entities. Social aspect of business is becoming a necessary component of success, increase of profitability and competitiveness as well as minimization of risks. Social effectiveness is referred to as correspondence between economic activity and main social needs and aims of society, interests of the staff and interests of a certain person. Investigation of social effectiveness of business entities is suggested to analyze with the help of social factors. Social factors are characterized by variability of expectations, relations and interests of society, staff and individuals. We suggest generalized classification of factors which have an impact on social effectiveness of business, we single out external and internal factors. To external factors belong: income of the population, differentiation of population according to income, migration, level of salaries, level of legality of income of population, family status of  population, employment rate, age structure of population etc. As for internal factors we single out the following: low level of basic professional training, use of unskilled workers, absence of conditions for creativity, imperfection of system of motivation of professional growth, absence of specialized centers for certification training etc. Quantity and quality analysis of the above mentioned factors will enable to determine the level of social effectiveness of business entities. For analyses of degree of influence of factors on effectiveness we worked out a questionnaire of expert assessment which is represented in the form of assessment scale. We conducted a questionnaire and analyzed expert results and determined degree of influence of factors on social effectiveness of business. Assessment of level of social effectiveness of business entities was carried out by expert method of certain factor and was represented by a formula. The scale of assessment of

  7. HGNET-BCOR Tumors of the Cerebellum: Clinicopathologic and Molecular Characterization of 3 Cases.

    Science.gov (United States)

    Appay, Romain; Macagno, Nicolas; Padovani, Laetitia; Korshunov, Andrey; Kool, Marcel; André, Nicolas; Scavarda, Didier; Pietsch, Torsten; Figarella-Branger, Dominique

    2017-09-01

    The central nervous system (CNS) high-grade neuroepithelial tumor with BCOR alteration (CNS HGNET-BCOR) is a recently described molecular entity. We report 3 new CNS HGNET-BCOR cases sharing common clinical presentation and pathologic features. The 3 cases concerned children aged 3 to 7 years who presented with a voluminous mass of the cerebellum. Pathologic features included proliferation of uniform spindle to ovoid cells with fine chromatin associated with a rich arborizing capillary network. Methylation profiling classified these cases as CNS HGNET-BCOR tumors. Polymerase chain reaction analysis confirmed the presence of internal tandem duplications in the C-terminus of BCOR (BCOR-ITD), a characteristic of these tumors, in all 3 cases. Immunohistochemistry showed a strong nuclear BCOR expression. In 2 cases, local recurrence occurred within 6 months. The third case, a patient who received a craniospinal irradiation after total surgical removal followed by a metronomics maintenance with irinotecan, temozolomide, and itraconazole, is still free of disease 14 months after diagnosis. In summary, CNS HGNET-BCOR represents a rare tumor occurring in young patients with dismal prognosis. BCOR nuclear immunoreactivity is highly suggestive of a BCOR-ITD. Whether CNS HGNET-BCOR should be classified among the category of "embryonal tumors" or within the category of "mesenchymal, nonmeningothelial tumors" remains to be clarified. Because CNS HGNET-BCOR share pathologic features and characteristic BCOR-ITD with clear cell sarcoma of the kidney, these tumors may represent local variants of the same entity.

  8. Primary tubercular abscess of the breast – an unusual entity

    Science.gov (United States)

    Gupta, R; Singal, RP; Gupta, A; Singal, S; Shahi, SR; Singal, R

    2012-01-01

    Primary breast tuberculosis manifested as abscess is a rare entity. We are reporting a case of primary breast tuberculosis, which presented as breast abscess. Abscess was drained and tissue sent for histopathology. To our surprise, diagnosis came as breast tuberculosis. Aspiration cytology was not done, as it is not a routine test for abscess cases. Patient was put on anti- tubercular drugs. In the follow-up of 6 months, she was asymptomatic and advised to continue medicine. PMID:22574095

  9. Primary tubercular abscess of the breast--an unusual entity.

    Science.gov (United States)

    Gupta, R; Singal, R P; Gupta, A; Singal, S; Shahi, S R; Singal, R

    2012-02-22

    Primary breast tuberculosis manifested as abscess is a rare entity. We are reporting a case of primary breast tuberculosis, which presented as breast abscess. Abscess was drained and tissue sent for histopathology. To our surprise, diagnosis came as breast tuberculosis. Aspiration cytology was not done, as it is not a routine test for abscess cases. Patient was put on anti- tubercular drugs. In the follow-up of 6 months, she was asymptomatic and advised to continue medicine.

  10. Ulcerative giant solitary trichoepithelioma of scalp: a rare presentation

    Directory of Open Access Journals (Sweden)

    Sundeep Chowdhry

    2016-07-01

    Full Text Available Trichoepithelioma is a trichogenic tumor which arises from the inferior segment of hair follicle epithelium as hamartoma. Giant solitary trichoepithelioma (GST has been defined as a solitary trichoepithelioma with a diameter greater than 2 cm. A 49-year-old female presented with a slow growing skin coloured swelling on the scalp of 8 years duration with recent history of ulceration and occasional bleeding. The local examination revealed a single well defined nodular swelling which was irregular in shape measuring approximately 2 × 2.5 cm. Histopathology from biopsy specimen revealed dark basaloid cells with scanty cytoplasm and darkly stained nucleus arranged in nests with horn cysts lacking high-grade atypia and mitosis, which was consistent with features of trichoepithelioma. Giant solitary trichoepithelioma of scalp is itself a rare entity and the present case is being reported with the additional component of ulceration in the lesion.

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

    Directory of Open Access Journals (Sweden)

    B R Vani

    2014-01-01

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

  12. Compulsive buying: an overlooked entity.

    Science.gov (United States)

    Basu, Bishnupriya; Basu, Saikat; Basu, Jharna

    2011-08-01

    Compulsive buying is an under-recognised entity among Indian psychiatrists. A Medline search, hand searching of journals and direct communications with lead investigators in compulsive buying have generated numerous studies. Overseas data indicate a community prevalence between 1% and 8% . The phenomenon can be an independent entity or appears as a comorbidity with another axis I or axis II disorder. A degree of suspicion on part of clinician regarding its possible presence is the key to its detection. A few rating instruments are available to quantify the morbidity and screening for compulsive buying. Management involves pharmacotherapy with SSRIs, psychotherapy, self-help groups and self-help books. Epidemiological and clinical studies on compulsive buying should be undertaken by Indian psychiatrists to provide better services for people suffering from compulsive buying.

  13. Rare earths

    International Nuclear Information System (INIS)

    1984-01-01

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

  14. Multiple Ewing Sarcoma/Primitive Neuroectodermal Tumors in the Mediastinum: A Case Report and Literature Review.

    Science.gov (United States)

    Bae, Sung Hwan; Hwang, Jung Hwa; Da Nam, Bo; Kim, Hyun Jo; Kim, Ki-Up; Kim, Dong Won; Choi, In Ho

    2016-02-01

    Ewing sarcoma/primitive neuroectodermal tumors (ES/PNET) are high-grade malignant neoplasms. These malignancies present very rare tumors of thoracopulmonary area and even rarer in the mediastinum. In our knowledge, ES/PNET presented with multiple mediastinal masses has not been reported previously. We experienced a case of a 42-year-old man presented with gradual onset of left-side pleuritic chest pain. A contrast-enhanced chest computed tomography (CT) scan showed separate 2 large heterogeneously enhancing masses in each anterior and middle mediastinum of the left hemithorax. Positron emission tomography-computed tomography (PET-CT) scan revealed high fluorodeoxyglucose (FDG) uptake in the mediastinal masses. After surgical excision for the mediastinal masses, both of the masses were diagnosed as the ES/PNET group of tumors on the histopathologic examination. The patient refused postoperative adjuvant chemotherapy and came back with local tumor recurrence and distant metastasis on 4-month follow-up after surgical resection. We report this uncommon form of ES/PNET. We are to raise awareness that this rare malignancy should be considered as a differential diagnosis of the malignant mediastinal tumors and which can be manifested as multiple masses in a patient. Understanding this rare entity of extra-skeletal ES/PNET and characteristic imaging findings can help radiologists and clinicians to approach proper diagnosis and better management for this highly malignant tumor.

  15. Entity Framework 41 Expert's Cookbook

    CERN Document Server

    Liles, Devlin

    2012-01-01

    This book takes a step-by-step problem solving approach that leads you through a problem and explains each step in detail to accomplish the goal. This books gives you concrete code examples as well as detailed information on the decisions involved. This book is for intermediate to advanced .NET developers who have used LINQ to SQL or Entity Framework in some form but want to have more control over the implementation.

  16. Rare particles

    International Nuclear Information System (INIS)

    Kutschera, W.

    1984-01-01

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

  17. The role of imaging for the surgeon in primary malignant bone tumors of the chest wall

    Energy Technology Data Exchange (ETDEWEB)

    Rocca, M., E-mail: michele.rocca@ior.it [General and Thoracic Surgery, The Rizzoli Orthopaedic Institute, Via Pupilli 1, 40136 Bologna (Italy); Salone, M. [General and Thoracic Surgery, The Rizzoli Orthopaedic Institute, Via Pupilli 1, 40136 Bologna (Italy); Galletti, S. [Ultrasound Unit, The Rizzoli Orthopaedic Institute, Bologna (Italy); Balladelli, A. [Laboratory of Experimental Oncology, The Rizzoli Orthopaedic Institute, Bologna (Italy); Vanel, D. [Research in Imaging Musculo Skeletal Tumors, The Rizzoli Orthopaedic Institute, Bologna (Italy); Briccoli, A. [General and Thoracic Surgery, The Rizzoli Orthopaedic Institute, Via Pupilli 1, 40136 Bologna (Italy)

    2013-12-01

    Primary malignant chest wall tumors are rare. The most frequent primary malignant tumor of the chest wall is chondrosarcoma, less common are primary bone tumors belonging to the Ewing Family Bone Tumors (EFBT), or even rarer are osteosarcomas. They represent a challenging clinical entities for surgeons as the treatment of choice for these neoplasms is surgical resection, excluding EFBT which are normally treated by a multidisciplinary approach. Positive margins after surgical procedure are the principal risk factor of local recurrence, therefore to perform adequate surgery a correct preoperative staging is mandatory. Imaging techniques are used for diagnosis, to determine anatomic site and extension, to perform a guided biopsy, for local and general staging, to evaluate chemotherapy response, to detect the presence of a recurrence. This article will focus on the role of imaging in guiding this often difficult surgery and the different technical possibilities adopted in our department to restore the mechanics of the thoracic cage after wide resections.

  18. Neuroendocrine tumors of the pancreas; Multimodale Bildgebung bei neuroendokrinen Tumoren des Pankreas

    Energy Technology Data Exchange (ETDEWEB)

    Holzapfel, Konstantin; Rummeny, Ernst J. [Technische Univ. Muenchen (Germany). Inst. fuer Radiologie; Gaertner, Florian C. [Technische Univ. Muenchen (Germany). Nuklearmedizinische Klinik

    2011-12-15

    Neuroendocrine tumors (NET) of the pancreas are rare entities. Functioning tumors tend to present early with specific symptoms and typical abnormalities in laboratory values. In contrast, non-functioning NET are often diagnosed with delay and become evident by tumor-related symptoms like pain, weight-loss or jaundice. The role of imaging is to localize and delineate the primary tumor and to detect metastases. In the diagnosis of NET radiologic techniques like computed tomography (CT) and magnetic resonance imaging (MRI) are applied. In certain cases nuclear medicine techniques like somatostatin receptor scintigraphy (SRS) and positron emission tomography (PET) using radioactively labelled somatostatin analogues are used. The present article reviews characteristic imaging findings of both functioning and non-functioning NET of the pancreas. (orig.)

  19. 31 CFR 596.308 - Person; entity.

    Science.gov (United States)

    2010-07-01

    ... FOREIGN ASSETS CONTROL, DEPARTMENT OF THE TREASURY TERRORISM LIST GOVERNMENTS SANCTIONS REGULATIONS General Definitions § 596.308 Person; entity. (a) The term person means an individual or entity. (b) The...

  20. Ranking related entities: components and analyses

    NARCIS (Netherlands)

    Bron, M.; Balog, K.; de Rijke, M.

    2010-01-01

    Related entity finding is the task of returning a ranked list of homepages of relevant entities of a specified type that need to engage in a given relationship with a given source entity. We propose a framework for addressing this task and perform a detailed analysis of four core components;

  1. Entity Ranking using Wikipedia as a Pivot

    NARCIS (Netherlands)

    R. Kaptein; P. Serdyukov; A.P. de Vries (Arjen); J. Kamps

    2010-01-01

    htmlabstractIn this paper we investigate the task of Entity Ranking on the Web. Searchers looking for entities are arguably better served by presenting a ranked list of entities directly, rather than a list of web pages with relevant but also potentially redundant information about

  2. Locally aggressive and multifocal phosphaturic mesenchymal tumors: two unusual cases of tumor-induced osteomalacia.

    Science.gov (United States)

    Higley, Meghan; Beckett, Brooke; Schmahmann, Sandra; Dacey, Elizabeth; Foss, Erik

    2015-12-01

    Tumor-induced osteomalacia (TIO) has long been recognized as a clinical paraneoplastic syndrome. The identification of a unique histopathologic entity, the phosphaturic mesenchymal tumor (PMT), as a distinct etiology for TIO has been a more recent discovery. The majority of published cases describe a solitary, non-aggressive appearing soft tissue or osseous lesions in patients with osteomalacia; aggressive appearing or multifocal lesions appear to be exceedingly rare. These tumors characteristically secrete fibroblast growth factor 23 (FGF23). Elevated serum levels of FGF23 result in phosphate wasting and osteomalacia. In the majority of cases, laboratory abnormalities and clinical signs and symptoms of osteomalacia precede identification of the causative lesion by years. Following diagnosis, complete resection with wide margins to prevent local recurrence is most often curative. Imaging characteristics of PMT are diverse and remain incompletely defined, as the majority of previous publications are outside of the radiologic literature. We present multiple imaging modalities in two cases of patients with debilitating osteomalacia and unusual appearing PMTs: one with a locally aggressive lesion leading to pathologic fracture, the second presenting with exceedingly rare multifocal PMT.

  3. Primary Ewing's Sarcoma of the Temporal Bone: A Rare Case Report and Literature Review.

    Science.gov (United States)

    Gupta, Divya; Gulati, Achal; Purnima

    2017-09-01

    Ewing's sarcoma is a malignant, round cell tumor arising from the bones and primarily affecting children and adolescent, accounting for 3 % of all childhood malignancies. Although the long bones and the trunk are typically affected, rare cases of it involving isolated bones throughout the body have been reported. Involvement of the skull bones is rare, constituting 1-6 % of the total Ewing's sarcoma cases but those affecting the cranial bones are rarer still, constituting only 1 %. We describe an 8 months old infant having Ewing sarcoma, of the petrous and mastoid parts of temporal bone along with the occipital bone, whose clinical presentation mimicked mastoiditis with facial nerve palsy. We discuss the clinical and therapeutic course of an extensive primary Ewing sarcoma of the temporal bone, which was treated without performing surgery and review this entity's literature in detail.

  4. Phosphaturic mesenchymal tumor, mixed connective tissue variant, of the mandible: report of a case and review of the literature.

    Science.gov (United States)

    Woo, Victoria L; Landesberg, Regina; Imel, Erik A; Singer, Steven R; Folpe, Andrew L; Econs, Michael J; Kim, Taeyun; Harik, Lara R; Jacobs, Thomas P

    2009-12-01

    Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome that results in renal phosphate wasting with hypophosphatemia. In most cases, the underlying cause of TIO is a small mesenchymal neoplasm that is often difficult to detect, resulting in delayed diagnosis. One such neoplasm is the phosphaturic mesenchymal tumor, mixed connective tissue variant (PMTMCT), an unusual entity with unique morphologic and biochemical features. Most of these tumors are found at appendicular sites with only rare cases reported in the jaws. We describe a PMTMCT involving the mandible in a patient with a protracted history of osteomalacia. A review of the current literature is provided with emphasis on the clinical and histologic features, etiopathogenesis, and management of PMTMCT in the setting of TIO.

  5. A rare adrenal incidentaloma: adrenal schwannoma.

    Science.gov (United States)

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

    2013-01-01

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

  6. Painful constipation: a neglected entity?

    Directory of Open Access Journals (Sweden)

    G. Bassotti

    2011-01-01

    Full Text Available Functional chronic constipation is a common symptom in daily clinical practice. Although the definition of constipation may be variable, there is usually agreement that (at least for research purposes the definition given by the Rome Committee are useful. However, some blind spots or hidden angles remain, even in the more thorough classifications; among these, there is painful constipation, a poorly defined yet clinically encountered entity. The present article reviews the current knowledge about painful constipation, trying to put together the scarce data available, and to frame it in the more general context of chronic constipation.

  7. Painful constipation: a neglected entity?

    Directory of Open Access Journals (Sweden)

    G. Bassotti

    Full Text Available Functional chronic constipation is a common symptom in daily clinical practice. Although the definition of constipation may be variable, there is usually agreement that (at least for research purposes the definition given by the Rome Committee are useful. However, some blind spots or hidden angles remain, even in the more thorough classifications; among these, there is painful constipation, a poorly defined yet clinically encountered entity. The present article reviews the current knowledge about painful constipation, trying to put together the scarce data available, and to frame it in the more general context of chronic constipation.

  8. TAMOXIFEN RETINOPATHY DURING TREATMENT OF AN INOPERABLE DESMOID TUMOR.

    Science.gov (United States)

    Furst, Meredith; Somogyi, Marie B; Wong, Robert W; Araujo, Dejka; Harper, Clio A

    2017-12-08

    To evaluate the clinical significance and rarity of tamoxifen retinopathy after a long-term tamoxifen treatment for an inoperable desmoid tumor. Case report. Tamoxifen retinopathy is a condition rarely observed in clinical practice. Although tamoxifen is typically a treatment for breast cancer patients, we present a 68-year-old woman taking tamoxifen for an inoperable desmoid tumor, an equally rare condition. She presented with bilaterally deteriorating vision over the course of a year. Fundoscopic examination revealed parafoveal deposits bilaterally. Spectral domain optical coherence tomography exhibited hyperreflective deposits in all layers of the retina. She had a cumulative treatment dose of 292 g of tamoxifen, and the medication was subsequently stopped. Her vision remained stable 3 months after the cessation of tamoxifen. The development of tamoxifen retinopathy in the treatment of a desmoid tumor makes this case a rare entity, and this is the first reported case of these two concomitant conditions to our knowledge. With the use of long-term tamoxifen as a primary treatment, we recommend screening at regular intervals by an ophthalmologist as an integral part of treatment.

  9. Large keratocystic odontogenic tumor of the mandible

    Directory of Open Access Journals (Sweden)

    Sri Krishna Koppula

    2015-01-01

    Full Text Available The odontogenic keratocyst (OKC is a rare odontogenic cyst of epithelial origin. There are many types of cysts of the jaws, but what make the odontogenic keratocyst unusual are its characteristic features including its potentially aggressive behavior, high recurrence rate, and an association with the nevoid basal cell carcinoma syndrome. Odontogenic keratocysts, now reclassified as keratocystic odontogenic tumors (KCOTs by the World Health Organization, are a clinical entity with a characteristic microscopic picture, kinetic growth, and biological behavior. The characteristic features are unique among all the different inflammatory and developmental cysts that occur in the jaws. In this report, we present a 35-year-old female patient with a massive KCOT with buccal cortical plate expansion and unusual anteroposterior extension extending from the premolar area up to the condyle.

  10. Tremor entities and their classification: an update.

    Science.gov (United States)

    Gövert, Felix; Deuschl, Günther

    2015-08-01

    This review focuses on important new findings in the field of tremor and illustrates the consequences for the current definition and classification of tremor. Since 1998 when the consensus criteria for tremor were proposed, new variants of tremors and new diagnostic methods were discovered that have changed particularly the concepts of essential tremor and dystonic tremor. Accumulating evidence exists that essential tremor is not a single entity rather different conditions that share the common symptom action tremor without other major abnormalities. Tremor is a common feature in patients with adult-onset focal dystonia and may involve several different body parts and forms of tremor. Recent advances, in particular, in the field of genetics, suggest that dystonic tremor may even be present without overt dystonia. Monosymptomatic asymmetric rest and postural tremor has been further delineated, and apart from tremor-dominant Parkinson's disease, there are several rare conditions including rest and action tremor with normal dopamine transporter imaging (scans without evidence of dopaminergic deficit) and essential tremor with tremor at rest. Increasing knowledge in the last decades changed the view on tremors and highlights several caveats in the current tremor classification. Given the ambiguous assignment between tremor phenomenology and tremor etiology, a more cautious definition of tremors on the basis of clinical assessment data is needed.

  11. Gastric Collision Tumors: An Insight into Their Origin and Clinical Significance

    Directory of Open Access Journals (Sweden)

    Adamantios Michalinos

    2015-01-01

    Full Text Available Collision tumors are rare neoplasms displaying two distinct cell populations developing in juxtaposition to one another without areas of intermingling. They are rare entities with only 63 cases described in English literature. Tumors encountered are gastric adenocarcinomas colliding with lymphomas, gastrointestinal stromal tumors, squamous cell carcinomas, and neuroendocrine tumors. Their cell origin is obsolete by the time of diagnosis. Different tumorigenesis theories have been suggested to explain their behavior, yet none has managed to provide satisfactory explanation for all cases. Clinically they are indistinguishable from the dominant tumor. Lack of data does not allow detailed assessment of their behavior yet they seem aggressive neoplasms with dismal prognosis. The majority of cases have been diagnosed postoperatively during histologic examination of specimens. There are no guidelines or concrete evidence to support best way of adjuvant or other types of treatment. However, these rare neoplasms might help in unlocking secrets of cancer behavior including tumorigenesis, differentiation, and adhesion and thus clinicians should be aware of their existence.

  12. Acute lymphoblastic leukemia mimicking Wilms tumor at presentation.

    Science.gov (United States)

    Singh, Amitabh; Mandal, Anirban; Guru, Vijay; Seth, Rachna

    2016-09-01

    Acute lymphoblastic leukemia (ALL), the commonest malignancy of childhood, is known to manifest with a myriad of atypical presentations. Nephromegaly is a rare presentation of childhood ALL with hepatic mass being even rarer. We present a 3 year-old child with unilateral renal mass and hepatic mass lesion with normal blood counts, initially suspected to have metastatic Wilms tumor based on clinical, radiological and WT1 positivity on immunocytochemistry of renal mass. He was later diagnosed as ALL with peripheral blood flowcytometry and bone marrow examination. Renomegaly at presentation of acute leukemia is not necessarily due to leukemic infiltration and rarely leads to renal impairment. The radiological differential of such a renal mass includes both benign and malignant entities including metastasis. Over-expression of WT1 mRNA has been found in a number of solid tumors and hematological malignancies and is far from being diagnostic of Wilms tumor. Again, a small number of children with acute leukemia may have a deceptively normal complete blood count at presentation. Though, initial all (clinical, radiological, hematological, and immunocytological) parameters pointed towards a diagnosis of Wilms tumor in our case, the subsequent development of thrombocytopenia and lymphocytic leukocytosis prompted further investigation and final diagnosis of ALL. WT1 positivity is a known phenomenon in childhood ALL and undifferentiated lymphoblasts may be positive for WT1 and negative for Leucocyte common antigen. Acute leukemia with renal and hepatic mass with normal blood counts at presentation is a diagnostic challenge.

  13. Two patients with rare mixed adenoneuroendocrine carcinomas of the rectum.

    Science.gov (United States)

    Gül-Klein, Safak; Sinn, Marianne; Jurmeister, Philipp Sebastian; Biebl, Matthias; Weiß, Sascha; Rau, Beate; Bläker, Hendrik; Pratschke, Johann; Aigner, Felix

    2018-01-01

    Mixed adenoneuroendocrine carcinomas of the gastrointestinal tract are until today poorly understood and thus very challenging for interdisciplinary therapy. We herewith report the first case series of patients with a primary mixed adenoneuroendocrine carcinoma of the rectum. Both cases were initially diagnosed as adenocarcinoma and only secondarily with mixed adenoneuroendocrine carcinoma and had a poor outcome due to a rapid tumor progression and resistance to chemotherapy. A 65-year-old female presented with local tumor recurrence and hepatopulmonary metastasis 1 year after primary surgery for adenocarcinoma of the rectum and consecutive radiochemotherapy regimen. Fluorouracil (5-FU) was followed by bevacizumab- and capecitabine-based chemotherapy but had to be discontinued due to side effects and progressive disease. Progressive local pain syndrome accompanied by recurrent bleeding episodes led to a local tumor-debulking operation. Afterward, mixed adenoneuroendocrine carcinoma as the underlying diagnosis in the final histopathological examination was detected. The patient died 3 months after the operation in the context of a fulminant tumor progress. A 63-year-old male patient underwent neoadjuvant radiochemotherapy and laparoscopic rectum resection. After 5 months, postoperative oxaliplatin/capecitabine-based adjuvant chemotherapy was switched to carboplatin/etopsid due to a progressive polyneuropathy and biopsy-proven pulmonary metastasis. The patient then had to be switched to local radiation of cerebral metastases and Topotecan due to cerebral bleeding episodes but died 18 months after the initial diagnosis. In conclusion of our case series, mixed adenoneuroendocrine carcinomas of the rectum should be considered as a rare but aggressive tumor entity. An early and detailed histopathological diagnosis is required in order to establish an individual interdisciplinary treatment concept.

  14. Inflammatory myofibroblastic tumor appendix with concomitant mucosal dysplasia, simulating pseudomyxoma on preoperative aspiration cytology

    Directory of Open Access Journals (Sweden)

    Kaushik Majumdar

    2012-01-01

    Full Text Available Inflammatory myofibroblastic tumor (IMT has been described as a pseudosarcomatous proliferation of spindled myofibroblasts admixed with lymphoplasmacytic cells. The various terminologies like inflammatory pseudotumor, plasma cell granuloma, and inflammatory myofibrohistiocytic proliferation, used to describe this entity, highlight the controversial etiopathogenesis of this relatively indolent neoplasm. IMT has now been described in different anatomic locations. However, cases occurring in the gastrointestinal tract are rare with very few cases described in the appendix. We present a case of inflammatory myofibroblastic tumor appendix with mucosal dysplasia in a 41-year-old male, presenting with abdominal pain and lump in the right iliac fossa. Aspiration cytology yielded few atypical epithelial cells and spindle cells in a mucinous background, suggesting the possibility of pseudomyxoma peritonei. Awareness of IMT appendix with rare presence of mucosal dysplasia may help in preventing overzealous resection, especially in situations that on preoperative evaluation may suggest malignancy.

  15. Neuroendocrine tumors of the gastrointestinal tract; Multimodale Bildgebung neuroendokriner Tumoren des Gastrointestinaltrakts

    Energy Technology Data Exchange (ETDEWEB)

    Holzapfel, Konstantin; Eiber, Matthias; Rummeny, Ernst J. [Klinikum rechts der Isar der Technischen Univ. Muenchen (Germany). Inst. fuer Radiologie; Gaertner, Florian C. [Bonn Univ. (Germany). Klinik und Poliklinik fuer Nuklearmedizin

    2014-03-15

    Neuroendocrine tumors (neuroendokrine Tumoren) are rare entities. They can be found in all organs and show substantial biologic heterogeneity depending on involved organ, clinical symptoms and histopathologic morphology. Involvement of organs like larynx, cervix uteri, ovary, gallbladder, liver or kidney is extensively rare. The majority of neuroendokrine Tumoren are found in gastrointestinal tract and lung and are classified as neuroendokrine Tumoren of foregut (stomach, duodenum, pancreas, lung), midgut (jejunum, ileum, appendix, right side of the colon) and hindgut (left side of the colon, rectum). The role of imaging is to localize and delineate the primary tumor and to detect metastases. In the diagnosis of neuroendokrine Tumoren radiologic techniques like computed tomography (CT) and magnetic resonance imaging (MRI) are applied. In certain cases nuclear medicine techniques like somatostatin receptor scintigraphy (SRS) and positron emission tomography (PET) using radioactively labelled somatostatin analogues are used. The present article reviews characteristic imaging findings of neuroendokrine Tumoren of the gastrointestinal tract. (orig.)

  16. Cross sectional imaging of cardiac tumors

    International Nuclear Information System (INIS)

    Maksimovic, R.

    2012-01-01

    Full text: Primary cardiac tumors are a rare entity whose incidence, according to surgery and autopsy reports, is 0.3% to 0.7% of all cardiac tumors. Metastasis to the heart from other primary cancers is 30 times more common. Only 25% of primary cardiac tumors are malignant, and, of these, 75% are sarcomas. Malignant primary cardiac sarcomas are usually located in the right atrium and are most commonly angiosarcoma. In the left atrium, the most common malignant tumors are pleomorphic sarcoma and leiomyosarcoma. Symptom presentation for cardiac tumors is quite varied, but it is dependent upon tumor location and size, rather than upon histologic characteristics. Presentation includes congestive heart failure from intracardiac obstruction, systemic embolization, constitutional symptoms, and arrhythmias. Left atrial sarcomas tend to be more solid and less infiltrative than right-sided sarcomas; consequently, they tend to metastasize later. They usually present with symptoms of blood-flow obstruction and substantial, life-threatening congestive heart failure. Right-sided cardiac tumors are usually malignant and appear as bulky, infiltrative masses that grow in an outward pattern. These are usually fast-growing tumors that metastasize early and do not present with congestive heart failure until late in the disease. The diagnosis of cardiac tumors relies heavily on the use of multiple imaging techniques, including cardiac computed tomography (CT), cardiovascular magnetic resonance (CMR), and echocardiography. Important imaging data to collect include information on the size of the intracardiac mass, the mobility of the mass (an important predictor of prognosis and embolic potential), myocardial invasion, and cardiac chamber location. These factors will provide the means to diagnosis and prognosis. Other important data to collect include the mechanism of tumor implantation, the relationship of the tumor with adjacent structures, the surgeon route of access to the heart

  17. Collision tumor of the thyroid: follicular variant of papillary carcinoma and squamous carcinoma

    Directory of Open Access Journals (Sweden)

    Kane Subhadra V

    2006-09-01

    Full Text Available Abstract Background Collision tumors of the thyroid gland are a rare entity. We present a case of a follicular variant of papillary carcinoma and squamous carcinoma in the thyroid. To the best of our knowledge, this is the first documentation of a collision tumor with a papillary carcinoma and a squamous carcinoma within the thyroid gland. The clinicopathological features and immunohistochemical profile are reported. The theories of origin, epidemiology and management are discussed with a literature review. Case presentation A 65 year old woman presented with a large thyroid swelling of 10 years duration and with swellings on the back and scalp which were diagnosed to be a follicular variant of papillary thyroid carcinoma with metastasis. Clinical examination, radiology and endoscopy ruled out any other abnormality of the upper aerodigestive tract. The patient was treated surgically with a total thyroidectomy with central compartment clearance and bilateral selective neck dissections. The histopathology revealed a collision tumor with components of both a follicular variant of papillary carcinoma and a squamous carcinoma. Immunohistochemical analysis confirmed the independent origin of these two primary tumors. Adjuvant radio iodine therapy directed toward the follicular derived component of the thyroid tumor and external beam radiotherapy for the squamous component was planned. Conclusion Collision tumors of the thyroid gland pose a diagnostic as well as therapeutic challenge. Metastasis from distant organs and contiguous primary tumors should be excluded. The origins of squamous cancer in the thyroid gland must be established to support the true evolution of a collision tumor and to plan treatment. Treatment for collision tumors depends upon the combination of primary tumors involved and each component of the combination should be treated like an independent primary. The reporting of similar cases with longer follow-up will help define the

  18. Rare Events

    Science.gov (United States)

    2009-10-01

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

  19. Ewing tumors in infants

    NARCIS (Netherlands)

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

    2008-01-01

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

  20. 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...

  1. Entity resolution in the web of data

    CERN Document Server

    Christophides, Vassilis; Stefanidis, Kostas

    2015-01-01

    In recent years, several knowledge bases have been built to enable large-scale knowledge sharing, but also an entity-centric Web search, mixing both structured data and text querying. These knowledge bases offer machine-readable descriptions of real-world entities, e.g., persons, places, published on the Web as Linked Data. However, due to the different information extraction tools and curation policies employed by knowledge bases, multiple, complementary and sometimes conflicting descriptions of the same real-world entities may be provided. Entity resolution aims to identify different descrip

  2. 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.

  3. A Rare Presentation of Odontogenic Keratocyst Mimicking an Antral Polyp

    OpenAIRE

    Sudhakar, S; Geethika, V; Ramaswamy, P; Kumar, PK; Sreenivasulu, P

    2014-01-01

    Maxillary sinus harbours many pathological lesions. Many of those presents as a sinonasal mass and are rarely symptomatic. These masses are usually an antral polyp, mucoceles or mucous retention cysts. Odontogenic keratocyst, a benign odontogenic lesion presenting within the maxillary sinus is a rare entity. We present a case of odontogenic keratocyst of the maxillary sinus in a 35 years old female.

  4. Hepatogastric fistula: A rare complication of liver abscess

    Directory of Open Access Journals (Sweden)

    Shrihari Anikhindi

    2015-01-01

    Full Text Available Rupture of amebic liver abscess into stomach is a rare complication. We report a case of a young male presenting with haematemesis due to a rupture of left lobe amebic liver abscess into stomach. We discuss the diagnosis and management of this rare clinical entity.

  5. [The nosological entity bulimia nerviosa].

    Science.gov (United States)

    López-Ibor Aliño, J J; Cervera Enguix, S

    1991-01-01

    Anorexia nervosa and bulimia nervosa are at the present moment, two well defined clinical entities among the group of the eating disorders. The psychopathological differentiation of both syndromes has a great importance for diagnosis and therapy. The authors make a phenomenological description, based on case histories of patients with diagnostics of anorexia and bulimia nervosa, and try to establish an approach to the essential symptomatology of those disorders. The presence of affective symptomatology--depressive, but not exclusively--in the eating behaviour disorders in general and particularly in bulimia nervosa, is nowadays interpreted as an unspecific emotional lability as a response to stressing situations. That is to say, it is a secondary depressive symptomatology, more than a primary mood disorder preceding or underlying bulimia. There is strong evidence in favour of a dysregulation of serotonin metabolism in patients with bulimia nervosa, in the sense of a reduced activity, which manifest itself clinically by binges with food with a high content in carbohydrates. High levels of 5-HT seem to induce increasing feelings of safety, fullness and lead to an interruption of eating. Fluoxetine and this active metabolite are selective inhibitors of the reuptake of 5-HT and their antibulimic effect could be mediated by this mechanism.

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

    Directory of Open Access Journals (Sweden)

    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.

  7. LEFT RENAL TUMOR: A RARE CADAVERIC FINDINGS

    Directory of Open Access Journals (Sweden)

    Siva Prasad

    2015-08-01

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

  8. Cytomorphology of unusual infectious entities in the Pap test

    Directory of Open Access Journals (Sweden)

    Walid E Khalbuss

    2012-01-01

    Full Text Available Rare entities in the Pap test, including neoplastic and non-neoplastic conditions, pose challenges due to their infrequent occurrence in the daily practice of cytology. Furthermore, these conditions give rise to important diagnostic pitfalls. Infections such as tuberculosis cervicitis may be erroneously diagnosed as carcinoma, whereas others, such as schistosomiasis, are associated with squamous cell carcinoma. These cases include granuloma inguinale (donovanosis, tuberculosis, coccidioidomycosis, schistosomiasis, taeniasis, and molluscum contagiosum diagnosed in Pap tests. Granuloma inguinale shows histiocytes that contain intracytoplasmic bacteria (Donovan bodies. Tuberculosis is characterized by necrotizing granulomatous inflammation with Langhans-multinucleated giant cells. Coccidioidomycosis may show large intact or ruptured fungal spherules associated with endospores. Schistosoma haematobium is diagnosed by finding characteristic ova with a terminal spine. Molluscum contagiosum is characterized by the appearance of squamous cells with molluscum bodies. This article reviews the cytomorphology of selected rare infections and focuses on their cytomorphology, differential diagnosis, and role of ancillary diagnostic studies.

  9. Papillary tumor of the pineal region: two case studies and a review of the literature.

    Science.gov (United States)

    Rickard, Kyle A; Parker, John R; Vitaz, Todd W; Plaga, Alexis R; Wagner, Stephanie; Parker, Joseph C

    2011-01-01

    Papillary tumor of the pineal region (PTPR) is a newly recognized distinct entity in the 2007 World Health Organization nomenclature. This tumor is characterized by epithelial-appearing areas with papillary features and more densely cellular areas that often display ependymal-like differentiation. Ultrastructurally, this rare neuroepithelial tumor possesses neuroendocrine, secretory, and ependymal organelles that likely originate from the subcommissural organ (SCO) near the aqueduct of Sylvius. To date, approximately fifty-seven described cases worldwide have been recognized, with ages ranging from 5 years to 66 years (mean age=32 years). Clinical presentation most often includes headache and obstructive hydrocephalus. The tumor, which is well circumscribed, may be cystic and radiographically is often considered to be consistent with the findings of a pineocytoma. Microscopic evaluation often demonstrates a lesion with papillary areas lined by epithelioid tumor cells with eosinophilic cytoplasm and more cellular areas with cells exhibiting clear or vacuolated cytoplasm. Perivascular and true rosettes may be identified. Distinctive immunohistochemical features including reactivity for keratins (AE1/AE3, CAM 5.2, CK18) and only focal GFAP staining help distinguish this neoplasm from an ependymoma. The relative paucity of data compiled for this tumor makes giving an accurate diagnosis and prognosis a daunting task. We discuss two additional cases of PTPR that presented to us within a three-month span in order to more fully elucidate the possible presentations of this rare entity. Furthermore, we examine now 59 reported cases of PTPR in order to review the current diagnostic and treatment modalities in addition to exploring emerging research encompassing this unusual neoplasm.

  10. 46 CFR 403.110 - Accounting entities.

    Science.gov (United States)

    2010-10-01

    ... 46 Shipping 8 2010-10-01 2010-10-01 false Accounting entities. 403.110 Section 403.110 Shipping COAST GUARD (GREAT LAKES PILOTAGE), DEPARTMENT OF HOMELAND SECURITY GREAT LAKES PILOTAGE UNIFORM ACCOUNTING SYSTEM General § 403.110 Accounting entities. Each Association shall be a separate accounting...

  11. 78 FR 31822 - Unincorporated Business Entities

    Science.gov (United States)

    2013-05-28

    ... framework for Farm Credit System (System) institutions' use of unincorporated business entities (UBEs) organized under State law for certain business activities. A UBE includes limited partnerships (LPs...-AC65 Unincorporated Business Entities AGENCY: Farm Credit Administration. ACTION: Final rule. SUMMARY...

  12. Entity models for trigger-reaction documents

    NARCIS (Netherlands)

    Khalid, M.A.; Marx, M.; Makkes, M.X.

    2008-01-01

    We define the notion of an entity model for a special kind of document popular on the web: an article followed by a list of reactions on that article, usually by many authors, usually inverse chronologically ordered. We call these documents trigger-reactions pairs. The entity model describes which

  13. Ranking Very Many Typed Entities on Wikipedia

    NARCIS (Netherlands)

    Zaragoza, Hugo; Rode, H.; Mika, Peter; Atserias, Jordi; Ciaramita, Massimiliano; Attardi, Guiseppe

    2007-01-01

    We discuss the problem of ranking very many entities of different types. In particular we deal with a heterogeneous set of types, some being very generic and some very specific. We discuss two approaches for this problem: i) exploiting the entity containment graph and ii) using a Web search engine

  14. 31 CFR 595.303 - Entity.

    Science.gov (United States)

    2010-07-01

    ... 31 Money and Finance: Treasury 3 2010-07-01 2010-07-01 false Entity. 595.303 Section 595.303 Money and Finance: Treasury Regulations Relating to Money and Finance (Continued) OFFICE OF FOREIGN ASSETS CONTROL, DEPARTMENT OF THE TREASURY TERRORISM SANCTIONS REGULATIONS General Definitions § 595.303 Entity...

  15. 31 CFR 800.211 - Entity.

    Science.gov (United States)

    2010-07-01

    ... 31 Money and Finance: Treasury 3 2010-07-01 2010-07-01 false Entity. 800.211 Section 800.211 Money and Finance: Treasury Regulations Relating to Money and Finance (Continued) OFFICE OF INVESTMENT... separate legal entity) operated by any one of the foregoing as a business undertaking in a particular...

  16. Code-first development with Entity Framework

    CERN Document Server

    Barskiy, Sergey

    2015-01-01

    This book is intended for software developers with some prior experience with the Microsoft .NET framework who want to learn how to use Entity Framework. This book will get you up and running quickly, providing many examples that illustrate all the key concepts of Entity Framework.

  17. 22 CFR 96.5 - Requirement that accrediting entity be a nonprofit or public entity.

    Science.gov (United States)

    2010-04-01

    ... administering standards for entities providing child welfare services; or (b) A public entity (other than a... political subdivision, agency, or instrumentality thereof, that is responsible for licensing adoption agencies in a State and that has expertise in developing and administering standards for entities providing...

  18. Testing the Application for Analyzing Structured Entities

    Directory of Open Access Journals (Sweden)

    Ion IVAN

    2011-01-01

    Full Text Available The paper presents the testing process of the application for the analysis of structured text entities. The structured entities are presented. Quality characteristics of structured entities are identified and analyzed. The design and building processes are presented. Rules for building structured entities are described. The steps of building the application for the analysis of structured text entities are presented. The objective of the testing process is defined. Ways of testing the application on components and as a whole are established. A testing strategy for different objectives is proposed. The behavior of users during the testing period is analyzed. Statistical analysis regarding the behavior of users in processes of infinite resources access are realized.

  19. Intraosseous verrucous carcinoma arising from an orthokeratinized odontogenic keratocyst: A report of a rarest entity

    Directory of Open Access Journals (Sweden)

    Nagaraju Kamarthi

    2016-01-01

    Full Text Available Intraosseous verrucous carcinomas (VCs arising from odontogenic cysts are a rare entity. An unusual case of a VC arising from the orthokeratinized odontogenic cyst is described for the first time. The microscopic features of the lesion are presented.

  20. Intimal aortic sarcoma mimicking ruptured thoracoabdominal type IV aneurysm. a rare case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Dedeilias Panagiotis

    2011-12-01

    Full Text Available Abstract Primary intimal aortic sarcoma represents a very rare and highly lethal medical entity. Diagnosis is made either by embolic events caused by the tumor or by surrounding tissue symptoms such as pain. Herein we report an extremely rare case of a 51-year-old man previously operated for ascending aortic aneurysm, who presented with clinical and radiological findings suggestive of a ruptured thoracoabdominal type IV aneurysm. The patient underwent radical resection of the aorta and surrounding tissue with placement of a composite 4-branched graft. The diagnosis was made by frozen section and regular histopathologic examination of the specimen and the patient received adjuvant chemotherapy. Nine months after surgery the patient is still alive and has no signs of recurrence. We review the literature and discuss the option of postoperative chemotherapy.

  1. Awareness of Entities, Activities and Contexts in Ambient Systems

    DEFF Research Database (Denmark)

    Kristensen, Bent Bruun

    2013-01-01

    Ambient systems are modeled by entities, activities and contexts, where entities exist in contexts and engage in activities. A context supports a dynamic collection of entities by services and offers awareness information about the entities. Activities also exist in contexts and model ongoing...... collaborations between entities. Activities and local contexts also obtain awareness information from the context about the dynamic collection of entities. Similarly activities, local contexts and entities are offered awareness information about activities and local contexts....

  2. Radiotherapy for pediatric brain tumors: Standards of care, current clinical trials, and new directions

    International Nuclear Information System (INIS)

    Goldwein, Joel W.

    1995-01-01

    The objectives of the course are to evaluate the role of radiation therapy in the treatment of pediatric brain tumors. Areas where the role is evolving will be identified, and the results of clinical trials which been mounted to clarify radiotherapy's role will be reviewed. Brain tumors are the second most common malignancy of childhood after leukemias and lymphomas. However, they remain the most common group of childhood tumors to require radiation therapy. Therefore, a thorough understanding of these tumors, and the appropriate role of surgery, radiation and chemotherapy is critical. Issues surrounding the management of sequelae are no less important. The role of radiotherapy for the treatment of these tumors is far different from that for adults. These differences relate to the profound potential for sequelae from therapy, the higher overall cure rates, and the utility of multimodality therapies. In addition, the rarity of childhood brain tumors compared with adults' makes them more difficult to study. In this session, the following issues will be reviewed; 1. Incidence of pediatric brain tumors, 2. General issues regarding symptoms, diagnosis, diagnostic tests and evaluation, 3. Importance of a team approach, 4. General issues regarding treatment sequelae, 5. Specific tumor types/entities; a. Cerebellar Astrocytomas b. Benign and malignant Gliomas including brainstem and chiasmatic lesions c. Primitive Neuroectodermal Tumors (PNET) and Medulloblastoma d. Ependymomas e. Craniopharyngiomas f. Germ cell tumors g. Miscellaneous and rare pediatric brain tumors 6. Management of sequelae 7. New and future directions a. Treatment of infants b. The expanding role of chemotherapy c. Advances in radiotherapy. The attendees will complete the course with a better understanding of the role that radiation therapy plays in the treatment of pediatric brain tumors. They will be knowledgeable in the foundation for that role, and the changes which are likely to take place in the

  3. Cellular solitary fibrous tumor (hemangiopericytoma) with anaplasia at cerebellopontine angle--a case report.

    Science.gov (United States)

    Zeng, Jianying; Ogera, Patricia; Benardete, Ethan A; Nicastri, Anthony D; Rao, Chandrakant

    2012-08-15

    Cellular solitary fibrous tumor is currently considered a synonym for hemangiopericytoma, as it became increasingly clear that the morphological and immunohistochemical features that separate these two entities have become tenuous, and evidence for a unifying concept has emerged. Furthermore, as no evidence of pericytic differentiation is given in most cases of hemangiopericytoma, this diagnostic term is waning in popularity. We present here a case of cellular solitary fibrous tumor in a 22-year-old man. Neuroimaging revealed a right cerebellopontine angle tumor. Most of the tumor was cellular although some less cellular areas were seen. Sinusoidally dilated large vessels, including staghorn type, were seen. Nuclear pleomorphism and increased mitotic activity (5 mitosis/10 high power field) were regarded as evidence of anaplasia. Diffuse CD34 immunoreactivity and focal positivity for Factor XIIIa were seen in the tumor, which was negative for EMA and S100. The tumor also displayed rich reticulin network. Solitary fibrous tumor at cerebellopontine angle is rare, and 20 such cases (five reported as hemangiopericytoma) have been reported in the English literature. Copyright © 2012 Elsevier GmbH. All rights reserved.

  4. CT and MR imaging features in phosphaturic mesenchymal tumor-mixed connective tissue: A case report.

    Science.gov (United States)

    Shi, Zhenshan; Deng, Yiqiong; Li, Xiumei; Li, Yueming; Cao, Dairong; Coossa, Vikash Sahadeo

    2018-04-01

    Phosphaturic mesenchymal tumor-mixed connective tissue (PMT-MCT) is rare and usually benign and slow-growing. The majority of these tumors is associated with sporadic tumor-induced osteomalacia (TIO) or rickets, affect middle-aged individuals and are located in the extremities. Previous imaging studies often focused on seeking the causative tumors of TIO, not on the radiological features of these tumors, especially magnetic resonance imaging (MRI) features. PMT-MCT remains a largely misdiagnosed, ignored or unknown entity by most radiologists and clinicians. In the present case report, a review of the known literature of PMT-MCT was conducted and the CT and MRI findings from three patient cases were described for diagnosing the small subcutaneous tumor. Typical MRI appearances of PMT-MCT were isointense relative to the muscles on T1-weighted imaging, and markedly hyperintense on T2-weighted imaging containing variably flow voids, with markedly heterogeneous/homogenous enhancement on post contrast T1-weighted fat-suppression imaging. Short time inversion recovery was demonstrated to be the optimal sequence in localizing the tumor.

  5. On the Evaluation of Entity Profiles

    DEFF Research Database (Denmark)

    de Rijke, Maarten; Balog, Krisztian; Bogers, Toine

    be assessed by means of precision and recall values of the descriptive terms produced. However, recent evidence suggests that more sophisticated metrics are needed that go beyond mere lexical matching of system-produced descriptors against a ground truth, allowing for graded relevance and rewarding diversity......Entity profiling is the task of identifying and ranking descriptions of a given entity. The task may be viewed as one where the descriptions being sought are terms that need to be selected from a knowledge source (such as an ontology or thesaurus). In this case, entity profiling systems can...... in the list of descriptors returned. In this note, we motivate and propose such a metric....

  6. Ranking Entities in Networks via Lefschetz Duality

    DEFF Research Database (Denmark)

    Aabrandt, Andreas; Hansen, Vagn Lundsgaard; Poulsen, Bjarne

    2014-01-01

    then be ranked according to how essential their positions are in the network by considering the effect of their respective absences. Defining a ranking of a network which takes the individual position of each entity into account has the purpose of assigning different roles to the entities, e.g. agents......, in the network. In this paper it is shown that the topology of a given network induces a ranking of the entities in the network. Further, it is demonstrated how to calculate this ranking and thus how to identify weak sub-networks in any given network....

  7. Central nervous system tumors

    International Nuclear Information System (INIS)

    Curran, W.J. Jr.

    1991-01-01

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

  8. GASTROINTESTINAL STROMAL TUMOR (GIST

    Directory of Open Access Journals (Sweden)

    Luigi eTornillo

    2014-11-01

    Full Text Available Gastrointestinal stromal tumors are the most frequent mesenchymal tumors of the gastrointestinal tract. The discovery that these tumors, formerly thought of smooth muscle origin, are indeed better characterized by specific activating mutation in genes coding for the receptor tyrosine kinases CKIT and PDGFRA and that these mutations are strongly predictive for the response to targeted therapy with receptor tyrosine kinase inhibitors has made GISTs the typical example of the integration of basic molecular knowledge in the daily clinical activity. The information on the mutational status of these tumors is essential to predict (and subsequently to plan the therapy. As resistant cases are frequently wild-type, other possible oncogenic events, defining other entities, have been discovered (e.g. succinil dehydrogenase mutation/dysregulation, insuline growth factor expression, mutations in the RAS-RAF-MAPK pathway. The classification of disease must nowadays rely on the integration of the clinico-morphological characteristics with the molecular data.

  9. Primitive myxoid mesenchymal tumor of infancy in a preterm infant.

    Science.gov (United States)

    Lam, Joseph; Lara-Corrales, Irene; Cammisuli, Salvatore; Somers, Gino R; Pope, Elena

    2010-01-01

    Primitive myxoid mesenchymal tumor of infancy is a recently recognized entity that has been added to the differential diagnosis of myxoid tumors of the soft tissue. Few cases have been reported of this entity in the literature, but none presenting in a preterm infant. We present the case and clinical course of a preterm boy with a primitive myxoid mesenchymal tumor of infancy that occurred following excision of a congenital juvenile xanthogranuloma. © 2010 Wiley Periodicals, Inc.

  10. Bilateral ossification of the auricles: an unusual entity and review of the literature

    Directory of Open Access Journals (Sweden)

    Siabi Vassiliki

    2009-10-01

    Full Text Available Abstract Background True ossification of the auricle with cartilage replacement by bone, is a very rare clinical entity and can result in an entirely rigid auricle. Case presentation We present a rare case of bilateral ossification of the auricles in a 75-years old man with profound progressive rigidity of both auricles. His main complaint was a mild discomfort during resting making sleeping unpleasant without any other serious symptoms. His medical history was significant for predisposing factors for this condition such as, Addison's disease and diabetes mellitus. Excisional biopsy was performed confirming the ossified nature of the auricles. Further treatment deemed unnecessary in our case due to his mild clinical picture. Conclusion True auricular ossification is a quite rare clinical entity with unclear pathogenesis and one should have in mind that there is always the possibility of a serious co-existed disease like endocrinopathy.

  11. 47 CFR 22.229 - Designated entities.

    Science.gov (United States)

    2010-10-01

    ... entrepreneur is an entity that, together with its controlling interests and affiliates, has average annual... entrepreneur, as defined in this section, or a consortium of entrepreneurs may use the bidding credit specified...

  12. 47 CFR 101.538 - Designated entities.

    Science.gov (United States)

    2010-10-01

    ... entrepreneur is an entity that, together with its controlling interests and affiliates, has average gross... entrepreneur, as defined in this section, or a consortium of entrepreneurs may use the bidding credit specified...

  13. Bone tumor

    Science.gov (United States)

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

  14. Tumor markers in colorectal cancer

    OpenAIRE

    Fernandes, Luís César [UNIFESP; Matos, Delcio [UNIFESP

    2002-01-01

    Colorectal cancer is a clinical entity of a persistent relevance in clinical practice and its early diagnosis is a determinant factor to obtain better therapeutic results. Tumor markers are helpful means for a better approach to individuals with such neoplasm. In the present review, the authors analyze the phases in which surgical-clinical treatment markers must be used: diagnosis, determination of tumor stage, establishment of prognosis and detection of recurrence. Current and future markers...

  15. FIBROMATOSIS (DESMOID TUMOR OF THE BREAST. Fibromatosis (tumor desmoide de mama

    Directory of Open Access Journals (Sweden)

    Zhaneta P Boceska

    2016-03-01

    Full Text Available El tumor desmoide (fibromatosis es una entidad patológica extremadamente rara que se desarrolla de la fascia muscular y la aponeusorsis. Aunque sin potencial metastático, estos tumores son localmente muy agresivos y tienden a infiltrarse en los tejidos circundantes. Nosotros presentamos un caso de tumour desmoide de mama, que tuvo apariencias clínicas sugestivas a carcinoma. La paciente, de 56 años presentó una masa palpable de mama derecho. La citología por aspiracion con aguja fina (AGF no detectó ninguna célula maligna, por lo que se hizo una escisión local conservadora. La paciente no recibió ningun tratamiento postoperatorio adicional, y continúa viva y sana en los siguientes 18 meses. Desmoid tumor (fibromatosis is extremely rare benign pathological entity that develops from muscular fasciae and aponeuroses. Although without metastatic potential, these tumors are locally very aggressive and tend to infiltrate the surrounding tissues. We present a case of a desmoid tumor of the breast that had clinical appearance suggestive of carcinoma. The patient was 56 years old female with a previous history of surgical trauma who presented with a palpable mass in the right breast. A fine needle aspiration (FNA cytology did not reveal any malignant cells, thus conservative local excision was performed. The patient did not receive any additional postoperative treatment and was alive and free of disease after 18 months of follow-up. 

  16. Extracranial metastasizing solitary fibrous tumors (SFT) of meninges: histopathological features of a case with long-term follow-up.

    Science.gov (United States)

    Gessi, Marco; Gielen, Gerrit H; Roeder-Geyer, Eva-Dorette; Sommer, Clemens; Vieth, Michael; Braun, Veit; Kuchelmeister, Klaus; Pietsch, Torsten

    2013-02-01

    Extrapleural solitary fibrous tumors are uncommon mesenchymal neoplasms frequently observed in middle-aged adults and are classified, according to the WHO classification of soft tissue tumors, as part of the hemangiopericytoma tumor group. However, these two entities remain separated in the WHO classification of tumors of the central nervous system. In fact, meningeal solitary fibrous tumors are believed to be benign lesion and only in a minority of cases local relapses have been described, although detailed survival clinical studies on solitary fibrous tumors of meninges are rare. In contrast to hemangiopericytoma, which frequently shows distant extracranial metastases, such an event is exceptional in patients with meningeal solitary fibrous tumors and has been clinically reported in a handful of cases only and their histopathological features have not been investigated in detail. In this report, we describe the detailed clinico-pathological features of a meningeal solitary fibrous tumor presenting during a 17-year follow-up period, multiple intra-, extracranial relapses and lung metastases. © 2012 Japanese Society of Neuropathology.

  17. Integrated tumor and germline whole-exome sequencing identifies mutations in MAPK and PI3K pathway genes in an adolescent with rosette-forming glioneuronal tumor of the fourth ventricle

    Science.gov (United States)

    Lin, Frank Y.; Bergstrom, Katie; Person, Richard; Bavle, Abhishek; Ballester, Leomar Y.; Scollon, Sarah; Raesz-Martinez, Robin; Jea, Andrew; Birchansky, Sherri; Wheeler, David A.; Berg, Stacey L.; Chintagumpala, Murali M.; Adesina, Adekunle M.; Eng, Christine; Roy, Angshumoy; Plon, Sharon E.; Parsons, D. Williams

    2016-01-01

    The integration of genome-scale studies such as whole-exome sequencing (WES) into the clinical care of children with cancer has the potential to provide insight into the genetic basis of an individual's cancer with implications for clinical management. This report describes the results of clinical tumor and germline WES for a patient with a rare tumor diagnosis, rosette-forming glioneuronal tumor of the fourth ventricle (RGNT). Three pathogenic gene alterations with implications for clinical care were identified: somatic activating hotspot mutations in FGFR1 (p.N546K) and PIK3CA (p.H1047R) and a germline pathogenic variant in PTPN11 (p.N308S) diagnostic for Noonan syndrome. The molecular landscape of RGNT is not well-described, but these data are consistent with prior observations regarding the importance of the interconnected MAPK and PI3K/AKT/mTOR signaling pathways in this rare tumor. The co-occurrence of FGFR1, PIK3CA, and PTPN11 alterations provides further evidence for consideration of RGNT as a distinct molecular entity from pediatric low-grade gliomas and suggests potential therapeutic strategies for this patient in the event of tumor recurrence as novel agents targeting these pathways enter pediatric clinical trials. Although RGNT has not been definitively linked with cancer predisposition syndromes, two prior cases have been reported in patients with RASopathies (Noonan syndrome and neurofibromatosis type 1 [NF1]), providing an additional link between these tumors and the mitogen-activated protein kinase (MAPK) signaling pathway. In summary, this case provides an example of the potential for genome-scale sequencing technologies to provide insight into the biology of rare tumors and yield both tumor and germline results of potential relevance to patient care. PMID:27626068

  18. Dissecting disease entities out of the broad spectrum of bipolar-disorders.

    Science.gov (United States)

    Levine, Joseph; Toker, Lilach; Agam, Galila

    2018-01-01

    The etiopathology of bipolar disorders is yet unraveled and new avenues should be pursued. One such avenue may be based on the assumption that the bipolar broad spectrum includes, among others, an array of rare medical disease entities. Towards this aim we propose a dissecting approach based on a search for rare medical diseases with known etiopathology which also exhibit bipolar disorders symptomatology. We further suggest that the etiopathologic mechanisms underlying such rare medical diseases may also underlie a rare variant of bipolar disorder. Such an assumption may be further reinforced if both the rare medical disease and its bipolar clinical phenotype demonstrate a] a similar mode of inheritance (i.e, autosomal dominant); b] brain involvement; and c] data implicating that the etiopathological mechanisms underlying the rare diseases affect biological processes reported to be associated with bipolar disorders and their treatment. We exemplify our suggested approach by a rare case of autosomal dominant leucodystrophy, a disease entity exhibiting nuclear lamin B1 pathology also presenting bipolar symptomatology. Copyright © 2017 Elsevier B.V. All rights reserved.

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

    DEFF Research Database (Denmark)

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

    2011-01-01

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

  20. Rare cancers in The Netherlands: a population-based study.

    Science.gov (United States)

    van der Zwan, Jan M; van Dijk, Boukje A C; Visser, Otto; van Krieken, Han J H J M; Capocaccia, Riccardo; Siesling, Sabine

    2018-07-01

    The conventional definition for rare disease is based on prevalence. Because of differences in prognosis, a definition on the basis of incidence was deemed to be more appropriate for rare cancers. Within the European RARECARE project, a definition was introduced that defines cancers as rare when the crude incidence rate is less than six per 100 000 per year. In this study, we applied the RARECARE definition for rare cancer to the Netherlands; this to identify the usefulness of the definition in a single country and to provide more insight into the burden of rare cancers in the Netherlands. Data for 2004 through 2008 were extracted from the Netherlands Cancer Registry and classified according to the RARECARE entities (tumour groupings). Crude and European standardized incidence rates were calculated. Out of the 260 entities, 223 (86%) were rare according to the definition, accounting for 14 000 cancers (17% of all). Considerable fluctuations in crude rates over years were observed for the major group of cancers. Rare tumours in the Netherlands constituted 17% of all newly diagnosed tumours, but were divided over 223 different entities, indicating the challenge that faces clinicians. To make the definition of rare cancers better applicable, it should be refined by taking into consideration the sex-specific incidence for sex-specific cancer sites. Moreover, a mean incidence over 5 years will provide more solid insight into the burden, eliminating large fluctuations in time of most of the cancers.

  1. A rare cause of hemoptysis

    Directory of Open Access Journals (Sweden)

    Meghan Aversa

    2014-01-01

    Full Text Available Angiosarcomas are rare, malignant, endothelial-cell tumors of vascular origin that can arise at any body site. They frequently metastasize to the lung, heralded by dyspnea, hemoptysis, chest pain, pneumothoraces, and diffuse pulmonary hemorrhage. However, in most cases lung metastases are discovered after the diagnosis of a primary angiosarcoma has already been established. Very rarely will an undiagnosed metastatic angiosarcoma present as diffuse pulmonary hemorrhage. We describe the case of a 59-year-old male who presented to hospital with dyspnea and hemoptysis. CT chest revealed rapidly progressing nodular changes and broncho-alveolar lavage returns were progressively bloody. Open lung wedge biopsy ultimately revealed metastatic angiosarcoma and extensive pulmonary hemorrhage. Our case highlights the key clinical, radiological, and pathological features of this rare malignancy that frequently metastasizes to the lung and reminds clinicians to consider it as a cause of hemoptysis and pulmonary hemorrhage.

  2. Gastric Volvulus and Wandering Spleen: A Rare Surgical Emergency

    Directory of Open Access Journals (Sweden)

    Georgios Lianos

    2013-01-01

    Full Text Available Gastric volvulus is a rare but potentially life-threatening clinical entity due to possible gastric necrosis. A wandering spleen may also be associated with gastric volvulus. Patients presenting with the triad epigastralgia, vomiting followed by retching, and difficulty or inability to pass a nasogastric tube into the stomach are likely to have gastric volvulus. The operating surgeon should include this rare entity in the differential diagnosis when dealing with a patient with such a clinical profile. Herein, we present a case of gastric volvulus associated with a wandering spleen in a 28-year-old Caucasian woman and we provide a brief review of the literature on this issue.

  3. Herpes zoster infection: a rare cause of acute urinary retention.

    Science.gov (United States)

    Chan, Jonathan E; Kapoor, Anil

    2003-06-01

    Herpes zoster (HZ) infection has been reported as a rare cause of acute urinary retention. HZ infection involving sacral, thoracolumbar, and rarely high thoracic dermatomes is believed to occasionally cause motor and sensory neuropathy of the bladder. This is specifically achieved by the interruption of the detrusor reflex causing subsequent bladder atonia. As the course and management of this entity is quite benign, HZ should remain a diagnostic consideration in the management of urinary retention. We report a case of acute urinary retention of approximately 2.5 liters associated with HZ infection and review the proposed pathogenesis and therapeutic considerations in the management of this entity.

  4. Breast abscess as the initial manifestation of primary pure squamous cell carcinoma: a rare presentation and literature review.

    Science.gov (United States)

    Salemis, Nikolaos S

    2011-01-01

    Primary squamous cell carcinoma of the breast is a very rare tumor accounting for less than 0.4% of all breast cancers. Fewer than 100 cases have been reported in the literature so far. The diagnosis requires strict pathologic criteria to be fulfilled. Due to the rarity of this tumor the optimal treatment and prognosis are both unclear. Breast abscess as the initial presentation of a primary squamous cell breast carcinoma is an extremely rare clinical entity. In this study, we describe a case of a 61-year-old postmenopausal woman who presented with typical manifestations of a breast abscess and was diagnosed with a pure primary squamous cell breast carcinoma. Diagnostic evaluation and management of the patient are discussed along with a review of the literature. Despite its rarity, the possibility of a primary pure squamous cell breast carcinoma should always be considered in the differential diagnosis in postmenopausal patients presenting with manifestations of a breast abscess, especially in those who respond poorly to the initial treatment. Physicians should be aware of this rare malignancy in order to avoid delays in diagnosis and treatment.

  5. Cerebellar liponeurocytoma: a newly recognized clinico-pathological entity Liponeurocitoma cerebelar: uma nova entidade clínico-patológica

    Directory of Open Access Journals (Sweden)

    Nádia Montagna

    2002-09-01

    Full Text Available The term "cerebellar liponeurocytoma", recently adopted by the World Health Organization Working Group (WHO, replaced many other different terms used up to now to give name to this rare tumor. To our knowledge, less than 20 cases have been related up to now under different names like as "lipomatous medulloblastoma, lipidized medulloblastoma, neurolipocytoma, medullocytoma and lipomatous glioneurocytoma". The new nomenclature eliminates the word "medulloblastoma", reinforces its benign caracter, and includes it in the category of glioneuronal tumors. We describe an adictional case of this distinct clinico-pathological entity removed from the right cerebellar hemisphere of a 53-year-old woman. With the present case report, we hope to contribute to the knowledge on the diagnostic and prognostic implications derived from the finding of mature adipose-like tissue within a medulloblastomatous tumour.O termo "liponeurocitoma cerebelar" recentemente adotado pela Organização Mundial de Saúde (Classificação de Tumores Cerebrais - versão 2000, surgiu em substituição a vários outros utilizados até então, para denominar esta rara neoplasia. De nosso conhecimento há na literatura menos de 20 casos relatados sob termos diferentes tais como "meduloblastoma lipomatoso, meduloblastoma lipidizado, neurolipocitoma, medulocitoma e glioneurocitoma lipomatoso". A nova nomenclatura elimina a palavra "meduloblastoma", enfatiza seu caráter benigno e o coloca na categoria dos tumores glioneuronais. Descrevemos mais um caso desta rara entidade clínico-patológica, ressecada do hemisfério cerebelar direito em uma mulher de 53 anos. Com este caso esperamos contribuir para o melhor conhecimento sobre o diagnóstico, prognóstico e possibilidades terapêuticas advindas da presença de tecido adiposo em tumor meduloblastomatoso de adultos.

  6. Intravascular Papillary Endothelial Hyperplasia (Masson’s Tumor) of the Radial Artery: A Case Report

    International Nuclear Information System (INIS)

    Stark, Christopher; Olsen, Daniel; Morris, Christopher; Bertges, Daniel; Najarian, Kenneth

    2016-01-01

    Intravascular papillary endothelial hyperplasia (IPEH), often referred to as Masson’s tumor, is a benign non-neoplastic vascular lesion of the skin and subcutaneous tissues. Although it is rare, knowledge of the existence of IPEH is important as it can mimic other benign and malignant tumors, most notably angiosarcoma. IPEH remains an incompletely understood entity; however, most consider it to be the result of reactive endothelial proliferation following thrombus formation within a vessel, vascular malformation, or adjacent to a vessel. In this article, we report a case of IPEH arising within an arteriovenous malformation of the radial artery and present accompanying multimodality imaging and pathology figures. We will also describe the clinical presentation, pathophysiology, histology, imaging features, and management of IPEH.

  7. Intravascular Papillary Endothelial Hyperplasia (Masson’s Tumor) of the Radial Artery: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Stark, Christopher, E-mail: Christopher.stark@uvmhealth.org [University of Vermont Medical Center, Department of Radiology (United States); Olsen, Daniel [Mayo Clinic, Department of Pathology (United States); Morris, Christopher [University of Vermont Medical Center, Department of Radiology (United States); Bertges, Daniel [University of Vermont Medical Center, Department of Surgery (United States); Najarian, Kenneth [University of Vermont Medical Center, Department of Radiology (United States)

    2016-11-15

    Intravascular papillary endothelial hyperplasia (IPEH), often referred to as Masson’s tumor, is a benign non-neoplastic vascular lesion of the skin and subcutaneous tissues. Although it is rare, knowledge of the existence of IPEH is important as it can mimic other benign and malignant tumors, most notably angiosarcoma. IPEH remains an incompletely understood entity; however, most consider it to be the result of reactive endothelial proliferation following thrombus formation within a vessel, vascular malformation, or adjacent to a vessel. In this article, we report a case of IPEH arising within an arteriovenous malformation of the radial artery and present accompanying multimodality imaging and pathology figures. We will also describe the clinical presentation, pathophysiology, histology, imaging features, and management of IPEH.

  8. Tension pneumoventricle after excision of third ventricular tumor in sitting position

    Directory of Open Access Journals (Sweden)

    Nidhi Gupta

    2011-01-01

    Full Text Available Occurrence of tension pneumoventricle (symptomatic intraventricular air can result in rapid clinical deterioration in an otherwise stable patient. It is a rare clinical entity, mentioned in relation to cerebrospinal fluid (CSF diversion procedures, during the late postoperative period. We present a patient with posterior third ventricular tumor who underwent excision by midline suboccipital craniotomy in sitting position. Neurological status of the patient deteriorated rapidly in the immediate postoperative period owing to development of tension pneumoventricle. The condition improved after twist-drill burr-hole evacuation of air under water-seal. Pre-existing gross hydrocephalus, exploration of third ventricle in sitting position, and residual tumor in third ventricle were possibly the factors responsible for this complication.

  9. Cesarean section after abdominal mesh repair for pregnancy-related desmoid tumor: a case report

    Directory of Open Access Journals (Sweden)

    Ooi S

    2017-07-01

    Full Text Available Sara Ooi, Harry Ngo Obstetrics and Gynaecology Department, Liverpool Hospital, Liverpool, NSW, Australia Abstract: We report the case of a 32-year-old gravida 2 para 1 woman with a background of partially resected desmoid tumor (DT arising from the previous cesarean section (CS scar. This case details the management of her DT by surgical resection and mesh repair and second pregnancy following this. Pregnancy-related DTs are a relatively rare entity, and there is a paucity of literature regarding their management during pregnancy. There are only five reported cases of DTs arising from CS scars. To our knowledge, this is the only report to illustrate that subsequent CS is possible after desmoid resection and abdominal mesh repair. It provides evidence that CS can be safely accomplished following abdominal wall reconstructions and further arguments against elective lower segment CS. Keywords: abdominal wall, cesarean section, complications, desmoid tumor, surgical mesh

  10. Isolated nonpulsatile enophthalmos in neurofibromatosis: An uncommon entity

    Directory of Open Access Journals (Sweden)

    Swati Singh

    2017-01-01

    Full Text Available Isolated enophthalmos is a rarely observed entity in neurofibromatosis (NF. A 12-year-old male presented with right lower eyelid fat prolapse and enophthalmos for the past 7 years. There was no history of antecedent trauma/surgery. Computed tomography of orbit revealed an ill-defined intraconal hyperdense lesion located between lateral and inferior rectus along with an enlarged inferior orbital fissure (IOF. Superior orbital fissure was minimally widened without prolapse of any intracranial contents. Excision biopsy along with repair of widened IOF was performed through inferior transconjunctival route. Histopathology was suggestive of plexiform neurofibroma with positivity for S-100 and epithelial membrane antigen. No associated cutaneous lesions were present. Nonpulsatile enophthalmos with eyelid fat prolapse can be a presenting sign of NF.

  11. Isolated nonpulsatile enophthalmos in neurofibromatosis: An uncommon entity

    Science.gov (United States)

    Singh, Swati; Mulay, Kaustubh; Mittal, Vikas

    2017-01-01

    Isolated enophthalmos is a rarely observed entity in neurofibromatosis (NF). A 12-year-old male presented with right lower eyelid fat prolapse and enophthalmos for the past 7 years. There was no history of antecedent trauma/surgery. Computed tomography of orbit revealed an ill-defined intraconal hyperdense lesion located between lateral and inferior rectus along with an enlarged inferior orbital fissure (IOF). Superior orbital fissure was minimally widened without prolapse of any intracranial contents. Excision biopsy along with repair of widened IOF was performed through inferior transconjunctival route. Histopathology was suggestive of plexiform neurofibroma with positivity for S-100 and epithelial membrane antigen. No associated cutaneous lesions were present. Nonpulsatile enophthalmos with eyelid fat prolapse can be a presenting sign of NF. PMID:29044092

  12. Tagging Named Entities in Croatian Tweets

    Directory of Open Access Journals (Sweden)

    Krešimir Baksa

    2017-01-01

    Full Text Available Named entity extraction tools designed for recognizing named entities in texts written in standard language (e.g., news stories or legal texts have been shown to be inadequate for user-generated textual content (e.g., tweets, forum posts. In this work, we propose a supervised approach to named entity recognition and classification for Croatian tweets. We compare two sequence labelling models: a hidden Markov model (HMM and conditional random fields (CRF. Our experiments reveal that CRF is the best model for the task, achieving a very good performance of over 87% micro-averaged F1 score. We analyse the contributions of different feature groups and influence of the training set size on the performance of the CRF model.

  13. Public administration social responsibility of business entities

    Directory of Open Access Journals (Sweden)

    N. H. Shpankovskaya

    2016-03-01

    Full Text Available Social responsibility of a business entity is seen as an effective tool of public administration. The current stage of development of social responsibility in Ukraine requires state involvement, as its vision by business entities are different, and there is also a need to develop a national model of social responsibility on the basis of international standards, because Ukraine, on the one hand, has the national characteristics of implementation of social initiatives and, on the other, the conditions and resources for their implementation is different from developed market economies. The visions of on social responsibility in the scientific literature are also different. This was the basis for the determination of her essence. We analyzed the interpretations of social responsibility and identified their advantages and disadvantages. Formulation of social responsibility, which is submitted in article, actualizes ecological orientation of the business entity taking into account the need for responsible behavior, and responsibility for actions, which violate the norms of society.

  14. Intraabdominal desmoplastic small round cell tumor: Report of a case and literature review.

    Science.gov (United States)

    Koniari, Katerina; Mahera, Helen; Nikolaou, Marinos; Chatzis, Odysseas; Glezakou, Ourania; Magiasis, Vasilios; Kiratzis, Georgios

    2011-01-01

    Desmoplastic small round cell tumor is a rare malignancy with poor prognosis that predominantly affects young males. Its etiopathogenesis is still unknown and diagnosis can be achieved only by immunohistochemistry and cytogenetic studies. Due to our limited knowledge of the pathologic and clinical nature of this disease, there is no clear consensus regarding the optimal therapeutic procedures for treating this neoplasm. A high degree of care and improvements in diagnostic capabilities are required in order to identify this entity and avoid misdiagnosis. We report a new case of a 29-year-old male who proceeded to our Emergency Department complaining about non-specific abdominal pain. Physical examination revealed no abnormalities except for a palpable mass in the lower abdomen and a diffuse abdominal pain. Computed Tomography scan showed enlarged paraortic and mesenteric lymphadenopathy, thickness of the small bowel wall and dispersed masses intraperitoneally. He underwent an exploratory laparotomy and the resultant biopsy revealed desmoplastic small round cell tumor. Diagnosis of desmoplastic small round cell tumor can easily be missed because it presents with few early warning symptoms and signs, while the routine blood tests are within normal limits. A high degree of suspicion, a thorough physical examination, a full imaging check and an aggressive therapeutic approach are required in order to identify this disease and fight for a better quality of life for these patients. In addition we make a review of the literature in an effort to clarify the epidemiological, clinical and pathological aspects of this entity.

  15. Rectal squamous cell carcinoma in immunosuppressed populations: is this a distinct entity from anal cancer?

    Science.gov (United States)

    COGHILL, Anna E.; SHIELS, Meredith S.; RYCROFT, Randi K.; COPELAND, Glenn; FINCH, Jack L.; HAKENEWERTH, Anne M.; PAWLISH, Karen S.; ENGELS, Eric A.

    2015-01-01

    Objective Squamous cell carcinoma (SCC) of the rectum is rare, but as with anal cancer, risk may be increased among immunosuppressed individuals. We assessed risk of rectal SCC in HIV-infected people. Design Population-based registry Methods We utilized the HIV/AIDS Cancer Match, a linkage of US HIV and cancer registries (1991–2010), to ascertain cases of anal SCC, rectal SCC, rectal non-SCC, and colon non-SCC. We compared risk in HIV-infected persons to the general population using standardized incidence ratios (SIRs) and evaluated risk factors using Poisson regression. We reviewed cancer registry case notes to confirm site and histology for a subset of cases. Results HIV-infected persons had an excess risk of rectal SCC compared to the general population (SIR=28.9; 95%CI 23.2–35.6), similar to the increase for anal SCC (SIR=37.3). Excess rectal SCC risk was most pronounced among HIV-infected men who have sex with men (MSM, SIR=61.2). Risk was not elevated for rectal non-SCC (SIR=0.88) or colon non-SCC (SIR=0.63). Individuals diagnosed with AIDS had higher rectal SCC rates than those with HIV-only (incidence rate ratio=1.86; 95%CI 1.04–3.31). Based on available information, one-third of rectal SCCs were determined to be misclassified anal cancer. Conclusions HIV-infected individuals, especially with advanced immunosuppression, appear to have substantially elevated risk for rectal SCC. As for anal SCC, rectal SCC risk was highest in MSM, pointing to involvement of a sexually transmitted infection such as human papillomavirus. Site misclassification was present, and detailed information on tumor location is needed to prove that rectal SCC is a distinct entity. PMID:26372482

  16. Bone tumors

    International Nuclear Information System (INIS)

    Unni, K.K.

    1988-01-01

    This book contains the proceedings on bone tumors. Topics covered include: Bone tumor imaging: Contribution of CT and MRI, staging of bone tumors, perind cell tumors of bone, and metastatic bone disease

  17. Cross domains Arabic named entity recognition system

    Science.gov (United States)

    Al-Ahmari, S. Saad; Abdullatif Al-Johar, B.

    2016-07-01

    Named Entity Recognition (NER) plays an important role in many Natural Language Processing (NLP) applications such as; Information Extraction (IE), Question Answering (QA), Text Clustering, Text Summarization and Word Sense Disambiguation. This paper presents the development and implementation of domain independent system to recognize three types of Arabic named entities. The system works based on a set of domain independent grammar-rules along with Arabic part of speech tagger in addition to gazetteers and lists of trigger words. The experimental results shown, that the system performed as good as other systems with better results in some cases of cross-domains corpora.

  18. 17 CFR Appendix A to Part 420 - Separate Reporting Entity

    Science.gov (United States)

    2010-04-01

    ... 17 Commodity and Securities Exchanges 3 2010-04-01 2010-04-01 false Separate Reporting Entity A... Part 420—Separate Reporting Entity Subject to the following conditions, one or more aggregating entity(ies) (e.g., parent, subsidiary, or organizational component) in a reporting entity, either separately...

  19. 26 CFR 301.7701-2 - Business entities; definitions.

    Science.gov (United States)

    2010-04-01

    ... 26 Internal Revenue 18 2010-04-01 2010-04-01 false Business entities; definitions. 301.7701-2...) PROCEDURE AND ADMINISTRATION PROCEDURE AND ADMINISTRATION Definitions § 301.7701-2 Business entities; definitions. (a) Business entities. For purposes of this section and § 301.7701-3, a business entity is any...

  20. Neuroendocrine Tumor, Well Differentiated, of the Breast: A Relatively High-Grade Case in the Histological Subtype

    Directory of Open Access Journals (Sweden)

    Shogo Tajima

    2013-01-01

    Full Text Available Primary neuroendocrine carcinoma of the breast is a rare entity, comprising <1% of breast carcinomas. Described here is the case of a 78-year-old woman who developed an invasive tumor in the left breast measuring 2.0 cm x 1.5 cm x 1.2 cm. The tumor was composed of only endocrine elements in the invasive part. It infiltrated in a nested fashion with no tubular formation. Intraductal components were present both inside and outside of the invasive portion. Almost all carcinoma cells consisting of invasive and intraductal parts were positive for synaptophysin and neuron-specific enolase. According to the World Health Organization classification 2012, this tumor was subclassified as neuroendocrine tumor, well-differentiated. Among the subgroup, this tumor was relatively high-grade because it was grade 3 tumor with a few mitotic figures. Vascular and lymphatic permeation and lymph node metastases were noted. In the lymph nodes, the morphology of the tumor was similar to the primary site. No distant metastasis and no relapse was seen for one year after surgery. The prognosis of neuroendocrine carcinomas is thought to be worse than invasive mammary carcinomas, not otherwise specified. Therefore, immunohistochemistry for neuroendocrine markers is important in the routine practice to prevent overlooking neuroendocrine carcinomas.