			Home About us Contact

Rare Malignancy (rare + malignancy)

Distribution by Scientific Domains

Medical Sciences	92%

Selected Abstracts

Mesothelioma of the tunica vaginalis testis: A rare malignancy mimicking more common inguino-scrotal masses

JOURNAL OF SURGICAL ONCOLOGY, Issue 2 2006
P.J.C.M. Schure
Abstract Malignant pleural mesotheliomas are rare malignancies associated with asbestos exposure and these tumors are infamous for their poor prognosis. Mesotheliomas in other body areas are much rarer. They may occur in the abdominal cavity and also in the inguinal region. In the latter area they are commonly confused with much commoner benign conditions. We present three cases of mesotheliomas in the tunica vaginalis testis. J. Surg. Oncol. 2006;94:162,164. © 2006 Wiley-Liss, Inc. [source]

Primary salivary gland type carcinoma of the nasopharynx: Therapeutic outcomes and prognostic factors

HEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 4 2010
Tian-Run Liu MD
Abstract Background. Primary salivary gland type nasopharyngeal carcinoma (SNPC) is a rare malignancy with diverse clinical behavior and different prognoses. Previous studies have reported on limited patient populations, and few long-term studies have outlined outcomes and prognostic factors. Furthermore, controversy exists as to the treatment policy of SNPC. The aim of this study was to define management approaches, therapeutic outcomes, and prognostic factors of SNPC. Methods. The medical records of 67 patients with SNPC at 1 institution between 1977 and 2005 were reviewed. Patient records were analyzed for management approaches, outcomes, and prognostic factors. Results. SNPC is a rare malignancy accounting for only 0.29% of nasopharyngeal malignancies, and the lymphatic metastases and distant metastases rates were 28.4% and 23.9%, respectively. The 5-year disease-free survival (DFS) and overall survival (OS) rates were 41.1% and 57.1%, respectively; no significant differences were found in DFS or OS between different histological subtypes. A significant difference was found in OS between surgical treatment and nonsurgical treatment in T1,T2 patients with well-differentiated tumors. Multivariate analyses indicated that lymph node metastases, stage, and distant metastases were independent factors for DFS, whereas cranial nerve invasion, tumor residue, and distant metastases were independent factors affecting OS. Conclusions. SNPC is a malignancy with generally favorable prognosis. In T1,T2 patients with well-differentiated tumors, SNPC should be treated by combined surgical operation and radiotherapy. Cranial nerve invasion, tumor residue, and distant metastases were independent factors affecting OS. © 2009 Wiley Periodicals, Inc. Head Neck, 2010 [source]

Peritoneal mesothelioma presenting as an acute surgical abdomen due to jejunal perforation

JOURNAL OF DIGESTIVE DISEASES, Issue 4 2007
Nikolaos S SALEMIS
BACKGROUND: Peritoneal mesothelioma is a rare disease associated with poor prognosis. Acute abdomen as the first presentation is an extremely rare occurrence. We report an exceptional case of a patient who was found to have a jejunal perforation due to infiltration of peritoneal mesothelioma. METHODS: A 62-year-old man was admitted with clinical signs of peritonitis. Computerized tomographic scans showed a mass distal to the ligament of Treitz, thickening of the mesentery and a small amount of ascites. RESULTS: Emergency laparotomy revealed a perforated tumor 15 cm distal to the ligament of Treitz and diffuse peritoneal disease. Segmental small bowel resection and suboptimal cytoreduction were performed. Histopathology and immunohistochemistry showed infiltration of malignant mesothelioma. During the postoperative period pleural mesothelioma was also diagnosed. Despite adjuvant chemotherapy, the patient died of disseminated progressive disease 7 months after surgery. CONCLUSIONS: Peritoneal mesothelioma is a rare malignancy with grim prognosis. Small bowel involvement is a poor prognostic indicator. Our case of a small bowel perforation due to direct infiltration by peritoneal mesothelioma appears to be the first reported in the English literature. [source]

Detection of Merkel cell polyomavirus in Merkel cell carcinoma and Kaposi's sarcoma

JOURNAL OF MEDICAL VIROLOGY, Issue 11 2009
Harutaka Katano
Abstract Merkel cell carcinoma is a rare malignancy that sometimes occurs in the skin of elderly people. Recently, a new human polyomavirus, Merkel cell polyomavirus (MCPyV) was identified in Merkel cell carcinoma. In the present study, MCPyV-DNA was detected in 6 of 11 (55%) cases of Merkel cell carcinoma by nested PCR and real-time PCR. Histologically, MCPyV-positive cases showed round and vesicular nuclei with a fine granular chromatin and small nucleoli, whereas MCPyV-negative cases showed polygonal nuclei with diffusely distributed chromatin. Real-time PCR analysis to detect the MCPyV gene revealed that viral copy numbers ranged 0.04,0.43 per cell in cases of Merkel cell carcinoma. MCPyV was also detected in 3 of 49 (6.1%) cases of Kaposi's sarcoma (KS), but not in 192 DNA samples of other diseases including 142 autopsy samples from 20 immunodeficient patients. The MCPyV copy number in KS was lower than that in Merkel cell carcinoma. PCR successfully amplified a full-length MCPyV genome from a case of KS. Sequence analysis revealed that the MCPyV isolated from KS had 98% homology to the previously reported MCPyV genomes. These data suggest that the prevalence of MCPyV is low in Japan, and is at least partly associated with the pathogenesis of Merkel cell carcinoma. J. Med. Virol. 81:1951,1958, 2009. © 2009 Wiley-Liss, Inc. [source]

Adjuvant radiation therapy is associated with improved survival for gallbladder carcinoma with regional metastatic disease

JOURNAL OF SURGICAL ONCOLOGY, Issue 1 2007
Pablo Mojica MD
Abstract Background Gallbladder carcinoma is a rare malignancy and is associated with dismal outcomes. The aim of this study was to better define the role of adjuvant radiation therapy in the management of gallbladder carcinoma. Methods The Surveillance, Epidemiological, and End Results (SEER) survey from the National Cancer Institute was queried from 1992 to 2002. Retrospective analysis was done. The end-point of the study was overall survival. Results There were a total of 3,187 cases of gallbladder carcinoma in the registry from 1992 to 2002. Of the surgical group, 35% were stage I, 36% were stage II, 6% were stage III, and 21% were stage IV. Adjuvant radiation was used in 17% of the cases. The median survival for those patients receiving adjuvant radiation therapy was 14 months compared to an 8 months median survival for those treated without adjuvant radiation therapy (P,,,0.001). The survival benefit associated with radiation use was only presenting those patients with regional spread (P,=,0.0001) and tumors infiltrating the liver (P,=,0.011). Conclusion The use of adjuvant radiation therapy is associated with improved survival in patients with locally advanced gallbladder cancer or gallbladder cancer with regional disease. J. Surg. Oncol. 2007;96:8,13 © 2007 Wiley-Liss, Inc. [source]

Mesothelioma of the tunica vaginalis testis: A rare malignancy mimicking more common inguino-scrotal masses

Peripheral T-cell lymphoma in a five year old

PEDIATRIC BLOOD & CANCER, Issue 1 2008
M.W. Beresford PhD
Abstract We report a case presenting with persistent pyrexia that led to the diagnosis of peripheral T-cell lymphoma, a rare malignancy in childhood. The case illustrates diagnostic conundrums in a patient who is not responding as expected to treatment. Pediatr Blood Cancer 2008;50:145,147. © 2006 Wiley-Liss, Inc. [source]

Leiomyosarcoma of the main bronchus in a girl: A long-time survivor with multiple lung metastases

PEDIATRIC PULMONOLOGY, Issue 4 2004
Fumihiro Takeda MD
Abstract Primary leiomyosarcoma of the respiratory tract is a very rare malignancy, especially in childhood, with only 15 cases in patients under 16 years old having been reported. In the present case, the survival period from the onset of symptoms has been over 7 years, despite incomplete resection. Based on the 15 published cases, the prognosis is poorer when the tumor is unresected or incompletely resected, but under favorable circumstances, prolonged survival is possible. Pediatr Pulmonol. 2004; 37:368,374. © 2004 Wiely-Liss, Inc. [source]

Diagnostic and surgical dilemmas in hereditary medullary thyroid carcinoma

THE LARYNGOSCOPE, Issue 7 2009
Shawn M. Allen MD
Abstract Medullary thyroid carcinoma (MTC) is a rare malignancy arising from the parafollicular C cells within the thyroid gland. The majority of cases are sporadic, but at least 30% are hereditary in nature. Inherited forms of MTC occur as familial MTC or as a manifestation of multiple endocrine neoplasia type 2. Early diagnosis and aggressive surgical management, including prophylactic thyroidectomy, improve the prognosis of patients with hereditary MTC. Several issues regarding the diagnosis and treatment of MTC remain controversial. Genetic penetrance and virulence are variable. We present an index case of familial MTC to illustrate common difficulties in the initial diagnosis and dilemmas in the surgical approach, followed by a review of current literature relevant to the management of hereditary MTC. Laryngoscope, 2009 [source]

Interdigitating dendritic cell sarcoma of the tonsil

ASIA-PACIFIC JOURNAL OF CLINICAL ONCOLOGY, Issue 3 2010
Sun Young KIM
Interdigitating dendritic cell sarcoma (IDCS) is an extremely rare malignancy derived from antigen-presenting cells, with 55 cases reported thus far. A standard treatment modality is still being debated. This report describes a 56-year-old female who presented with right tonsillar enlargement and right submandibular swelling for 6 months. Treatment with empiric antibiotics did not result in improvement of her symptoms. Fine needle aspiration of the tonsil revealed no malignant cells. Tonsillectomy was eventually performed due to persistent symptoms. Based on microscopic findings, immunohistochemical stains, and review of the literature, the present case was finally diagnosed as IDCS of the tonsil with cervical lymph node involvement. The patient received four cycles of CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) chemotherapy, and a clinically complete response was achieved followed by adjuvant radiation. [source]

Clinical characteristics and prognostic factors for primary appendiceal carcinoma

ASIA-PACIFIC JOURNAL OF CLINICAL ONCOLOGY, Issue 1 2010
Yoon Ho KO
Abstract Aim: Primary adenocarcinoma of the appendix is a rare malignancy. This study assessed prognostic factors affecting the clinical outcome in patients with appendiceal neoplasms. Methods: We performed a retrospective analysis of patients who had appendectomies between 1991 and 2007 at five centers in South Korea. Results: Overall 55 patients (19 men, 36 women, median age 61 years) were identified. Of these, 37 (67.3%) were mucinous adenocarcinomas, 14 (25.5%) were intestinal-type adenocarcinomas, and four (7.3%) were signet ring cell carcinomas. The distribution of stages was: 26 (47.3%) with localized disease, five (9.1%) with regional disease, and 24 (43.6%) with distant metastatic disease. The overall 3- and 5-year survival rates among all patients were 72.2% and 64.0%, respectively, with 20 deaths during the follow-up period. In a multivariate analysis, high histological grade (hazard ratio [HR]vs low grade 15.7; P = 0.001) and pathological stage (distant vs loco-regional, HR 6.2; P = 0.021) were independent predictors of overall survival. Of the 34 patients who underwent curative resections of primary appendiceal carcinomas, the 3- and 5-year disease-free survival rates were 66.4% and 53.3%, respectively. The recurrence rate was higher in patients with regional lymph node metastasis (HR vs node negative disease 23.4; P = 0.005) and high-grade tumors (HR vs low grade 6.3; P = 0.029). Additionally, a right hemicolectomy reduced the risk of recurrence (HR vs lesser procedures 0.05; P = 0.005). Conclusion: High tumor grade and advanced stage were significantly predictive of poor survival outcome in patients with primary appendiceal carcinomas. [source]

A couple with gastrointestinal stromal tumor (GIST)

ASIA-PACIFIC JOURNAL OF CLINICAL ONCOLOGY, Issue 2 2009
Pirooz POURSOLTAN
We present a 70-year old woman with metastatic gastrointestinal stromal tumor (GIST) and her partner, a 79-year old man with multiple gastric GIST tumors. This tumor is considered a rare malignancy with a reported incidence of 6,13 new cases per million. Our patients were found to have different genetic mutations in the C-KIT gene as the cause of their disease but, given the rarity of this tumor, it raises a question about their possible exposure to carcinogens or another shared mechanism that might have been involved in the pathogenesis of this cancer. [source]

Parosteal osteosarcoma: report of a case and review of the literature

AUSTRALIAN DENTAL JOURNAL, Issue 1 2010
TC Huang
Abstract Parosteal osteosarcoma is a rare malignancy of the bone that usually arises in the long bones. Involvement of the oral cavity is rare. Only 12 cases of intraoral parosteal osteosarcoma have been reported in the English language literature. This paper defines the major clinical, radiographic and histologic features of parosteal osteosarcoma and illustrates these with a case of a 33-year-old male presenting with a three-month history of a painless enlarging lump in the right maxilla. A critical and comprehensive review of the English language literature is also provided. [source]