Management of mucinous urachal neoplasm presenting as pseudomyxoma peritonei.

Background: Mucinous neoplasms of the urachus are rare malignancies so that the physicians' index of suspicion for a timely and accurate diagnosis is low. Also, this disease may present with a wide variety of symptoms and signs.

Methods: Two patients with pseudomyxoma peritonei as the initial presentation of urachal mucinous adenocarcinoma were treated successfully.

Thin-walled cysts as a pathognomonic CT finding in cystic mesothelioma.

Aim: Cystic mesothelioma is a rare disease that results in abdominal distention and poorly defined abdominal pain. Diagnosis has always been made by tissue biopsy rather than by radiologic studies.

Materials And Methods: Our experience with 7 patients with cystic mesothelioma includes 4 patients who had not had prior surgery before the performance of a high resolution CT scan.

Discordant histology of primary appendiceal adenocarcinoid neoplasms with peritoneal dissemination.

Background: Adenocarcinoid is a rare appendiceal tumor that shows histologically both epithelial and neuroendocrine components. We observed that some patients have peritoneal dissemination, which shows adenocarcinoma but lacks the neuroendocrine histology. The objective of the present study was to quantitate these discordant histopathological findings and examine its clinical implication.

Prognostic significance of histomorphologic parameters in diffuse malignant peritoneal mesothelioma.

Context: Diffuse malignant peritoneal mesothelioma is currently regarded as a rare and lethal primary tumor arising from the peritoneal membrane. In the past, treatment plans with variable combinations of surgery and systemic chemotherapy were associated with a median survival of approximately 1 year. Standardized treatments using cytoreductive surgery and perioperative intraperitoneal chemotherapy have extended this survival.

Prognostic indicators for patients undergoing cytoreductive surgery and perioperative intraperitoneal chemotherapy for diffuse malignant peritoneal mesothelioma.

Background: This study evaluates clinical, radiological and histopathological prognostic indicators for survival of patients undergoing cytoreductive surgery and perioperative intraperitoneal chemotherapy for diffuse malignant peritoneal mesothelioma (DMPM).

Methods: Sixty-two consecutive patients with DMPM underwent cytoreduction and perioperative intraperitoneal chemotherapy at the Washington Cancer Institute. Twenty-six clinical, radiological and histopathological parameters were analyzed in univariate and multivariate analyses using overall survival as an endpoint.

Clinical perspective on desmoplastic small round-cell tumor.

Rare diseases are often associated with uninformed medical decisions and poorly executed treatments because of inexperience of the physicians. Desmoplastic small round-cell tumor is a rare disease that is a form of peritoneal surface malignancy usually affecting young males, with a mean survival of 29 months. In order to begin to build a more knowledgeable clinical pathway all 7 patients treated at the Washington Hospital Center were studied and compared to patients described in the medical literature.

Glomus tumor of the duodenum: a case report.

We describe a rare case of glomus tumor of the duodenum. The tumor was detected in a 46-year-old man being investigated for episodes of gastrointestinal bleeding. The excised specimen showed a 2.

Clinically aggressive pseudomyxoma peritonei: a variant of a histologically indolent process.

Background: Three distinct morphologic types of pseudomyxoma peritonei syndrome have been defined: disseminated peritoneal adenomucinosis (DPAM), peritoneal mucinous carcinoma (PMCA), and a hybrid morphologic type. Prognosis is best in patients with DPAM; unfortunately, some patients with DPAM succumb to a rapidly progressive disease process.

Methods: We identified a subset of 11 patients with a histopathology of DPAM but a clinical course showing an invasive disease process.

Prognostic assessment of gastrointestinal stromal tumor.

The term "gastrointestinal stromal tumor" (GIST) has been applied to a collection of distinctive mesenchymal tumors occurring within the human gastrointestinal tract. As new drug therapy becomes available, data regarding the natural history of these unusual tumors are necessary to provide selection factors for treatment. Ninety-eight patients had light microscopy compatible with GIST at a single institution from 1989 to 2000.

An unusual case of multiple papillary fibroelastoma, review of literature.

Primary neoplasms of the cardiac valves are extremely rare. However, papillary fibroelastoma is the third most common primary tumor of the heart [Ann Thorac Surg 52 (1991) 1127]. These tumors can be found anywhere in the heart, but most commonly involve the cardiac valves [Ann Thorac Surg 52 (1991) 1127; McAllister HA, Fenoglio JJ.