A large retroperitoneal Mullerian cyst: case report and review of the literature.

BACKGROUND: Mullerian duct cysts are considered to be part of the urogenital cysts. Mullerian cysts, anatomically distant to the genitourinary organs or the pelvis, are extremely rare entities. MATERIAL AND METHODS: Case report and review of the literature using key word "Mullerian cyst", "retroperitoneal" and "mediastinal". RESULTS: A 48-year-old woman with abdominal discomfort and nausea was referred to our hospital. Further work-up with CT and MRI showed a large retrogastric, retropancreatic cyst, herniating through the diaphragm in the mediastinum. As etiology was unsure, a laparoscopic cystectomy was performed. Histological examination showed a benign cyst of Mullerian origin. A review of the literature confirmed that extrapelvic Mullerian cysts are rare, with only 28 mediastinal cysts and 12 extrapelvic retroperitoneal cysts being described. CONCLUSION: Retroperitoneal Mullerian cysts outside the pelvis have infrequently been described in the literature. As correct preoperative diagnosis is seldom possible, surgical resection is the gold standard of treatment.

A primary extraskeletal osteosarcoma of the mesentery: a case report.

Extraskeletal osteosarcoma is a rare and invasive malignancy, typically located in the soft tissue without attachment to the skeleton. The present study reports a case of a primary mesenteric extraskeletal osteosarcoma of a 71-year-old woman. The patient complained of an incomplete defecation. Colonoscopy showed an ulcer with impression of external compression of the sigmoid. An additional abdominal computed tomography (CT) scan revealed a large, almost completely calcified, mass in the left lower abdomen causing hydronephrosis of the left kidney. The patient underwent surgery and the mass was resected completely. The histopathological diagnosis was a primary abdominal extraskeletal osteosarcoma arising from the mesocolon with local invasion of the sigmoid. She was in follow-up without adjuvant chemo- or radiotherapy. Five months after initial surgery the tumor recurred with widespread peritoneal metastasis.

High-dose lanreotide in the treatment of poorly differentiated pancreatic neuroendocrine carcinoma: a case report.

Pancreatic neuroendocrine tumors (NETs), including poorly differentiated carcinomas (NECs), are rarely encountered. The majority of these tumors do not secrete excess hormones, but functioning NETs produce large amounts of vasoactive peptides and may cause carcinoid syndrome. Synthetic somatostatin analogs (SSAs) have been widely used in NETs for control of hormonal syndromes. Here, we present a case of poorly differentiated, grade 3 pancreatic NEC associated with carcinoid syndrome, for which adequate symptom control was achieved for 2 years and 4 months using the long-acting SSA lanreotide Autogel(®). In February 2009, a 55-year-old woman presented with episodes of flushing, diarrhea and epigastric pain. Imaging techniques revealed the presence of a metabolically active mass expressing somatostatin receptors in the hilar area of the liver. Histopathological examination confirmed the malignant nature of the mass, which was identified as a poorly differentiated grade 3 pancreatic NEC (TNM staging: T4NxM0). Therapeutic options were limited for the patient because of the extent of the primary mass involving the celiac axis, severe gastrointestinal toxicity experienced as a side effect of chemotherapy with cisplatin-etoposide and, later in the course of the disease, extensive liver metastases and carcinoid heart syndrome. Along with a palliative debulking surgery and right portal vein embolization, biotherapy with a high dose of lanreotide Autogel (120 mg/14 days) contributed to alleviation of symptoms caused by hormone overproduction, even after the development of liver metastases. These results suggest that patients with poorly differentiated NECs who exhibit signs of carcinoid syndrome can benefit from treatment with somatostatin analogs.