Malignant extra-adrenal pancreatic paraganglioma: case report and literature review.

BACKGROUND: Pancreatic paragangliomas are rare tumors, with only 16 reported cases to date. One of these cases demonstrates metastasis to lymph node, while another case was functional, however, none of these cases showed malignant and large, pancreatic paraganglioma with marked invasion. Also another unique feature was the age of our patient compared to the average reported ages in published literature (42-85 years). CASE PRESENTATION: A 19-year-old woman presented with a one-year history of intermittent abdominal pain. Physical examination showed a palpable mass in the right upper abdomen, but initial laboratory results were within normal ranges; tumor markers (CEA, AFP, and CA19-9) were negative. An abdominal and pelvic computed tomography (CT) scan showed a well-defined retroperitoneal para-aortic mass. The CT scan revealed that the surrounding lymph nodes were not enlarged, but the liver showed evidence of parenchymal infiltration. Intraoperatively, a large, firm tumor originating from the head of pancreas was found pushing on the caudate hepatic lobe and the inferior vena cava (IVC). The tumor was resected through a pancreaticoduodenectomy, involving segment VI of the liver and a small segment of the IVC. The blood pressure spiked (>220 mm Hg) when the tumor was manipulated during the operation. The final pathology report showed a 9-cm tumor with lymphovascular invasions; immunohistochemistry was positive for synaptophysin and chromogranin. All resection margins were negative and 1/15 lymph nodes was positive for metastasis. Post-operative recovery was unremarkable. One month after discharge, the patient was re-admitted with abdominal pain and found to have an abdominal collection at the resection site, which was drained under CT guidance. She received a therapeutic dose of I131-metaiodobenzylguanidine (MIBG). Follow-ups showed the absence of recurrence, and she has remained disease free. CONCLUSION: This patient was an extraordinary example of a rare tumor. Even more remarkable was that the tumor was malignant with lymph node invasion. To our knowledge, a case similar to that presented here has not been previously reported in the literature.

Telangiectatic osteosarcoma of the patella.

Although osteosarcoma is the most common primary malignancy of bone, it has only been reported to arise from the patella in a handful of cases. Telangiectatic osteosarcoma accounts for <5% of all osteosarcomas, and it is distinguished histologically by spaces, often blood filled, separated by septa containing highly malignant cells and radiographically by a predominately lytic and/or expansile component. Telangiectatic osteosarcoma can be radiologically confused with aneurysmal bone cyst or giant cell tumor. A 22-year-old otherwise healthy man presented with increasing pain, swelling, and limited flexion of the right knee after failing physical therapy for anterior knee pain. Standard anteroposterior and lateral radiographs demonstrated a diffuse destructive process involving the majority of the patella (including loss of the inferior patellar cortex) and a lytic lesion of the proximal tibia. Apparent osteoid matrix was visible in the soft tissue extension along the inferior pole of the patella. A computed tomography scan of the chest showed 2 pulmonary nodules consistent with metastatic disease. Evaluation of core needle biopsy showed osteosarcoma with telangiectatic features. Given that the majority of the tumor involved the patella/extensor mechanism, it was clear that the tumor originated in the patella. This case presents the first published report of a telangiectatic osteosarcoma arising from the patella.

Benign intrascrotal lipoblastoma in a 4-month-old infant: a case report and review of literature.

Lipoblastomas are rare benign soft tissue tumors that occur primarily in young children. Most lipoblastomas occur in the extremities, trunk, head, and neck. An intrascrotal location is unusual. We describe a case of a 4-month-old infant with an intrascrotal lipoblastoma and discuss the differential diagnosis and review the literature.

Testing of two binary grading systems for FIGO stage III serous carcinoma of the ovary and peritoneum.

OBJECTIVE: A variety of histologic grading systems for ovarian carcinoma have been used, but there is no widely accepted system. Binary grading systems are inherently superior to the more common three-grade systems because they are more reproducible and they correspond to the number of options in the binary treatment decision for which grade is considered important: the use of or withholding of chemotherapy. METHODS: One hundred thirteen unselected FIGO stage III serous carcinomas of the ovary and peritoneum were tested with two grading systems: a binary system recently proposed by investigators at MD Anderson Cancer Center (MDACC) and a new binary system we formulated at the Washington Hospital Center (WHC). Both of these systems are based on nuclear grade. The WHC system has a higher threshold of nuclear size for diagnosing high-grade tumors. RESULTS: The WHC system separated the cases into 89 high-grade and 24 low-grade tumors. The median survival rates were 30 and 49 months for high and low grade respectively, and the actuarial survival curves were not significantly different (P > 0.10). The MDACC system separated the cases into 103 high-grade and 10 low-grade tumors. With this system, low-grade tumors were significantly more likely than high grade to be stage IIIA (P < 0.05) and occurred at a mean age of 57 years compared to 65 years for high-grade tumors (P < 0.05). Low-grade tumors were suboptimally debulked in 10% of cases compared to 27% for high-grade tumors (P > 0.05). The median survival for high-grade tumors was 34 months, and the median for low grade has not been reached. The actuarial survival curves were not significantly different (P = 0.065). CONCLUSION: The MDACC grading system appears more promising than the WHC system. The MDACC system separates a small (9% of advanced stage serous carcinomas) but distinctive well-differentiated tumor which usually has the appearance of invasive low-grade (micropapillary) serous carcinoma. The rarity of this tumor, however, will require a larger series to demonstrate prognostic value. The WHC system, which was designed to enlarge the low-grade group to a size that would be more meaningful in clinical practice, did not demonstrate a survival difference. The failure of the WHC system suggests that attempts to enlarge the low-grade group using histologic features alone are unlikely to be successful. The potential for confounding of grade with substage, volume of residual disease and patient age are issues that may impede determination of the independence of tumor grade in prognosis, and more data, especially for low-grade tumors, are needed.

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. As a comparison group, from a database of 501 patients with pseudomyxoma peritonei, we selected 22 age- and sex-matched controls with a DPAM histology who are alive with no evidence of disease. Clinical factors were identified for comparison of case and control groups. Expression of mucin antigens, mucin (MUC)1 and MUC2, were evaluated using immunohistochemistry. RESULTS: The study group consisted of 11 patients (five men and six women), with a median survival of 52.2 months (SD 7.46) and a 31% 5-year survival. All 22 matched control cases (10 men and 12 women) are alive and disease-free. Clinical data on the study and control groups including co-morbidity were similar. No significant difference in the expression of MUC1 (P = 0.74, Fisher's exact test) or MUC2 (P = 0.34, Fisher's exact test) was demonstrated between groups. CONCLUSIONS: Further investigation of pseudomyxoma peritonei at a molecular and genetic level may help to formulate a more comprehensive classification.

The histologic type and stage distribution of ovarian carcinomas of surface epithelial origin.

Advances over the past decade suggest a need to reassess the distribution of ovarian surface epithelial tumors. A series of 220 consecutive invasive ovarian carcinomas, including carcinosarcomas and peritoneal carcinomas, was reviewed. Notable findings include: 7% of tumors were carcinosarcomas; 22% of cases of peritoneal serous carcinomatosis were of peritoneal origin; <3% of cases were mucinous carcinomas; and only one malignant Brenner tumor (0.5%) and no pure transitional cell carcinomas were identified. If peritoneal carcinomas, carcinosarcomas, and mixed carcinomas with a serous component are combined with serous carcinomas, this group accounts for 78% of all cases and 87% of advanced stage cases, suggesting a greater uniformity to epithelial ovarian cancer than previously appreciated.