Malignant craniopharyngioma.

Craniopharyngiomas are histologically and cytologically benign epithelial tumors of the central nervous system that may be locally aggressive and tend to recur after excision. Malignant change in craniopharyngiomas is extremely rare; we found only 4 such reports in the literature. In this report, we describe a case of squamous cell carcinoma arising in a previously benign craniopharyngioma in a 42-year-old woman. The patient was diagnosed with craniopharyngioma in 1982; during the subsequent 15 years she experienced 7 tumor recurrences, for which surgical resections and 3 courses of radiotherapy were performed. In 1998, the tumor recurred with involvement of the nasal cavity and sphenoid and ethmoid sinuses. Histologic evaluation revealed foci of typical adamantinomatous craniopharyngioma associated with a moderately differentiated squamous cell carcinoma. The transition of typical craniopharyngioma to squamous cell carcinoma was well demonstrated, suggesting that carcinoma arose from the underlying craniopharyngioma. Radiation may have been a contributing factor to carcinogenesis in this case.

Orbital Wegener granulomatosis without systemic findings.

PURPOSE: To describe a case of orbital Wegener granulomatosis without systemic disease. METHOD: Case report. RESULTS: A 69-year-old patient with bilateral inflammatory lacrimal gland masses underwent multiple biopsies that showed a nonspecific lymphoplasmacytic infiltrate consistent with orbital pseudotumor. After unsuccessful treatment with systemic corticosteroids and radiation, severe orbital disease rapidly progressed and the patient underwent unilateral enucleation. The enucleated specimen showed multifocal vasculitis, tissue necrosis, and granulomas consistent with Wegener granulomatosis (WG). Elevated antineutrophil cytoplasmic antibody titers supported the diagnosis of WG. The patient did not have any extraocular signs of WG and continues to be disease-free systemically. CONCLUSION: The authors believe this is the first report of bilateral lacrimal gland masses presenting as a localized form of WG in the total absence of systemic disease.

Clostridium perfringens: a rare cause of postoperative spinal surgery meningitis.

BACKGROUND: Clostridium perfringens is a rare cause of central nervous system infections, particularly meningitis. The case of a 76-year-old man who developed fatal C. perfringens meningitis after routine decompressive laminectomy for spinal stenosis is described. CASE REPORT: Twelve days after surgery the patient presented with pain and serosangiunous drainage from the surgical incision site. A swab of the drainage revealed Gram-positive bacilli; MRI of the lumbosacral spine showed the appearance of air around the laminectomy site. The patient died within 6 hours of presentation. Autopsy revealed acute cranial and spinal meningitis and choroid plexitis with organisms consistent with C. perfringens. CONCLUSION: No significant enteral pathology or source of endogenous infection was determined, suggesting postoperative wound contamination and meningeal seeding with this ubiquitous organism. Clostridial infection, although rare, should be considered in any patient with meningitis with a history of surgical intervention. Survival with minimal neurological deficits was achieved in half of the previously reported cases.

Cystic glucagonoma: A rare variant of an uncommon neuroendocrine pancreas tumor.

Glucagon-producing neuroendocrine tumors typically present with a characteristic constellation of symptoms including necrolytic migratory erythema, non-insulin-dependent diabetes, weight loss, anemia, glossitis, and an increased thrombotic tendency. Most glucagonomas are solid and arise in the body or tail of the pancreas. We report two cases of cystic glucagonoma, one found incidentally in an asymptomatic patient and one in a patient with weight loss and diabetes but no rash. In the first patient, distal pancreatectomy and splenectomy were curative, whereas the second patient continued to exhibit elevated serum glucagon levels and symptoms of glucose intolerance in the absence of demonstrable metastases. Cystic glucagonoma is a unique variant of classic glucagonoma and should be considered in the differential diagnosis of cystic pancreatic neoplasms.

Giant antral ulcer: a rare presentation of eosinophilic gastroenteritis--case report and review of the literature.

A 24-yr-old female presented with a giant gastric ulcer and anemia. She suffered from a transient infantile malabsorption syndrome with eosinophilia. The diagnosis of eosinophilic gastroenteritis associated with the gastric ulcer was made by endoscopic biopsy. Ulcer healing was refractory to medical therapy and partial gastrectomy was performed. Histologic examination revealed transmural eosinophilic infiltrates with mast cell infiltrates in the gastric wall. This case illustrates (1) an extremely rare presentation of eosinophilic gastroenteritis--giant, refractory, gastric ulcer; (2) a potential pathogenic role for mast cells in this syndrome; and (3) the chronic and relapsing nature of the syndrome.