Isolated intracranial involvement in Rosai-Dorfman disease: a report of two cases and review of the literature.

Rosai-Dorfman disease is an idiopathic histiocytic disorder of lymph nodes and extranodal sites. Central nervous system manifestations of this disease are rare, and to our knowledge only 16 cases of intracranial involvement have been reported previously. Intracranial Rosai-Dorfman disease clinically and radiologically resembles meningioma, and histologic examination is essential for a definitive diagnosis. We report two cases of isolated, intracranial, dural-based Rosai-Dorfman disease, review the literature, and discuss the differential diagnoses of this lesion.

Adrenal histoplasmosis: diagnosis by fine-needle aspiration biopsy.

A 54-year-old man presented with a 6-month history of fever, night sweats, and weight loss. He had hepatosplenomegaly, and bilateral adrenal masses were discovered on computed tomographic (CT) scan. CT-guided fine-needle aspiration biopsy (FNAB) of the right adrenal mass demonstrated purulent material. Special stains done on this material showed organisms with morphologic features of Histoplasma capsulatum. The patient was started on antifungal therapy and discharged. FNAB of the adrenal gland is an effective method in the diagnosis of unusual infectious diseases. Special stains for micro-organisms proved helpful in the initial diagnosis of histoplasmosis.

Granulocytic sarcoma of the spine in nonleukemic patients: report of three cases.

OBJECTIVE AND IMPORTANCE: Granulocytic sarcomas involving the spine in patients without myelogenous leukemia are rare. We report three cases and review the literature. CLINICAL PRESENTATION: Three patients presented with spinal epidural tumors, which caused spinal cord compression in one and cauda equina compression in two. INTERVENTION: All patients underwent surgery, and biopsies revealed histological features of granulocytic sarcomas. Bone marrow aspirates and biopsies showed no evidence of acute leukemia at initial presentation, for all three patients. CONCLUSION: Granulocytic sarcomas in nonleukemic patients are rare, and when they affect the spine they are frequently misdiagnosed. Appropriate therapy for these tumors requires early identification.

Adrenal pheochromocytoma mimicking small cell carcinoma on fine needle aspiration biopsy. A case report.

BACKGROUND: Fine needle aspiration biopsy (FNAB) is a sensitive and specific technique in the diagnosis of adrenal tumors. However, in rare cases the cytomorphologic features may mimic small cell neoplasms. CASE: A 54-year-old male presented with a history of hypertension and left flank pain of recent onset. Abdominal computed tomography (CT) showed a 4-cm mass involving the left adrenal gland. Fine needle aspiration biopsy (FNAB) showed groups of small round cells with hyperchromatic nuclei. The findings were highly suspicious for metastatic small cell carcinoma. Subsequently it was learned that the mass had been noted three years earlier on CT studies but had grown from 2.5 to 4 cm. A chest radiograph was unremarkable. The clinical findings were more in keeping with a primary adrenal tumor. Immunohistochemical staining done retrospectively on the cell block showed positive reactivity for chromogranin and neuron-specific enolase. These findings, correlated with the clinical features, were in keeping with a diagnosis of pheochromocytoma. Left adrenalectomy revealed a pheochromocytoma. CONCLUSION: Adrenal pheochromocytoma should be included in the differential diagnosis of small round cell neoplasms seen on FNAB of the adrenal gland. Immunohistochemistry and clinical findings are helpful in reaching the correct diagnosis.

Paraneoplastic limbic encephalitis in Hodgkin's disease.

BACKGROUND: Oat cell carcinoma of the lung is the most common cause of paraneoplastic limbic encephalitis. Association with other malignancies, in particular Hodgkin's disease, is very rare. CASE REPORT: This 23-year-old male presented with a six month history of progressive alteration in mental status, which consisted of insomnia, short-term memory loss, depression and cognitive impairment. Gadolinium MRI of the head showed intense bilateral contrast enhancement affecting the medial aspects of the temporal lobes in the region of the amygdala and hippocampus. The brain biopsy showed minimal neuronal loss with intense perivascular lymphocytic cuffing and microglial nodules. Polymerase chain reaction for herpes simplex and cytomegalovirus were negative. With prednisone treatment, the patient's neurologic status stabilized but did not improve. Four months later, he presented with left axillary lymphadenopathy. Lymph node biopsy was diagnostic of Hodgkin's disease. During the chemotherapy, his lymphadenopathy subsided and his neurologic and mental status improved. When seen last after completion of his chemotherapy, one year after presentation, he had resumed normal social activities and was enrolled in a university language course. CONCLUSION: This is the first reported case in the English literature of a biopsy proven paraneoplastic limbic encephalitis associated with Hodgkin's disease. Hodgkin's disease should be thought of as a possible cause of paraneoplastic limbic encephalitis in the appropriate clinical setting.

Gastrocolic fistula secondary to primary gastric lymphoma.

Gastrocolic fistula in primary non-Hodgkin's lymphoma (NHL) of the stomach is rare; in a review of the literature we found only four cases, all in association with disseminated (stage IV) disease. We describe the first case of a gastrocolic fistula in a patient with stage IE lymphoma. The diagnosis was suggested by feculent vomiting, and the fistula was located using barium enema and CT scan. Therapy consisted of local resection followed by combination chemotherapy.

The significance of spinal cord compression as the initial manifestation of lymphoma.

Eighteen patients with spinal cord compression caused by previously undiagnosed lymphoma were treated at our institution between 1976 and 1991. There were 14 male and 4 female patients (mean age, 58.2 years). The absence of bony involvement on radiographic images was a feature in 16 of the cases. All patients underwent laminectomy for decompression and tissue diagnosis, after which 5 underwent radiotherapy, 3 underwent chemotherapy, and 10 underwent combined-modality treatment. The functional outcome was improvement in 8 patients and no change in 10; no patient worsened after surgery. Eleven had advanced disease at diagnosis, while seven had limited disease, including three patients with localized extradural lymphoma. There were 16 cases of non-Hodgkin's lymphoma and 2 of Hodgkin's disease. Two patients had T-cell lymphoma and were among the longest survivors. DNA flow cytometry identified the low-grade tumors as diploid with very low proliferative indices, while the high-grade tumors all had high indices. At a mean observation time of 41.7 months, five patients have died of their disease, and seven remain in complete remission. Survival is markedly better than that reported for other malignant extradural tumors; however, even limited stage lymphoma can behave aggressively. Similarities in age, sex distribution, histological features, and the results of flow cytometry suggest behavior similar to extranodal lymphoma at other sites. Surgery to provide a tissue diagnosis, followed by combined radiotherapy and chemotherapy, is indicated for all cases.

Diffuse epithelial mesothelioma: a review of the ultrastructural spectrum.

Traditionally, diffuse epithelial mesotheliomas are mainly identified at the ultrastructural level by the numerous, long, wavy-appearing surface microvilli. By electron microscopy of a series of diffuse mesotheliomas of varying subtype (epithelial, biphasic, sarcomatous, and poorly differentiated), it can be demonstrated that the differentiation of this specialized surface organelle is quite variable even in well-differentiated lesions. The presence of only a few, scattered, short microvilli does not exclude a diagnosis of epithelial mesothelioma, particularly if historical, surgical, and radiologic findings support this diagnostic conclusion. Indeed, even the complete absence of surface microvilli is compatible with a diagnosis of diffuse epithelial mesothelioma. It is important to become aware of the spectrum of tumor cell differentiation in serosal tumors, as all of the fine structural diagnostic criteria in mesotheliomas are expressed to varying degrees in individual cases.

Leiomyoma of Meckel's diverticulum associated with adenocarcinoma of the descending colon.

A case of leiomyoma of Meckel's diverticulum associated with a carcinoma of the descending colon, presenting with intestinal obstruction, is described. Only two cases of smooth muscle tumors of the small intestine associated with carcinoma of the large bowel have been described so far, and ours is probably the first case of its kind to be reported. The importance of angiography and a through intraoperative exploration is stressed.

Necrotizing enteritis.

Clinicopathological features and follow-up of 150 cases of necrotizing enteritis observed over the period of 8 years at Miraj, India, are presented. This is a distinct pathological entity with seasonal occurrence, frequently affecting male patients of the low socioeconomic groups between the third and fifth decades of life. The symptoms are disproportionately greater than the physical signs. The ischaemic lesions, which mainly affect the jejunum, show a variable degree of severity ranging from segmental congestion and ulceration to extensive gangrene and perforation. In the early stage of the disease recovery is complete. Conservative treatment is preferred in early cases, surgery being indicated only for advanced lesions. No definite aetiology has been detected.