Interdigitating dendritic cell sarcoma. A report of four cases and review of the literature.

To better define the clinical and pathologic features of interdigitating dendritic cell sarcoma (IDCS), we report 4 cases, including the first reported in the tonsil. There were 2 male and 2 female patients (mean age, 70 years). Sites of tumor included 1 case each in the right cervical lymph node, left axillary lymph node, right tonsil, and right inguinal lymph node. Histologically, all showed diffuse effacement of the lymphoid tissue by pleomorphic round to spindled cells with convoluted nuclei and abundant eosinophilic cytoplasm. All were immunoreactive for S-100, CD68, lysozyme, and vimentin. CD45 was positive in 3 cases and CD1a in 1 case. Fascin was positive in 3 cases. Other immunostains, including CD3, CD20, CD21, CD30, actin, cytokeratin, and HMB-45, were negative. Ultrastructurally, the tumor cells were elongated and showed indented nuclei, variable numbers of lysosomes, and interdigitating cytoplasmic processes. Follow-up was available for all cases. One patient died of widespread disease 2 months after diagnosis. One was alive with metastatic lung disease at 12 months. Two patients were disease free at 5 and 9 months.

Intravascular lymphomatosis presenting within angiolipomas.

This report presents a case of intravascular lymphomatosis identified within an angiolipoma. The patient was a 73-year-old woman with a history of lobular carcinoma of the breast who presented with a chest wall nodule near the site of prior mastectomy. Microscopically, the nodule was composed of encapsulated adipose tissue with an associated vascular proliferation. Many of the vascular lumina were expanded by collections of large pleomorphic cells with vesicular nuclei and prominent nucleoli. These atypical cells displayed immunoreactivity for CD45RB (leukocyte common antigen) and the pan-B cell marker CD20 (L26). The patient subsequently developed multiple lesions of the extremities and died soon after developing symptoms referable to the central nervous system. Intravascular lymphomatosis most commonly presents with central nervous system and dermatologic involvement although any organ system may be affected. Intravascular lymphomatosis is an aggressive neoplasm that is generally diagnosed at postmortem examination. This case shows the protean manifestations of intravascular lymphomatosis and highlights the necessity of considering this malignancy in the differential diagnosis when entertaining the diagnosis of vascular invasion by carcinoma. Ann Diagn pathol 5:103-106, 2001. This is a US government work. There are no restrictions on its use.

Splenic inflammatory myofibroblastic tumor (inflammatory pseudotumor): a clinicopathologic and immunophenotypic study of 12 cases.

CONTEXT: Inflammatory pseudotumor is an uncommon and enigmatic lesion. The spindle cells found in this tumor have features of myofibroblasts. Because of the indefinite relationship of these lesions with inflammatory fibrosarcoma and their indefinite biologic behavior, inflammatory pseudotumor is currently classified as inflammatory myofibroblastic tumor (IMT). To date, only case reports or small series have been published on these tumors, which are primary in the spleen. DESIGN: In this study, we describe the clinical, morphologic, and immunophenotypic findings of 12 cases of splenic IMT and examine their relationship to Epstein-Barr virus (EBV). RESULTS: The patients included 8 women and 3 men, ranging from 19 to 77 years of age (mean, 53 years; median, 60 years). Demographic data were unavailable for 1 patient. Patients generally presented with abdominal pain (n = 5) and fever (n = 4). Associated lesions included renal cell carcinoma (n = 2), colonic adenocarcinoma (n = 1), and cholecystitis (n = 1). All tumors were composed of a bland spindle cell proliferation in association with a variable mixed inflammatory component. There were 2 growth patterns, namely, a cellular spindle cell pattern and a hypocellular fibrous pattern. An immunohistochemical panel confirmed the myofibroblastic nature of the spindle cells. The spindle cells of 2 cases were immunoreactive for EBV latent membrane protein 1, whereas 6 of 10 cases were positive for EBV-encoded RNA using in situ hybridization. Follow-up was available for 8 patients; 6 were alive with no evidence of recurrence and 2 were dead of other causes. CONCLUSION: Splenic IMTs are uncommon lesions that can be distinguished from other conditions using a combination of clinical, histologic, and immunophenotypic findings. Epstein-Barr virus may play a role in the pathogenesis of splenic IMT, and there may be an association of splenic IMT with concomitant disease or malignancy. Most splenic IMTs have an excellent long-term prognosis.

Follicle center lymphoma involving the female genital tract: a morphologic and molecular genetic study of three cases.

Primary female reproductive system lymphomas are distinctly uncommon, and are defined as lymphomas that present primarily as gynecologic tumors. We describe 3 cases which presented in this location, 1 primary ovarian disease and 2 others presenting the initial manifestations of disseminated lymphoma. Clinical history, follow-up information, and paraffin embedded archival tissue were available for all 3 cases. A panel of immunoperoxidase studies and molecular genetic studies were performed for each case. The primary ovarian follicle center lymphoma was a grade III/III (large cell) while the cases representing secondary involvement were grade I/III (small cell). Immunohistochemistry demonstrated reactivity of the malignant cells in each case with CD20 and bcl-2. In the grade I/III cases the cells were immunoreactive for CD45RA and CD10. Molecular genetic analysis demonstrated the t(14;18) translocation in the case of primary ovarian follicle center lymphoma. Follicle center lymphoma uncommonly presents in the female genital system, and may rarely be primary to this site. Immunoperoxidase and molecular studies in concert with the morphology are invaluable in rendering a correct diagnosis and ensuring correct treatment of the patient.

Splenic angiosarcoma: a clinicopathologic and immunophenotypic study of 28 cases.

Primary angiosarcoma of the spleen is a rare neoplasm that has not been well characterized. We describe the clinical, morphologic, and immunophenotypic findings of 28 cases of primary splenic angiosarcoma, including one case that shares features of lymphangioma/lymphangiosarcoma. The patients included 16 men and 12 women, aged 29 to 85 years, with a mean of 59 years and median of 63 years. The majority of patients (75%) complained of abdominal pain, and 25% presented with splenic rupture. The most common physical finding was splenomegaly (71%). Seventeen of 21 patients were reported to have anemia. Macroscopic examination showed splenomegaly in 85% cases. Sectioning revealed discrete lesions in 88% of cases, ranging from well-circumscribed firm nodules to poorly delineated foci of necrosis and hemorrhage associated with cystic spaces. Microscopically, the tumors were heterogenous; however, all cases demonstrated at least a focal vasoformative component lined by atypical endothelial cells. Solid sarcomatous, papillary, and epithelioid growth patterns were observed. The solid sarcomatous component resembled fibrosarcoma in two cases and malignant fibroushistiocytoma in one case. Hemorrhage, necrosis, hemosiderin, extramedullary hematopoiesis, and intracytoplasmic hyaline globules were frequently identified. A panel of immunohistochemical studies revealed that the majority of tumors were immunoreactive for at least two markers of vascular differentiation (CD34, FVIIIRAg, VEGFR3, and CD31) and at least one marker of histiocytic differentiation (CD68 and/or lysozyme). Metastases developed in 100% of patients during the course of their disease. Twenty-six patients died of disease despite aggressive therapy, whereas only two patients are alive at last follow-up, one with disease at 8 years and the other without disease at 10 years. In conclusion, primary splenic angiosarcoma is an extremely aggressive neoplasm that is almost universally fatal. The majority of splenic angiosarcomas coexpress histiocytic and endothelial markers by immunohistochemical analysis, which suggest that some tumors may originate from splenic lining cells.

Death among U.S. Air Force basic trainees, 1956 to 1996.

BACKGROUND: Basic military training in the U.S. Air Force exposes recruits to multiple environmental and psychological stressors. Deaths often prompt examination of the training process. METHODS: This retrospective case study evaluates recruit deaths at Lackland Air Force Base between 1956 and 1996 in terms of demographic, clinical, and environmental variables. RESULTS: Eighty-five deaths occurred, with 81% being natural, 13% suicide, 4% accidental, and 2% not classified. Ninety-four percent of recruits who died were male, and 60% were 17 to 19 years of age. The average death rate was 2.8/100,000 recruits. Seven recruits were sickle cell trait (SCT)-positive. The relative risk for nontraumatic deaths between expected SCT-positive and non-SCT-positive populations was 23.53 (confidence interval, 19.55-30.01). Thirty-five percent (30 recruits) died from cardiac causes, resulting in a death rate of 1.0/100,000 trainees. Thirty-three percent (28 recruits) died primarily from infections. Six deaths were due to heat stroke, 11 to suicide, and 3 to accidents. CONCLUSION: As a result of improvements in immunizations, changes in hydration and exercise policies, limited access to vehicles, close supervision, the "buddy system," and the institution of the Navy-Air Force Medical Evaluation Test, only a few deaths occurred in any given year.

Rapidly progressive T cell lymphoma presenting as acute renal failure: case report and review of the literature.

We describe a case of peripheral T cell lymphoma that is remarkable for its fulminate course and selective targeting of both kidneys. The patient was a 6-year-old girl who was in her usual state of good health until the onset of abdominal pain and fever. She was treated for acute oliguric renal failure and visual disturbances. A renal biopsy was performed. Biopsy findings were interpreted as suggestive of a vasculitic process, and treatment was initiated for a presumptive diagnosis of Wegener's granulomatosis. The patient died 3 days following admission, and autopsy revealed extensive bilateral kidney infiltration by a peripheral T cell lymphoma. The remainder of the body was spared with the exception of mild infiltration of the pulmonary parenchyma and choroid plexus by neoplastic lymphocytes. The neoplastic nature of the disease was confirmed utilizing immunoperoxidase stains and T cell receptor gene rearrangement. Primary renal lymphoma and renal failure attributable to involvement by lymphoma are rare findings that should be considered when other more common causes of renal insufficiency have been excluded. The presenting clinical complaints are generally of short duration, nonspecific, and atypical. Most patients exhibit oliguria. Physical examination may reveal hepatosplenomegaly, lymphadenopathy, and flank and/or abdominal mass(es). Laboratory findings frequently include an elevated serum creatinine, blood urea nitrogen, lactate dehydrogenase, and a mild proteinuria. Electrolyte abnormalities are variably present. Possible radiographic findings include hypodense or hypoechoic renal lesions and diffuse bilateral renal enlargement. Although the prognosis is dismal, survival may be prolonged utilizing current treatment modalities, and rare patients may be "cured" of disease. The clinical presentation, radiological findings, and prognosis of patients with clinically evident renal involvement by non-Hodgkin's lymphoma are discussed.