Combined partial cystectomy and cesarean delivery in a pregnant female with bladder pheochromocytoma.

We report the first known case of concurrent partial cystectomy and cesarean delivery in a pregnant female with bladder pheochromocytoma. A 28-year-old G4P2 female presented at 28 weeks gestation with labile blood pressures requiring three antihypertensive medications. Urinary catecholamines were elevated, and a subsequent MRI showed a 2.6 cm x 3.2 cm bladder wall mass. She underwent combined cesarian section and partial cystectomy at 37 weeks. Fluid resuscitation and vasopressors were required in the immediate postoperative period. While bladder pheochromocytoma with pregnancy is a rare occurrence, concurrent delivery and removal of the bladder tumor can be performed safely.

Sclerosing angiomatoid nodular transformation (SANT) of the spleen: a report of 3 cases.

The authors describe 3 cases of sclerosing angiomatoid nodular transformation (SANT) of the spleen diagnosed at Memorial Sloan-Kettering Cancer Center within a 1-year period (July 2008 to June 2009). All patients were female, older than 50, with lesions ranging in size from 2 to 4 cm. All were alive and well after splenectomy. All the cases showed characteristic histological and immunophenotypical findings as previously described in the literature, including scattered IgG4positive plasma cells in the fibrosclerotic stroma. Of the 3 patients, 2 had a history of carcinoma, and metastasis was of concern, but a PET scan in one of these patients showed minimal to absent FDG activity suggesting that this process was of a benign indolent nature. However, in 1 patient, a PET scan revealed positive FDG activity, heightening clinical concern for malignancy.

Synchronous follicular lymphoma, kaposi sarcoma, and castleman's disease in a HIV-negative patient with EBV and HHV-8 coinfection.

The authors describe the case of a 65-year-old woman who was HIV negative and had a lymph node biopsy that showed concurrent follicular lymphoma (FL; grade 3A), Kaposi sarcoma (KS), and Castleman's disease (CD) with coinfection by human herpes virus-8 (HHV-8) and Epstein-Barr virus (EBV). The lymphoma was positive for CD20, CD10, and BCL6 and negative for BCL2. Flow cytometry showed a clonal lambda B-cell population, and polymerase chain reaction (PCR) showed a clonal immunoglobulin heavy chain gene rearrangement, confirming a neoplastic B-cell process. Focally, the FL component showed numerous EBER1-positive cells, with rare HHV-8-positive cells. The KS component showed strong HHV-8 expression with rare EBER1-positive cells. The CD component showed scattered HHV-8, viral interleukin-6, and EBER1-positive cells. The simultaneous occurrence of a FL, KS, and CD in an HIV-negative patient expands the spectrum of HHV-8-positive neoplasms and suggests the possibility of HHV-8 rendering mature B-cells hyperresponsive to antigenic stimulation, providing an expanded target for second site mutations or cytokine-driven hyperplasia, culminating in lymphoma.

The role of cytotoxic and regulatory T cells in relapsed/refractory Hodgkin lymphoma.

Recent data suggests the presence of cytotoxic (TIA-1 and granzyme B+) and regulatory T-cells (FOXP3+) in classical Hodgkin lymphoma (cHL) tissues has been shown to correlate with poor overall survival in mainly diagnostic biopsies. By tissue microarray analyses, we extend this observation to a cohort of relapsed/refractory cHL tissue biopsies and analyze immunohistochemical expression of FOXP3, TIA-1, and granzyme B in the inflammatory background and the tumor microenvironment. High expression of TIA-1 (>50%) correlated with poor overall survival (P<0.0001), low expression of FOXP3 (<25%) correlated with poor overall survival (P<0.01), and combined high TIA-1 (>50%) and low FOXP3 (<25%) correlated with poor overall survival (P<0.0001). Expression of cytotoxic and regulatory T-cells shows prognostic significance in the relapsed/refractory clinical setting of cHL.

Nephrogenic systemic fibrosis: a pathologic study of autopsy cases.

CONTEXT: -Nephrogenic systemic fibrosis (NSF) is a rare but serious disorder initially described as a purely dermatologic process. Isolated autopsy reports have described multiorgan involvement by this disease. OBJECTIVE: -To further illustrate the varied and systemic involvement of NSF by describing the autopsy experience at the Massachusetts General Hospital. DESIGN: -We describe the findings in a series of 4 autopsy cases of patients diagnosed with NSF. This report describes the history of renal dysfunction, exposure to gadolinium-containing contrast agents, specific laboratory parameters, and the extent of systemic involvement identified by postmortem examination. RESULTS: -Causes of death included systemic thromboembolic disease (n = 3) and pneumonia (n = 1). Laboratory parameters and type, dose, or timing of gadolinium-containing contrast-agent exposure did not correlate with clinical findings and outcomes. All patients demonstrated cutaneous manifestations of the disease and nephrocalcinosis, with some exhibiting calcification and fibrosis of the dura, thyroid, and heart including the cardiac conduction system, on postmortem examination. Soft tissue calcification was associated with concurrent hyperparathyroidism or high serum parathyroid hormone levels. CONCLUSIONS: -Thromboembolic disease can be a significant clinical complication of NSF. Patients with NSF may also develop characteristic histologic features of fibrosis and calcification in multiple organs, with significant morbidity and mortality. This autopsy series highlights the variability of systemic manifestations of NSF.