PubMed · 7408474
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C A Gundersen. 1980. Multiple sclerosis simulating pheochromocytoma.. https://pubmed.ncbi.nlm.nih.gov/7408474/
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OBJECTIVES: We undertook this study to establish criteria for adrenalectomy in patients with renal cell carcinoma. METHODS: We retrospectively reviewed the records of 162 patients undergoing radical nephrectomy from 1979 to 1993 at University Hospitals of Cleveland. Simultaneous ipsilateral adrenalectomy was performed in 57 patients (35%). RESULTS: Three of these 57 patients (5.3%) had ipsilateral adrenal metastases. All 3 patients had large, left-sided, upper-pole tumors that extended through the renal capsule (Stage T3a). All 3 patients with adrenal metastases had progression to disseminated disease, with an average time to progression of 7.2 months, whereas only 13 (24%) of the 54 patients without adrenal metastases developed metastatic disease (none to adrenal), with an average time to progression of 27.6 months. No patient with organ-confined disease (Stage T1 or T2) or extracapsular disease in the midkidney or lower pole had adrenal metastases identified histologically. CONCLUSIONS: The prognosis is poor for renal cell carcinoma with ipsilateral adrenal involvement, even with complete removal. Because of this poor prognosis, we believe that adrenal involvement should constitute a separate stage category. We propose that patients with ipsilateral adrenal metastases via direct extension should be classified as having pathologic Stage pT3d. If the patient has an ipsilateral adrenal metastasis not via direct extension, contralateral adrenal metastasis, or bilateral adrenal metastases, the pathologic stage should be M1. Ipsilateral adrenalectomy should only be performed if a lesion is seen preoperatively on computed tomographic scan or if gross disease is seen at the time of nephrectomy although its removal may not benefit the patient.
Extra-adrenal myelolipomas are rare, benign tumors composed of hematopoietic and adipose elements. Although these tumors can cause local symptoms or hemorrhage, they are generally asymptomatic. However, when discovered intraoperatively, they pose a diagnostic dilemma to the urologic surgeon. We present a case of perirenal extra-adrenal myelolipoma discovered intraoperatively in a patient with von Hippel-Lindau disease undergoing partial nephrectomy.
We report a case of composite pheochromocytoma/ganglioneuroma arising in a background of diffuse and nodular medullary hyperplasia in the adrenal gland of a 34-year-old man with multiple endocrine neoplasia 2a (MEN 2a). Cells were histologically classified as chromaffin or chromaffin-like (small typical-appearing pheochromocytoma cells), neuron-like (possessing ganglion cell morphology), and intermediate. We speculate that these cell types may represent a spectrum of differentiation of a neoplastic clone, with the intermediate cells representing a transitional stage between chromaffin cells and neurons. All three cell types in the composite tumor and all chromaffin cells in both nodular and nonnodular areas of the remaining medulla were strongly immunoreactive for tyrosine hydroxylase, the rate-limiting enzyme in catecholamine synthesis. In contrast, neuron-like cells (and to a variable extent intermediate cells) displayed selective loss of expression of phenylethanolamine-N-methyltransferase (PNMT), the enzyme that synthesizes epinephrine. Proliferative activity of the composite tumor and both the nodular and nonnodular medulla was studied by staining for the endogenous cell proliferation antigen Ki-67, using monoclonal antibody MIB-1. MIB-1 labeling was highest in Schwann cell areas of the composite tumor, followed by chromaffin-like cells in the composite tumor and in the separate nodules. Labeling was absent in neuron-like cells, consistent with the cells' postulated status as terminally differentiated derivatives of a chromaffin cell precursor, and was highly variable in nonnodular areas of the medulla. The latter observation suggests topographical variation in signals that drive chromaffin cell proliferation in MEN.