From the radiologic pathology archives: Adrenal tumors and tumor-like conditions in the adult: radiologic-pathologic correlation.

Advanced imaging often reveals adrenal tumors and tumor-like conditions in both symptomatic and asymptomatic patients. When adrenal disease is clinically suspected, cross-sectional imaging can be helpful in evaluating the etiology of the patient's symptoms. When adrenal disease is incidentally identified, what the clinician and patient really want to know is whether the findings are benign or malignant, as this ultimately will affect their next step in management. Using radiologic-pathologic correlation, we broadly classify common, uncommon, and rare tumors and tumor-like conditions that can occur in the adrenal as benign or malignant. This classification follows predominant trends in observed biologic behavior while acknowledging those tumors that may behave in the minority in an unpredictable manner. We review the clinical background and presentation of functional adrenal tumors including Conn syndrome, Cushing syndrome, and catecholamine-secreting tumors, as well as their relationship with adrenal anatomy. We discuss a variety of benign tumors, including adrenal cortical adenoma (including oncocytoma) and pheochromocytoma, as well as uncommonly and rarely encountered tumors such as myelolipoma, hemangioma, lymphangioma, schwannoma, ganglioneuroma, and adenomatoid tumor. A variety of tumefactive but nonneoplastic lesions are addressed, including adrenal cortical hyperplasia, adrenal hemorrhage, adrenal cysts, and infections. Malignant tumors discussed include adrenal cortical carcinoma, the rare malignant pheochromocytoma, lymphoma, metastases, and sarcomas. For each tumor and tumor-like lesion, the clinical presentation, epidemiology, key imaging findings, diagnostic differential considerations, and management options are briefly addressed. Finally, an approach to the workup of suspected or incidentally discovered tumors is presented based on a selected literature survey and our clinical experience. Radiologists play an important role in identification and diagnosis of adrenal tumors and tumor-like conditions in both symptomatic and asymptomatic patients.

The Reemergence of Measles.

OBJECTIVES: Present-day pathologists may be unfamiliar with the histopathologic features of measles, which is a reemerging disease. Awareness of these features may enable early diagnosis of measles in unsuspected cases, including those with an atypical presentation. Using archived tissue samples from historic patients, a unique source of histopathologic information about measles and other reemerging infectious diseases, we performed a comprehensive analysis of the histopathologic features of measles seen in commonly infected tissues during prodrome, active, and late phases of the disease. METHODS: Subspecialty pathologists analyzed H&E-stained slides of specimens from 89 patients accessioned from 1919 to 1998 and correlated the histopathologic findings with clinical data. RESULTS: Measles caused acute and chronic histopathologic changes, especially in the respiratory, lymphoid (including appendix and tonsils), and central nervous systems. Bacterial infections in lung and other organs contributed significantly to adverse outcomes, especially in immunocompromised patients. CONCLUSIONS: Certain histopathologic features, especially Warthin-Finkeldey cells and multinucleated giant cells without inclusions, allow pathologists to diagnose or suggest the diagnosis of measles in unsuspected cases.

Data set for the reporting of pheochromocytoma and paraganglioma: explanations and recommendations of the guidelines from the International Collaboration on Cancer Reporting.

BACKGROUND AND OBJECTIVES: The International Collaboration on Cancer Reporting (ICCR) is a not-for-profit to develop evidence-based, internationally agreed-upon standardized data sets for each anatomic site, to be used throughout the world. Providing global standardization of pathology tumor classification, staging, and other reporting elements will lead to improved patient management and enhanced epidemiological research. METHODS: Pheochromocytoma and paraganglioma are uncommon and are frequently overlooked in registry data sets. Malignant criteria have previously been defined only when there was metastatic disease. RESULTS: With recent recognition of a significant inheritance association and the development of risk stratification tools, this data set was created in order to obtain more meaningful outcomes and management data, using similar criteria across the global pathology community. Issues related to key core and non-core elements, especially clinical hormonal status, familial history, tumor focality, proliferative fraction, adverse or risk stratification features, and ancillary techniques, are discussed in the context of daily application to these types of specimens. CONCLUSIONS: The ICCR data set, developed by an international panel of endocrine organ specialists, establishes a pathology-standardized reporting guide for pheochromocytoma and paraganglioma.

Primary thyroid teratomas in children: a report of 11 cases with a proposal of criteria for their diagnosis.

Cervical teratomas are uncommon neoplasms, although the commonest neck tumors in newborns and infants. Presence of associated thyroid tissue often causes speculation as to the site of origin, ie, arising from within thyroid, adjacent soft tissue with secondary involvement of thyroid, or as innate part of a cervical teratoma. Twenty-eight cases of cervical teratomas were identified over 75 years, including 11 cases containing associated thyroid tissue. Clinical history, treatment, and follow-up were reviewed and the neoplasms analyzed regarding location, size, degree of maturity, and relative arrangement of thyroid and other tissues. All thyroid teratomas were congenital, measured 3.5 to 13.5 cm in diameter (median size, 6.9 cm), and were resected. Follow-up ranged from 1 to 45 years (median, 17 years) without recurrent disease in any patient. Neuroglial tissue predominated in 10 of 11 tumors. Intimate admixture of thyroid and other tissues with or without surrounding fibrous pseudocapsule was present in 8 cases, suggesting thyroid as origin. Histologic immaturity in congenital thyroid teratomas is not the harbinger of adverse behavior as seen in adolescents and adults. Intimate intermingling of thyroid tissue with teratoma and presence of a pseudocapsule seem to be the most significant criteria for establishing thyroid as origin.

Glomus tumor of the duodenum: a case report.

We describe a rare case of glomus tumor of the duodenum. The tumor was detected in a 46-year-old man being investigated for episodes of gastrointestinal bleeding. The excised specimen showed a 2.3 x 1.5 x 1.5 cm gray-tan tumor involving the mucosa and the submucosa. The overlying mucosa was focally ulcerated. Histologically, the tumor had a solid pattern of sharply demarcated, round glomus cells with prominent pericytoma-like vessels of varying sizes. The neoplastic cells stained for vimentin, smooth muscle actin, and focally for synaptophysin. These histologic and immunohistochemical features are consistent with a diagnosis of glomus tumor of the duodenum.

Retroperitoneal ganglioneuroma encasing the celiac and superior mesenteric arteries.

Ganglioneuroma is a rare neoplasm arising from the sympathoadrenal neuroendocrine system and has anatomic distribution paralleling the sympathetic chain ganglia and the adrenal medulla. In some cases, ganglioneuroma is the end stage maturation of less-differentiated neoplasms such as neuroblastoma or ganglioneuroblastoma, but based on age at diagnosis (over 10 years of age) and anatomic location, many of these tumors appear to arise de novo. It must be included in the differential diagnosis of posterior mediastinal and retroperitoneal mass. We report a case of retroperitoneal ganglioneuroma involving the celiac axis and superior mesenteric arteries in a 40-year-old female.

Adrenocortical neoplasms in childhood and adolescence: Analysis of prognostic factors including DNA content.

Thirty-two adrenocortical neoplasms in children and adolescents were evaluated for prognostic factors including clinical and morphological parameters and DNA ploidy. The patients were segregated into two groups according to clinical outcome: group A, represented by patients with clinically benign neoplasms (n = 15), and group B, patients with clinically malignant tumors as evidenced by local recurrence, metastases, or fatal outcome (n = 17). Clinical and morphological parameters in these two groups were evaluated using appropriate statistical methods. Parameters with a significant predictive value in terms of prognosis were age [p = .04], tumor size (p = .0003), median tumor weight (p = .0001), mitotic count (p = 0.04), and 25% tumor necrosis or more (p = .03). Twenty-three cases were studied for DNA ploidy: 10 cases by image analysis and 13 by both image analysis and flow cytometry. By ploidy analysis, 17 of 23 cases-12 of 14 in group A and 5 of 9 in group B-were found to be aneuploid. Multiple aneuploid peaks were found in 5 of 23 cases-4 of 14 cases in group A and 1 of 9 cases in group B. In tumors studied by both image analysis and flow cytometry, there was no discrepancy between results of ploidy analysis. There was no statistically significant association demonstrated between clinical outcome and DNA ploidy pattern. DNA ploidy heterogeneity, characterized by multiple aneuploid populations of cells, was also detected in both benign and malignant neoplasms. Based on our results, aneuploidy is relatively frequent in pediatrie adrenocortical tumors and does not appear to have predictive value for biological behavior.