15 YEARS OF PARAGANGLIOMA: Pathology of pheochromocytoma and paraganglioma

Abstract

Pathologists using their routine diagnostic tools can contribute both to the care of patients with pheochromocytoma/paraganglioma and to understanding the pathobiology of the tumors. They can document details of tissue organization and cytology that are accessible only by microscopy and can characterize admixtures of cell types that are morphologically distinct or show differential expression of immunohistochemical markers. Current roles and challenges for pathologists include differential diagnosis, identifying clues to the presence of hereditary disease, and effective communication of pathology information for clinical and research purposes. Future roles will increasingly involve risk stratification, identification of actionable targets for personalized therapies, and aiding the interpretation of molecular tests by helping characterize genetic variants of unknown significance. It remains to be determined to what extent the need for pathology input will be overshadowed by the availability of genetic testing and other molecular analyses at ever-decreasing cost, together with very effective clinical paradigms for risk stratification and patient care.

Nasal Cavity Paraganglioma: Literature Review and Discussion of a Rare Case

“…The classic Zellballen pattern is shown in most paragangliomas, with cell nests separated by capillaries and sustentacular cells at its periphery [51]. This pattern was observed in our case, but an uncommon morphology was described with lipoblast-vacuolated cells instead of the typical pattern [23].…”

“…This pattern was observed in our case, but an uncommon morphology was described with lipoblast-vacuolated cells instead of the typical pattern [23]. Other variations in the tumor architecture include diffuse and trabecular growth patterns, extensive sclerosis that may mimic invasive carcinoma, and unusual vascular patterns that can mimic angiomas [51]. …”

“…Immunohistochemical staining of head and neck paragangliomas can be negative or focally positive for chromogranin A and tyrosine hydroxylase, and sometimes preferably express chromogranin B. The absence of tyrosine hydroxylase explains the usual nonfunctionality of these tumors [51]. Immunohistochemical analysis of our patient's specimen showed positivity only for synaptophysin, and was negative for S100 and chromogranin, which is a compatible pattern for head and neck paraganglioma.…”

Recurrent, bilateral, and metastatic pheochromocytoma in a young patient with Beckwith-Wiedemann syndrome: A genetic link?

“…Additionally, the histopathology of the PHEO/PGL of our patient had low-risk features, which is unusual in cases of metastases, occurring in less than 4% of cases. 1 This case suggests that the BWS in itself could predispose to formation of a PHEO/PGL phenotype with aggressive behaviour. The following manuscript summarizes the case report, reviews pertinent literature, and proposes a possible mechanism for this association.…”

“…Four percent of well-differentiated/low-risk tumours metastasize. 1 A lumbar puncture to evaluate for leptomeningeal spread was negative. Two years later, there is no evidence of residual disease biochemically or radiologically; the patient is asymptomatic and presumed in remission.…”