Paraganglioma: Cytomorphologic features, radiologic and clinical findings in 12 cases

Abstract

An accurate diagnosis of paraganglioma on FNA specimens is crucial for proper treatment. SDH status should be considered for all patients with paraganglioma as it may be important for patients' lifelong follow-up as well as for familial considerations. Paraganglioma is a rare entity with wide age and anatomic distribution and variable cytomorphological features that often overlap with those of malignant neoplasms. Possible aggressive behavior such as recurrence and metastasis to lymph nodes, bone, and lung as well as Succinate Dehydrogenase complex mutations warrant an accurate diagnosis on aspirated material for appropriate clinical management.

INSM1, a Novel Biomarker for Detection of Neuroendocrine Neoplasms: Cytopathologists’ View

“…Paraganglioma is often found in the neck area as a primary tumor or metastasis to the cervical lymph nodes. However, large cytological series of paraganglioma cases did not study INSM1 expression as NE marker in cytological material [ 33 ]. Thyroid paraganglioma is supposedly INSM1 positive, but no data is available in the sparse literature on this rare entity [ 34 ].…”

“…Pancreatic specimens may pose diagnostic challenges as well, due to nuclear expression of INSM1 in islet cells (pancreatic neuroendocrine cells) [ 25 ]. In addition, normal cortical adrenal cells and also a very small percentage of prostate cells are positive for INSM1 [ 25 , 26 , 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 ].…”

Thyroid paraganglioma: A diagnostic pitfall in thyroid FNA

“…However, cytologic features such as papillary fragments, nuclear grooves, psammoma bodies, and dense gum-like colloid would help support the diagnosis of papillary thyroid carcinoma. 24 On immunohistochemistry, papillary thyroid carcinoma would be positive for TTF-1, thyroglobulin, PAX-8, CK19, and HBME, whereas paraganglioma would be negative. 23 Of note, most thyroid paragangliomas may also be misdiagnosed on intraoperative frozen sections as follicular carcinoma, Hurthle cell carcinoma, MTC, metastatic carcinoma, or they may end up being deferred.…”

CytoJounal quiz case: Fine-needle aspiration of peripancreatic mass clinically mimicking a lymphoma

“…Cytologic diagnosis of extra-adrenal paraganglioma presenting as a peripancreatic mass is challenging with a high error rate due to its rarity. [1] Diagnosis on cytologic (FNA specimens) is usually challenging. To render the correct diagnosis, the pathologist should keep in mind the anatomic location and cytologic features reminiscent of an endocrine neoplasm.…”