Journal of Pathology and Translational Medicine

Composite Pheochromocytoma or Paraganglioma of Adrenal Gland: A Case Report with Immunohistochemical Studies and Electron Microscopic Examination.: Hyeyoon Chang, Hoiseon Jeong, Younghye Kim, Sung Hye Park, Aeree Kim; Korean J Pathol. 2011;45(3):306-310.; DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.3.306

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Composite pheochromocytoma or paraganglioma of the adrenal gland is a well-recognized, yet extremely rare tumor with only one case reported in Korea. We report a case of incidentally found composite pheochromocytoma and ganglioneuroma of the adrenal gland in a 44-year-old female composed of intermingled components of pheochromocytom, ganglioneuroma, and cells with intermediate features. On immunohistochemical staining, the pheochromocytoma component was positive for synaptophysin and chromogranin, but negative for S-100 protein. Staining for the S-100 protein revealed sustentacular cells which formed a peripheral coat around the "Zellballen" and Schwann cells. The Fontana-Masson stain defined neuromelanin granules of ganglion cells and the ganglion cells expressed neural markers such as neurofilament proteins. Ultrastructural findings revealed pheochromocytes with a round or ovoid nucleus and occasionally prominent nucleolus containing numerous adrenaline and noradrenaline granules.

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Bilateral pheochromocytoma with ganglioneuroma component associated with multiple neuroendocrine neoplasia type 2A: a case report
Boubacar Efared, Gabrielle Atsame-Ebang, Soufiane Tahirou, Khalid Mazaz, Nawal Hammas, Hinde El Fatemi, Laila Chbani
Journal of Medical Case Reports.2017;[Epub] CrossRef

Jugulotympanic Paraganglioma, Mimicking a Vascular Tumor: A Brief Case Report.: Ji Youn Sung, Chang Il Cha, Yong Koo Park; Korean J Pathol. 2010;44(5):543-546.; DOI: https://doi.org/10.4132/KoreanJPathol.2010.44.5.543

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Abstract PDF: Jugulotympanic paragangliomas (JTPs) known as glomus tumors, are neoplasms of variable invasiveness that arise from the paraganglia situated around the jugular bulb or middle ear. We now report a rare case of JTP in an 18-year-old male. Preoperative diagnoses through external auditory canal biopsy and radiologic examination both failed. Even using a frozen section, an informative finding was not obtained because mostly granulation tissue was present along with associated squeezing artifacts. On permanent histologic examination, small cell nests between many ectatic small vessels and fibrotic stroma were seen, and those cells were positive for CD56, synaptophysin and chromogranin. Because JTPs are rare and have rather different histologic findings - higher vascularity, smaller and less uniform tumor cells than other paragangliomas - they are easy to misdiagnose. However, remembering those differences may help the physician avoid missing JTPs.

First Report of a Gangliocytic Paraganglioma Arising in a Tailgut Cyst.: Yosep Chong, Mee Yon Cho; Korean J Pathol. 2010;44(4):435-440.; DOI: https://doi.org/10.4132/KoreanJPathol.2010.44.4.435

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Here we present the first report of a gangliocytic paraganglioma arising in a tailgut cyst; it occurred in a 56-year-old man. Tailgut cysts are uncommon congenital hamartomatous lesions that arise in the retrorectal presacral space in infants or adults. Benign or malignant tumors associated with tailgut cysts are rarely described; the most common tumors are adenocarcinomas and carcinoid tumors. A gangliocytic paraganglioma is a rare benign tumor that occurs nearly exclusively in the second portion of the duodenum. Rare cases have been reported at other locations, but a tailgut cyst has never been described. In our case, a resected 3.9 x 3.3 x 3 cm mass was composed predominantly of a solid yellow white neuroendocrine tumor within the area of a tailgut cyst. The neuroendocrine component of this tumor was different from previously described carcinoid tumors with respect to the histologic findings of neural differentiation as well as the intermixed typical gangliocytic features highlighted by immunohistochemical stains for S-100 protein and neurofilament. Although an intermixed area of the tailgut cyst and gangliocytic paraganglioma were found in some areas, the pathogenesis of this tumor remains to be elucidated.

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Diagnosis of Tailgut Cyst in Gynecologic Patients: Systematic Review of the Literature
Polina Schwarzman, Salvatore Andrea Mastrolia, Yael Sciaky-Tamir, Joel Baron, Boaz Sheizaf, Giuseppe Trojano, Reli Hershkovitz
Journal of Endometriosis and Pelvic Pain Disorders.2017; 9(3): 168. CrossRef
Fine Needle Aspiration Cytology Diagnosis of Tailgut Cyst: A Rare Entity
Farhan Asif Siddiqui, Rajan Chopra, Yusef Al-Marzooq
Acta Cytologica.2014; 58(2): 217. CrossRef

Paraganglioma of Cauda Equina.: Seok Jin Kang, Youn Soo Lee, Byung Kee Kim, Sang In Shim; Korean J Pathol. 1997;31(9):895-897.

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Abstract PDF: This case report describes a paraganglioma of the cauda equina in a 37-year-old man, as documented by light microscopy and immunohistochemistry. The patient experienced low back pain of 3 years duration, with the recent onset of sciatic pain and altered sensation in the right leg. Magnetic resonance imaging of L4 vertebral level revealed an ovoid, solid mass in the cauda equina. The mass was measured 1.5 cm in the greatest diameter. The histologic appearance was characterized by organoid pattern with clusters of chief cells (zellballen). Immunohistochemically, tumor cells are positive for keratin, epithelial membrane antigen, vimentin, neuron specific enolase and chromogranin.

Hyalinizing Trabecular Adenoma of the Thyroid: A case report.: Hyun ee Yim, Chull Shim, Euy Young Soh; Korean J Pathol. 1998;32(3):226-230.

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Abstract PDF: We report a case of hyalinizing trabecular adenoma of the thyroid gland with its immunohistochemical and ultrastructural features. A 53 year-old euthyroid woman presented a well defined small cold nodule on a thyroid iodine scan. Microscopically, oval and elongated tumor cells were arranged in trabeculae, clusters and a "zellballen" pattern resembling paraganglioma with scattered follicles. Nuclear features were characterized by fine nuclear grooves, acidophilic intranuclear cytoplasmic inclusions and perinucleolar halos. Abundant extracellular eosinophilic fibrohyaline matrix resembling amyloid were also noted. Immunostaining of tumor cells was positive for thyroglobulin and negative for calcitonin. In addition, tumor cells displayed an unexpected, unique cytoplasmic immunoreactivity for MIB1. Electron microscopy revealed euchromatic nuclei with grooves, intranuclear cytoplasmic inclusions, intermediate filament stuffed cytoplasms and abundant extracellular basal lamina material.

Pigmented Mediastinal Paraganglioma: A case report.: Seong Ho Kim, Yoon Hee Jin, Eun Kyung Hong, Moon Hyang Park; Korean J Pathol. 2000;34(8):597-600.

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Abstract PDF: Pigmented extraadrenal paraganglioma is an unusual neoplasm that has rarely been reported in the literature. Based on histochemical staining or electron microscopy, pigment has been classified as lipofuscin, neuromelanin or true melanin. We report a case of pigmented extraadrenal paraganglioma in the posterior mediastinum of a 70-year-old woman. Histologically, the tumor had a characteristic organoid architecture of "zellballen" pattern with rich delicate microvasculature. Tumor cells contained numerous coarse brown-black pigment granules. Ultrastructurally, the tumor showed abundant large electron-dense pigment granules that vary in size and shape and smaller membrane-bound neurosecretory granules. The larger granules were consistent with neuromelanin or lipofuscin. Histochemically, the pigment is most likely neuromelanin, which is a waste product of catecholamine metabolism.

Primary Spinal Oncocytic Paraganglioma.: Ji Hye Lee, Seong Hwan Park, Duk Hyun Cho, Bum Woo Yeom, Jong Sang Choi, Chul Hwan Kim, Yeon Lim Suh; Korean J Pathol. 2001;35(6):561-564.

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Abstract: Paraganglioma is a generic term applied to tumors of paraganglia, regardless of location, and composed largely of paraganglionic chief cells. It is a rare tumor, especially in the spinal region. When it appears in the craniospinal axis, it is restricted to the cauda equina or filum terminale, and less commonly, the spinal nerve root. We report a case of oncocytic paraganglioma in the spinal nerve root of 13-year-old girl. The tumor was located in intradural and extramedullary areas from the 12th thoracic to the 1st lumbar vertebra. Histologically, the tumor cells with abundant eosinophilic cytoplasms show diffuse compact clusters, which are surrounded by fibers in a reticulin stain, like a nested pattern. The nuclei are round to ovoid in shape with mild atypia. Immunohistochemically, the tumor cells are positive for synaptophysin, neuron-specific enolase and vimentin but are negative for cytokeratin, chromogranin and glial fibrillary acidic protein. Some cells are positive for S-100 protein. The MIB-1 labeling index is low. Ultrastructurally, dense core neurosecretory granules are not found but mitochondrias are commonly noted.

Fine Needle Aspiration Cytology of Retroperitoneal Paraganglioma with an Unusual Pattern: A Case Report.: Jean A Kim, Young Shin Kim, Chang Suk Kang, An Hi Lee, Byung Kee Kim, Sang In Shim, Sun Moo Kim; Korean J Cytopathol. 1994;5(1):74-78.

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Abstract PDF: A case of retroperitoneal paraganglioma is presented with fine needle aspiration cytologic features.

Paraganglioma of the Thyroid: A Case Report.: Jin Joo Baek, Youn Soo Lee, Chang Seok Kang, Sang In Sim, Kyo Young Lee; Korean J Pathol. 2008;42(6):401-404.

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Abstract PDF: Paragangliomas (PGs) of the thyroid are very rare tumors. Based on the medical literature, thyroid PGs invariably affected women and they were always thought to be benign, and they have always been mistaken for other more common lesions. We report here on the first case of PG of the thyroid in a 45-year-old man in Korea. Ultrasonographically, the tumor showed hypoechoic features. Microscopically, the tumor showed the typical zellballen pattern. The small to medium-sized tumor cells contained moderate amounts of finely granular eosinophilic cytoplasm and round to oval nuclei with fine chromatin. The tumor invaded the thyroid capsule and the extrathyroidal tissue with vascular and perineural invasion. Immunohistochemically, the tumor showed positivity for chromogranin, S-100 protein, CD56a, and synaptophysin and negativity for calcitonin, thyroglobulin, galectin-3, p53, CK19, and EMA. The Ki-67 labeling Index was 10%. We concluded that our case has a high potential of metastasis, and a close follow up would be important.

Gangliocytic Paraganglioma.: Joon Mee Kim, Suk Jin Choi, Lucia Kim, In Suh Park, Jee Young Han, Young Chae Chu, Hyung Gil Kim, Sun Geun Choi; Korean J Pathol. 2005;39(6):428-432.

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Abstract PDF: Gangliocytic paraganglioma (GP) is a rare benign tumor that is usually seen in the duodenum. It shows unique histologic features that are composed of a carcinoid or paraganglioma-like appearance, ganglion cells, and Schwann cells. The common presenting symptoms are abdominal pain, gastrointestinal bleeding and obstruction. The lesion can sometimes be asymptomatic and they are discovered incidentally. We experienced a case of incidentally found GP in a 73 year-old-man who had a colon cancer with liver metastasis. During the preoperative workup, a submucosal tumor was found in the duodenal papilla. The frozen diagnosis of the duodenal mass was GP, which was confirmed by the permanent sections and immunohistochemical staining. Pathologists should be alert to recognize and diagnose this rare, but benign disease, especially in the patient suffering with gastrointestinal cancer.

Paraganglioma of Cauda Equina: A case report.: Ji Hwa Kim, Sang Han Lee, Yoon Kyung Shon, Jyung Sik Kwak, Tae Joong Shon; Korean J Pathol. 1994;28(5):528-532.

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Abstract PDF: The clinical and pathological features of a paraganglioma arising in the cauda equina is described and compared with previous reports. The right microscopic fetures were similar to those of paragangliomas from other sites, with a 'Zellballen' pattern of cells containing arzyrophil granules. Immunohistocytochemical stains for neurone specific enolase, S-100 protein, cytokeratin were positive, but stains for glial fibrillary acidic protein were negative. Electron microscopy showed densely staining membrane-bound granules, cilia like structures and fibros bodies in the cytoplasm. The last two features only occur in paragangliomas from this site. The pathological findings suggest that paragangliomas in this site arise from pre-existing paraganglia, possibly of the visceral autonomic group.

Paraganglioma of the Urinary Bladder: A case report.: H K Lee, K M Lee, D K Chung; Korean J Pathol. 1988;22(2):164-168.

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Abstract PDF: We herein report a case of paraganglioma arising from the urinary bladder in a 67-year-old woman. Her chief complaint was gross hematuria. However, she didn't manifest any other catecholamine-associated symptoms such as hypertension, palpitation and micturitional attack. Grossly, the tumor revealed bilobular external appearance with foci of ulceration and hemorrhage. The cut surface showed pinkish yellow homogeneous soft tissue. Microscopically, the tumor was composed of polygonal cells with basophilic granular plump cytocopic study, numerous membrane-bound electron dense granules were noted in the cytoplasm.

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