Journal of Pathology and Translational Medicine

Case Reports

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

Polypoid Ganglioneuromatosis of Colon: A case report.: Jin Ja Park, Kyung Chan Choi, Young Hee Choi, Young Euy Park; Korean J Pathol. 1998;32(5):388-390.

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Abstract: Gastrointestinal ganglioneuromatosis is an extremely rare lesion which typically occurs with a significant systemic syndrome. It is known to be a major component of multiple endocrine neoplasia, type 2b. We presented a case of polypoid ganglioneuromatosis of the colon in a 3-year-old female with abdominal pain and diarrhea. She had no clinical evidence of the systemic syndrome or von Recklinghausen's neurofibromatosis, conditions in which intestinal ganglioneuromatosis can occur. Gross examination showed diffuse polypoid masses in ascending and transverse colons with normal-appearing mucosa. Microscopic examination revealed a proliferation of spindle-shaped neuronal cells containing multiple clusters of mature ganglion cells in the mucosa, submucosa and proper muscle. We describe a case of colonic ganglioneuromatosis without any component of multiple endocrine neoplasia or family history.

Isolated Polypoid Ganglioneuroma in the Rectum.: Se Hoon Kim, Chang Hwan Choi, Yong Han Paik, Won Ho Kim, Hoguen Kim; Korean J Pathol. 2001;35(4):344-346.

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Abstract PDF: Gastrointestinal ganglioneuroma is a rare benign neoplasm, composed of ganglion cells, nerve fibers, and supporting cells. Ganglioneuromas are presented as isolated polypoid ganglioneuroma, ganglioneuromatous polyposis, and diffuse ganglioneuromas. We have experienced a case of an isolated ganglioneuromatous polyp in the rectum. The patient was a 58-year-old female who had experienced low abdominal discomfort and tenesmus for 6 to 7 months. Colonoscopic examination revealed a polypoid tumor in the rectum. Microscopically, the tumor showed cystic glands, expanded lamina propria, and smooth surface epithelium. Many proliferated ganglion cells with nerve fibers were evident in the lamina propria which was extended to the submucosa.

Fine Needle Aspiration Cytology of Parapharyngeal Ganglioneuroma: A Case Report.: Ji Shin Lee, Hyang Jeong Cho, Ki Jung Yun, Hyung Bae Moon; Korean J Cytopathol. 1995;6(2):179-182.

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Abstract PDF: Ganglioneuromas are a fully differentiated tumor that contains no immature elements. The majority of ganglioneuromas are diagnosed in patients older than 10 years and are most often located in the posterior mediastinum, followed by the retroperitoneum. The location of these tumors in the parapharyngeal region is extremely uncommon and there are only a few reports on the cytologic appearance of the tumor. We report a case of ganglioneuroma presenting in a parapharyngeal location in a 4 year-old boy, diagnosed by fine needle aspiration cytology. The smears revealed scattered large oval to polygonal cells with voluminous, granular cytoplasms. The nuclei were one to two in number and had a prominent nucleolus. Clusters of benign spindie-shaped cells were also present.

Fine Needle Aspiration Cytology of Ganglioneuroma: A Case Report.: Hee Kyung Kim, Dong Wha Lee, So Young Jin; Korean J Cytopathol. 2003;14(1):12-16.

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Abstract PDF: Ganglioneuroma is a well-differentiated, benign tumor of the sympathetic nervous system. These tumors belong to a family of neoplasm that exhibit a wide range of differentiation, with neuroblastoma at one end and ganglioneuroma at the other. Because it share morphologic features with other both benign and malignant neural tumors, accurate preoperative diagnosis is often difficult. Nonetheless, it is critical for proper management. Fine needle aspiration cytology(FNAC) in the diagnosis of the ganglioneuroma has been a little documented. We describe a case of mediastinal ganglioneuroma in a 33-month-old girl. The diagnosis was suggested on FNAC and was confirmed by histopathologic examination later.

Diffuse Ganglioneuromatosis of Appendix associated with Von Recklinghausen;s Disease.: Young Su Kim, Sang Chul Nam, Man Hoon Han, Jeong Shik Kim, Ji Yun Jeong, Han Ik Bae; Korean J Pathol. 2007;41(5):334-336.

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Abstract PDF: Gastrointestinal ganglioneuromatosis is a rare neoplastic condition that can occur in association with von Recklinghausen's disease with multiple endocrine neoplasia type II B. The main locations are the ileum, colon, and appendix. We report a case of diffuse ganglioneuromatosis of the appendix associated with a mesenteric and ileocecal plexiform neurofibroma in von Recklinghausen's disease.

Original Article

Immunohistochemical Characteristics According to Histologic Differentiation and Flow Cytometric Analysis of DNA Ploidy in Neuroblastic Tumors.: Jai Hyang Go, Woo Hee Jung, Soon Hee Jung, Tai Seung Kim, Chanil Park; Korean J Pathol. 1995;29(1):52-60.

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Abstract PDF: Neuroblastoma, ganglioneuroblastoma and ganglioneuroma are derived from primordial neural crest cells and can be conceptualized as three different maturational manifestations of a common neoplasm. To assess the validity of immunohistochemistry and DNA Ploidy in the diagnosis of neuroblastic tumor in terms of prognostication, histologic and immunohistochemical evaluation with NB-84, neuron specific enolase(NSE) and S-100 protein and flow Cytometric DNA analysis were done on 21 neuroblastomas and 19 ganglioneuromas. Thirteen of 21 neuroblastomas were undifferentiated and 8 differentiating in type. Eleven of the 19 ganglioneuromas were mature in type and 8 had immature foci. Eighty one percent of neuroblastomas were positive for NB-84, 100% for NSE and 67% for S-100 protein, respectively. All ganglioneuromas were positive for NSE and S-100 protein, in contrast, only immature foci in ganglioneuroma were positive for NB-84. B-84 reacted positively with undifferentiated and differentiating neuroblasts including neuropil but not with mature ganglion cells. In contrast, NSE reacted positively with all components of neuroblastic tumor and S-100 protein mainly with cells of Schwannian differentiation. Three of eight(37.5%) differentiating neuroblastomas were strongly positive for NB-84 in contrast with seven of thirteen(53.8%) undifferentiated tumors, reflecting that undifferentiated cells tended to be positive for NB-84 in neuroblastoma. Twenty two percent of neuroblastoma showed diploidy and 78% aneuploidy including 11% of near-diploidy. Seven of eight(87.5%) differentiating neuroblastomas in contrast with seven of ten(70%) undifferentiated tumors showed aneuploidy. By contrast, 53% of ganglioneuroma showed diploidy and 47% aneuploidy with DNA index ranged from 1.12 to 1.19. Three of nine(33.3%) mature ganglioneuromas in contrast with five of eight(62.5%) ganglioneuromas with immature foci showed aneupolidy. Differentiating neuroblastoma tended to be aneuploid and ganglioneuroma with immature foci tended to be near-diploid. In conclusion, immunohistochemistry for NB-84, NSE and S-100 protein is useful for confirming neuronal, both neuronal and Schwannian, and Schwannian differentiation, respectively. Immunohistochemistry together with flow cytometric DNA analysis would be helpful to confirm the immature foci in ganglioneuroma.

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