Gangliocytic paraganglioma arising from mature cystic teratoma of the ovary

doi:10.1016/S0090-8258(03)00272-5

Gynecologic Oncology

Volume 90, Issue 2, August 2003, Pages 482-485

https://doi.org/10.1016/S0090-8258(03)00272-5 Get rights and content

Abstract

Background

Gangliocytic paraganglioma is a rare neoplasm involving the small intestine, stomach, and spinal cord. Ovarian gangliocytic paraganglioma has not been reported in the medical literature.

Case

A 55-year-old caucasian woman underwent exploratory laparotomy, total abdominal hysterectomy, and bilateral salpingo-oophorectomy for evaluation of a persistent right adnexal mass. Microscopic examination of the right ovary revealed a mature cystic teratoma with a mural nodule consistent with gangliocytic paraganglioma. As there was no evidence of significant pleomorphism or neoplastic infiltration, surgical staging was not performed.

Conclusion

Gangliocytic paraganglioma may arise from ovarian cystic teratoma. Although most cases of gangliocytic paragangliomas are benign, surgical staging and retroperitoneal lymphadenectomy may be required if histopathology is suggestive of invasive disease or if enlarged lymph nodes are noted.

Introduction

The dispersed neuroendocrine system contains goups of neurosecretory cells scattered throughout the body. It may give rise to several neoplasms with both neuronal and neuroendocrine features such as ganglioneuroma, ganglioneuroblastoma, neuroblastoma, and paraganglioma [1]. Gangliocytic paragangliomas are rare neoplasms that are characterized by the presence of three different cells: (1) epithelioid cells with endocrine growth pattern, resembling paraganglioma or carcinoid tumor cells, (2) spindle cells and reminiscent of schwann cells, and (3) ganglion or ganglion-like cells. Gangliocytic paragangliomas have been reported in duodenum, jejunum, pylorus of stomach, and cauda equina region of spinal cord. These are nonsecretory tumors of benign or uncertain biologic nature and in a few instances metastasis to regional lymph nodes has been described [2]. We report a case of gangliocytic paraganglioma of the ovary which, to the best of our knowledge, is the first case at this particular site to be described in the medical literature (see Fig. 1, Fig. 2) .

Section snippets

Case report

A 55-year-old, gravida 5, para 5 postmenopausal caucasian woman was found to have a palpable right adnexal mass on routine gynecologic examination. She had been treated with combination hormone replacement for 5 years. Her past medical, gynecologic, and surgical histories were unremarkable except for a history of osteopenia, treated with intranasal calcitonin. Pelvic ultrasound demonstrated a normal-sized uterus, a 3.4-cm complex right adnexal mass with a solid component, and no free fluid in

Discussion

Gangliocytic paraganglioma is a rare lesion of the digestive tract that occurs most commonly in the second part of the duodenum [3]. Histologically, the tumor is a mixture of neuroendocrine cells, mature nerve elements, and supporting schwann cells. Immunohistochemical studies have demonstrated the presence of neuropeptides including somatostatin, serotonin, and human pancreatic polypeptide [3], [4]. Histologically similar tumors that arise from the cauda equina also contain peptide hormones.

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Incidental bilateral ovarian paraganglioma of uterine fibroid with widespread adenomyosis: A case report
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Case report of a paraganglioma arising from a mature cystic teratoma of the ovary
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Citation Excerpt :
Most are localized but about 15 percent can become metastatic (Erickson et al., 2001; Chen et al., 2010). Only two previous cases of paraganglioma within a mature cystic teratoma have been reported in the literature (Mahdavi et al., 2003; Elliot et al., 2012). In addition, McCluggage and Young reported a case series of probable primary ovarian paragangliomas but this is likely a separate entity (McCluggage and Young, 2006).
Paragangliomas are rare neuroendocrine neoplasms derived from sympathetic or parasympathetic paraganglia and have the ability to secrete catecholamines. We present the case of a 37-year-old asymptomatic female who underwent right ovarian cystectomy for a mature cystic teratoma and was found to have an intra-tumoral paraganglioma. More research is needed to determine metastatic potential as well as the likelihood of recurrence of these unique neoplasms.
Chapter 26 - Germ Cell Tumors of the Ovary
2017, Diagnostic Gynecologic and Obstetric Pathology
Germ cell tumors of the ovary are presumed to derive from the pathogenic transformation of ovarian germ cells at different stages of development. Germ cell tumors account for 20% to 25% of all ovarian neoplasms in females. The great majority are mature teratomas, which are benign. Only 3% to 5% of all germ cell tumors in females are malignant, of which the majority are dysgerminomas, immature teratomas, and yolk sac tumors in a pure form or as part of a mixed germ cell tumor, all occurring in young patients. Somatic-type malignant transformation in mature teratoma is rare and corresponds to an additional 1% to 2% of cases; it is encountered predominantly in older patients.
Endoscopic resection of a periampullary gangliocytic paraganglioma of the duodenum: A case report
2016, International Journal of Surgery Case Reports
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The tumour was removed and a GP was found. Moreover Mahdavi et al. published a case report of a 55-year-old Caucasian women suffering from a cystic teratoma with nodules consisting of GP [16]. Furthermore Sinkre et al. treated a 44-year-old woman with a GP in the nasopharynx.
The gangliocytic paraganglioma (GP) is an extremely rare neuroendocrine tumour originating from the second part of the duodenum. Generally GP shows benign clinical behaviour. The GP is typically characterized by consisting of three tumour components: the epithelioid, the spindle-shaped and the ganglion-like cells.
We present a female patient at the age of 65, who underwent a routine gastroscopy due to known gastro-oesophageal reflux. Accidentally a 2 × 1,5 cm sized mass of unknown entity was revealed in the duodenum. The magnet resonance tomography neither detected distant metastasis nor any local lymphadenopathy. After endosonographically guided punctures of the submucosal mass, a malignant tumour could not be diagnosed thus the decision to perform an endoscopical resection was made and successfully conducted. Immunohistochemical examination revealed a total resected GP.
In literature malignant transformation with distant metastasis and local recurrences has been described. Furthermore the clinical manifestation and location varies. The GP has often been misdiagnosed as a neuroendocrine tumour (NET) G1.
Due to published cases of metastasising GPs, we recommend a long term follow-up. In a non-metastatic stage the endoscopic resection should be the therapy of choice in order to prevent unnecessary major surgical interventions. In this case report we will discuss the clinical appearance, behaviour and differential diagnosis of GP.
Paragangliomas: Update on differential diagnostic considerations, composite tumors, and recent genetic developments
2013, Seminars in Diagnostic Pathology
Citation Excerpt :
Reported sites include skin, liver, gallbladder/biliary system, inferior vena cava, kidney, ureter, urethra, prostate gland, spermatic cord, vulva, vagina, uterus, ovary, CNS (intracerebral and intracerebellar), orbit, nasal cavity, paranasal sinuses, trachea, tongue, hypoglossal nerve, thyroid gland, and parathyroid gland.47–70 Two ovarian paragangliomas, one of which was gangliocytic, have been reported to arise in mature cystic teratoma.71,72 Given their extreme rarity and the fact that normal paraganglionic tissue has yet to be well characterized in some of these anatomic locations, histopathologists may understandably not consider PGLs or even misinterpret them as metastatic deposits, especially in the familial/syndromic setting, where multifocal PGLs are present.
Recent developments in molecular genetics have expanded the spectrum of disorders associated with pheochromocytomas (PCCs) and extra-adrenal paragangliomas (PGLs) and have increased the roles of pathologists in helping to guide patient care. At least 30% of these tumors are now known to be hereditary, and germline mutations of at least 10 genes are known to cause the tumors to develop. Genotype–phenotype correlations have been identified, including differences in tumor distribution, catecholamine production, and risk of metastasis, and types of tumors not previously associated with PCC/PGL are now considered in the spectrum of hereditary disease. Important new findings are that mutations of succinate dehydrogenase genes SDHA, SDHB, SDHC, SDHD, and SDHAF2 (collectively “SDHx”) are responsible for a large percentage of hereditary PCC/PGL and that SDHB mutations are strongly correlated with extra-adrenal tumor location, metastasis, and poor prognosis. Further, gastrointestinal stromal tumors and renal tumors are now associated with SDHx mutations. A PCC or PGL caused by any of the hereditary susceptibility genes can present as a solitary, apparently sporadic, tumor, and substantial numbers of patients presenting with apparently sporadic tumors harbor occult germline mutations of susceptibility genes. Current roles of pathologists are differential diagnosis of primary tumors and metastases, identification of clues to occult hereditary disease, and triaging of patients for optimal genetic testing by immunohistochemical staining of tumor tissue for the loss of SDHB and SDHA protein. Diagnostic pitfalls are posed by morphological variants of PCC/PGL, unusual anatomic sites of occurrence, and coexisting neuroendocrine tumors of other types in some hereditary syndromes. These pitfalls can be avoided by judicious use of appropriate immunohistochemical stains. Aside from loss of staining for SDHB, criteria for predicting risk of metastasis are still controversial, and “malignancy” is diagnosed only after metastases have occurred. All PCCs/PGLs are considered to pose some risk of metastasis, and long-term follow-up is advised.
Pancreatic gangliocytic paraganglioma with thick tortuous drainage veins
2009, European Journal of Radiology Extra
Citation Excerpt :
The CTA of the upper abdomen showed no sings of disease involvement in the liver, spleen and bilateral kidneys. Gangliocytic paraganglioma is a rare, benign lesion encountered most commonly in the second portion of the duodenum [1], with rare cases reported in the jejunum, pylorus [2], esophagus [3], nasopharynx [4], ovary [5], lung [6] and pancreas [7–9]. There are only three reported cases in the literature about the pancreatic gangliocytic paraganglioma.
We report herein the case of a 60-year-old Chinese woman found to have a rare pancreatic gangliocytic paraganglioma (PGP) with thick tortuous drainage veins. Gangliocytic paraganglioma is a rare, benign lesion encountered most commonly in the second portion of the duodenum. It is extremely rare in the pancreas and there are only three reported cases in the literature. Ours is the first case of PGP with thick tortuous drainage vein. Interestingly, preoperative data and images showed similar characteristics to pancreatic endocrine tumors. So it is difficult to establish a correct diagnosis of PGP preoperatively. However some imaging features of the mass that may be helpful in determining the diagnosis include a solid appearance with thick tortuous drainage veins, comparatively homogeneous contrast enhancement, and usually benign behavior. PGP sometimes shows some premalignant features, so radical resection is recommended to achieve curability with a good prognosis.

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Short communicationGangliocytic paraganglioma arising from mature cystic teratoma of the ovary

Abstract

Background

Case

Conclusion

Introduction

Section snippets

Case report

Discussion

Gangliocytic paraganglioma

Am J Clin Pathol

Duodenal gangliocytic paragangliomaclinicopathologic and immunocytochemical study of 11 cases

Am J Clin Pathol

Short communication
Gangliocytic paraganglioma arising from mature cystic teratoma of the ovary