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 Table of Contents  
Year : 2018  |  Volume : 23  |  Issue : 1  |  Page : 37-39

Retroperitoneal paraganglioma: A case report with review of literature

1 Department of Pathology, Jawaharlal Nehru Medical College, KLE Academy of Higher Education and Research (KAHER), Belgavi, Karnataka, India
2 Department of Surgery, JNMC, KLE University, Belgavi, Karnataka, India

Date of Web Publication3-Apr-2018

Correspondence Address:
Dr. Bhagyashri R Hungund
F-2/Nityadeep Comforts, Shivbasav Nagar, Belgavi, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jmgims.jmgims_3_16

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Retroperitoneal paragangliomas are rare neoplasms usually seen as histological surprises and can be confused with more common retroperitoneal neoplasms. The present case is reported to discuss the clinical presentation, radiological features, and histological features of a nonfunctioning retroperitoneal paraganglioma of unusual histology which presented as a diagnostic dilemma which was confirmed by immunohistochemistry.

Keywords: Diagnostic dilemma, immunohistochemistry, paraganglioma, retroperitoneal

How to cite this article:
Hungund BR, Pattanshetti VM, Pilli GS, Kangle R, Chavan RY. Retroperitoneal paraganglioma: A case report with review of literature. J Mahatma Gandhi Inst Med Sci 2018;23:37-9

How to cite this URL:
Hungund BR, Pattanshetti VM, Pilli GS, Kangle R, Chavan RY. Retroperitoneal paraganglioma: A case report with review of literature. J Mahatma Gandhi Inst Med Sci [serial online] 2018 [cited 2023 Mar 28];23:37-9. Available from: https://www.jmgims.co.in/text.asp?2018/23/1/37/229153

  Introduction Top

Paragangliomas are rare neuroendocrine neoplasms that are derived from the paraganglia, a diffuse neuroendocrine system dispersed from the skull base to the pelvic floor. They are found in many tissues such as the adrenal medulla, the carotid bodies, the organs of Zuckerkandl, and the paraganglia of the sympathetic and parasympathetic neurons. Paragangliomas usually occur in the head and neck region but are rare in the retroperitoneum.[1]

The exact incidence of extra-adrenal paragangliomas is not known, accounting for 10% of adult pheochromocytomas.[2] The diagnosis of retroperitoneal paraganglioma is infrequently made unless the tumor is functional. They are usually confused with the more common retroperitoneal tumors, and histological examination is needed for the diagnosis, especially when they are nonfunctional in nature. Microscopically, a cellular arrangement in close groups, large nucleus, and granular cytoplasm is characteristic of paraganglioma.[2] However, unusual histology, like pleomorphism of cells, poses diagnostic challenges, and immunohistochemistry (IHC) is needed for confirmation of diagnosis.

The present case is reported to describe the clinical presentation, radiological features, and histological features of a nonfunctioning retroperitoneal paraganglioma of unusual histology which presented as a diagnostic dilemma which was further confirmed by IHC.

  Case Report Top

A 60-year-old female presented with a history of vague right upper quadrant abdominal pain and pedal edema for 15 days which was sudden in onset and progressive in nature. The pain was nonradiating, dull aching and was not associated with aggravating or relieving factors. The patient had a history of hypertension, well controlled with medications since 10 years. General physical examination revealed mild pallor, blood pressure of 130/70 mmHg with a pulse rate of 80/min. Per abdominal examination revealed a mass in the right hypochondrium, firm in consistency, nonmobile, and nontender measuring 15 cm × 10 cm × 10 cm. Computed tomography (CT) scan [Figure 1] of abdomen revealed a mixed density mass with enhancing septae and calcification measuring 15.2 cm × 10.7 cm × 10.3 cm in the subhepatic region. Superiorly, the mass was extending up to the porta hepatis. The mass was seen displacing the head and uncinate process of pancreas medially, the ascending colon hepatic flexure and transverse colon inferiorly, and right kidney posteriorly. However, the fat planes are maintained. The CT anatomy of pancreas, stomach, kidneys, bowel loops, and genitourinary organs is normal. There was no evidence of thrombi in the inferior vena cava, portal or iliac vessels. Clinical diagnosis of gastrointestinal tumor with cystic degeneration was made based on CT findings.
Figure 1: Computed tomography scan of the abdomen depicting the retroperitoneal lesion showing cystic changes

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Exploratory laparotomy was done, and the mass was excised and sent for histopathological examination. Intraoperative and postoperative course was uneventful.

Gross examination of mass showed a well-encapsulated mass measuring 15.5 cm × 15 cm × 10 cm with smooth external surface and cut section showing solid and cystic areas with areas of hemorrhage and necrosis [Figure 2].
Figure 2: The gross appearance of the retroperitoneal mass. Cystic degeneration and areas of hemorrhage are evident

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Hematoxylin- and eosin-stained sections studied revealed cells with moderate-to-abundant amount of granular to vacuolated cytoplasm and ill-defined borders. These cells were arranged in sheets to ill-defined lobules separated by fibrovascular connective tissue. The nuclei were bizarre with pleomorphism and hyperchromatic appearance. Lymphocytic infiltrate was seen in the septa.

Differential diagnosis of paraganglioma, germ cell tumor, and liposarcoma was considered. Immunohistochemistry showed strong positivity for chromogranin and synaptophysin and negativity for vimentin, smooth muscle actin, desmin, pan-cytokeratin, and S100 which confirmed the diagnosis of paraganglioma [Figure 3].
Figure 3: The microscopy of the retroperitoneal mass: (a) H and E, ×100 depicting the lobular arrangement of cells with fibrous septae. (b) H and E, ×200, depicting the good number of blood vessels and abrupt nuclear atypia. (c) H and E, ×400, showing the nuclear atypia. (d) IHC stain showing strong positivity for chromogranin, ×200

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Patient was followed up for 1 year without any adjuvant therapy, and the patient did not show any evidence of any complications or recurrence.

  Discussion Top

Paragangliomas are rare tumors arising from the collection of neuroendocrine cells dispersed throughout the body called paraganglia. These paraganglia comprise chromaffin cells which secrete and store catecholamines.[3] Those arising in aggregates in the adrenal medulla are called pheochromocytomas. Grimley and Glenner, in 1969, divided the extra-adrenal paraganglia into anatomical groups as branchiomeric, intravagal, and aorticosympathetic.[4]

They may be found in skull base, neck, chest, and abdomen. Only 10%–20% of retroperitoneal paragangliomas are extra-adrenal. The reported incidence of extra-adrenal retroperitoneal paragangliomas in a case series is 2–8/million. Gallbladder, mesentery, prostate, and ovary are the other rare sites of paragangliomas reported in literature.[3] The extra-adrenal retroperitoneal commonly arise from the collection of paraganglia overlying the aorta at the level of inferior mesenteric artery, called organs of Zuckerkandl. They may occur sporadically or as a part of hereditary syndromes such as MEN-2 (Ret mutation), Von Hippel-Lindau disease mutations, paraganglioma/pheochromocytomas (SDHx mutations), and neurofibromatosis type I mutations.[5] Occasionally, these tumors are multiple, or they may be associated with paragangliomas of other sites or with other tumors such as gastrointestinal stromal tumors of the stomach and pulmonary chondromas as a component of Carney's triad.[4]

When presenting as retroperitoneal neoplasm within the abdominal cavity, they may mimic vascular malformations or conditions related to retroperitoneal organs such as pancreas, kidneys, or adrenals.[6],[7]

Although laboratory values of catecholamine are the most efficacious way of preoperative diagnosis of retroperitoneal mass detected radiologically, symptoms due to excess catecholamine secretion such as chronic or intermittent hypertension, palpitations, and headache are seen in 25%–60% of the cases. However, in nonfunctional tumors, the diagnosis becomes more difficult, and the diagnosis of retroperitoneal paraganglioma is often seen as a histological surprise [2],[8],[9] as is the scenario in the present case.

Although radiological investigations such as CT scan and MRI have been used as sensitive tools in diagnosis, histology and immunohistochemical analysis remain the mainstay for the final diagnosis of paraganglioma. Histologically, these tumors are composed of cells which are small polygonal or slightly spindly with an amphophilic or eosinophilic cytoplasm. The cells are arranged in short irregular anastomosing sheets around a delicate vasculature. The cell outlines are often indistinct, thus having a syncytial quality.[4] Rarely, retroperitoneal paragangliomas may have highly pleomorphic cells, spindly or angular, often lacking the organization of usual paraganglioma and can mimic other malignant tumors.[4],[10] The present case also had cells which showed marked pleomorphic hyperchromatic nuclei which created dilemma and thus other differential diagnoses such as liposarcoma and germ cell tumors were considered in the present case.

There are no histological criteria for distinguishing the benign from malignant paragangliomas. Metastasis and recurrence are the only criteria for malignancy. However, as it is a potentially malignant tumor, complete surgical excision is the treatment of choice.[4],[11]

  Conclusion Top

Clinically nonfunctional paragangliomas at rare sites and unusual histology can pose diagnostic challenges in the diagnosis of paragangliomas and IHC can help in the diagnosis of such cases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

He J, Wang X, Zheng W, Zhao Y. Retroperitoneal paraganglioma with metastasis to the abdominal vertebra: A case report. Diagn Pathol 2013;8:52.  Back to cited text no. 1
Goel N, Gupta N, Nag HH, Saran RK, Mishra PK. Nonfunctional retroperitoneal paraganglioma: A Histological surprise. World J Endocr Surg 2012;4:60-2.  Back to cited text no. 2
Jahangir N, Shirazi B. Paraganglioma: A diagnostic dilemma. J Pak Med Assoc 2010;60:789-91.  Back to cited text no. 3
Enzinger FM, Weiss SW. Paraganglioma. In: Soft Tissue Tumours. 3rd ed. St. Louis, Missouri, USA: Mosby; 1995. p. 965-90.  Back to cited text no. 4
Wen J, Li HZ, Ji ZG, Mao QZ, Shi BB, Yan WG, et al. Adecade of clinical experience with extra-adrenal paragangliomas of retroperitoneum: Report of 67 cases and a literature review. Urol Ann 2010;2:12-6.  Back to cited text no. 5
[PUBMED]  [Full text]  
Sangster G, Do D, Previgliano C, Li B, LaFrance D, Heldmann M, et al. Primary retroperitoneal paraganglioma simulating a pancreatic mass: A case report and review of the literature. HPB Surg 2010;2010:645728.  Back to cited text no. 6
Singhi AD, Hruban RH, Fabre M, Imura J, Schulick R, Wolfgang C, et al. Peripancreatic paraganglioma: A potential diagnostic challenge in cytopathology and surgical pathology. Am J Surg Pathol 2011;35:1498-504.  Back to cited text no. 7
Hayes WS, Davidson AJ, Grimley PM, Hartman DS. Extraadrenal retroperitoneal paraganglioma: Clinical, pathologic, and CT findings. AJR Am J Roentgenol 1990;155:1247-50.  Back to cited text no. 8
Noraziana AW, Hakim B, Alik R, Mokhtar A. An undiagnosed nonfunctioning rertroperitoneal paraganglioma complicating a gynaecological surgery; A case report. Int Med J 2010;9:59-61.  Back to cited text no. 9
Gupta P, Chakraborty S, Deb AR, Aich RK. Diagnostic enigma in retroperitoneal paraganglioma – A case report. J Indian Med Assoc 2012;110:184-6.  Back to cited text no. 10
Hasbi S, Khader AE, Fahssi ME, Kaoui HE, Sall I, Ali AA, et al. Retroperitoneal nonfunctioning paraganglioma. Can J Surg 2010;53:E3-4.  Back to cited text no. 11


  [Figure 1], [Figure 2], [Figure 3]


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