Extra-adrenal Mesenteric Pigmented Paraganglioma: A Rare Case Report
AbstractParagangliomas are rare neoplasms that arise from neural crest cells of the autonomous system. It has been estimated that 5% to 10% of paragangliomas occur in extra-adrenal sites, which can extend from the upper cervical region to the pelvis, parallel to the autonomic nervous system. This distribution corresponds to the embryologic development of the paraganglia from neural crest cells. Rarely, extra-adrenal paragangliomas can also occur outside this distribution. Herein, we present a case of a 70-year-old female with history of abdominal pain, in whom an abdominal mass was identified during ultrasonography. CT scan shows solid cystic mass arising from the right side of the pelvic cavity. Exploration laparotomy reveal well-circumscribed, encapsulated, ovoid and blackish mesenteric mass. After thorough microscopic and immunohistochemistry examination, the features were that of Pigmented Paraganglioma. This case report expands the morphologic spectrum of extra-adrenal paragangliomas and emphasizes the need to consider these tumors in the differential diagnosis of pigmented neoplasms.
Goldblum JR, Lamps LW, Mckenney JK, Myers JL. Adrenal Gland and Other Paraganglia. In: McKenney JK, editor. Rosai J Ackerman’s Surgical Pathology. Philadelphia: Elsevier 11th ed. 2018. p. 1209–12.
Mills SE, Greenson JK, Hornick JL, Longacre TA, Reuter VE. Paragangliomas. In Lack EE editor: Sternbergs Diagnostic Surgical Pathology. New york:Wolters Kluwer 6th ed. 2015.p.647-68
Rocco R, Murphy BL, Patel VP, et al. A rare case of a 65 year old female with a mesenteric paraganglioma. Hum Pathol Case Reports. 2020;19.
Moran CA, Albores-Saavedra J, Wenig BM et al. Pigmented extraadrenal paragangliomas. A clinicopathologic and immunohistochemical study of five cases. Cancer. 1997 Jan;79(2):398–402.
Dundr P, Dudorkinova D, Povysil C, et al. Pigmented Composite Paraganglioma-Ganglioneuroma of the Urinary Bladder. Pathol Res Pract. 2003;199(11):765–9.
Chetty R, Clark SP, Taylor DA. Pigmented pheochromocytomas of the adrenal medulla. Hum Pathol. 1993;24(4):420–3.
Reddy CEE, Panda NK, Vaiphei K, et al. Pigmented vagal paraganglioma. J Laryngol Otol. 2003;117(7):584–7.
Lloyd RV, Osamura RY, Kloppel G, Rosai J. Extra-adrenal paragangliomas. In Kmura N, Capella C, et al editors: WHO Classification of Tumours of Endocrine Organs. 4th ed. 2017. p. 190–5.
Jaffer S, Harpaz N. Mesenteric paraganglioma: a case report and review of the literature. Arch Pathol Lab Med. 2002 Mar;126(3):362–4.
Hayes WS, Davidson AJ, Grirnley PM, et al. Extraadrenal Paraganglioma : and CT Findings Retroperitoneal. Am J Roentgenol. 1990;155(6):1247–50.
Chetrit M, Dube P, Royal V, et al. Malignant paraganglioma of the mesentery: a case report and review of literature. World J Surg Oncol 2012;10(1):46.
Granger J, Mahapatra R, Hamid B, et al. Incidental mesenteric paraganglioma: A case report and literature review. Ann Coloproctol. 2017;33(5):197–200.
Young WF. Paragangliomas: Clinical overview. Ann N Y Acad Sci. 2006;1073:21–9.
Ntanasis-Stathopoulos I, Tsilimigras DI, Klapsinou E, et al. Challenging differential diagnosis of an extra-adrenal paraganglioma; the role of fine needle aspiration cytology. Diagn Cytopathol. 2017;45(6):565–8.
Zhao L, Luo J, Zhang H et al. Pigmented paraganglioma of the kidney: a case report. Diagn Pathol. 2012;7(1):3–7.
Iliesiu A, Ungureanu IA, Petca A, et al. Paraganglioma presenting as a mesenteric cystic mass : A case report. 2020;2489–92.
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
Authors who publish with this journal agree to the following terms:
- Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
- Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
- Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work (See The Effect of Open Access at http://opcit.eprints.org/oacitation-biblio.html).