| Abstract|| |
Teratomas are neoplasms comprising of tissues derived from more than one germ layer. Most common sites are gonads, sacrococcygeal, mediastinal, retroperitoneal, and pineal region. Here, we report a case of 2 -year-old previously healthy female toddlerwho presented with fever and right upper quadrant abdominal pain. Magnetic resonance imaging (MRI) of the abdomen revealed a right suprarenal nonenhancing cystic mass. Focal calcification was seen in the inferomedial aspect of the cyst. Complete surgical resection of the right adrenal gland was done. The patient had an uneventful postoperative course and was free of recurrence at 8 months follow-up. Primary retroperitoneal teratomas of the adrenal gland are very uncommon (4% of all primary teratomas) and can mimic a primary adrenal tumor, hence should be included in the differential diagnosis of adrenal masses.
Keywords: Adrenal, primary, retroperitoneal, teratomas
|How to cite this article:|
Narla SL, Jacob S, Kurian A, Parameswaran A. Primary mature cystic teratoma with carcinoid mimicking an adrenal tumor: Report of a rare association and review of literature. Indian J Pathol Microbiol 2016;59:200-2
|How to cite this URL:|
Narla SL, Jacob S, Kurian A, Parameswaran A. Primary mature cystic teratoma with carcinoid mimicking an adrenal tumor: Report of a rare association and review of literature. Indian J Pathol Microbiol [serial online] 2016 [cited 2020 Jul 9];59:200-2. Available from: http://www.ijpmonline.org/text.asp?2016/59/2/200/182012
| Introduction|| |
Teratomas are tumors derived from embryonal tissue composed of cell types derived from two or more germ layers (ectoderm, mesoderm or endoderm). Most common sites are gonads, sacrococcygeal, mediastinal, retroperitoneal, and pineal region. Primary teratomas in retroperitoneum are very rare, and primary adrenal teratomas are extremely rare accounting for only 4% of all primary teratomas., Most of these cases are asymptomatic or present with nonspecific symptoms. Surgical excision which is required for definite diagnosis by histopathological examination is the main line of treatment. Here, we report a case of primary adrenal mature teratoma with carcinoid mimicking a primary adrenal tumor in a toddler.
| Case Report|| |
A 2 -year-old previously healthy female toddler developed fever and right upper quadrant abdominal pain. Physical examination revealed a palpable nontender and limitedly movable mass in the right flank. All laboratory investigations are done including hemogram, chest X-ray, liver function test, renal function test, 24 h urine vanillylmandelic acid (VMA), and spot urine VMA were unremarkable. Magnetic resonance imaging abdomen revealed a right suprarenal nonenhancing cystic mass measuring 5.3 cm × 4.8 cm × 3.8 cm, pushing the inferior vena cava anteriorly and to the right [Figure 1]. There was no spinal cord/vertebral extension. Both ovaries and other visceral organs were unremarkable. Complete surgical resection of the right adrenal gland was done. Grossly, the mass measured 6 cm × 5 cm × 3 cm was well circumscribed and with smooth borders. Cut surface revealed predominantly cystic areas filled with thick yellowish white fluid. Focal solid yellowish white areas were also noted. Microscopic examination showed cystic spaces lined by stratified squamous epithelium with adnexal structures, smooth muscle and fibroadipose tissue beneath, along with mature neuroglial tissue, nerve bundles, ganglion cells, and focal choroid plexus like structures. Beneath the epithelium, solid areas showed an organoid or insular proliferation of cells separated by thin fibrovascular septae that showed strong positivity for Grimelius stain. Mitosis was sparse. Immunohistochemistry revealed diffuse and strong positivity for Synaptophysin, focal positivity for Chromogranin A and Cytokeratin in this area of organoid neoplasm. S100 was negative. Proliferation marker Ki-67 labeling index was <1% [Figure 2]. A diagnosis of primary mature cystic teratoma with carcinoid tumor was made (WHO grading system for neuroendocrine tumors 2010). Postoperative period was uneventful. 8 months postoperative period did not show any recurrence.
|Figure 1: Magnetic resonance imaging scan image showing a right suprarenal nonenhancing cystic mass pushing inferior vena cava anteriorly and to the right side|
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|Figure 2: Mature cystic teratoma showing squamous epithelium with adnexal structures (a, H and E ×40). Adipose tissue and smooth muscle bundles (b, H and E ×40). Choroid plexus like structures (c, H and E ×40). Cyst wall with an organoid proliferation of cells having salt and pepper chromatin (a-d, ×40). Organoid cells staining positive for Grimelius (e, ×100) immunohistochemical stains for Synaptophysin (f, ×100) cytokeratin (g, ×100), and Ki-67 (h, ×100)|
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| Discussion|| |
Teratomas are neoplasms comprising of tissues derived from more than one germ layer. During the 4th week of embryonic development, germ cells developing from yolk sac migrate to the midline of the fetus to become the future gonads. Those cells that do not migrate completely may differentiate into extragonadal teratomas., Based on the location, teratomas can be categorized into gonadal and extragonadal teratomas. Extragonadal ones are uncommon compared to gonadal ones and usually occur in the sacrococcygeal, mediastinal, retroperitoneal, and pineal region., Primary retroperitoneal teratomas of adrenal are extremely uncommon, accounting for 4% of all primary teratomas.,, Differential diagnosis of retroperitoneal teratomas includes ovarian tumors, renal cysts, adrenal tumors, Wilm's tumor, sarcomas, retroperitoneal fibromas, cystic neuroblastoma, congenital mesoblastic nephroma, hemangiomas, xanthogranuloma, enlarged lymph nodes, and perirenal masses. Differential diagnosis of adrenal neuroendocrine tumors with solid pattern includes medullary paraganglioma and pheochromocytoma. All of these derive from a neuroendocrine cell line and ultrastructurally contain neurosecretory granules. Lack of Zellbalen pattern, the presence of monotonous structure, diffuse strong staining with Grimelius, and negative staining for S100 eliminated the possibilities of paraganglioma and pheochromocytoma. Tumor cells showed positivity for two neuroendocrine markers-Synaptophysin and Chromogranin A.
Based on the degree of cellular differentiation, teratomas can be classified into mature and immature. Mature teratomas are benign, more common, may be cystic, solid, or mixed. 3–6% of benign teratomas can undergo malignant transformation. Cases of germ cell tumor, adenocarcinoma, sarcoma, squamous cell carcinoma, and neuroendocrine carcinoma have been reported within retroperitoneal teratomas. The stratified squamous component is the one that is at increased risk of undergoing malignant transformation. Retroperitoneal immature teratomas are predominantly solid, can be benign/malignant, and around 26% are malignant. Cases of germ cell tumor, adenocarcinoma, sarcoma, squamous cell carcinoma, and neuroendocrine carcinoma have been reported within retroperitoneal teratomas.
| Conclusion|| |
Primary retroperitoneal teratomas of the adrenal gland are very uncommon tumors (4% of all primary teratomas) and can mimic a primary adrenal tumor and hence should be included in the differential diagnosis of adrenal masses. Histopathological examination of the resected tumor is mandatory for diagnosis, and careful grossing of the solid areas is very important for detection of malignancies as prognosis differs. Complete surgical excision is the mainstay of treatment with an excellent an overall 5-year survival of nearly 100%.
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Conflicts of interest
There are no conflicts of interest.
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Swetha Lakshmi Narla
Department of Histopathology, Apollo Hospitals, Greams Lane, Off. Greams Road, Chennai - 600 006, Tamil Nadu
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2]