Journal of Indian Association of Pediatric Surgeons
Journal of Indian Association of Pediatric Surgeons
                                                   Official journal of the Indian Association of Pediatric Surgeons                           
Year : 2020  |  Volume : 25  |  Issue : 2  |  Page : 128--129

Primary carcinoid tumor in a retroperitoneal teratoma in a child

Mahadevappa Kiran1, Rajalakshmi Tirumalae2, Attibele Mahadevaiah Shubha1, Kanishka Das3,  
1 Department of Paediatric Surgery, St. John's Medical College Hospital, St. John's National Academy of Health Sciences, Bengaluru, Karnataka, India
2 Department of Pathology, St. John's Medical College Hospital, St. John's National Academy of Health Sciences, Bengaluru, Karnataka, India
3 Department of Paediatric Surgery, AIIMS, Bhubaneswar, Odisha, India

Correspondence Address:
Dr. Kanishka Das
Department of Paediatric Surgery, All India Institute of Medical Sciences, Bhubaneswar - 751 019, Odisha

How to cite this article:
Kiran M, Tirumalae R, Shubha AM, Das K. Primary carcinoid tumor in a retroperitoneal teratoma in a child.J Indian Assoc Pediatr Surg 2020;25:128-129

How to cite this URL:
Kiran M, Tirumalae R, Shubha AM, Das K. Primary carcinoid tumor in a retroperitoneal teratoma in a child. J Indian Assoc Pediatr Surg [serial online] 2020 [cited 2020 Apr 10 ];25:128-129
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Full Text


A 2-year-old male child presented elsewhere with an increasing, painless abdominal lump since infancy. A laparotomy was abandoned due to the large size of the lump and involvement of major vessels. The mass had further increased in size; he was discomfited on a liquid diet and had lost weight. He was pale and normotensive. A firm, nontender, lobulated mass occupied the entire abdomen [Figure 1]a.{Figure 1}

Contrast-enhanced computed tomography showed a 13 cm × 8 cm × 13 cm, well-defined, heterogeneous, solid, cystic retroperitoneal mass with echogenic areas and focal calcification displacing the liver, right kidney/adrenal, and bowel. The inferior vena cava (IVC) was not visualized in its course near the mass [Figure 1]b. Routine laboratory investigations including serum alphafetoprotein (2.47 ng/ml) and β-human chorionic gonadotropin (0.5 mIU/ml) were normal. A provisional diagnosis of a mature retroperitoneal teratoma with caval compression/encasement was made.

At laparotomy, the tumor was circumscribed but densely adherent to the aorta, superior mesenteric artery, and left renal artery. The right renal vein was splayed, and the IVC (infrahepatic region to the right renal vein) was encased in a deep groove at the extreme right of the mass [Figure 1]c. An accidental division of the grossly displaced IVC was recognized which was repaired without any hemodynamic disturbances. The postoperative Doppler evaluation confirmed normal patency and flow.

The tumor was nodulocystic and bosselated. The cut surface showed heterogenous areas: cysts – mucoid, pultaceous, and hemorrhagic; calcification; solid whitish; and fatty. Microscopic examination showed features of a mature cystic teratoma (skin, adipose tissue, mature glial tissue, skeletal muscle, mucous glands, pancreatic tissue, mature cartilage, choroid plexus, and cysts with pseudostratified ciliated columnar epithelium) and a histological surprise – a focus of carcinoid tumor without atypia [Figure 1]d and [Figure 1]e. At 42-month follow-up, he is asymptomatic; the physical examination and imaging findings are normal.

Teratomas are uncommon pediatric germ cell tumors, with 3.5%–4% being retroperitoneal. Malignant transformation in a mature teratoma occurs in 1%–3% of cases, commonly into a squamous cell carcinoma (75%–87%). A carcinoid tumor is documented in only 6% of cases.[1],[2],[3] Primary carcinoid tumors are low-grade malignancies with neuroendocrine differentiation, commonly encountered in the gastrointestinal tract (85%) or bronchopulmonary tree (10%). In adults, most carcinoid tumors arise in gonadal teratomas from such cells. Only 10% secrete vasoactive peptides to manifest the carcinoid syndrome and increased urinary 5-hydroxyindoleacetic acid.[4] Isolated reports document their occurrence elsewhere – thymus, spinal cord, kidney, retroperitoneum, adrenal, and pelvis.[5] Their clinical behavior and prognosis is unclear, and it is recommended to evaluate for metastases and a cryptic primary site.[4]

In the present case, the carcinoid focus was incidentally detected during extensive histopathological evaluation. No sites of primary tumor or metastasis were evident. This is the youngest reported case with a retroperitoneal teratoma harboring a primary carcinoid tumor reported till date. Complete surgical excision and meticulous sampling from the solid parts of a teratoma characterizes rare tumoral components that impact on the prognosis. The lack of hemodynamic effects after accidental division of the IVC implies adequate retroperitoneal collateralization with the chronic occlusion.

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.


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