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Immature duodenal teratoma: Problems with occurrence of a common tumor at a rare site

1 Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India
2 Department of Surgical Gastroenterology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry, India

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Date of Submission11-Sep-2021
Date of Decision20-Nov-2021
Date of Acceptance10-Dec-2021
Date of Web Publication03-Feb-2023

How to cite this URL:
Muralidaran M, Arumugam P, Rekha JS, Potakkat B. Immature duodenal teratoma: Problems with occurrence of a common tumor at a rare site. Indian J Pathol Microbiol [Epub ahead of print] [cited 2023 Mar 27]. Available from:

Teratomas are non-seminomatous germ cell tumors (GCT) with a predilection to gonads. Extragonadal germ cell tumors represent 1–5% of all GCT.[1] Extragonadal teratomas occur commonly in the midline at the sacrococcygeal region, head and neck, retroperitoneum, mediastinum, brain, and spine, in descending order of frequency.[2] Gastrointestinal teratomas are rare and are seen predominantly in neonates and children and usually present as gastric teratomas.[3] Rare sites like the duodenum, liver, pancreas, lesser omentum, mesentery, and caecum have been documented.[1],[4],[5]

A 22-year-old male patient was self-referred to the surgical gastroenterology department of our institute with the diagnosis of duodenal carcinoma. He presented to a local hospital with pain in the right flank and melena for 2 weeks. At presentation, there was no history of jaundice, abdominal distension, nausea, or vomiting. The patient did not have any significant loss of weight or appetite. His routine hematological and biochemical parameters were within normal limits.

The upper gastrointestinal endoscopy showed a proliferative mass with surface ulceration and luminal narrowing in D3. The Computed tomography (CT) scan showed a large heterogeneously-enhancing lobulated predominantly exophytic mass arising from the duodenal serosa with a small luminal component in D3 and D4, measuring 12 cm × 7 cm × 5 cm. There was no evidence of liver or lung lesions and free fluid in the abdomen.

The patient underwent a biopsy at the local hospital and a preoperative histopathology report of poorly differentiated carcinoma was made. The patient was treated as a locally advanced duodenal carcinoma and received eight cycles of preoperative chemotherapy with two different regimens and did not respond. Staging laparotomy performed at our institute showed a large, firm mass arising from the D2-D3 junction with a large extramural component. The mass densely adhered to the inferior vena cava and aorta. Multiple paraaortic and interaortocaval nodes were present with extensive desmoplastic reaction. A segmental duodenal resection with interaortocaval lymph node dissection was performed and sent for histopathological examination.

The segmental duodenal excision specimen showed a nodular tumor on the serosal aspect of the duodenum measuring 12 cm × 9 cm × 4.5 cm and causing ulceration of the mucosa. On cut surface, the mass showed a solid cystic lesion with grayish white solid areas and multiple cystic areas filled with inspissated mucoid material [Figure 1]a. Additionally, there were pale yellow tiny nodular areas seen throughout the mass. The microscopic examination showed the tumor composed of tissues arising from all three germinal layers with a preponderance of ectodermal derivatives like stratified squamous epithelium with lamellated keratinous material. Mesodermal derivatives such as lobules of mature cartilage, smooth muscle, and endodermal derivatives such as respiratory and colonic epithelium were seen. In addition, there were neuroectodermal derivatives like immature neural tissue, comprising four low-power fields on one slide [Figure 1]b, [Figure 1]c, [Figure 1]d. A single focus of retinal pigment epithelium with melanin, choroid plexus like epithelium, and occasional foci of mature glial tissue with nerve bundles and ganglion cells were also noted. The separately sent interaortocaval node also showed an only tumor with ectodermal derivatives. On immunohistochemistry, the immature neural component showed patchy positivity for neuron-specific enolase (NSE). The serum markers showed elevation of the alpha fetoprotein. Beta Human Chorionic Gonadotrophin (HCG) was within normal limits. He is currently on chemotherapy with paclitaxel, ifosfamide, and cisplatin.
Figure 1: (a) Cut surface of the tumor shows multiple cysts filled with mucoid and pultaceous material. (b) Ectodermal derivatives like squamous epithelium, lamellated keratin, and mesodermal derivatives like cartilage (arrow); (c) endodermal derivatives like colonic epithelium with goblet cells; (d )immature neuroepithelial cells with a hyperchromatic nucleus and frequent mitosis highlighted with neuron-specific enolase (NSE—inset)

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There are three cases of duodenal teratomas reported in the literature so far.[4],[5],[6] Abdominal pain and intestinal obstruction were the most common symptoms at presentation. Histologically, all three were mature cystic teratomas with no immature elements, and underwent a curative excision, and were asymptomatic at follow-up.[4],[5],[6]

This is the first reported case of duodenal teratoma showing immature neural elements. Owing to the rare occurrence in Gastrointestinal Tract (GIT) and limited material in small biopsy chances of misdiagnosis are high. In our case, the poorly differentiated carcinoma diagnosed earlier was probably the undifferentiated immature component. This tumor comes as a surprise to the pathologist and clinician, if not suspected radiologically.

According to the study by Sun H et al.,[7] extragonadal immature teratomas comprise about 10–17% of all immature teratomas. Immature teratomas exhibited two peaks, one in less than 1 year of age and the other between 20 and 30 years. The percentage of immature teratomas with locoregional spread was seen more commonly in the males as compared to females. Our case also showed a young adult male in the second decade with a locally advanced immature teratoma.

The diagnosis and follow-up of extragonadal immature teratomas are crucial as they have locally advanced disease and poor prognosis. Extragonadal immature teratomas are treated with surgical resection with or without chemotherapy. They have a poorer prognosis as compared to the gonadal immature teratomas with variable responses to chemotherapy.[7]

The possibility of a germ cell tumor must be kept in mind when dealing with mass lesions of GIT in young adults. The pitfall occurring by sampling only the immature elements in a small biopsy may lead to an erroneous diagnosis of undifferentiated malignancy and unnecessary chemotherapy in such cases.

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There are no conflicts of interest.

   References Top

McKenney JK, Heerema-McKenney A, Rouse RV. Extragonadal germ cell tumors: A review with emphasis on pathologic features, clinical prognostic variables, and differential diagnostic considerations. Adv Anat Pathol 2007;14:69–92.  Back to cited text no. 1
Tapper D, Lack EE. Teratomas in Infancy and Childhood. A 54 year experience at the children's hospital medical center. Ann Surg 1983;198:398-410.  Back to cited text no. 2
Sharma A, Arora R, Gupta R, Dinda AK. Immature gastric teratoma in an infant: Report of a case and review of the literature. Indian J Pathol Microbiol 2010;53:868-70.  Back to cited text no. 3
[PUBMED]  [Full text]  
Joshi P, Parelkar S, Shetty S, Sanghvi B, Mundada D, Kapadnis S, et al. Mature duodenal teratoma in a neonate with exomphalos minor with intestinal obstruction: First case report. Eur J Pediatr Surg 2014;24:187–9.  Back to cited text no. 4
Puri A, Chauhan A, Bhalla S. Duodenal teratoma: A rare diagnostic and therapeutic challenge. J Indian Assoc Pediatr Surg 2019;24:313-4.  Back to cited text no. 5
[PUBMED]  [Full text]  
Chansoon T, Angkathunyakul N, Aroonroch R, Jirasiritham J. Duodenal mature teratoma causing partial intestinal obstruction: A first case report in an adult. World J Clin Cases 2020;8:1489–94.  Back to cited text no. 6
Sun H, Ding H, Wang J, Zhang E, Fang Y, Li Z, et al. The differences between gonadal and extragonadal malignant teratomas in both genders and the effects of chemotherapy. BMC Cancer 2019;19:408.  Back to cited text no. 7

Correspondence Address:
Jinkala Sree Rekha,
Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Puducherry – 605 006
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpm.ijpm_913_21


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