Duodenal Teratoma: A Rare Diagnostic and Therapeutic Challenge.

Sir,

We read with interest the article titled “Teratoma in the hepatoduodenal ligament,” published in the November issue of this journal.[1] Recently, we managed a patient with duodenal teratoma which is yet another rare site for extragonadal teratoma and a close differential to that reported in this article.[1] It poses a diagnostic and therapeutic challenge due to its proximity to the ampulla of Vater, superior mesenteric vessels, and portal vein. A 2-year-old girl presented with 8 months history of severe anemia (hemoglobin: 6.8 mg %), generalized body swelling (anasarca), and incidentally detected upper abdominal mass. She had melena and had received multiple blood transfusions. On examination, she had 12 cm × 10 cm well-circumscribed, firm, mobile, nontender mass in the right hypochondrium encroaching on to adjoining abdominal quadrants. Abdominal ultrasound and contrast-enhanced computed tomography (CECT) revealed a complex bilobed solid-cystic mass in anterior pararenal space below the splenic portal confluence with multiple foci of adipose tissue and calcification [Figure 1a]. The mass (M) caused widening of the C-loop of the duodenum and anterosuperior displacement of the pancreas. There was no dilatation of intrahepatic biliary radicles, and serum alpha-fetoprotein was normal. On exploration, she had a large intraluminal mass in the duodenum involving the second, third, and fourth part of the duodenum extending into the duodenojejunal flexure [Figure 1b]. There was a clear plane of cleavage from the pancreas and inferior vena cava. The mass, however, was abutting the superior mesenteric vessels and a subadventitial dissection was done. Duodenum was divided just distal to the opening of the ampulla of Vater with a 1-cm tumor-free proximal margin and 3-cm distal to duodenojejunal flexure. A trans-anastomotic nasojejunal tube ensured early postoperative feeding. The mass was excised completely with no spillage. Histopathology report confirmed the diagnosis of subserosal mature duodenal teratoma comprising stratified squamous and respiratory epithelium with skin appendages, tooth analage, cartilage, and bone with no immature element or malignant transformation [Figure 1c and d]. At 10-months follow-up, the patient is asymptomatic with no evidence of recurrence on the postoperative ultrasound and CECT abdomen.

Figure 1

(a) Contrast-enhanced computed tomography of the abdomen axial view reveals a bilobed complex solid-cystic mass (M) containing adipose tissue and calcification in anterior pararenal space; (b) Operative view showing the intraluminal duodenal mass (M) with a clear plane of cleavage from the pancreas (P). The proximal and distal level of duodenal transection (d) are shown; (c and d) Light microscopy (×10) view of two sections from tumor showing intestinal mucosa, squamous and respiratory epithelium, glial tissue (G), adipose tissue, skin appendage, and lymphoid tissue (L)

Gastrointestinal (GIT) teratomas as such are uncommon and duodenal teratoma is a rarity with only a few reported cases.[2] Pediatric GIT teratomas mostly occur in the stomach, with a distinct preponderance for male infants and present with palpable lump and GIT bleeding.[2] Duodenal teratoma, just as teratoma of the hepatoduodenal ligament, is a rare differential for an upper abdominal mobile and midline solid-cystic right upper abdominal lump. It is usually associated with GIT bleeding and jaundice and may masquerade as choledochal cyst. The presence of a well-circumscribed complex mass having fat attenuation and calcification as reported herein, however, clinches the diagnosis of teratoma.[2] Another important differential for duodenal teratoma is GIT stromal tumor. They, however, have a median age of presentation in the fifth decade and have distinct spindle cell morphology with CD 117 positivity.[3] Complete surgical excision with clear margins, as done in this patient, is the mainstay of treatment for duodenal teratoma. This, however, may be difficult for tumors that are abutting ampulla of Vater and may require complex reconstructive procedures and at times even a pancreatoduodenectomy.

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Conflicts of interest

There are no conflicts of interest.