|Year : 2015 | Volume
| Issue : 1 | Page : 106-107
Pathological aspects of a rare association of duodenal teratoma with exomphalos minor
Prachi S Chaudhari, Ratnaprabha K Ghodke, Pragati A Sathe, Bhuvaneshwari M Kandalkar
Department of Pathology, Seth G. S. Medical College and KEM Hospital, Parel, Mumbai, Maharashtra, India
|Date of Web Publication||8-Jan-2015|
Prachi S Chaudhari
203, Sterling Tower, Sector 21, Plot no 232, Kamothe, New Mumbai - 410 209
Source of Support: None, Conflict of Interest: None
Teratomas are neoplasms, which are foreign to the anatomic site of occurrence. They occur in children, most commonly in sacrococcygeal region and gonads and less frequently at other sites. Teratomas of gastrointestinal tract are very rare. We report pathological aspects of an unusual case of teratoma having communication with the duodenum with exomphalos minor and malrotation of the intestine.
Keywords: Duodenum, examphalos minor, intestinal obstruction, malrotation of intestine, neonate, teratoma
|How to cite this article:|
Chaudhari PS, Ghodke RK, Sathe PA, Kandalkar BM. Pathological aspects of a rare association of duodenal teratoma with exomphalos minor. Med J DY Patil Univ 2015;8:106-7
| Introduction|| |
Teratomas are neoplasms arising from pluripotent cells showing mixture of tissues of tridermal or bidermal origin, foreign to the anatomic site of occurrence.  Teratomas are commonest in the sacrococcygeal area and the gonads. Less frequent locations include the neck, mediastinum, retroperitoneum, cranial cavity, nasopharynx, and upper jaw.  Gastrointestinal tract is a rare site.  We report an unusual case of a duodenal teratoma communicating with its lumen and associated with exomphalos minor and malrotation of the intestine.
| Case Report|| |
A seven-day-old female child came with complaints of bilious vomiting following feeds since day three of life. In view of oligohydramnios, the child was delivered at 32 weeks of gestation, by lower uterine segment cesarean section. Birth weight was 2.2 kilograms. Significant obstetric history of the mother mentioned was a previous intrauterine fetal death at seven months of gestation, details of which were not available.
General examination of the baby revealed an exomphalos minor. Roentgenograms and ultrasonography of the abdomen were suggestive of intestinal obstruction, with multiple air fluid levels, at the level of exomphalos. Hematological and biochemical investigations were within normal limits. Emergency laparotomy was performed to relieve intestinal obstruction.
The surgeons reported a plethora of findings intraoperatively. The exomphalos sac showed a portion of malrotated small bowel and appendix. Also, a Meckel's diverticulum was found 50 cm proximal to ileocecal junction. Additionally, a mesenteric cyst measuring one centimeter (cm) in diameter was seen at jejunum. A hard cystic mass measuring 3 × 2 cm was seen attached to the duodenum, which on probing was found to communicate with duodenum. Clinical impression of this lesion was an intestinal duplication cyst. Pancreas was free from the lesion. Appendicectomy, mesenteric cyst excision, and excision of the duplication cyst were performed.
At histopathology, the excised duplication cyst was a solid cystic mass measuring 3 × 2 cm. The solid areas were composed of fat, few strands of hair, and hard glistening white tissue resembling cartilage. Cystic portion was irregular and showed greenish secretions resembling bile [Figure 1]a. The mesenteric cyst was a small cyst measuring 1 cm in diameter containing thick inspissated mucin. Microscopy from the duodenal mass showed a tumor composed of multiple tissues namely, stratified keratinized squamous epithelium with hair follicles, sebaceous glands, mature cartilage, lymphoid follicle, adipose tissue, fibrous tissue, and pancreatic tissue [Figure 1]b-f. All tissues were mature. Diagnosis of mature teratoma of the duodenum was given. Microscopic examination of the mesenteric cyst revealed features of a foregut cyst. Appendicectomy specimen showed normal histology.
|Figure 1: Gross and Microscopic features of duodenal cystic mass. (a) Gross photograph showing solid cystic mass with yellowish areas of fat with embedded glistening white cartilage. (b) Microscopic photo showing squamous epithelium with keratin flakes and adipose tissue. Hematoxylin and eosin stain (H and E) 100x. (c) Areas showing mature cartilage, fat and glands. (H and E) 100x. (d) Stratifi ed squamous epithelium with pancreatic tissue in the periphery. (H and E) 100x. (e) Area showing mature lymphoid tissue. (H and E) 100x. (f) Pancreatic tissue. (H and E) 400x|
Click here to view
After diagnosis of teratoma, serum tumor markers were done. Serum alfa-fetoprotein levels were 1151.45 ng/ml, β human chorionic gonadotropin was less than 1.20 units, and serum lactate dehydrogenase was 555 units/ liter; normal for the age of the child. Subsequently, antibiotic therapy and supportive care was given, and child was discharged on 12 th post-operative day. Child is fine 18 months post-surgery.
| Discussion|| |
Gastrointestinal teratomas are rare, with female predilection and hindgut being the common site.  However, the exact histogenesis is not yet clearly known. It is postulated that, during embryogenesis, rests of totipotential germ cells become sequestered along the path of migration from endoderm of yolk sac to gonads via dorsal mesentery of the hindgut, which explains the common occurrence of teratomas in gonads, presacral regions, and distal gastrointestinal tract.  Foregut teratomas are believed to originate from migrated totipotent germ cells. 
Teratomas occurring in the sacrococcyx and gonads cause local developmental defects, which lead to various anomalies. 
Mature cystic teratomas of gastrointestinal tract are generally benign with excellent prognosis, and complete excision is the treatment of choice.  Majority of the teratomas can be diagnosed pre-operatively. Pre-operative diagnosis of teratoma was not suspected in our case, probably due to rare location. Also, the presence of air-fluid levels possibly obscured the radiologic image of a teratoma.
The highlights of the present case are, a teratoma in a female child arising in the wall of duodenum and its unusual association with malrotation and exomphalos. Communication with the duodenal lumen was suggested by presence of bile. Patient also had Meckel's diverticulum and foregut cyst. These associated anomalies in gastrointestinal tract may be due to local developmental defect caused by the teratoma. In spite of so many associated conditions, our patient was operated successfully for the teratoma as well as the anomalies.
Teratoma arising in the duodenal wall communicating with the lumen along with Meckel's diverticulum and foregut cyst, and associated with malrotated small intestine and exomphalos, is a very rare association. 
| Conclusion|| |
Teratoma should be included in the differential diagnosis of cystic lesions of the gastrointestinal tract, even when it communicates with the intestinal lumen and more so when there are associated developmental anomalies.
| References|| |
Al-Arfaj AA, El-Shawarby MA, Al-Mulhim FA, Lardhi AA. Mesenteric cystic teratoma in children. Saudi Med J 2003;24:1388-90.
Schuetz MJ 3 rd
, Elsheikh TM. Dermoid cyst (mature cystic teratoma) of the cecum. Histologic and cytologic features with review of the literature. Arch Pathol Lab Med 2002;126:97-9.
Joo M, Kang YK, Lee HK, Lee HS, Yum HK, Bang SW, et al
. Intrapulmonary and gastric teratoma: Report of two cases. J Korean Med Sci 1999;14:330-4.
Berry CL, Keeling J, Hilton C. Coincidence of congenital malformation and embryonic tumours of childhood. Arch Dis Child 1970;45:229-31.
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 2013 Jan 4. [Epub ahead of print]