Immature mesenteric teratoma causing intestinal obstruction
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《美国医学杂志》
1 Department of Pediatric Surgery, Pt BDSharma PGIMS, Rohtak, Haryana, India
2 Department of Pathology,Pt BDSharma PGIMS, Rohtak, Haryana, India
3 Department of Radiodiagnosis, Pt BDSharma PGIMS, Rohtak, Haryana, India
Extragonadal intraperitoneal teratomas, especially those arising from mesentery and mesocolon, are very rare.[1],[2],[3],[4],[5],[6] We describe the case of a 7-year-old girl with abdominal mass leading to intestinal obstruction due to an immature teratoma arising from mesentery of jejunum.To the best of our knowledge only one case of immature mesenteric teratoma has been reported in the English literature earlier.
A seven year old female child presented with a history of abdominal pain for one month and bilious vomiting and constipation for 3 days. There was a painless, firm, globular lump of about 10x10cm size in the umbilical region extending on to the left iliac fossa. The lump was mobile side to side. X-ray of the abdomen showed multiple air -fluid levels and ultrasound revealed a huge mass of mixed echogenicity arising from pelvis. CT scan of the abdomen showed a large intraperitoneal mass with heterogenous enchancement occupying the lower abdomen and displacing the gut loops. Para aortic lymphadenopathy was also present [Figure - 1]. The tentative diagnosis of ovarian mass was made. However, at laparaotomy a large nodular mesenteric growth 15× 15 cm in size invading the jejuno-ileal region and compressing the jejunal lumen was found. Resection of growth alongwith adherent jejunoileal portion of gut was done and gastro-intestinal continuity was restored. Remaining viscera was found to be normal. Histopathological findings of immature epithelial component, mature cartilage, fat, intestinal glands alongwith areas of necrosis and hemorrhage were suggestive of immature teratoma of the mesentery [Figure - 2]. Tumour nodules were present on the gut surface but they were not infiltrating the gut wall.
The post operative recovery was uneventful. Chemotherapy was started in view of tumour infiltration upto serosa. However, soon after the first cycle of chemotherapy, the patient was lost to follow up
Most benign teratomas are composed of the mature cells. However, 20-25% of these also contain immature elements, mostly the neuroepithelium.[2] Most common site of teratorma is sacroccygeal region followed by gonads, mediastinum, central nervous system, retroperitoneum, neck and head etc.[2] Only few cases of extragonadal intrapperitoneal teratomas have been reported. These include gastric,[7],[8] hepatic, mesocolic, mesosigmoidal[6] and mesenteric,[1],[2],[3],[4],[5] teratomas. Most of the reported cases of mesenteric teratoma have been mature teratoma and presented as a cystic lesion in the mesentery. Their differential diagnosis included dermoid cyst, intestinal duplication cyst, ovarian, choledochal, pancreatic splenic or renal cysts, hydronephrosis, hydatid cyst or loculated ascites in different cases. In our patient the pre-operative investigations suggested on ovarian mass of mixed echogenicity, thus, hinting at the presence of some immature or malignant components while the final diagnosis was established at surgery and histopathology of the resected specimen.
In view of paucity of the reported cases of immature teratoma, no clearcut guidelines are available in the literature as to the treatment and follow up of the patients with the immature teratoma; though it is well established that the total excision is the optimal treatment for the benign lesions. It has been generally agreed that the amount of the neuroepithelium should guide the prognosis and treatment in these patients.[10] In our patient, however, we adopted a safer approach by giving chemotherapy (Vincristine, Adriamycin, Cyclophosphamide) despite an absence of the neuroepithelium since the tumour had infiltrated the intestinal serosa and micrometastasis could not be ruled out. Unfortunately, the patient was lost to follow up after the first chemotherapy cycle itself. Needless to say that alfafetoprotein monitoring constitutes the integral part of follow up of the patients of teratoma.
Intrabdominal teratomas generally present as abdominal masses and entail good prognosis if timely treatment is given. Mesenteric teratomas, though rare, must be kept in mind as one of the differential diagnosis in the intra-peritoneal abdominal masses in children.
References
1.Marcolongo A, Divirgilio G, Bettili G, Saverio Camoglio F, Fasoli L, Marradi L, Balter R.Immature mesenteric teratoma in a male newborn infant: prenatal ultrosonographic diagnosis and surgical treatment. Prenat Diagn 1997; 17(7) : 686-688.
2.Laberage JM Nguyen LT, Shaw KS. Teratomas, dermoids and other soft tissue tumours. In Aschcraft KW, ed. Pediatric Surgery . 3rd ed. Philadelphia : WB Saunders, 2000; 915-917.
3.Chiba T, Iwami D, Kikuuchi Y. Mesenteric teratoma in an 8 months old girl. J Pediatr Surg 1995; 30 : 120.
4.Prieto ML, Casanova A, Delgado J, Zabalza R. Cystic teratoma of the mesentery. Padiatr Radio 1989; 19 : 439. [PUBMED]
5.Nagashima K, Matsuyama S. A case of mature teratoma of the mesentery in a three year old girl. J Jpn Assoc Pediatr Surg 1977; 13 : 637-641.
6.Ratan SK, Ratan J, Kalra R. Large benign cystic teratoma of mesosigmoid causing intestinal obstruction: report of a case. Surgery Today 2002; 32(10) : 922-924.
7.Bhattacharjee PK, Ray D, Sarkar AN, Biswas PC, Dermoid Cyst of the mesentery in infant. J Ind Assoc Pediatr Surg 2005; 10: 254-255.
8.Ratan SK, Kulshrestha R. Immature gastric teratoma in an infant(letter). Ind Pediatr 1999; 6 : 847-849.
9.Rattan KN, Mathur SK, Marwah N, Purwar P, Rohilla S, Balasubramanium G. Gastroc Teratoma. Ind J Pediatr 2004; 71.
10.Ablin A,Isaacs H,Jr. Germ cell tumors. In Pizo PA,Poplack DG, eds. Principles and Practice of Pediatric Oncology , Chapter 33. London; Lippincott, 1989; 713-731.(Rattan Kamal Nain, Ratan Simmi K, Jhanwa)
2 Department of Pathology,Pt BDSharma PGIMS, Rohtak, Haryana, India
3 Department of Radiodiagnosis, Pt BDSharma PGIMS, Rohtak, Haryana, India
Extragonadal intraperitoneal teratomas, especially those arising from mesentery and mesocolon, are very rare.[1],[2],[3],[4],[5],[6] We describe the case of a 7-year-old girl with abdominal mass leading to intestinal obstruction due to an immature teratoma arising from mesentery of jejunum.To the best of our knowledge only one case of immature mesenteric teratoma has been reported in the English literature earlier.
A seven year old female child presented with a history of abdominal pain for one month and bilious vomiting and constipation for 3 days. There was a painless, firm, globular lump of about 10x10cm size in the umbilical region extending on to the left iliac fossa. The lump was mobile side to side. X-ray of the abdomen showed multiple air -fluid levels and ultrasound revealed a huge mass of mixed echogenicity arising from pelvis. CT scan of the abdomen showed a large intraperitoneal mass with heterogenous enchancement occupying the lower abdomen and displacing the gut loops. Para aortic lymphadenopathy was also present [Figure - 1]. The tentative diagnosis of ovarian mass was made. However, at laparaotomy a large nodular mesenteric growth 15× 15 cm in size invading the jejuno-ileal region and compressing the jejunal lumen was found. Resection of growth alongwith adherent jejunoileal portion of gut was done and gastro-intestinal continuity was restored. Remaining viscera was found to be normal. Histopathological findings of immature epithelial component, mature cartilage, fat, intestinal glands alongwith areas of necrosis and hemorrhage were suggestive of immature teratoma of the mesentery [Figure - 2]. Tumour nodules were present on the gut surface but they were not infiltrating the gut wall.
The post operative recovery was uneventful. Chemotherapy was started in view of tumour infiltration upto serosa. However, soon after the first cycle of chemotherapy, the patient was lost to follow up
Most benign teratomas are composed of the mature cells. However, 20-25% of these also contain immature elements, mostly the neuroepithelium.[2] Most common site of teratorma is sacroccygeal region followed by gonads, mediastinum, central nervous system, retroperitoneum, neck and head etc.[2] Only few cases of extragonadal intrapperitoneal teratomas have been reported. These include gastric,[7],[8] hepatic, mesocolic, mesosigmoidal[6] and mesenteric,[1],[2],[3],[4],[5] teratomas. Most of the reported cases of mesenteric teratoma have been mature teratoma and presented as a cystic lesion in the mesentery. Their differential diagnosis included dermoid cyst, intestinal duplication cyst, ovarian, choledochal, pancreatic splenic or renal cysts, hydronephrosis, hydatid cyst or loculated ascites in different cases. In our patient the pre-operative investigations suggested on ovarian mass of mixed echogenicity, thus, hinting at the presence of some immature or malignant components while the final diagnosis was established at surgery and histopathology of the resected specimen.
In view of paucity of the reported cases of immature teratoma, no clearcut guidelines are available in the literature as to the treatment and follow up of the patients with the immature teratoma; though it is well established that the total excision is the optimal treatment for the benign lesions. It has been generally agreed that the amount of the neuroepithelium should guide the prognosis and treatment in these patients.[10] In our patient, however, we adopted a safer approach by giving chemotherapy (Vincristine, Adriamycin, Cyclophosphamide) despite an absence of the neuroepithelium since the tumour had infiltrated the intestinal serosa and micrometastasis could not be ruled out. Unfortunately, the patient was lost to follow up after the first chemotherapy cycle itself. Needless to say that alfafetoprotein monitoring constitutes the integral part of follow up of the patients of teratoma.
Intrabdominal teratomas generally present as abdominal masses and entail good prognosis if timely treatment is given. Mesenteric teratomas, though rare, must be kept in mind as one of the differential diagnosis in the intra-peritoneal abdominal masses in children.
References
1.Marcolongo A, Divirgilio G, Bettili G, Saverio Camoglio F, Fasoli L, Marradi L, Balter R.Immature mesenteric teratoma in a male newborn infant: prenatal ultrosonographic diagnosis and surgical treatment. Prenat Diagn 1997; 17(7) : 686-688.
2.Laberage JM Nguyen LT, Shaw KS. Teratomas, dermoids and other soft tissue tumours. In Aschcraft KW, ed. Pediatric Surgery . 3rd ed. Philadelphia : WB Saunders, 2000; 915-917.
3.Chiba T, Iwami D, Kikuuchi Y. Mesenteric teratoma in an 8 months old girl. J Pediatr Surg 1995; 30 : 120.
4.Prieto ML, Casanova A, Delgado J, Zabalza R. Cystic teratoma of the mesentery. Padiatr Radio 1989; 19 : 439. [PUBMED]
5.Nagashima K, Matsuyama S. A case of mature teratoma of the mesentery in a three year old girl. J Jpn Assoc Pediatr Surg 1977; 13 : 637-641.
6.Ratan SK, Ratan J, Kalra R. Large benign cystic teratoma of mesosigmoid causing intestinal obstruction: report of a case. Surgery Today 2002; 32(10) : 922-924.
7.Bhattacharjee PK, Ray D, Sarkar AN, Biswas PC, Dermoid Cyst of the mesentery in infant. J Ind Assoc Pediatr Surg 2005; 10: 254-255.
8.Ratan SK, Kulshrestha R. Immature gastric teratoma in an infant(letter). Ind Pediatr 1999; 6 : 847-849.
9.Rattan KN, Mathur SK, Marwah N, Purwar P, Rohilla S, Balasubramanium G. Gastroc Teratoma. Ind J Pediatr 2004; 71.
10.Ablin A,Isaacs H,Jr. Germ cell tumors. In Pizo PA,Poplack DG, eds. Principles and Practice of Pediatric Oncology , Chapter 33. London; Lippincott, 1989; 713-731.(Rattan Kamal Nain, Ratan Simmi K, Jhanwa)