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Neonatal colorectal spindle cell sarcoma
Neonatal Colorectal Spindle Cell Sarcoma By B.A. Madarikan, E.N. Thompson, and J. Lari Cardiff, Wales and South Glamorgan, Wales l Intestinal spindle cell sarcomas neonatal period. There have been cases. A neonate with colorectal reported. The clinical features of prognosis of the tumor are discussed. Copyright o 1991 by W.B. Saunders INDEX
WORDS:
Sarcoma,
occur very rarely in the 10 previous reported spindle cell sarcoma is the 11 cases and the Company
colorectal,
CASE
neonatal.
REPORT
A 2,225-g girl was born to a 31-year-old gravida 4 mother by emergency cesarean section at 38 weeks’ gestation. Apgar scores were 8 and 9 at 1 and 5 minutes, respectively. Postnatal examination showed a pale baby with a bilobar mass arising in the pelvis and extending to the visceral surface of the liver on the right side. Abdominal ultrasonagraphy performed on the second day of life showed normal kidneys and a mass lying anterior to the right kidney and crossing the midline. Her hemoglobin level was 12.2 g/dL. Intravenous urography confirmed the presence of normal kidneys with the right kidney being compressed by the mass. Serum c*-fetoprotein level was normal. Laparotomy performed on the 9th day of life showed a dumbbell tumor surrounding the sigmoid colon and upper rectum and extending from the pelvis and lower abdomen to the visceral surface of the liver. The tumor was resected in continuity with the sigmoid colon and upper rectum. An end colostomy was fashioned and the distal rectum was oversewn. Histologically, the tumor consisted of oval and spindle cells involving all the coats of the large bowel with serosal and submucosal spread. The tumour had a moderate to high mitotic rate but without apparent differentiation. It was classified as a spindle cell sarcoma. Postoperatively, the patient remained well and thrived. At the age of 7 months a further laparotomy showed no macroscopic or microscopic tumor and a colorectal anastomosis was performed. At the time of writing she is aged 2 years 2 months, is thriving, and is free of recurrence. DISCUSSION
The sites of the tumor in the 10 previously reported cases were 1 in the stomach, 4 in the small bowel, and 5 in the large bowel.‘-’ Of these 10 infants there were 5 boys, 4 girls, and the sex of the 10th infant was not reported. All 10 infants presented during the neonatal period (8 within the first week). Eight presented with an abdominal mass, 9 with signs of intestinal obstruction, and in 5 there was a perforation. From the Regional Centre for Paediatric Surgery, University Hospital of Wales, Cardifl and the Department of Child Health, Llandough Hospital, South Glamorgan, Wales. Address reprint requests to B.A. Madarikan, MD, Department of Surgery University College Hospital, Ibadan, Nigetia. Copyright o 1991 by WB. Saunders Company
0022-3468/9112612-0021$03.00/O 1416
The treatment in all cases was resection. Local recurrence was reported in two patients at 3 and 4 months after the initial laparotomy. However, the foci of recurrence were not palpable and were visible only at “second-look” laparotomies. Both patients were alive and well at the ages of 8 years 8 months and 6 years, respectively. The only death in the series occurred in the early postoperative period and was the result of anastomotic dehiscence. Postmortem examination confirmed that the tumor had been completely resected. The mean follow-up period in the 10 survivors (including the present patient) was 46 months (range, 3 to 156 months). The histological diagnosis in the majority of the previously reported cases has been leiomyosarcoma. In one of the other cases4a spindle cell sarcoma of the left transverse colon was resected at the age of 3 days. When colostomy closure was performed at the age of 4 months there was no visible macroscopic tumor. However, histological examination showed two small foci of residual tumor. Differentiation toward smooth muscle had occurred and the residual tumor could be classified as leiomyosarcoma. This would suggest a spectrum of differentiation between spindle cell sarcoma and leiomyosarcoma. These tumors can very rarely present in neonates of either sex. Adequate local resection appears to be curative. Even when recurrent (or probably residual) tumor is discovered at second-look laparotomy, the prognosis does not appear to be adversely affected. Radiotherapy and/or chemotherapy would appear to have no role in the management of this tumor. ACKNOWLEDGMENT The authors are greatly indebted to Dr Geraint Williams for reviewing the histology. REFERENCES 1. Roth D, Farinaci CJ: Jejunal leiomyosarcoma in a newborn. Cancer 3:1039-1043,195O 2. Kriss N: Leiomyosarcoma of the colon in an infant. AJR Am J Roentgen01 84:540-545,196O 3. El Shafie M, Spitz L, Ikeda S: Malignant tumours of the small bowel in neonates presenting with perforation. J Pediatr Surg 6:62-64,197l 4. Ein SH, Beck AR, Allen JE: Colon sarcoma in the newborn. J Pediatr Surg 14:455-457,1979 5. Sherman MP, Neustein HB: Congenital spindle-cell neoplasms of the intestine: A case report and literature review. Am J Pediatr Hematol Oncol7:380-384,1985 JournalofPediatric
Surgery,
Vol26,
No 12 (December),
1991: p 1416
WORDS:
Sarcoma,
occur very rarely in the 10 previous reported spindle cell sarcoma is the 11 cases and the Company
colorectal,
CASE
neonatal.
REPORT
A 2,225-g girl was born to a 31-year-old gravida 4 mother by emergency cesarean section at 38 weeks’ gestation. Apgar scores were 8 and 9 at 1 and 5 minutes, respectively. Postnatal examination showed a pale baby with a bilobar mass arising in the pelvis and extending to the visceral surface of the liver on the right side. Abdominal ultrasonagraphy performed on the second day of life showed normal kidneys and a mass lying anterior to the right kidney and crossing the midline. Her hemoglobin level was 12.2 g/dL. Intravenous urography confirmed the presence of normal kidneys with the right kidney being compressed by the mass. Serum c*-fetoprotein level was normal. Laparotomy performed on the 9th day of life showed a dumbbell tumor surrounding the sigmoid colon and upper rectum and extending from the pelvis and lower abdomen to the visceral surface of the liver. The tumor was resected in continuity with the sigmoid colon and upper rectum. An end colostomy was fashioned and the distal rectum was oversewn. Histologically, the tumor consisted of oval and spindle cells involving all the coats of the large bowel with serosal and submucosal spread. The tumour had a moderate to high mitotic rate but without apparent differentiation. It was classified as a spindle cell sarcoma. Postoperatively, the patient remained well and thrived. At the age of 7 months a further laparotomy showed no macroscopic or microscopic tumor and a colorectal anastomosis was performed. At the time of writing she is aged 2 years 2 months, is thriving, and is free of recurrence. DISCUSSION
The sites of the tumor in the 10 previously reported cases were 1 in the stomach, 4 in the small bowel, and 5 in the large bowel.‘-’ Of these 10 infants there were 5 boys, 4 girls, and the sex of the 10th infant was not reported. All 10 infants presented during the neonatal period (8 within the first week). Eight presented with an abdominal mass, 9 with signs of intestinal obstruction, and in 5 there was a perforation. From the Regional Centre for Paediatric Surgery, University Hospital of Wales, Cardifl and the Department of Child Health, Llandough Hospital, South Glamorgan, Wales. Address reprint requests to B.A. Madarikan, MD, Department of Surgery University College Hospital, Ibadan, Nigetia. Copyright o 1991 by WB. Saunders Company
0022-3468/9112612-0021$03.00/O 1416
The treatment in all cases was resection. Local recurrence was reported in two patients at 3 and 4 months after the initial laparotomy. However, the foci of recurrence were not palpable and were visible only at “second-look” laparotomies. Both patients were alive and well at the ages of 8 years 8 months and 6 years, respectively. The only death in the series occurred in the early postoperative period and was the result of anastomotic dehiscence. Postmortem examination confirmed that the tumor had been completely resected. The mean follow-up period in the 10 survivors (including the present patient) was 46 months (range, 3 to 156 months). The histological diagnosis in the majority of the previously reported cases has been leiomyosarcoma. In one of the other cases4a spindle cell sarcoma of the left transverse colon was resected at the age of 3 days. When colostomy closure was performed at the age of 4 months there was no visible macroscopic tumor. However, histological examination showed two small foci of residual tumor. Differentiation toward smooth muscle had occurred and the residual tumor could be classified as leiomyosarcoma. This would suggest a spectrum of differentiation between spindle cell sarcoma and leiomyosarcoma. These tumors can very rarely present in neonates of either sex. Adequate local resection appears to be curative. Even when recurrent (or probably residual) tumor is discovered at second-look laparotomy, the prognosis does not appear to be adversely affected. Radiotherapy and/or chemotherapy would appear to have no role in the management of this tumor. ACKNOWLEDGMENT The authors are greatly indebted to Dr Geraint Williams for reviewing the histology. REFERENCES 1. Roth D, Farinaci CJ: Jejunal leiomyosarcoma in a newborn. Cancer 3:1039-1043,195O 2. Kriss N: Leiomyosarcoma of the colon in an infant. AJR Am J Roentgen01 84:540-545,196O 3. El Shafie M, Spitz L, Ikeda S: Malignant tumours of the small bowel in neonates presenting with perforation. J Pediatr Surg 6:62-64,197l 4. Ein SH, Beck AR, Allen JE: Colon sarcoma in the newborn. J Pediatr Surg 14:455-457,1979 5. Sherman MP, Neustein HB: Congenital spindle-cell neoplasms of the intestine: A case report and literature review. Am J Pediatr Hematol Oncol7:380-384,1985 JournalofPediatric
Surgery,
Vol26,
No 12 (December),
1991: p 1416