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Pancreatoduodenectomy for neonatal myofibromatosis of the pancreas
Pancreatoduodenectomy for Neonatal Myofibromatosis of the Pancreas By Stephen
E. Morrow,
Gerald
M. Woods,
Kansas
Robert
E. Garola,
and
Ronald
J. Sharp
City, Missouri
Myofibromatosis is a rare congenital disorder consisting of one or more fibrous nodules in the skin, soft tissues, bones, and internal organs. The authors report the unique case of a newborn who presented with obstructive jaundice caused by a single myofibroma in the head of the pancreas that was treated successfully by pancreatoduodenectomy on the eighth
day
0
decision was made to follow the infant’s progress with laboratory monitoring and CT imaging. Although glucose levels have been normal throughout the postoperative course without the need for insulin, the baby did require pancreatic enzyme supplementation. The enzymes were gradually withdrawn and recently discontinued without ill effect. The baby remains clinically well with normal weight gain 10 months after resection.
BSTRUCTIVE JAUNDICE in the newborn is rarely caused by a pancreatic neoplasm. We report the unique case of a pancreatic myofibroma successfully treated by primary pancreatoduodenectomy in an %dayold infant. CASE
ofPediatric
Surgery,
Vol34,
INDEX WORDS: denectomy.
Copyright
Myofibromatosis,
o 1999 by W.B. Saun-
pancreas,
pancreatoduo-
REPORT
A 39.week-gestation white boy was born to a healthy mother after an uncomplicated pregnancy. The baby became jaundiced on the second day of life (total bilirubin, 9.4 mg/dL; unconjugated, 7.4). Coomb’s test, complete blood count (CBC), and routine screening test results were normal. There was no family history of hepatobiliary disorders. On the fifth day of life, the bilirubin level had risen to 13.8, with a direct fraction of 8.9. Physical examination was otherwise unremarkable. Serum electrolytes and CBC findings were normal. Additional blood tests included albumin, 3.5 g/dL; amylase, <5 U/L; AST, 46 IUIL; ALT, 32 ILK: alkaline phosphatase, 308 U/L; and GGT, 1,329 U/L. An ultrasound scan demonstrated a solid mass in the head of the pancreas without definite vascularity. The mass displaced the superior mesenteric artery and portal vein and obstructed the common bile duct, which measured 4 mm in diameter. The liver, intrahepatic ducts, and remaining abdominal organs were unremarkable. A computed tomography (CT) scan of the chest, abdomen, and pelvis confirmed these fmdings and demonstrated duodenal and caval compression. No other abnormalities were noted. During laparotomy, a tirm mass in the head of the pancreas with several peripancreatic nodules was found. Frozen-section analysis was interpreted as neoplastic tissue consisting of nests of pleomorphic cells growing in a well-differentiated stroma. A pancreatoduodenectomy was performed and reconstruction was accomplished with end-to-end pancreatoduodenostomy, end-to-side choledochoduodenostomy, and end-toend Roux-en-Y gastrojejunostomy. Laboratory examination of the specimen showed a 1.9-cm yellowbrown tumor with irregular borders and a lobulated, focally necrotic surface (Fig 1). The mass infiltrated the muscularis propria of the duodenal wall but spared the mucosa. Microscopic examination demonstrated interlacing fascicles of spindle cells, with a central vascular pattern similar to a hemangiopericytoma (Fig 2). Few mitotic figures were identified, and normal pancreatic acinar and endocrine cells were seen. The majority of cells expressed vimentin. with focal actin and rare desmin expression. A diagnosis of infantile myofibromatosis was made, which also was found m the peripancreatic lymph nodes. The postoperative course was unremarkable. A skeletal survey did not demonstrate any additional foci of disease. After multidisciplinary review, the Journal
of life.
J Pediatr Surg 34:609-611. ders Company.
No 4 (April),
1999: pp 609-611
DISCUSSION
First described in 1954 by Stout,’ infantile myofibromatosis is a rare disorder distinguished histologically by the presence of interlacing fascicles of spindle cells with central vascular areas.2 Other names for myofibromatosis include congenital mesenchymal tumor, congenital generalized myfibromatosis, generalized hamartomatosis, congenital multiple fibromatosis, and multiple vascular leiomyomas of the newborn.3-6 The entity must be distinguished from similar lesions such as fibromatosis, desmoid tumors, hemangiopericytoma, and low-grade fibrosarcoma. Some investigators believe the myofibromatosis consists of a spectrum of spindle cell tumors with varying degrees of fibroblastic, myofibroblastic, and leiomyomatous features4.’ After vascular tumors, fibromatoses are the most common neonatal tumors8 and occur with a male to female ratio of 1.6: 1.9 Although the etiology is unclear, the lesion is believed to originate from the myofibroblast.4 Although most cases appear sporadic, there is evidence for both autosomal dominant and autosomal recessive inheritance.2~10,11Apoptosis may explain cases of spontaneous regression. l2 Associated anomalies occasionally have been reported, as in a case of gingival
From the Departments of Surgery Oncology, and Pathology, The Children’s Mercy Hospital and the Unrversity of Missouri-Kansas City School of Medicme, 24th and Gillham Rd, Kansas Cizy, MO 64108. Copyright 0 1999 by WB. Saunders Company 0022-3468/99/3404-0021$03.00/O 609
610
MORROW
I,@:..
.
Fig 1. Gross specimen, myofibromatosis of the pancreas. The mass measured 1.9 cm in largest diameter and had slightly lobulated borders and a smooth surface with focal necrosis. The duodenum (at left) has been opened anteriorly. The tubular structure at the upper left of the specimen is the gallbladder; normal pancreas is at the extreme right of the specimen.
hypertrophy13 and a case of esophageal atresia, annular pancreas, and renal hypoplasia. l4 Whereas three clinical forms of myofibromatosis have been described,5,8J5each is histologically identical to the others4 Myofibromatosis most often presents as a solitary nodule in the skin or soft tissues. These lesions most commonly are found in the head and neck, followed by the extremities and trunk.2J In 54% of cases, solitary fibromatosis is present at birth and is discovered within the first 2 years of life in 88% of cases2 Patients with
Fig 2. Hemangiopericytomelike pattern characteristic of congenital myofibromatosis.
in the center
of the lesion
ET AL
solitary nodules have an excellent prognosis after local excision. A local recurrence rate of 6.7% to 10.6% has been reported.2,g The second form is the multicentric variety consisting of multiple nodules in the skin, soft tissues, and bones. Like the solitary form, these nodules are usually present at bum9 The multicentric form generally has a good prognosis and spontaneously regresses in about one third of cases.2 The diagnosis is confirmed with excisional biopsy. The remaining nodules may be observed because they rarely cause harm even when persistent. Symptomatic or enlarging nodules are excised. Visceral involvement complicates about one third of the multicentric cases.gAlthough they do not metastasize, visceral lesions may enlarge and exert significant local tissue effects, as in the current case. In contrast to the solitary and multicentric forms, the visceral form usually is fatal, most often secondary to cardiopulmonary or gastrointestinal complications.g However, if a single visceral lesion is present and can be extirpated successfully, survival is anticipated. Infants with myofibromatosis should be evaluated with a skeletal survey; CT of the chest, abdomen, and pelvis; and possibly an echocardiogram. Bone scans are not necessary because the lytic skeletal lesions are seen easily on plain films. Although the primary treatment of myofibromatosis is surgical excision, symptomatic, progressive, and unresectable disease may respond to chemotherapeutic agents.16 To our knowledge, this is only the second report of a myofibroma isolated to the pancreas presenting with obstructive jaundice at birth and the first successfully treated by immediate pancreatoduodenectomy. In 1973, Amann and Klingenbergi7 reported a case of a girl born with obstructive jaundice who underwent surgical exploration at 8 months of age when cirrhosis developed. Congenital fibromatosis of the pancreatic head was diagnosed, and the lesion was bypassed. The same year, Chappellls reported a benign hemangioendothelioma in the head of the pancreas in an 1l-month-old boy successfully treated with pancreatoduodenectomy. In 1986 Rich et all9 reported on a 3-week-old infant with pancreatoblastoma treated with pancreatoduodenectomy who was healthy 4 years postoperatively. In 1988 Marks et a120 reported on an l&week-old girl with a 3-cm obstructing myofibroma of the head of the pancreas. The child was treated initially with T-tube drainage of the common bile duct. However, obstruction persisted and internal billiary diversion was performed. Eight months later a gastrojejunostomy became necessary when the tumor enlarged.20 In 1990 Grosfeld et a121reviewed 13 cases of pancreatic
EXCISION
OF NEONATAL
PANCREATIC
MYOFIBROMA
611
tumors in childhood treated at a single institution over a 20-year period. None of the lesions were discovered in the neonatal period, and none were myofibromas. In the current case, we were able to anastamose the distal duodenum to the pancreas because it was mobilized easily. The distal common bile duct reached the duodenum without tension and was sewn to it end to side. A roux limb of jejunum sewn end to end to the stomach completed the reconstruction. We felt that this arrangement was preferable to oversewing the duodenum and bringing up a roux limb of jejunum to drain the pancreas, stomach, and bile duct because it prevents reflux of bile and pancreatic juice into the stomach.
The preoperative diagnosis was pancreatoblastoma, which is the most common malignant pancreatic tumor in newborns.** Our frozen section biopsy result was consistent with malignancy, and thus added some reassurance in proceeding with pancreatoduodenectomy. However, the clinically ominous nature of the mass and the risk of diagnostic error with frozen section analysis would have compelled us to perform pancreatoduodenectomy even if the intraoperative biopsy interpretation had been benign. Bypass procedures can preclude potentially curative resections in cases of missed malignancy and can be compromised by the local tissue effects of persistent tumor growth.
REFERENCES 1. Stout AP: Juvenile fibromatosis. Cancer 7:953-978, 1954 2. Chung EB, Enzigner FM: Infantile myofibromatosis. Cancer 48:1807-1818,198l 3. Lin JJ, Svoboda DJ: Multiple congenital mesenchymal tumors: Multiple vascular leiomyomas in several organs of a newborn. Cancer 28:1046-1053,197l 4. Coffin CM, Neilson KA, Ingels S, et al: Congenital generalized myofibromatosis: A disseminated angiocentric myofibromatosis. Ped Path01 Lab Med 15:571-587, 1995 5. Morettin IB, Mueller E, Schreiber M: Generalized hamartomatosis (congenital generalized fibromatosis). AJR 114:722-734, 1972 6. Baer JW, Radkowski MA: Congenital multiple fibromatosis: A case report with review of the world literature. Am J Roentgen01 Rad Ther Nucl Med 118:200-205, 1973 7. Mentzel T, Calonje E, Nascimento AG, et al: Infantile hemangiopericytoma versus infantile myofibromatosis: Study of a series suggesting a continuous spectrum of infantile myofibroblastic lesions. Am J Surg Path01 18:922-930, 1994 8. Kaufman SL. Stout AP: Congenital mesenchymal tumors. Cancer 18:460-476,
1965
9. Wiswell TE, Sakas EL, Stephenson SR, et al: Infantile myofibromatosis. Pediatrics 76:981-984, 1985 10. Jennings TA, Duray PH, Collins FS, et al: Infantile myofibromatosis: Evidence of an autosomal-dominant disorder. Am J Surg Path01 8:529-538,
1984
11. Baird PA, Worth AJ: Congenital generalized autosomal recessive condition? Clin Genet 9:488-494,
fibromatosis: 1976
An
12. Fukasawa Y, Ishikura H, Takada A, et al: Massive apoptosis in infantile myofibromatosis. A putative mechanism of tumor regression. Am J Path01 144480-485, 1994 13. Familusi JB, Nottidge BA, Antia AU, et al: Congenital generalized fibromatosis. An African case with gingival hypertrophy and other unusual features. Am J Dis Child 130:1215-1217, 1976 14. Michel M, Ninane J, Claus D, et al: Major malformations in a case of infantile myofibromatosis. Eur J Pediatr 149:251-252, 1990 15. Mande R Hennequet A, Loubry P, et al: Fibmmatose congenitale diffuse du nouveau-ne a evolution regressive. Ann Pediatr (Paris) 12:692701,1%5 16. Raney B, Evans A. Granowetter L, et al: Nonsurgical management of children with recurrent or unresectable fibromatosis. Pediatrics 79:394-398,
1987
17. Amann FW, Klingenberg A: Localized congenital fibromatosis of the head of the pancreas as a cause of neonatal icterus. Z Kmderchir l&399-405,1974 18. Chappell JS: Benign hemangioendothelioma of the head of the pancreas treated by pancreaticoduodenectomy. J Pediatr Surg 8:431432.1973 19. Rich
RH, Weber JL, Shandling B: Adenocarcinoma of the pancreas in a neonate managed by pancreatoduodenectomy. J Pediatr Surg 21:806-808, 1986 20. Marks MK, Dewan PA, Stokes KB, et al: Infantile myofibromatosis causing billiary and pancreatic obstruction: A case report. Med Pediatr Oncol 16:363-365, 1988 21. Grosfeld JL, Vane DW, Rescorla FJ, et al: Pancreatic tumors in childhood: Analysis of 13 cases. J Pediatr Surg 25: 1057- 1062, 1990 22. Isaacs H: Pancreatic tumors, in Tumors of the Fetus and Newborn, Philadelphia, PA, Saunders, 1997, pp 317-327
E. Morrow,
Gerald
M. Woods,
Kansas
Robert
E. Garola,
and
Ronald
J. Sharp
City, Missouri
Myofibromatosis is a rare congenital disorder consisting of one or more fibrous nodules in the skin, soft tissues, bones, and internal organs. The authors report the unique case of a newborn who presented with obstructive jaundice caused by a single myofibroma in the head of the pancreas that was treated successfully by pancreatoduodenectomy on the eighth
day
0
decision was made to follow the infant’s progress with laboratory monitoring and CT imaging. Although glucose levels have been normal throughout the postoperative course without the need for insulin, the baby did require pancreatic enzyme supplementation. The enzymes were gradually withdrawn and recently discontinued without ill effect. The baby remains clinically well with normal weight gain 10 months after resection.
BSTRUCTIVE JAUNDICE in the newborn is rarely caused by a pancreatic neoplasm. We report the unique case of a pancreatic myofibroma successfully treated by primary pancreatoduodenectomy in an %dayold infant. CASE
ofPediatric
Surgery,
Vol34,
INDEX WORDS: denectomy.
Copyright
Myofibromatosis,
o 1999 by W.B. Saun-
pancreas,
pancreatoduo-
REPORT
A 39.week-gestation white boy was born to a healthy mother after an uncomplicated pregnancy. The baby became jaundiced on the second day of life (total bilirubin, 9.4 mg/dL; unconjugated, 7.4). Coomb’s test, complete blood count (CBC), and routine screening test results were normal. There was no family history of hepatobiliary disorders. On the fifth day of life, the bilirubin level had risen to 13.8, with a direct fraction of 8.9. Physical examination was otherwise unremarkable. Serum electrolytes and CBC findings were normal. Additional blood tests included albumin, 3.5 g/dL; amylase, <5 U/L; AST, 46 IUIL; ALT, 32 ILK: alkaline phosphatase, 308 U/L; and GGT, 1,329 U/L. An ultrasound scan demonstrated a solid mass in the head of the pancreas without definite vascularity. The mass displaced the superior mesenteric artery and portal vein and obstructed the common bile duct, which measured 4 mm in diameter. The liver, intrahepatic ducts, and remaining abdominal organs were unremarkable. A computed tomography (CT) scan of the chest, abdomen, and pelvis confirmed these fmdings and demonstrated duodenal and caval compression. No other abnormalities were noted. During laparotomy, a tirm mass in the head of the pancreas with several peripancreatic nodules was found. Frozen-section analysis was interpreted as neoplastic tissue consisting of nests of pleomorphic cells growing in a well-differentiated stroma. A pancreatoduodenectomy was performed and reconstruction was accomplished with end-to-end pancreatoduodenostomy, end-to-side choledochoduodenostomy, and end-toend Roux-en-Y gastrojejunostomy. Laboratory examination of the specimen showed a 1.9-cm yellowbrown tumor with irregular borders and a lobulated, focally necrotic surface (Fig 1). The mass infiltrated the muscularis propria of the duodenal wall but spared the mucosa. Microscopic examination demonstrated interlacing fascicles of spindle cells, with a central vascular pattern similar to a hemangiopericytoma (Fig 2). Few mitotic figures were identified, and normal pancreatic acinar and endocrine cells were seen. The majority of cells expressed vimentin. with focal actin and rare desmin expression. A diagnosis of infantile myofibromatosis was made, which also was found m the peripancreatic lymph nodes. The postoperative course was unremarkable. A skeletal survey did not demonstrate any additional foci of disease. After multidisciplinary review, the Journal
of life.
J Pediatr Surg 34:609-611. ders Company.
No 4 (April),
1999: pp 609-611
DISCUSSION
First described in 1954 by Stout,’ infantile myofibromatosis is a rare disorder distinguished histologically by the presence of interlacing fascicles of spindle cells with central vascular areas.2 Other names for myofibromatosis include congenital mesenchymal tumor, congenital generalized myfibromatosis, generalized hamartomatosis, congenital multiple fibromatosis, and multiple vascular leiomyomas of the newborn.3-6 The entity must be distinguished from similar lesions such as fibromatosis, desmoid tumors, hemangiopericytoma, and low-grade fibrosarcoma. Some investigators believe the myofibromatosis consists of a spectrum of spindle cell tumors with varying degrees of fibroblastic, myofibroblastic, and leiomyomatous features4.’ After vascular tumors, fibromatoses are the most common neonatal tumors8 and occur with a male to female ratio of 1.6: 1.9 Although the etiology is unclear, the lesion is believed to originate from the myofibroblast.4 Although most cases appear sporadic, there is evidence for both autosomal dominant and autosomal recessive inheritance.2~10,11Apoptosis may explain cases of spontaneous regression. l2 Associated anomalies occasionally have been reported, as in a case of gingival
From the Departments of Surgery Oncology, and Pathology, The Children’s Mercy Hospital and the Unrversity of Missouri-Kansas City School of Medicme, 24th and Gillham Rd, Kansas Cizy, MO 64108. Copyright 0 1999 by WB. Saunders Company 0022-3468/99/3404-0021$03.00/O 609
610
MORROW
I,@:..
.
Fig 1. Gross specimen, myofibromatosis of the pancreas. The mass measured 1.9 cm in largest diameter and had slightly lobulated borders and a smooth surface with focal necrosis. The duodenum (at left) has been opened anteriorly. The tubular structure at the upper left of the specimen is the gallbladder; normal pancreas is at the extreme right of the specimen.
hypertrophy13 and a case of esophageal atresia, annular pancreas, and renal hypoplasia. l4 Whereas three clinical forms of myofibromatosis have been described,5,8J5each is histologically identical to the others4 Myofibromatosis most often presents as a solitary nodule in the skin or soft tissues. These lesions most commonly are found in the head and neck, followed by the extremities and trunk.2J In 54% of cases, solitary fibromatosis is present at birth and is discovered within the first 2 years of life in 88% of cases2 Patients with
Fig 2. Hemangiopericytomelike pattern characteristic of congenital myofibromatosis.
in the center
of the lesion
ET AL
solitary nodules have an excellent prognosis after local excision. A local recurrence rate of 6.7% to 10.6% has been reported.2,g The second form is the multicentric variety consisting of multiple nodules in the skin, soft tissues, and bones. Like the solitary form, these nodules are usually present at bum9 The multicentric form generally has a good prognosis and spontaneously regresses in about one third of cases.2 The diagnosis is confirmed with excisional biopsy. The remaining nodules may be observed because they rarely cause harm even when persistent. Symptomatic or enlarging nodules are excised. Visceral involvement complicates about one third of the multicentric cases.gAlthough they do not metastasize, visceral lesions may enlarge and exert significant local tissue effects, as in the current case. In contrast to the solitary and multicentric forms, the visceral form usually is fatal, most often secondary to cardiopulmonary or gastrointestinal complications.g However, if a single visceral lesion is present and can be extirpated successfully, survival is anticipated. Infants with myofibromatosis should be evaluated with a skeletal survey; CT of the chest, abdomen, and pelvis; and possibly an echocardiogram. Bone scans are not necessary because the lytic skeletal lesions are seen easily on plain films. Although the primary treatment of myofibromatosis is surgical excision, symptomatic, progressive, and unresectable disease may respond to chemotherapeutic agents.16 To our knowledge, this is only the second report of a myofibroma isolated to the pancreas presenting with obstructive jaundice at birth and the first successfully treated by immediate pancreatoduodenectomy. In 1973, Amann and Klingenbergi7 reported a case of a girl born with obstructive jaundice who underwent surgical exploration at 8 months of age when cirrhosis developed. Congenital fibromatosis of the pancreatic head was diagnosed, and the lesion was bypassed. The same year, Chappellls reported a benign hemangioendothelioma in the head of the pancreas in an 1l-month-old boy successfully treated with pancreatoduodenectomy. In 1986 Rich et all9 reported on a 3-week-old infant with pancreatoblastoma treated with pancreatoduodenectomy who was healthy 4 years postoperatively. In 1988 Marks et a120 reported on an l&week-old girl with a 3-cm obstructing myofibroma of the head of the pancreas. The child was treated initially with T-tube drainage of the common bile duct. However, obstruction persisted and internal billiary diversion was performed. Eight months later a gastrojejunostomy became necessary when the tumor enlarged.20 In 1990 Grosfeld et a121reviewed 13 cases of pancreatic
EXCISION
OF NEONATAL
PANCREATIC
MYOFIBROMA
611
tumors in childhood treated at a single institution over a 20-year period. None of the lesions were discovered in the neonatal period, and none were myofibromas. In the current case, we were able to anastamose the distal duodenum to the pancreas because it was mobilized easily. The distal common bile duct reached the duodenum without tension and was sewn to it end to side. A roux limb of jejunum sewn end to end to the stomach completed the reconstruction. We felt that this arrangement was preferable to oversewing the duodenum and bringing up a roux limb of jejunum to drain the pancreas, stomach, and bile duct because it prevents reflux of bile and pancreatic juice into the stomach.
The preoperative diagnosis was pancreatoblastoma, which is the most common malignant pancreatic tumor in newborns.** Our frozen section biopsy result was consistent with malignancy, and thus added some reassurance in proceeding with pancreatoduodenectomy. However, the clinically ominous nature of the mass and the risk of diagnostic error with frozen section analysis would have compelled us to perform pancreatoduodenectomy even if the intraoperative biopsy interpretation had been benign. Bypass procedures can preclude potentially curative resections in cases of missed malignancy and can be compromised by the local tissue effects of persistent tumor growth.
REFERENCES 1. Stout AP: Juvenile fibromatosis. Cancer 7:953-978, 1954 2. Chung EB, Enzigner FM: Infantile myofibromatosis. Cancer 48:1807-1818,198l 3. Lin JJ, Svoboda DJ: Multiple congenital mesenchymal tumors: Multiple vascular leiomyomas in several organs of a newborn. Cancer 28:1046-1053,197l 4. Coffin CM, Neilson KA, Ingels S, et al: Congenital generalized myofibromatosis: A disseminated angiocentric myofibromatosis. Ped Path01 Lab Med 15:571-587, 1995 5. Morettin IB, Mueller E, Schreiber M: Generalized hamartomatosis (congenital generalized fibromatosis). AJR 114:722-734, 1972 6. Baer JW, Radkowski MA: Congenital multiple fibromatosis: A case report with review of the world literature. Am J Roentgen01 Rad Ther Nucl Med 118:200-205, 1973 7. Mentzel T, Calonje E, Nascimento AG, et al: Infantile hemangiopericytoma versus infantile myofibromatosis: Study of a series suggesting a continuous spectrum of infantile myofibroblastic lesions. Am J Surg Path01 18:922-930, 1994 8. Kaufman SL. Stout AP: Congenital mesenchymal tumors. Cancer 18:460-476,
1965
9. Wiswell TE, Sakas EL, Stephenson SR, et al: Infantile myofibromatosis. Pediatrics 76:981-984, 1985 10. Jennings TA, Duray PH, Collins FS, et al: Infantile myofibromatosis: Evidence of an autosomal-dominant disorder. Am J Surg Path01 8:529-538,
1984
11. Baird PA, Worth AJ: Congenital generalized autosomal recessive condition? Clin Genet 9:488-494,
fibromatosis: 1976
An
12. Fukasawa Y, Ishikura H, Takada A, et al: Massive apoptosis in infantile myofibromatosis. A putative mechanism of tumor regression. Am J Path01 144480-485, 1994 13. Familusi JB, Nottidge BA, Antia AU, et al: Congenital generalized fibromatosis. An African case with gingival hypertrophy and other unusual features. Am J Dis Child 130:1215-1217, 1976 14. Michel M, Ninane J, Claus D, et al: Major malformations in a case of infantile myofibromatosis. Eur J Pediatr 149:251-252, 1990 15. Mande R Hennequet A, Loubry P, et al: Fibmmatose congenitale diffuse du nouveau-ne a evolution regressive. Ann Pediatr (Paris) 12:692701,1%5 16. Raney B, Evans A. Granowetter L, et al: Nonsurgical management of children with recurrent or unresectable fibromatosis. Pediatrics 79:394-398,
1987
17. Amann FW, Klingenberg A: Localized congenital fibromatosis of the head of the pancreas as a cause of neonatal icterus. Z Kmderchir l&399-405,1974 18. Chappell JS: Benign hemangioendothelioma of the head of the pancreas treated by pancreaticoduodenectomy. J Pediatr Surg 8:431432.1973 19. Rich
RH, Weber JL, Shandling B: Adenocarcinoma of the pancreas in a neonate managed by pancreatoduodenectomy. J Pediatr Surg 21:806-808, 1986 20. Marks MK, Dewan PA, Stokes KB, et al: Infantile myofibromatosis causing billiary and pancreatic obstruction: A case report. Med Pediatr Oncol 16:363-365, 1988 21. Grosfeld JL, Vane DW, Rescorla FJ, et al: Pancreatic tumors in childhood: Analysis of 13 cases. J Pediatr Surg 25: 1057- 1062, 1990 22. Isaacs H: Pancreatic tumors, in Tumors of the Fetus and Newborn, Philadelphia, PA, Saunders, 1997, pp 317-327