FETAL RHABDOMYOMA AND NEVOID BASAL CELL CARCINOMA SYNDROME
Susan DiSanto, MD, and Arthur B. Abt, MD
Department of Pathology, Milton S. Hershey Medical Center, Pennsylvania State University, Hershey, Pennsylvania 17033
Danielle K. Boal, M D 0 Department of Radiology, Milton S. Hershey Medical Center, Pennsylvania State University, Hershey, Pennsylvania 17033 Fetal Pediatr Pathol Downloaded from informahealthcare.com by University of Newcastle on 01/02/15 For personal use only.
Thomas M. Krummel, M D
0 Department of Surgery, Milton S. Hershey Medical Center, Pennsylvania State University, Hershey, Pennsylvania 17033
0 A 6-year-old white female presented with a fetal rhabdomyoma of the posterior mediastinum and retroperitoneurn. Radiologic evaluation and f a m i b history revealed features of the nevoid basal cell carcinoma syndrome (NBS).Literature review disclosed two other children with NBS and fetal rhabdomyoma, which should be retarded as one of the soft tissue tumors associated with NBS.
KEY WORDS: j t a l rhabdomyoma, neuoid basal cell carcinoma
INTRODUCTION The nevoid basal cell carcinoma syndrome (NBS) is an autosomal dominant disorder with complete penetrance but variable expressivity (1). In addition to the presence of numerous basal cell carcinomas, skeletal, ocular, endocrine, and nervous system abnormalities and mesenteric cysts have been described (2, 3). The syndrome is usually recognized in older children and young adults with the premature appearance of basal cell carcinomas in both sun-exposed and nonexposed areas. We recently had the opportunity to study a fetal rhabdomyoma excised from a 6-year-old female presenting with a posterior mediastinal mass and subsequent recognition of features characterizing the NBS syndrome.
CASE HISTORY This 6-year-old white female with no significant past medical history was noted to have clubbing of the digits and a systolic murmur when examined by her family dentist. She was referred for a cardiology examination, and an echocardiogram revealed a structurally normal heart with normal function. A grade Address reprint requests to Arthur B. Abt, M.D., Department of Pathology, Milton S. Hershey Medical Center, PO Box 850, Hershey, Pennsylvania 17033. Pediatric Patholou, 12:441-447, 1992 Copyright 0 1992 by Hemisphere Publishing Corporation
441
Fetal Pediatr Pathol Downloaded from informahealthcare.com by University of Newcastle on 01/02/15 For personal use only.
442
S. DISANTO ET AL.
I I N I vibratory systolic ejection murmur was present. There was no evidence of cyanosis. A routine chest radiograph showed a left chest mass. A complete blood count and chemical analysis were within normal limits. The physical examination was noncontributory except for the previously noted findings. The patient made an eventful recovery following removal of the mass, diagnosed as a fetal rhabdomyoma. She continues to do well 1 year following surgery and the digital clubbing appears to be resolving. The patient’s family history is significant for NBS in her father and a paternal uncle. Her brother and a first cousin have Sprengel’s deformity, a scapular anomaly associated with NBS.
IMAGING EVALUATION Accompanying the referral chest X-ray, which demonstrated a large left intrathoracic mass and calcification in the left suprarenal area, was a chest Xray taken 4 years earlier and interpreted as normal but which, in retrospect, shows a 2-cm left thoracic mass at the diaphragmatic costovertebral angle. U1trasonography, computed tomography, and magnetic resonance imaging revealed a large solid bilobed intrathoracic mass with retroperitoneal extension into the left suprarenal area. Computed tomography demonstrated a whorled appearance of the tumor along with small punctate areas of calcification and dense calcification within the left adrenal. The mass, although contiguous, did not invade paraspinal structures, spinal canal, pericardium, or lung (Fig. 1). The remainder of the visualized upper abdomen was normal. A limited skeletal survey including chest, skull, mandible, and extremities was obtained. Pertinent findings included the following: early calcification of the falx, mild thoracic kyphosis and carinatum deformity, bifid right sixth rib, clinodactyly of fifth fingers, short broad distal phalanx of thumbs, dysplastic phalanges of toes, bipartite ossification centers of metatarsals, and focal radiolucencies in tubular bones of hands and feet.
OPERATIVE FINDINGS A large left thoracic tumor was excised. The mass was in the posterior mediastinum and contiguous with the diaphragm. Inferiorly, the tumor mass extended into the left retroperitoneal space but did not involve the suprarenal space. There was no attachment to the pericardium. The mass, including involved diaphragm, was completely excised and the diaphragm was repaired. PATHOLOGIC FINDINGS The mass measured 13 X 10 X 7 cm and weighed 448 g. It was well circumscribed but not encapsulated and had three lobes. A cuff of diaphragm
Fetal Pediatr Pathol Downloaded from informahealthcare.com by University of Newcastle on 01/02/15 For personal use only.
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443
FIGURE 1. Coronal T1-weighted, post-gadolinium enhancement magnetic resonance image demonstrates a mass with diffuse inhomogenous enhancement filling the lower left thorax, displacing the thoracic aorta, and extending into the upper abdomen. Lateral margin of hemidiaphragm is evident, but the mass replaces the medial two-thirds of the hemidiaphragm.
separated the upper two thoracic lobes from the retroperitoneal lobe (Fig. 2). The cut surface of the tumor was yellow-white, had a whorled pattern, and was myxoid. There was a 3-cm hemorrhagic cystic area in the retroperitoneal pole. The tumor was composed primarily of well-differentiated (striated) and immature skeletal muscle, Cells were disorganized and frequently arranged in a haphazard pattern. Admixed with the muscle cells were immature mesenchymal cells and a moderate number of mature lymphocytes and plasma cells (Fig. 3). In a few areas, small groups of wavy spindle cells formed relatively compact bundles suggesting neural differentiation. There was no significant mitotic activity, nuclear pleomorphism, or necrosis. A trichrome stain demonstrated redstaining muscle cells with interstitial areas demonstrating either pale or dark blue staining consistent with collagen. Sevier-Munger and SlOO stains failed to demonstrate neural elements within the tumor, but a few small nerves were
Fetal Pediatr Pathol Downloaded from informahealthcare.com by University of Newcastle on 01/02/15 For personal use only.
444
S. DISANTO E l AL.
FIGURE 2. T h e excised mass is unencapsulated and demonstrates three lobes. A small cuff of diaphragm (arrow) is noted.
noted near the resection margins. Staining for muscle-specific actin and myoglobin was noted in both mature and immature spindle cells. Ultrastructural studies further confirmed the skeletal muscle origin of the tumor cells. Histologic sections did not demonstrate calcifications and a specimen X-ray was not obtained.
DISCUSSION The NBS was first recognized by Gorlin and Goltz in 1960 (4). Since their description of the classic features, including basal cell carcinomas, dentigerous cysts, and bifid ribs, many other abnormalities have been reported. Ectopic calcification, palmar and/or plantar pits, and ovarian fibromas are now recognized as frequent components of the NBS syndrome (2). Many other abnormalities and neoplasms have been associated with the syndrome but are seen in less than 10% of patients. Ocular lesions including cataracts, glaucoma, and coloboma are well recognized, and central nervous system tumors include medulloblastoma and meningioma. The reader is referred to publications by Gorlin (3) and Taybi and Lachman ( 5 ) for a more complete listing of associated lesions and their estimated frequency. With the exception of ovarian fibromas, other stromal neoplasms are rela-
Fetal Pediatr Pathol Downloaded from informahealthcare.com by University of Newcastle on 01/02/15 For personal use only.
FETAL RHABDOMYOMA IN BASAL CELL SYNDROME
445
tively uncommon but have been described in a variety of organs in patients with NBS. More than 11 patients with cardiac neoplasms have been described (3). Cardiac fibromas are the most commonly reported thoracic neoplasm, but a cardiac fibrous histiocytoma has been reported (6). Other mesenchymal neoplasms include rhabdomyosarcoma originating in nasopharynx and presternal locations (7, 8). Tumors composed largely of skeletal muscle are uncommon. Some, admixed with neural elements, have been described as neuromuscular hamartoma or benign triton tumor (9, 10). This was excluded in our patient because immunoperoxidase stains for SlOO antigen revealed only rare peritumoral nerve fibers, and an association with a nerve trunk was not appreciated. Other tumors containing striated muscle must be considered in the differential diagnosis. Adult rhabdomyomas contain large ovoid cells with pink granular or vacuolated cytoplasm and occasional cross-striations (1 1). These tumors are most frequently seen in the heart and oral cavity. Fetal rhabdomyomas are rare neoplasms and are differentiated from embryonal rhabdomyosarcoma by being well circumscribed, exhibiting myogenic cells demonstrating all stages of differentiation and separation of myogenic cells by mononuclear mesenchymal cells.
FIGURE 3. Cells with cross-striations are admixed with stromal cells containing scant cytoplasm and lymphocytes ( X 350). Cross-striations are best seen in the insert ( X 600).
Fetal Pediatr Pathol Downloaded from informahealthcare.com by University of Newcastle on 01/02/15 For personal use only.
446
S. DISANTO ET AL.
Occasional mitotic figures may be present but are not numerous (12, 13). All of these features were observed in our patient. Dahl and colleagues reported thigh and chest wall fetal rhabdomyomas in an infant with NBS (14). The patient reported by Schweisguth et al. as having a presternal congenital rhabdomyosarcoma shows histologic features strongly suggesting a fetal rhabdomyoma (8). This case was published prior to the recognition of fetal rhabdomyoma by Dehner et al. (12). The other report of a rhabdomyosarcoma and NBS contains no photographs, but this nasopharyngeal tumor was treated with radiation and chemotherapy. Thus it appears that our patient is the third individual with NBS and a fetal rhabdomyoma. The rarity of this tumor further supports a relationship between this syndrome and fetal rhabdomyoma. Recognition of NBS in our patient resulted from X-ray studies showing the described abnormalities and subsequent disclosure of the appropriate family history. The tumor seen in this child involved the posterior mediastinum and extended below the diaphragm into the retroperitoneal region. Its point of origin is unknown but the diaphragm is suspected. Digital clubbing is not a reported feature of NBS. As the clubbing has largely resolved following surgery, it appears to be related to the presence of the posterior mediastinal mass. In summary, fetal rhabdomyoma should be recognized as one of the tumors associated with NBS. The lesion is histologically distinct from adult rhabdomyoma, rhabdomyosarcoma, and neuromuscular hamartoma. Some authors have questioned whether this is a neoplasm or a hamartoma analogous to the hamartomatous lesions seen in the phakomatoses (1 2). Previous authors reviewing NBS have included this syndrome as one of the phakomatoses (15). Finally, fetal rhabdomyoma presents in childhood before other stigmata of the syndrome are recognized. The diagnosis depends on identification of the associated abnormalities and the appropriate family history.
REFERENCES 1. Gorlin RJ, Sedano HO. The multiple nevoid basal cell carcinoma syndrome revisited. Birth Defects 1971 ;7: 140-8. 2. Case records of the Massachusetts General Hospital (case 10-1986). N Engl J Med 1986;314:700-6. 3. Gorlin RJ. Nevoid basal-cell carcinoma syndrome. Medicine 1987;66:98-113. 4. Gorlin RJ, Goltz RW. Multiple nevoid basal-cell epithelioma, jaw cysts and bifid rib: A syndrome. N Engl J Med 1960;262:908-12. 5. Taybi H , Lachman RS. Radiology of Syndromes, Metabolic Disorders, and Skeletal Dysplasias. 3rd ed. Chicago: Year Book Medical Publishers, 1990. 6. Jones KL, Wolf PL, Jensen P, Dittrich H, Benirschke K, Bloor C. The Gorlin syndrome: A genetically determined disorder associated with cardiac tumor. Am Heart J 1986;l 11:1013-5. 7. Beddis IR, Mott MG, Bullimore J. Case report: Nasopharyngeal rhabdomyosarcoma and Gorlin’s naevoid basal cell carcinoma syndrome. Med Pediatr Oncol 1983;ll: 178-9. 8. Schweisguth 0, Gerard-Marchant R , Lemerle J. Basal cell nevus syndrome. Association with congenital rhabdomyosarcoma. Arch Fr Pediatr 1968;25:1083-93 (in French).
FETAL RHABDOMYOMA IN BASAL CELL SYNDROME
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Fetal Pediatr Pathol Downloaded from informahealthcare.com by University of Newcastle on 01/02/15 For personal use only.
9. Bonneau R,Brochu P. Neuromuscular choristoma: A clinicopathologic study of two cases. Am J Surg Pathol 1983;7: 52 1-8. 10. Markel SF, Enzinger FM. Neuromuscular hamartoma-a benign “triton tumor” composed of mature neural and striated muscle elements. Cancer 1982;49:140-4. 11. DiSant’Agnese PA, Knowles DM. Extracardiac rhabdomyoma: A clinicopathologic study and review of the literature. Cancer 1980;46:780-9. 12. Dehner LP, Enzinger FM, Font RL. Fetal rhabdomyoma. An analysis of nine cases. Cancer 1972;30:160-6. 13. Dehner LP. Pediatric Surgical Pathology. 2nd ed. Baltimore: Williams & Wilkins, 1987. 14. Dahl I, Angervall L, Save-Soderbergh J. Foetal rhabdomyoma. Case report of a patient with two tumors. Acta Pathol Microbiol Scand [A] 1976;84:107-12. 15. Hermans EH, Grosfeld JC, Spaas JA. The fifth phacomatosis. Dermatologica 1965;130:446-76. Received May 13, 1991 Revision accepted Jub 15, 1991
Susan DiSanto, MD, and Arthur B. Abt, MD
Department of Pathology, Milton S. Hershey Medical Center, Pennsylvania State University, Hershey, Pennsylvania 17033
Danielle K. Boal, M D 0 Department of Radiology, Milton S. Hershey Medical Center, Pennsylvania State University, Hershey, Pennsylvania 17033 Fetal Pediatr Pathol Downloaded from informahealthcare.com by University of Newcastle on 01/02/15 For personal use only.
Thomas M. Krummel, M D
0 Department of Surgery, Milton S. Hershey Medical Center, Pennsylvania State University, Hershey, Pennsylvania 17033
0 A 6-year-old white female presented with a fetal rhabdomyoma of the posterior mediastinum and retroperitoneurn. Radiologic evaluation and f a m i b history revealed features of the nevoid basal cell carcinoma syndrome (NBS).Literature review disclosed two other children with NBS and fetal rhabdomyoma, which should be retarded as one of the soft tissue tumors associated with NBS.
KEY WORDS: j t a l rhabdomyoma, neuoid basal cell carcinoma
INTRODUCTION The nevoid basal cell carcinoma syndrome (NBS) is an autosomal dominant disorder with complete penetrance but variable expressivity (1). In addition to the presence of numerous basal cell carcinomas, skeletal, ocular, endocrine, and nervous system abnormalities and mesenteric cysts have been described (2, 3). The syndrome is usually recognized in older children and young adults with the premature appearance of basal cell carcinomas in both sun-exposed and nonexposed areas. We recently had the opportunity to study a fetal rhabdomyoma excised from a 6-year-old female presenting with a posterior mediastinal mass and subsequent recognition of features characterizing the NBS syndrome.
CASE HISTORY This 6-year-old white female with no significant past medical history was noted to have clubbing of the digits and a systolic murmur when examined by her family dentist. She was referred for a cardiology examination, and an echocardiogram revealed a structurally normal heart with normal function. A grade Address reprint requests to Arthur B. Abt, M.D., Department of Pathology, Milton S. Hershey Medical Center, PO Box 850, Hershey, Pennsylvania 17033. Pediatric Patholou, 12:441-447, 1992 Copyright 0 1992 by Hemisphere Publishing Corporation
441
Fetal Pediatr Pathol Downloaded from informahealthcare.com by University of Newcastle on 01/02/15 For personal use only.
442
S. DISANTO ET AL.
I I N I vibratory systolic ejection murmur was present. There was no evidence of cyanosis. A routine chest radiograph showed a left chest mass. A complete blood count and chemical analysis were within normal limits. The physical examination was noncontributory except for the previously noted findings. The patient made an eventful recovery following removal of the mass, diagnosed as a fetal rhabdomyoma. She continues to do well 1 year following surgery and the digital clubbing appears to be resolving. The patient’s family history is significant for NBS in her father and a paternal uncle. Her brother and a first cousin have Sprengel’s deformity, a scapular anomaly associated with NBS.
IMAGING EVALUATION Accompanying the referral chest X-ray, which demonstrated a large left intrathoracic mass and calcification in the left suprarenal area, was a chest Xray taken 4 years earlier and interpreted as normal but which, in retrospect, shows a 2-cm left thoracic mass at the diaphragmatic costovertebral angle. U1trasonography, computed tomography, and magnetic resonance imaging revealed a large solid bilobed intrathoracic mass with retroperitoneal extension into the left suprarenal area. Computed tomography demonstrated a whorled appearance of the tumor along with small punctate areas of calcification and dense calcification within the left adrenal. The mass, although contiguous, did not invade paraspinal structures, spinal canal, pericardium, or lung (Fig. 1). The remainder of the visualized upper abdomen was normal. A limited skeletal survey including chest, skull, mandible, and extremities was obtained. Pertinent findings included the following: early calcification of the falx, mild thoracic kyphosis and carinatum deformity, bifid right sixth rib, clinodactyly of fifth fingers, short broad distal phalanx of thumbs, dysplastic phalanges of toes, bipartite ossification centers of metatarsals, and focal radiolucencies in tubular bones of hands and feet.
OPERATIVE FINDINGS A large left thoracic tumor was excised. The mass was in the posterior mediastinum and contiguous with the diaphragm. Inferiorly, the tumor mass extended into the left retroperitoneal space but did not involve the suprarenal space. There was no attachment to the pericardium. The mass, including involved diaphragm, was completely excised and the diaphragm was repaired. PATHOLOGIC FINDINGS The mass measured 13 X 10 X 7 cm and weighed 448 g. It was well circumscribed but not encapsulated and had three lobes. A cuff of diaphragm
Fetal Pediatr Pathol Downloaded from informahealthcare.com by University of Newcastle on 01/02/15 For personal use only.
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FIGURE 1. Coronal T1-weighted, post-gadolinium enhancement magnetic resonance image demonstrates a mass with diffuse inhomogenous enhancement filling the lower left thorax, displacing the thoracic aorta, and extending into the upper abdomen. Lateral margin of hemidiaphragm is evident, but the mass replaces the medial two-thirds of the hemidiaphragm.
separated the upper two thoracic lobes from the retroperitoneal lobe (Fig. 2). The cut surface of the tumor was yellow-white, had a whorled pattern, and was myxoid. There was a 3-cm hemorrhagic cystic area in the retroperitoneal pole. The tumor was composed primarily of well-differentiated (striated) and immature skeletal muscle, Cells were disorganized and frequently arranged in a haphazard pattern. Admixed with the muscle cells were immature mesenchymal cells and a moderate number of mature lymphocytes and plasma cells (Fig. 3). In a few areas, small groups of wavy spindle cells formed relatively compact bundles suggesting neural differentiation. There was no significant mitotic activity, nuclear pleomorphism, or necrosis. A trichrome stain demonstrated redstaining muscle cells with interstitial areas demonstrating either pale or dark blue staining consistent with collagen. Sevier-Munger and SlOO stains failed to demonstrate neural elements within the tumor, but a few small nerves were
Fetal Pediatr Pathol Downloaded from informahealthcare.com by University of Newcastle on 01/02/15 For personal use only.
444
S. DISANTO E l AL.
FIGURE 2. T h e excised mass is unencapsulated and demonstrates three lobes. A small cuff of diaphragm (arrow) is noted.
noted near the resection margins. Staining for muscle-specific actin and myoglobin was noted in both mature and immature spindle cells. Ultrastructural studies further confirmed the skeletal muscle origin of the tumor cells. Histologic sections did not demonstrate calcifications and a specimen X-ray was not obtained.
DISCUSSION The NBS was first recognized by Gorlin and Goltz in 1960 (4). Since their description of the classic features, including basal cell carcinomas, dentigerous cysts, and bifid ribs, many other abnormalities have been reported. Ectopic calcification, palmar and/or plantar pits, and ovarian fibromas are now recognized as frequent components of the NBS syndrome (2). Many other abnormalities and neoplasms have been associated with the syndrome but are seen in less than 10% of patients. Ocular lesions including cataracts, glaucoma, and coloboma are well recognized, and central nervous system tumors include medulloblastoma and meningioma. The reader is referred to publications by Gorlin (3) and Taybi and Lachman ( 5 ) for a more complete listing of associated lesions and their estimated frequency. With the exception of ovarian fibromas, other stromal neoplasms are rela-
Fetal Pediatr Pathol Downloaded from informahealthcare.com by University of Newcastle on 01/02/15 For personal use only.
FETAL RHABDOMYOMA IN BASAL CELL SYNDROME
445
tively uncommon but have been described in a variety of organs in patients with NBS. More than 11 patients with cardiac neoplasms have been described (3). Cardiac fibromas are the most commonly reported thoracic neoplasm, but a cardiac fibrous histiocytoma has been reported (6). Other mesenchymal neoplasms include rhabdomyosarcoma originating in nasopharynx and presternal locations (7, 8). Tumors composed largely of skeletal muscle are uncommon. Some, admixed with neural elements, have been described as neuromuscular hamartoma or benign triton tumor (9, 10). This was excluded in our patient because immunoperoxidase stains for SlOO antigen revealed only rare peritumoral nerve fibers, and an association with a nerve trunk was not appreciated. Other tumors containing striated muscle must be considered in the differential diagnosis. Adult rhabdomyomas contain large ovoid cells with pink granular or vacuolated cytoplasm and occasional cross-striations (1 1). These tumors are most frequently seen in the heart and oral cavity. Fetal rhabdomyomas are rare neoplasms and are differentiated from embryonal rhabdomyosarcoma by being well circumscribed, exhibiting myogenic cells demonstrating all stages of differentiation and separation of myogenic cells by mononuclear mesenchymal cells.
FIGURE 3. Cells with cross-striations are admixed with stromal cells containing scant cytoplasm and lymphocytes ( X 350). Cross-striations are best seen in the insert ( X 600).
Fetal Pediatr Pathol Downloaded from informahealthcare.com by University of Newcastle on 01/02/15 For personal use only.
446
S. DISANTO ET AL.
Occasional mitotic figures may be present but are not numerous (12, 13). All of these features were observed in our patient. Dahl and colleagues reported thigh and chest wall fetal rhabdomyomas in an infant with NBS (14). The patient reported by Schweisguth et al. as having a presternal congenital rhabdomyosarcoma shows histologic features strongly suggesting a fetal rhabdomyoma (8). This case was published prior to the recognition of fetal rhabdomyoma by Dehner et al. (12). The other report of a rhabdomyosarcoma and NBS contains no photographs, but this nasopharyngeal tumor was treated with radiation and chemotherapy. Thus it appears that our patient is the third individual with NBS and a fetal rhabdomyoma. The rarity of this tumor further supports a relationship between this syndrome and fetal rhabdomyoma. Recognition of NBS in our patient resulted from X-ray studies showing the described abnormalities and subsequent disclosure of the appropriate family history. The tumor seen in this child involved the posterior mediastinum and extended below the diaphragm into the retroperitoneal region. Its point of origin is unknown but the diaphragm is suspected. Digital clubbing is not a reported feature of NBS. As the clubbing has largely resolved following surgery, it appears to be related to the presence of the posterior mediastinal mass. In summary, fetal rhabdomyoma should be recognized as one of the tumors associated with NBS. The lesion is histologically distinct from adult rhabdomyoma, rhabdomyosarcoma, and neuromuscular hamartoma. Some authors have questioned whether this is a neoplasm or a hamartoma analogous to the hamartomatous lesions seen in the phakomatoses (1 2). Previous authors reviewing NBS have included this syndrome as one of the phakomatoses (15). Finally, fetal rhabdomyoma presents in childhood before other stigmata of the syndrome are recognized. The diagnosis depends on identification of the associated abnormalities and the appropriate family history.
REFERENCES 1. Gorlin RJ, Sedano HO. The multiple nevoid basal cell carcinoma syndrome revisited. Birth Defects 1971 ;7: 140-8. 2. Case records of the Massachusetts General Hospital (case 10-1986). N Engl J Med 1986;314:700-6. 3. Gorlin RJ. Nevoid basal-cell carcinoma syndrome. Medicine 1987;66:98-113. 4. Gorlin RJ, Goltz RW. Multiple nevoid basal-cell epithelioma, jaw cysts and bifid rib: A syndrome. N Engl J Med 1960;262:908-12. 5. Taybi H , Lachman RS. Radiology of Syndromes, Metabolic Disorders, and Skeletal Dysplasias. 3rd ed. Chicago: Year Book Medical Publishers, 1990. 6. Jones KL, Wolf PL, Jensen P, Dittrich H, Benirschke K, Bloor C. The Gorlin syndrome: A genetically determined disorder associated with cardiac tumor. Am Heart J 1986;l 11:1013-5. 7. Beddis IR, Mott MG, Bullimore J. Case report: Nasopharyngeal rhabdomyosarcoma and Gorlin’s naevoid basal cell carcinoma syndrome. Med Pediatr Oncol 1983;ll: 178-9. 8. Schweisguth 0, Gerard-Marchant R , Lemerle J. Basal cell nevus syndrome. Association with congenital rhabdomyosarcoma. Arch Fr Pediatr 1968;25:1083-93 (in French).
FETAL RHABDOMYOMA IN BASAL CELL SYNDROME
447
Fetal Pediatr Pathol Downloaded from informahealthcare.com by University of Newcastle on 01/02/15 For personal use only.
9. Bonneau R,Brochu P. Neuromuscular choristoma: A clinicopathologic study of two cases. Am J Surg Pathol 1983;7: 52 1-8. 10. Markel SF, Enzinger FM. Neuromuscular hamartoma-a benign “triton tumor” composed of mature neural and striated muscle elements. Cancer 1982;49:140-4. 11. DiSant’Agnese PA, Knowles DM. Extracardiac rhabdomyoma: A clinicopathologic study and review of the literature. Cancer 1980;46:780-9. 12. Dehner LP, Enzinger FM, Font RL. Fetal rhabdomyoma. An analysis of nine cases. Cancer 1972;30:160-6. 13. Dehner LP. Pediatric Surgical Pathology. 2nd ed. Baltimore: Williams & Wilkins, 1987. 14. Dahl I, Angervall L, Save-Soderbergh J. Foetal rhabdomyoma. Case report of a patient with two tumors. Acta Pathol Microbiol Scand [A] 1976;84:107-12. 15. Hermans EH, Grosfeld JC, Spaas JA. The fifth phacomatosis. Dermatologica 1965;130:446-76. Received May 13, 1991 Revision accepted Jub 15, 1991
