L E T T E R S TO T H E E D I T O R Clinical notes
"Clinical notes" represent clinical and/or laboratory experiences which can be presented in 200 to 400 words, 3 or 4 references, and, if contributory, one illustration or short table. "Clinical notes" are subject to the same critical peer review and editing as papers published in other sections of THE JOtrRr~AL.
Wilms tumor and pigmented nevi To the Editor: This is a report of a four-year-old girl who developed multiple linear nevi coincident with the fortuitous discovery of a Wilms tumor. The association of pigmented nevi and Wilms tumor with or without hemihypertrophy has been noted in the past but has not received adequate emphasis. CASE REPORT Patient L. L., four-year-old Caucasian girl, presented with a two- to three-month history of an increasing number of pigmented lesions over the trunk and extremities. The lesions were first noted over the dorsal right forearm and then over the lower anterior and upper posterior thorax. She had been in good health during this time. There was no family history of nevi or anomalies associated with Wilms tumor. Physical examination revealed a healthy appearing girl. Moderately pigmented linear nevi were noted in the above described areas. A hard, 6 • 7 cm, nontender, laterally displaced mass was palpated in the left upper quadrant of the abdomen. There was no palpable lymphadenopathy. The remainder of the examination was negative. An intravenous pyleogram and ultrasound study demonstrated a solid structure in the lower pole of the left kidney that deformed the calyceal system. The tumor was surgically removed with no evidence on gross examination of involvement of the contralateral kidney or the liver. Microscopic studies revealed a welldifferentiated Wilms tumor with no capsular invasion, lymph node involvement, or vessel emboli. Chest roentgenogram and liver scan were interpreted as negative. The child was classified as having group t disease and chemotherapy was begun with actinomycin D and vincristine. DISCUSSION Children with Wilms tumor have demonstrated a variety of congenital anomalies, which can serve as external markers for the presence of occult disease. The National Wilms Tumor Study has tabulated the occurrence of congenital anomalies. In addition to the well-recognized associations of aniridia, hemihypertrophy,
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The Journal of P E D 1 A T R 1 C S VoL 90, No. 5, pp. 840-860
and genitourinary anomalies, hamartomas including "birthmarks" and multiple nevi were reported in 7.9% of the children. Our patient had an increasing number of pigmented linear nevi observed prior to the discovery of the abdominal mass. Following removal of the tumor and onset of chemotherapy, the nevi decreased in size and pigmentation. They have not intensified on re-exposure to sunlight. It is interesting to speculate that the tumor might have contained ACTH or active melaninstimulating hormone. ~. :~There was no evidence of osteoporosis or Cushing syndrome; however, hormonal analysis was not performed. The relationship between these findings is uncertain but provocative. Children with nevi should have frequent, careful abdominal examinations. The authors express their appreciation to Dr. R. Rodney Howell for his critical appraisal of the manuscript. We thank Mrs. Ruth Person for her assistance in the preparation of this paper. Frank A. Simon. M.D. Department of Pediatrics The University of Texas Medical School at Houston Houston. TX 77030 Jan E. Drutz, M.D. Department of Pediatrics Ba•lor College of Medicine Houston, TX 77030 Joseph N. Corriere, M.D. Department of Surgery The University of Texas Medical School at Houston Houston, TX 77030
REFERENCES 1. Pendergrass TW: Congenital anomalies in children with Wilms' tumor, Cancer 37:403, 1976. 2. Cummins GE, and Cohen D: Cushing's syndrome secondary to ACTH-secreting Wilms' tumor, J Pediatr Surg 9:535, 1974. 3. Omenn GS: Ectopic hormone syndromes associated with tumors in childhood, Pediatrics 47:613, 1971.
"Clinical notes" represent clinical and/or laboratory experiences which can be presented in 200 to 400 words, 3 or 4 references, and, if contributory, one illustration or short table. "Clinical notes" are subject to the same critical peer review and editing as papers published in other sections of THE JOtrRr~AL.
Wilms tumor and pigmented nevi To the Editor: This is a report of a four-year-old girl who developed multiple linear nevi coincident with the fortuitous discovery of a Wilms tumor. The association of pigmented nevi and Wilms tumor with or without hemihypertrophy has been noted in the past but has not received adequate emphasis. CASE REPORT Patient L. L., four-year-old Caucasian girl, presented with a two- to three-month history of an increasing number of pigmented lesions over the trunk and extremities. The lesions were first noted over the dorsal right forearm and then over the lower anterior and upper posterior thorax. She had been in good health during this time. There was no family history of nevi or anomalies associated with Wilms tumor. Physical examination revealed a healthy appearing girl. Moderately pigmented linear nevi were noted in the above described areas. A hard, 6 • 7 cm, nontender, laterally displaced mass was palpated in the left upper quadrant of the abdomen. There was no palpable lymphadenopathy. The remainder of the examination was negative. An intravenous pyleogram and ultrasound study demonstrated a solid structure in the lower pole of the left kidney that deformed the calyceal system. The tumor was surgically removed with no evidence on gross examination of involvement of the contralateral kidney or the liver. Microscopic studies revealed a welldifferentiated Wilms tumor with no capsular invasion, lymph node involvement, or vessel emboli. Chest roentgenogram and liver scan were interpreted as negative. The child was classified as having group t disease and chemotherapy was begun with actinomycin D and vincristine. DISCUSSION Children with Wilms tumor have demonstrated a variety of congenital anomalies, which can serve as external markers for the presence of occult disease. The National Wilms Tumor Study has tabulated the occurrence of congenital anomalies. In addition to the well-recognized associations of aniridia, hemihypertrophy,
840
The Journal of P E D 1 A T R 1 C S VoL 90, No. 5, pp. 840-860
and genitourinary anomalies, hamartomas including "birthmarks" and multiple nevi were reported in 7.9% of the children. Our patient had an increasing number of pigmented linear nevi observed prior to the discovery of the abdominal mass. Following removal of the tumor and onset of chemotherapy, the nevi decreased in size and pigmentation. They have not intensified on re-exposure to sunlight. It is interesting to speculate that the tumor might have contained ACTH or active melaninstimulating hormone. ~. :~There was no evidence of osteoporosis or Cushing syndrome; however, hormonal analysis was not performed. The relationship between these findings is uncertain but provocative. Children with nevi should have frequent, careful abdominal examinations. The authors express their appreciation to Dr. R. Rodney Howell for his critical appraisal of the manuscript. We thank Mrs. Ruth Person for her assistance in the preparation of this paper. Frank A. Simon. M.D. Department of Pediatrics The University of Texas Medical School at Houston Houston. TX 77030 Jan E. Drutz, M.D. Department of Pediatrics Ba•lor College of Medicine Houston, TX 77030 Joseph N. Corriere, M.D. Department of Surgery The University of Texas Medical School at Houston Houston, TX 77030
REFERENCES 1. Pendergrass TW: Congenital anomalies in children with Wilms' tumor, Cancer 37:403, 1976. 2. Cummins GE, and Cohen D: Cushing's syndrome secondary to ACTH-secreting Wilms' tumor, J Pediatr Surg 9:535, 1974. 3. Omenn GS: Ectopic hormone syndromes associated with tumors in childhood, Pediatrics 47:613, 1971.
