Adult rhabdomyoma of the esophagus

Adult Rhabdomyoma of the Esophagus By Ganesh K. Pai, Pushpalatha K. Pai, and Suresh M. Kamath Mangalore, India

9 Rhabdomyoma is a rare benign tumor of striated muscle origin. T w o distinct histologic groups, adult and fetal type, are recognized. So far only 66 cases of extracardiac rhabdomyomas (37 fetal and 29 adult) have been documented in the literature reviewed. The present report details a case of adult rhabdomyoma of the esophagus in an 8-year-old boy. 9 1987 by Grune & Stratton, Inc. INDEX WORDS: Adult rhabdomyoma; esophageal rhabdomyoma.

CASE REPORT An 8-year-old boy came to medical attention in October 1981 because of progressively increasing inspiratory stridor of 11/2years' duration. He had minimal difficulty in eating solids. Earlier, he was put on antitubercular drugs, with a clinical diagnosis of bronchial asthma with primary complex by a pediatrician. On examination the boy appeared to be in moderate respiratory distress. There was severe retraction of the lower intercostal spaces. Physical examination was unremarkable but for the bilateral basal crepitations. Laboratory evaluation of this patient revealed normal blood chemistry. A chest roentgenogram was normal. Plain roentgenogram of the neck and upper chest revealed a soft tissue density pushing the trachea in front like a bow string (Fig 1). Barium swallow outlined a mass between the trachea and the esophagus. Cervical and upper thoracic esophagus was pushed posteriorly (Fig 2) and to the left (Fig 3). The neck was explored from the right side. A well-encapsulated tumor measuring 9 x 5 x 4 cm arising from the musculature of the cervical and upper thoracic esophagus was removed. The boy made

Fig 1. Neck and upper chest, lateral view x-ray showing trachea compressed like a bow string.

Journal of PediatricSurgery, Vol 22, No 11 (November),1987: pp 991-992

Fig 2. Barium swallow. Lateral view showing esophagus compressed posteriorly by the tumor.

an uneventful postoperative recovery. There was no evidence of recurrence during 4 years of follow-up. Histologic examination revealed a well-encapsulated tumor composed of well-outlined muscle fibers of varying sizes arranged in different directions (Fig 4). Phospbotungstic acid hematoxylin stain showed clear cross striations in the majority of the cells (Fig 5). Some sections showed fibrous tissue and fat. DISCUSSION

Rhabdomyoma was first described by Zenker in 1864 as a tumor of striated muscle cells with varying degrees of differentiation and maturity. Extra cardiac rhabdomyomas are distinct from and should not be confused with cardiac rhabdomyomas. These so-called cardiac rhabdomyomas, seen in association with the tuberous sclerosis complex, are probably not true neoplasms. I Benign extracardiac rhabdomyomas have been divided into two distinct histologic groups, adult and fetal, depending on the degree of differentiation of the cells) Adult type rhabdomyomas are histologically characterized by well-differentiated cells with cross striations. ~ These occur exclusively in the head and neck region (93%), particularly larynx and pharynx of adult males) Occasional multifocal rhabdomyomas are reported in the literature.4-6 In 1975, Ferlito and Frugoni7 reviewed 50 cases of From the Departments of Pediatric Surgery and Pathology, Kasturba Medical College, Mangalore, India. Address reprint requests to Ganesh K. Pal, MS, Pediatric Surgeon, Hemavathi Building, Balmatta, Mangalore-575001, India. 9 1987 by Grune'& Stratton, Inc. 0022-3468/87/2211-0005503.00/0

991

992

PAl, PAl, AND KAMATH

Fig 5. Well-defined cross striations (arrow) in muscle fibers (Phosphotungstic acid hematoxylin, original magnification, x400).

Fig 3. Barium swallow. Anteroposterior view showing esophagus pushed to the left by the tumor.

Fig 4. Photomicrograph showing muscle fibers of varying sizes arranged in different directions (H&E, original magnification, x150}.

rhabdomyomas and documented one case of laryngeal rhabdomyoma in a 50-year-old male. In the same year, Shapiro et al 8 reviewed 20 cases of adult variant and added one from their own experience, occurring in a 221/2-month-old female child. In 1980, di Sant Agnese and Knowles3 reviewed 66 cases, 51 acceptable cases from the literature and 15 from their own experience. Out of these 66 cases, 29 were adult and 37 were of the fetal variant. Age incidence in the adult type ranged from 8 to 82 years with a male to female ratio of more than 4:1. Only two out of 29 adult rhabdomyomas occurred in children. After di Sant Agnese and Knowles, two more cases of rhabdomyomas have been added to the world literature, one by Scrivner and Meyer6 and the other by Modlin and Spring: both cases being found in adults. Tumors in reported cases were well encapsulated with few exceptions:s Local recurrence after excision biopsy is rare except for occasional reports in the literature: '9 Rhabdomyoma of the esophagus has not been reported previously in the English literature to the authors' knowledge.

REFERENCES I. Rosai J: Ackerman's Surgical Pathology (ed 6). St Louis, Mosby, 1981, p 1449 2. Stout AP, Lattes R: Tumors of the soft tissues. Armed Forces Institute of Pathology, Washington De, 1967, pp 64-66 3. di Sant Agnese PA, Knowles DM: Extracardiac rhabdomyoma, a clinicopathologic study and review of the literature. Cancer 46:780-789, 1980 4. Goldman RL: Multicentric benign rhabdomyoma of skeletal muscle. Cancer 16:1609-1613, 1963 5. Modlin B, Spring S: Rhabdomyoma of the larynx. Laryngoscope 92:580-582, 1982

6. Scrivner D, Meyer JS: Multifocal recurrent adult rhabdomyoma. Cancer 46:790-795, 1980 7. Ferlito A, Frugoni P: Rhabdomyoma purum of the larynx. J Laryngol Otol 89:1131-1141, 1975 8. Shapiro RS, Stool SE, Snow JB, et al: Parapharyngeal rhabdomyoma. Arch Otolaryngol 101:323-326, 1975 9. Fu YS, Perzin KH: Non epithelial tumors of the nasal cavity, paranasal sinuses and nasopharynx. A clinicopathologic study. V. Skeletal muscle tumors (rhabdomyoma and rhabdomyosarcoma). Cancer 37:364-376, 1976