Rhabdomyosarcoma: report of case

June 4, 2017 | Autor: James Gutmann | Categoria: Dentistry, Humans, Female, Rhabdomyosarcoma, Maxilla, Endodontics, Adult, Incisor, Endodontics, Adult, Incisor
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CASE REPORTS

RHABDOMYOSARCOMA: REPORT OF CASE

James L. Gutmann, DDS, Baltimore, and Joel L. Tillman, DMD, Springfield. Mass

Rhabdomyosarcoma of the head and neck is primarily a disease that occurs during the first two decades of life. It is the most common malignant softtissue neoplasm of that anatomic region to develop in childrenA In 1946, Stout 2 reported on 121 cases of rhabdomyosarcoma and presented the first detailed description of the disease. Stobbe and Dargeon '~ were the first to recognize rhabdomyosarcoma of the head and neck as a distinct pathologic entity. Further studies by Moore and Grossi, 4 Edland, 5 and Masson and Soule 6 have substantiated its presence in the head and neck region. Horn and Enterline 7 listed four histologic types of rhabdomyosarcoma--alveolar, embryonal, pleomorphic, and botryoid---and found the embryonal to be the most common form in the head and neck region. Dito and Batsakis s reported on 170 cases of rhabdomyosarcoma in which three had their site of origin in the maxillary antrum, and Masson and Soule 6 reported on 88 cases in which seven had that same site.

Case Report A 26-year-old woman was referred for endodontic therapy on the maxillary left lateral incisor. Her chief complaints were the presence of a diffuse swelling of the left cheek and a slight sensation of pressure felt on the inferior border of the left eye.

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She did not experience any discomfort. Her medical history showed that she had been treated three weeks previously by her physician and then by her general dentist for the swelling. She was taking 500-rag penicillin twice daily. The maxillary left lateral incisor had a lingual endodontic access opening. Periapical radiographs showed a radiolucent area about 4 mm in diameter at the apex of the left lateral incisor (Fig. 1). Attempts to establish drainage and secure resolution by instrumentation of the root canal (Fig 2) and by incision and trephination (Fig 3) were unsuccessful. Vitality tests of adjacent teeth were of no diagnostic value. Further radiographic examination showed complete loss of normal anatomic structure along the inferior border of the maxillary left antrum ( F i g 4). Clinically, the left eye was elevated and proptotic. The patient's discomfort was minimal or absent during treatment. She was referred for further evaluation. Complete skull radiographs (Fig 5) and tomograms taken of the area affected showed destruction of the floor of the orbit. The patient was admitted to a hospital. A Caldwell-Luc antrotomy was performed; the antrum was curetted, and a biopsy of the maxilla was taken. Histologic examination showed a definite embryonal rhabdomyosarcoma (Fig 6).

Further diagnostic tests were performed to ascertain the possible extent of metastasis. Brain and bone scans, radiographs of the long bones, and aspiration of bone marrow showed no abnormalities. Results of an SMA12 survey and a radiograph taken of the chest were normal also. Because of the significant involvment of the orbital contents, it was believed that radical surgery was necessary. An extensive Weber-Fergusson incision was made, and the entire left side of the maxilla, zygoma, orbital contents, and medial and lateral walls of the orbit were resected along with the ethmoid, sphenoid, and frontal sinuses. Skin grafts from the thigh were used to line the flap. The patient was maintained on a regimen of ampicillin and chloromycetin, postoperatively; radiation treatment was anticipated.

Discussion One of the first clinical signs of rhabdomyosarcoma is an asymptomatic swelling or a mass. Growth rate of the mass is very rapid, by way of expansion and infiltration. The majority of these neoplasms in the head and neck arise from unsegmented mesoderm, while a relatively small number arise from skeletal muscle. Histomorphologically, rhabdomyosarcoma can be considered as the neoplastic analogue of the embryogenesis of skeletal muscle. 1

JOURNAL OF ENDODONTICS ] VOL 2, NO 8, AUGUST 1976

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Fig I---Radiograph of maxillary left lateral incisor shows periapical radiolucent area.

Fig 2----Instrumentation of root canal to establish drainage.

Fig 4 Radiograph of maxillary left premolars shows absence of inferior border of sinus and motheaten appearance of lamina dura. Pulps tested vital.

Embryonal rhabdomyosarcoma shows various cellular types and tissue patterns. Spindle-shaped cells; small-to-moderate round eosinophilic cells; broad, elongated eosinophilic cells; polyhedral granular cells; and spider-shaped cells may be present in a loose myxoma or in a compact syncytium (Fig 7). Malignant myoblasts showing cross-

striations may or may not be seen, although their presence is not necessary to make an accurate diagnosis. 1,5 The tendency for distant metastasis is pronounced in all rhabdomyosarcomas, except those originating from the orbit. The lungs, lymph nodes, and bones are the most frequent sites of metastasis. Frequency of recurrence of the tumor is high.

Fig 3---Curet in periapical lesion during trephination.

Although the prognosis is guarded, early, radical surgery and a regimen of chemotherapy and radiation have been surprisingly effective in prolonging the patient's life. 9 Summary A 26-year-old woman with a swelling of the left cheek was treated endodontically for an apical lesion of the maxillary left lateral incisor. Failure to obtain immediate resolution resulted in referral of the patient for further consultation and evaluation of the condition. Diagnostic tests showed the presence of an embryonal rhabdomyosarcoma of the maxillary left antrum. Radical surgery was performed, followed by chemotherapy. The prognosis remains guarded. This case emphasizes the importance of a differential diagnosis based on complete evaluation of the patient's signs and symptoms. Radiographic evaluation alone is insufficient because of the potential for malignancies to 251

IOffRNAL OF ENDODONTICS t VOL 2, NO 8, AffGUST 1978

Fig 5---Complete skull radiograph shows cloudiness and destruction of lateral border and roof of left antrum.

Fig 6~Frozen section of maxillary antrum shows sheets of eosinophilic tumor cells (H & E, orig mag X 250).

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mimic periapical pathosis of pulpal origin. Patients who do not respond to the normal course of treatment should be considered for further diagnostic evaluation. Dr. Gutmann is chairman, department of endodontics, Baltimore College of Dental Surgery, University of Maryland. Dr. Tillman is a member of the attending staff, Medical Center of Western Massachusetts, Springfield. Requests for reprints should be directed to: Dr. James L. Gutmann, Baltimore College of Dental Surgery, University of Maryland, Baltimore, 21201. References 1. Batsakis, J.G. Tumors of the head and neck; clinical and pathological considerations. Baltimore, Williams & Wilkins Co., 1974, 203. 2. Stout, A.P. Rhabdomyosarcoma of skeletal muscles. Ann Surg 123:447 March 1946. 3. Stobbe, G.D., and Dargeon, H.W. 252

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Embryonal rhabdomyosarcoma of the head and neck in children and adolescents. Cancer 3:826 Sept 1950. 4. Moore, O., and Grossi, C. Embryonal rhabdomyosarcoma of the head and neck. Cancer 12:69 Jan-Feb 1959. 5. Edland, R.W. Embryonal rhabdomyosarcoma. Am J Roentgenol 93:671 March 1965. 6. Masson, J.K., and Soule, E.H. Embryonal rhabdomyosarcoma of the head and neck. Report on eighty-eight cases. Am J Surg 110:585 Oct 1965. 7. Horn, R.C., Jr., and Enterline, H.T. Rhabdomyosarcoma: a clinicopathological study and ~lassification of 39 cases. Cancer 11:181 Jan-Feb 1958. 8. Dito, W.R., and Batsakis, J.G. Rhabdomyosarcoma of the head and neck. An appraisal of the biologic behavior in 170 cases. Arch Surg 84:582 May 1962. 9. Nelson, A.J. Embryonal rhabdomyosarcoma. Report of twenty-four cases and study of the effectiveness of radiation therapy upon the primary tumor. Cancer 22:64 July 1968.

Fig 7--Top, sheets of elongated and round tumor cells from floor of an. trum (H & E, orig mag • 160). Center, polyhedral and elongated cells from maxillary sinus (H & E, orig mag • Bottom, neoplastic poly. hedral eosinophilic cells and elongated spindle-shaped cells from maxillary sinus (H • E, orig mag •

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