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Pleomorphic rhabdomyosarcoma of the gingiva
Pleomorphic rhabdomyosarcoma of the gingiva Report
of a case
Thanos M. Kaloyannides, DEPARTMENT
D.D.S., Thessalodii,
OF STOMATOLOGY,
SCHOOL
Greece
OF DENTISTRY,
THESSALONIKI
UNIVERSITY
R
habdomysarcoma is a malignant tumor of mesenchymal origin which occurs mainly in the extremities and skeletal muscles and is rarely seen in the region of the head and neck or in the oral cavity. Weber,2, 3 in 1854, was the first to describe such a malignant tumor of striated muscle located in the tongue. In 1946 Stout’ conducted recondite studies on rhabdomysarcomas, and in 1950 Sto’bbe and DargeorP described a completely different variety of rhabdomyosarcoma which presented embryonal histologic features and tended to appear in the head and neck region of children and young adults. In 1958 Horn and Enterline finally classified rhabdomyosarcomas into four groups-embryonal, pleomorphic, alveolar, and botryoid-and presented a detailed description of the main characteristics of each group. This classification subsequently was approved internationally. HISTOLOGY
Histologically, pleomorphic rhabdomyosarcoma consists of bundles of spindleshaped cells which show a variable number of mitoses as well as dark-staining and polymorphic features ; ‘I striated” or “racket” cells with multiple nuclei are found scattered in the field. These cells have abundant cytoplasm and may show oblong fibrils and cross striations which become evident when stained by special methods. The presence of cross striations establishes a secure diagnosis, but it is not altogether necessary. SYMPTOMS
AND
SIGNS
Because these tumors are completely painless, the main symptom in the first stage is the presence of a fleshy mass of variable shape which is hard and elastic, 150
Volume 27 Number 2
Pleomorphic rhabdomyosarcoma
151
strongly adherent to the surrounding tissues, and reddish in appearance. However, such symptoms as pain, dysphonia, dysphagia, and trismus may appear during the progress of the disease, depending upon the location of the tumor. The growth of the tumor is sometimes slow and sometimes rapid; the patient usually seeks medical care 1 to 6 months after the appearance of the first symptoms. Involvement of local lymph nodes is not frequent. Males are affected twice as often as females. Pleomorphic rhabdomyosarcoma occurs usually during the fifth decade of life. Differentiation from other malignant tumors will be necessary. RECURRENCE AND METASTASIS Rhabdomyosarcomas show either local recurrences or distal metastases. The latter follow the bloodstream rather than the lymph stream. The time of appearance of local recurrence and distal metastasis varies, according to various authors, from 2 weeks to 24 months, regardless of treatment, but they usually occur 3 months postoperatively. PROGNOSIS AND SURVIVAL The prognosis for patients with these tumors is very poor. Dito and Batsakis2 report a 5-year survival rate of only 5.94 per cent for rhabdomyosarcomas of the head and neck and 33.89 per cent for peripheral rhabdomyosarcomas. The same authors have found that survival from the time of diagnosis averages 22 months. Stobbe and Dargeon6 lowered this time to 17 months, and Moore and Gross? report that the average survival period is 10 months. TREATMENT Several authors recommend wide surgical excision of the tumor, whereas others insist on radiotherapy and still others favor a combination of wide surgical excision followed by irradiat.ion of the area. This latter process seems to give the best results.
Pig. 1. Site and gross appearance
of intraoral
tumor.
O.S., O.&l. & 0.1'. February,
1969
CASE REPORT A 51-year-old white man came lo the IDepartment of Stomatology of the University of Thessaloniki on Jan. 11, 1966, for treatment of a growth in the right half of the mandible. He had first noticed the tumor 22 days earlier, when it had the size of a hazelnut. The tumor had enlarged rapidly and impaired mastication, with pain am1 henlorrhage appearing in response to pressure from the t&h. Examination
Examination revealed an extraoral swelling of the right half of the mandible corresponding to the premolar and molar segments. Intraorally, a tumor was found arising from the gingiva of the missing first and second right premolars of the mandible. This tumor, which had the size of a small walnut, occupied the space between the second premolar and the third molar and expanded lingually and buccally. The growth rose to the height of the masticatory surfaces of the lower teeth and bore the imprints of the opponent upper teeth (Fig. 1). The upper surface of the tumor was whitish in color, while the lower surface was dark red. The tumor was hard and elastic in consistency. Infiltrated lymph nodes were not found. Intraoral x-ray examination revealed no osseous lesions (Figs. 2 and 3). Treatment
An excisional biopsy was performed immediately. Following administration of a local anesthetic, the tumor was excised with a scalpel and the surrounding tissues were coagulated by diathermy. The histologic examination of the surgical specimen was performed in the Department of Pathology of the Medical School of the University of Thessaloniki. The gross specimen consisted of a tumor measuring 3.5 by 1.8 by 1.4 cm. (Fig. 4). The surface of the
Figs.
I and 9. Roentgenograms
revealing
no osseous involvement.
Fig. 4. Gross appearance
Figs. 5 and 6. High-power stained nucIei with abundant (Magnification, x120.)
and size of surgical
specimen.
photomicrograph showing several hyperchromic, eosinophilic cytoplasm in which some striations
Fig.
5
Fig.
6
irregular, deeply can be observed.
Fig. 7. Photograph growth is clearly seen.
shol\-ing
recurrence
ef tumor
4 months
after
initial
operation.
New
showed focal ulcerations. The cut surfaces growth, covered mostly by squamous epithelium, were rather soft and grayish white in color. Microscopic examination revealed that the tumor was a pleomorphic rhabdomyosarcoma, composed of bundles of collagen and muscle fibers running in various directions and intertwining with each other. These bundles consisted of large spindle-shaped cells with abundant eosinophilic cytoplasm and large, bizarre nuclei. Numerous mitoses were observed. The squamous epithelium showed focal hyperplastic areas as well as ulcerations and polymorphonuclear leukoeytic exudate (Figs. 5 and 6). The postoperative course was uneventful, and the healing of the wound was complete. It was decided, in accordance with the radiologist’s suggestion, that it would be wise to avoid radiation, at least for the time being, and to keep in constant touch with the patient. On April 15, 1966, the patient returned, complaining of the tumor’s reappearance in the same place. The local recurrence had been noticed by the patient 15 days before. Examination revealed a small tumor, the size of a pea, situated in the same place as previously. It was hard and elastic in consistency, with a broad base and a deep red color (Fig. 7). Infiltrated lymph nodes were not found. X-ray examination showed no osseous involvement. A surgical excision of the tumor was performed, together with curettage of the bone and cauterization of the surrounding tissues with diathermy. Histologic examination confirmed the previous diagnosis of pleomorphic rhabdomyosarcoma. The patient was then taken to the Anticancer Institute, where immediate irradiation of the area was performed. Six months later the tumor recurred once more. The new growth was excised, along with a very wide area of healthy tissues, and the patient was released after receiving 5,000 r of radiation. The patient has shown no signs of rccurrcncc to date.
DISCUSSION
As mentioned previously, rhabdomyosarcoma rarely occurs in the oral cavity. We have not been able to find any record whatsoever of pleomophic rhabdomyosarcoma occurring primarily in the gingiva. Therefore, we have presented the foregoing case, believing it to be the first of its kind reported. We wish to point out that, clinically, this tumor resembles the common innocent giant-cell epulis. This fact emphasizes the importance of a histologic examination in every case of hyperplasia.
Pleomorphic
rhabdomyosarcoma
155
The age, the rapid growth, and the rapid reccurrence of the tumor in the present case are in complete agreement with the generally accepted behavior of the rhabdomyosarcoma. REFERENCES
1. Bernier, J. L.: The Management of Oral Disease, ed. 2, St. Louis, 1959, The C. V. Mosby Company, p. 832. 2. Dito, W. R., and Batsakis, 5. G.: Intraoral, Pharyngeal and Nasopharyngeal Rhabdomyosarcoma, Arch. Otolaryng. 77: 123-128, 1963. 3. Dito, W. R., and Batsakis, J. G.: Rhabdomyosarcoma of the Head and Neck, Arch. Burg. 84: 582-588, 1962. 4. Horn, R. C., and Enterline, H. T.: Rhabdomyosarcoma: A Clinico-pathological Study and Classification of 39 Cases, Cancer 11: 181-199, 1958. 5. Moore, O., and Grossi, C.: Embryonal Rhabdomyosarcoma of the Head and Neck, Cancer 12: 69-73, 1959. 6. Stobbe, G. D., and Dargeon, H. W.: Embryonal Rhabdomyosarcoma of Head and Neck in Children and Adolescents, Cancer 3: 826-836, 1950. 7. Stout, A. P.: Rhabclomyosarcoma of Skeletal Muscles, Ann. Surg. 123: 447-472, 1946. 8. Thoma, K. H., and Goldman, H. M.: Oral Pathology, ed. 5, St. Louis, 1960, The C. V. Mosby Company, pp. 903, 1388. 49 Hermou
St.
of a case
Thanos M. Kaloyannides, DEPARTMENT
D.D.S., Thessalodii,
OF STOMATOLOGY,
SCHOOL
Greece
OF DENTISTRY,
THESSALONIKI
UNIVERSITY
R
habdomysarcoma is a malignant tumor of mesenchymal origin which occurs mainly in the extremities and skeletal muscles and is rarely seen in the region of the head and neck or in the oral cavity. Weber,2, 3 in 1854, was the first to describe such a malignant tumor of striated muscle located in the tongue. In 1946 Stout’ conducted recondite studies on rhabdomysarcomas, and in 1950 Sto’bbe and DargeorP described a completely different variety of rhabdomyosarcoma which presented embryonal histologic features and tended to appear in the head and neck region of children and young adults. In 1958 Horn and Enterline finally classified rhabdomyosarcomas into four groups-embryonal, pleomorphic, alveolar, and botryoid-and presented a detailed description of the main characteristics of each group. This classification subsequently was approved internationally. HISTOLOGY
Histologically, pleomorphic rhabdomyosarcoma consists of bundles of spindleshaped cells which show a variable number of mitoses as well as dark-staining and polymorphic features ; ‘I striated” or “racket” cells with multiple nuclei are found scattered in the field. These cells have abundant cytoplasm and may show oblong fibrils and cross striations which become evident when stained by special methods. The presence of cross striations establishes a secure diagnosis, but it is not altogether necessary. SYMPTOMS
AND
SIGNS
Because these tumors are completely painless, the main symptom in the first stage is the presence of a fleshy mass of variable shape which is hard and elastic, 150
Volume 27 Number 2
Pleomorphic rhabdomyosarcoma
151
strongly adherent to the surrounding tissues, and reddish in appearance. However, such symptoms as pain, dysphonia, dysphagia, and trismus may appear during the progress of the disease, depending upon the location of the tumor. The growth of the tumor is sometimes slow and sometimes rapid; the patient usually seeks medical care 1 to 6 months after the appearance of the first symptoms. Involvement of local lymph nodes is not frequent. Males are affected twice as often as females. Pleomorphic rhabdomyosarcoma occurs usually during the fifth decade of life. Differentiation from other malignant tumors will be necessary. RECURRENCE AND METASTASIS Rhabdomyosarcomas show either local recurrences or distal metastases. The latter follow the bloodstream rather than the lymph stream. The time of appearance of local recurrence and distal metastasis varies, according to various authors, from 2 weeks to 24 months, regardless of treatment, but they usually occur 3 months postoperatively. PROGNOSIS AND SURVIVAL The prognosis for patients with these tumors is very poor. Dito and Batsakis2 report a 5-year survival rate of only 5.94 per cent for rhabdomyosarcomas of the head and neck and 33.89 per cent for peripheral rhabdomyosarcomas. The same authors have found that survival from the time of diagnosis averages 22 months. Stobbe and Dargeon6 lowered this time to 17 months, and Moore and Gross? report that the average survival period is 10 months. TREATMENT Several authors recommend wide surgical excision of the tumor, whereas others insist on radiotherapy and still others favor a combination of wide surgical excision followed by irradiat.ion of the area. This latter process seems to give the best results.
Pig. 1. Site and gross appearance
of intraoral
tumor.
O.S., O.&l. & 0.1'. February,
1969
CASE REPORT A 51-year-old white man came lo the IDepartment of Stomatology of the University of Thessaloniki on Jan. 11, 1966, for treatment of a growth in the right half of the mandible. He had first noticed the tumor 22 days earlier, when it had the size of a hazelnut. The tumor had enlarged rapidly and impaired mastication, with pain am1 henlorrhage appearing in response to pressure from the t&h. Examination
Examination revealed an extraoral swelling of the right half of the mandible corresponding to the premolar and molar segments. Intraorally, a tumor was found arising from the gingiva of the missing first and second right premolars of the mandible. This tumor, which had the size of a small walnut, occupied the space between the second premolar and the third molar and expanded lingually and buccally. The growth rose to the height of the masticatory surfaces of the lower teeth and bore the imprints of the opponent upper teeth (Fig. 1). The upper surface of the tumor was whitish in color, while the lower surface was dark red. The tumor was hard and elastic in consistency. Infiltrated lymph nodes were not found. Intraoral x-ray examination revealed no osseous lesions (Figs. 2 and 3). Treatment
An excisional biopsy was performed immediately. Following administration of a local anesthetic, the tumor was excised with a scalpel and the surrounding tissues were coagulated by diathermy. The histologic examination of the surgical specimen was performed in the Department of Pathology of the Medical School of the University of Thessaloniki. The gross specimen consisted of a tumor measuring 3.5 by 1.8 by 1.4 cm. (Fig. 4). The surface of the
Figs.
I and 9. Roentgenograms
revealing
no osseous involvement.
Fig. 4. Gross appearance
Figs. 5 and 6. High-power stained nucIei with abundant (Magnification, x120.)
and size of surgical
specimen.
photomicrograph showing several hyperchromic, eosinophilic cytoplasm in which some striations
Fig.
5
Fig.
6
irregular, deeply can be observed.
Fig. 7. Photograph growth is clearly seen.
shol\-ing
recurrence
ef tumor
4 months
after
initial
operation.
New
showed focal ulcerations. The cut surfaces growth, covered mostly by squamous epithelium, were rather soft and grayish white in color. Microscopic examination revealed that the tumor was a pleomorphic rhabdomyosarcoma, composed of bundles of collagen and muscle fibers running in various directions and intertwining with each other. These bundles consisted of large spindle-shaped cells with abundant eosinophilic cytoplasm and large, bizarre nuclei. Numerous mitoses were observed. The squamous epithelium showed focal hyperplastic areas as well as ulcerations and polymorphonuclear leukoeytic exudate (Figs. 5 and 6). The postoperative course was uneventful, and the healing of the wound was complete. It was decided, in accordance with the radiologist’s suggestion, that it would be wise to avoid radiation, at least for the time being, and to keep in constant touch with the patient. On April 15, 1966, the patient returned, complaining of the tumor’s reappearance in the same place. The local recurrence had been noticed by the patient 15 days before. Examination revealed a small tumor, the size of a pea, situated in the same place as previously. It was hard and elastic in consistency, with a broad base and a deep red color (Fig. 7). Infiltrated lymph nodes were not found. X-ray examination showed no osseous involvement. A surgical excision of the tumor was performed, together with curettage of the bone and cauterization of the surrounding tissues with diathermy. Histologic examination confirmed the previous diagnosis of pleomorphic rhabdomyosarcoma. The patient was then taken to the Anticancer Institute, where immediate irradiation of the area was performed. Six months later the tumor recurred once more. The new growth was excised, along with a very wide area of healthy tissues, and the patient was released after receiving 5,000 r of radiation. The patient has shown no signs of rccurrcncc to date.
DISCUSSION
As mentioned previously, rhabdomyosarcoma rarely occurs in the oral cavity. We have not been able to find any record whatsoever of pleomophic rhabdomyosarcoma occurring primarily in the gingiva. Therefore, we have presented the foregoing case, believing it to be the first of its kind reported. We wish to point out that, clinically, this tumor resembles the common innocent giant-cell epulis. This fact emphasizes the importance of a histologic examination in every case of hyperplasia.
Pleomorphic
rhabdomyosarcoma
155
The age, the rapid growth, and the rapid reccurrence of the tumor in the present case are in complete agreement with the generally accepted behavior of the rhabdomyosarcoma. REFERENCES
1. Bernier, J. L.: The Management of Oral Disease, ed. 2, St. Louis, 1959, The C. V. Mosby Company, p. 832. 2. Dito, W. R., and Batsakis, 5. G.: Intraoral, Pharyngeal and Nasopharyngeal Rhabdomyosarcoma, Arch. Otolaryng. 77: 123-128, 1963. 3. Dito, W. R., and Batsakis, J. G.: Rhabdomyosarcoma of the Head and Neck, Arch. Burg. 84: 582-588, 1962. 4. Horn, R. C., and Enterline, H. T.: Rhabdomyosarcoma: A Clinico-pathological Study and Classification of 39 Cases, Cancer 11: 181-199, 1958. 5. Moore, O., and Grossi, C.: Embryonal Rhabdomyosarcoma of the Head and Neck, Cancer 12: 69-73, 1959. 6. Stobbe, G. D., and Dargeon, H. W.: Embryonal Rhabdomyosarcoma of Head and Neck in Children and Adolescents, Cancer 3: 826-836, 1950. 7. Stout, A. P.: Rhabclomyosarcoma of Skeletal Muscles, Ann. Surg. 123: 447-472, 1946. 8. Thoma, K. H., and Goldman, H. M.: Oral Pathology, ed. 5, St. Louis, 1960, The C. V. Mosby Company, pp. 903, 1388. 49 Hermou
St.