Short communications & case reports
Clear-cell sarcoma of tendons and aponeuroses William M. Carpenter, D.D.S., M.S.,* Peter J. Tsaknis, D.D.S., M.S., M.Ed.,** Joseph L. Konzelman, D.D.S.,*** and William J. J. Logan, M.D., **** Washington, D. C. UNITED STATES ARMY INSTITUTE OF DENTAL RESEARCH, WALTER REED ARMY MEDICAL CENTER The natural history and histologic characteristics of a tare malignant neoplasm of tendons and aponeurosesare described, and a case is presentedwhich originated in the right foot of a 19-year-old Caucasianwoman who succumbedto the diseaseafter 4 years. This case representsthe first known example of metastasisto the oral cavity of this particular neoplasm.
M alignant neoplasmsthat originate in tendons and aponeurosesare rare, with most
caseshaving been classified as synovial sarcomaor fibrosarcoma until 1965, when Enzinger’ first reported twenty-one casesof a specific clinicopathologic entity. Since the histogenesis was not evident, he suggestedthe descriptive term “clear-cell sarcomaof tendons and aponeuroses.” The neoplasm has a characteristic natural history, and there appearsto be a slight female predilection. It hasoccurred in patients in the agerangeof 12 to 65 years, but the median age at onset of symptoms is approximately 24 years. The duration before treatment is 3.5 years, with an averagesurvival time of 6 years from time of initial treatment. The neoplasm begins as a slowly growing, painless, slightly movable mass. It arises from tendinous structures in the extremities, usually the lower ones, with the foot and ankle being the most common sites. The clinical course is slow but relentless, with a tendency toward repeatedrecurrencesand eventual metastasis.Most often the metastases involve the regional lymph nodes, lungs, skeleton, heart, liver, and brain. The tumors are usually firm and roughly spherical, with a smooth nodular or coarsely lobulated surface. Most of the tumors are well defined and some appear to be encapsulated. The size rangesbetween2 and 12 cm., although when the size was given, only four *Chief, m Pathology Section, Letterman General Hospital, San Francisco, Calif. **Fellow in Electron Microscopy, Division of Oral Biology, U. S. Army Institute of Dental Research,Washington, D. C. ***Office of the Director of Dentistry, HeadquartersHealth Services Command, Ft. Sam Houston, Texas. ****Department of Pathology. U. S. Army Hospital, Camp Zama, Japan.
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of forty tumors exceeded 6 cm. The microscopic features are distinctive and characterized by pale fusifonn cells of epithelioid appearance with indistiuct cytoplasnic borders, round pale nuclei, and prominent nucleoli. Mitotic activity, although present, is not a conspicuous feature. The cellular arrangement is that of solid nests and fascicles of vafying sizes separated by delicate fibrous connective tissue septa. There is a variation in the qpcarance of recurrent and metastatic lesions, with several exhibiting only slight resemblance to the primary lesion. A thorough review of the English language literature revealed only forty-nine cases,2-‘o and metastasis to the oral cavity had not occurred in any of these. Even though bilateral oral metastases occurred late in the course of the widely disseminated condition in the case that we present here, we believe that reporting it is warranted because of the paucity of cases recorded in the literature, as well as the need for prolonged palliative dental treatment of the condition. CASE REPORT
The patient was a 23-year-old Caucasianwoman who, at the age of 19 years, presentedwith a nodule on the dorsum of the right foot. Ihe nodule had been present for at least 5 years. In June, 1970,the patient underwent excision of the massafter it was diagnosedas being a clear-ceil sarcoma of tendonsand aponeuroses.Subsequentrecurrenceof the tumor resulted in a mid-thigh amputation. In mid- 1972, she becamepregnant, but after IO lo 12 weeksof gestation, she underwent therapeutic abortion at Walter Reed General Hospital, becausean examination of the pelvis revealed a Sby-6-cm. masson the pelvic wall. The relentless progressof her diseaseled to metaataticinvolvement of mostof the major organs(brain, liver, kidneys, spleen, adrenals,lungs, and heart), the ribs, both femurs and humeri, the vertebral column, and cranium and bilateral oral metastasesto the left zygomatic bone and the right angle of the mandible. Palpablecervical nodes were noted on the left side of the neck. Radical surgery, radiation, and chemotherapywere of no avail and the patient died in April, 1974. Dental history The patient was referred to the Department of Dentistry at Walter Reed General Hospital on Aug. 8, 1973, with the chief complaint of “numbness of the right lower lip.” No reaction to sharp stimuli could be elicited in this anza. The right mandibular second premolar displayed Class 2 mobility with no evidence of loss of alveolar bone and only a slightly diffuse apical radiolucency. All vitality testsgave normal findings. Her condition was postulatedlo representa metastasisof the primary clear-cell sarcomaand palliative care only was recommended.At the return visit on Oct. 15, 1973, the patient reported intermittent exacerbationand remission of anesthesiaand paresthesia of the left mental region. Swelling was also evident in the left infraorbital and canine fossaareas.NO clearly demarcatedradiographic features were visualized. Since the patient’s main concern was possible facial disfigurement and oral health, assurancewas the most important aspectof care at this time. She was Seenat 2-week intervals at her request. The primary purpose of these visits was emotional suppott. On December 11, the patient reported severepain in the right mandible which did not respond to analgesic medication with Dilaudid (hydromoxphone)2 mg. every 4 hours. The pain was allayed temporarily by injection of an anesthetic.A panograp4icfilm was taken at this time (Fig. I ). The last of the periodic visits occurred on March 28. 1974. In spite of her extremely debilitated physical condition, she was anxious lo have restorationsplaced in several caxiousteeth. The patient’s oral health had been excellent until her physical weaknessprecluded a&quate oral hygiene. The restoration of four molar teeth was accomplished. Extreme cant had to be exercisedin order to prevent iatrogenic fracture of the mandible. A great amount of facial swelling due 10 the
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Fig. 1. A panographic radiograph reveals an unusualreticulated trabecular pattern in the right mandibular angle and ramus.
presenceof intraoral metastasiswas then evident. The patient died 2 weeks later of widely disseminated disease. Microscopic
description
The original surgical specimen (Fig. 2) revealed nests of epithelioid-appearing cells with illdefined cytoplasmic borders. A clear or slightly granular cytoplasm was noted. Generally, round to oval nuclei were apparent,with obvious pleomorphism. A mitotic rate of six to eight atypical figures per ten high-power fields was noted. The nucleoli were distinct and sharply defined; however, the metastaticlesions (Fig. 3) demonstratedincreasedcellular pleomorphism and a decreasedtendency to form clusters or nests.The nucleoli were also less prominent. Special stains for melanin produced negative results. DISCUSSION
In 1947, Bennett” reported a series of malignant neoplasmsoriginating in synovial tissues. Two of the caseswere of a peculiar type. However, Enzinger’ was the first to separatetheseunusual tumors from synovial sarcomasand to delineatethe histopathologic differences. Other cases were previously diagnosed as fibrosarcoma, alveolar soft-part sarcoma, paraganglioma, malignant melanoma, and benign giant-cell tumor of tendon sheath. Although the neoplasm has been recognized for a decade, the cell of origin is still unknown. Electron microscopic studieshave offered two histogenic possibilities. K&o’s’ findings were strongly suggestiveof synovial lining cells. Hoffman and Carte? reported a case which demonstrated melanin in the cytoplasm, and theorized that it might be a melanoma originating from neural-crest cells. Similar findings were noted in Mackenzie’s’O report; however, melanin was not present in most of the casesreported. These findings suggesta variable histogenesis. Possibleroutes of metastasisinclude the systemiccirculation and the lymphatics, since tumor has been found to be present in the lungs and heart along with a histologically
confirmed generalized lymphadenopathy secondaryto the sarcoma.A secondpossibility is via Batson’s vertebral veins, since tumor cells have also been identified in the thoracic and lumbar vertebrae.
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Fig. 2. Photomicrographsof the primary nodule from the dorsnm of the foot. The specimen is composedof groups of round and spindle-shaped, sparsely granulated, or vacuolated cells with prominent nucleoli. (Hematoxylin and eosin stain. Magnification: X 100, inset, X 250.)
Fig. 3. Photomicrograpbof the mandibular metastasisshowing increasedcellular pieomorphismand lessdistinct nucleoli. (Hematoxylin and eosin stain. Magnification, x 350.)
Reportsin the literature involving metastasisto the jaws are uncommon. Metastasisto osseoustissue was found in 27 percent of 1,000 autopsied casesby Abrams and associates,‘* but Geschickter and Copelandt3reported that the jaws were involved in less than 1 percent. Undoubtedly, many casesare not reported or remain undiscovered becauseof a lack of careful observation of this anatomic areain the late terminal or postmortemperiod. In a series of casesof jaw metastasisby Meyer and Shklar,14seventeenof twenty tumors
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were metastatic to the mandible, and only three to the maxilla. The reason for this disparity is unknown but may be due to the contrasting vascularization of these bonesor their hemopoietic capacity. Even more uncommon are reports of metastaticlesions in the soft tissues of the oral cavity. In a review of the literature by Hatziotis and associates,‘” only forty-eight caseswere found from 1945 to 1970. The gingiva was the site of metastasis in nearly 50 percent in their series, whereasthe lung appearsto be the most common site of the primary tumor according to Solomon and associates.i6 The histopathologic diagnosis in a high percentageof casesof metastaticneoplasmsto the jaws is adenocarcinoma. The primary source should be diligently sought for in the breast, prostate, gastrointestinal tract, thyroid, and parotid gland. Although the diagnosis in this casewas known long before the oral metastasis,this is not always so. Bhaskar” has emphasizedthat oral metastatictumors are the initial indication of a distant primary tumor in one-third of cases.Ominous signs and symptoms normally associatedwith metastases to the jaws are pain, swelling, numbness,and loosening of teeth. Our patient experienced all these symptoms. Since our patient’s right mandible was extensively involved by the malignant process, there was very little hard, osseousstructure still present in the ramus area. However, spontaneousfracture had not occurred. Metastasesto intraoral soft tissue often appearas rapidly growing masseswhich tend to bleed and ulcerate. In this case a swelling was presenton the gingiva opposite the left maxillary first molar, and this probably represented a direct extension of the zygomatic mass. Unfortunately, this specimen could not be obtained to verify the true nature of the mass. The anterior two-thirds of the tongue was available for histologic examination, but neoplastic cells were not present. The baseof the tongue was not examined histologically. This areashould be evaluatedthoroughly. Zegarelli and associates’*found that the baseof the tongue was involved in eight of twelve casesof metastasisto this structure. It must be emphasizedthat the dental aspectof this patient’s terminal treatmentwas of the utmost importance. Even though the treatment was mainly supportive and palliative, it made the patient’s final suffering easier for her to endure. The empathy of the dentist is a factor which can completely change the outlook for the emotionally distraught patient. Our patient repeatedly expressed the feeling that oral care was one of her strongest terminal needs, and she was extremely grateful for the consideration shown. SUMMARY
Reported is a caseof clear-cell sarcomaof tendonsand aponeurosesoriginating in the right foot of a 19-year-old Caucasianwoman. The clinical and histologic characteristics are discussed. The patient died from widespread metastases4 years after the initial operative intervention. This is the first reported caseof metastasisof this neoplasmto the oral cavity. RERRENCES 1. Enzinger, F. M.: Clear-Cell Carcinoma of Tendons and Aponeuroses, An Analysis of 21 Cases, Cancer 18: 1163-I 174, 1965. 2. McClatchie, S.: An Example of Clear-Cell Sarcoma of Tendon, East Afr. Med. J. 46: 524-526, 1969. 3. Hirata, K.: Clear-Cell Sarcoma Arising From the Right Plantar Aponeurosis, Orthop. Surg. [Tokyo) 2& 1326-1328, 1969. 4. Morimoto, N.: Clear-Cell Sarcoma of the Heel, Jap. J. Clin. Pathol. 17: 60-61, 1969.
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5. Angervall. L.. and Stcner. B.: Clear-Cell Sarcoma of Tendons-A Study of Four Cases, Acta Pathol. Microbial. Scand. 7’1: 589-597, 1969. 6. Mackenzie, D. H.: Two Types of Soft Tissue Sarcoma of Uncertain Histogenesis. Br. J. Cancer. 25: 458-461, 1971. 7. Kuho, T.: Clear-Cell Sarcomaof Patellar Tendon Studied by Electron Microscopy. Cancer 2rl: 948-953. 1969. 8. Hoffman, G. J., and Carter, D.: Clear-Cell Sarcomaof Tendons and AponeurosesWith Melanin. Arch. Pathol. 95: 22-25, 1973. 9. Dutra, F. R.: Clear-Cell Sarcoma of Tendons and Aponeuroses; Three Additional Cases, Cancer 25: 942-946, 1970. IO. Mackenzie, D. H.: Clear-Cell Sarcomaof Tendonsand AponeurosesWith Melanin Production, J. Pathol. 114: 231-235, 1974. I I. Bennett, G. A.: Malignant Neoplasms Originating in Synovial Tissue, J. Bone Joint Surg. (Br.) 29: 259-291, 1947. 12. Abrams. H. L., Sprio, R., and Goldstein, N.: Metastasisin Carcinoma; Analysis of 1000Autopsied Cases. Cancer 3: 74-85, 1950. 13. Geschickter, C. F.. and Copeland, M. M.: Tumors of Bone, Philadelphia. 1949.J. B. Lippincott Company, pp. 472-536. 14. Meyer, I.. and Shklar, G.: Malignant Tumors Metastatic to Mouth and Jaws, ORAL SURG. 20: 350-362. 1965. 15. Hatziotis, J. C.. Constantinidou, H., and Papanayotou.P. H.: Me&static Tumors of the Oral Soft Tissues, ORALSURG.36: 544-550. 1973. 16. Solomon, M. P.. Rosen, Y., Gormley. M., and Jartett, W.: Metastatic Lesions to the oral Soft Tissues, J. Oral Surg. 33: 53-56. 1975. 17. Bhaskar, S. N.: Oral Manifestations of Metastatic Tumors, Postgrad. Med. 49: 155-158, 1971. 18. Zegarelli, D. J., Tsukada, Y., Pi&en, J. W., and Greene, G. W.: Metastatic Tumor to the Tongue, ORAL SURG. 3s: 202-211, 1973. Reprint
requests to:
Lt. Col. Peter J. Tsaknis Division of oral Biology U. S. Army Institute of Dental Research Walter Reed Army Medical Center Washington, D. C. 20012