0ncology

Primary intraosseoussquamous carcinoma

Susan M(iller, Charles A, Waldron Department of Oral Pathology, Emory University, Atlanta, Georgia, USA

Report of two cases S. Miiller, C. A. Waldron: Primary intraosseous squamous carcinoma. Int. J. Oral Maxillofae. Surg. 1991; 20: 362-365. Abstract. Two cases of primary intraosseous squamous carcinoma (PIOSC) are reported. One PIOSC is in the mandible of a 24-year-old m a n and appears to be a keratinizing PIOSC arising de novo. The other PIOSC presented in the edentulous maxilla of a 56-year-old m a n and was diagnosed as a PIOSC arising from an odontogenic cyst. The 2nd tumor subsequently metastasized to a cervical lymph node. Previous reports have noted a favorable prognosis for PIOC arising in odontogenic cysts, and only 3 other instances of metastases of a PIOSC in an odontogenic cyst have been documented.

An u n c o m m o n tumor, primary intraosseous carcinoma (PIOC) is a carcinoma arising within the jaw. According to the World Health Organization's (WHO) classification there must be no initial connection with the oral mucosa, overlying skin or antral or nasal mucosa. Metastases from distant sites must be excluded by clinical evaluation. Since PIOC is rare in bones other than the jaws, it presumably arises from residues of odontogenic epithelium. In a similar manner, the central mucoepidermoid carcinoma, in which salivary or mucous gland tissue undergoes neoplastic change, is found solely in the jaws. The terminology and classification of this entity has continued to evolve since 1913 when the first case of central epidermoid carcinoma of the jaw was described by Loos 8. ELZAY reviewed the subject of PIOC in 1982 and suggested a modification of the W H O classification 3. This proposed classification was modified slightly by SLOOTWEG & MOI~LER in 198514. More recently, WALDRON& MUSTOErecommended adding one more type to these classifications of PIOC 19. The proposed classification is as follows: Type 1: PIOC ex odontogenic cyst Type 2A: Malignant ameloblastoma Type 2B: Ameloblastic carcinoma arising de novo, ex ameloblastoma or ex odontogenic cyst Type 3: PIOC arising de novo; a)keratinizing type b)nonkeratinizing type Type 4: Intraosseous (central) mucoepidermoid carcinoma

This report describes 2 cases of PIOC. One is in the left mandible associated with the 1st and 2nd molars in a 24year-old male, the other presented in the maxilla of a 56-year-old edentulous male. The following discussion will be limited to the review of PIOSC of either Type 1 (with origin in an odontogenic cyst), or Type 3 (arising de novo). Report of two cases Case 1.

A 24-year-old man presented to his family dentist with a complaint of aching and pain in the left mandible molar area. After a preliminary evaluation revealed tooth mobility and bone loss, the patient was referred to an oral and maxillofacial surgeon for further evaluation.

Fig. 1. Panoramic radiograph revealing a radiolucency around tooth 18.

Key words: cyst; intraosseous squamous carcinoma Accepted for publication 31 July 1991

A panoramic radiograph demonstrated significant bone loss in the left mandibular area (Fig. 1). Exploration of the area around the molar teeth revealed granulation-like tissue with necrotic debris. This soft tissue was sent for microscopic examination and demonstrated marked proliferation of stratified squamous epithelium; however, no frank malignant changes were noted. The patient was scheduled for the removal of the mobile first molar. After extraction, an irregular rubber-like nodule of tissue was noted in the alveolar bone. Due to the involvement of the 2nd molar with this lesion, this tooth was removed as well. The lesion shelled out easily from the tooth socket. Histological examination revealed islands of keratinizing strati-

Fig. 2. Computed tomographic scan reveals an osteolytic defect of the lingual border of the left mandible.

P r i m a r y intraosseous s q u a m o u s carcinoma

Fig. 3. Several well-differentiated islands of s q u a m o u s cell carcin o m a are present adjacent to the root surface (H & E, x 100).

fled squamous epithelium showing numerous keratin pearls and hyperchromatic atypical cells. A diagnosis of well-differentiated squam o u s cell carcinoma was rendered and the patient was scheduled for a mandibular resection. A computed tomographic scan of the

head revealed a destructive t u m o r without soft tissue involvement (Fig. 2). Clinical and endoscopic evaluations were negative as was a chest X-ray. A left hemimandibulectomy with a radical neck dissection was performed and the specimen sent en bloc for histological

Fig. 4. Panoramic radiograph demonstrating a poorly circumscribed radiolucency in the left anterior maxilla.

363

examination. Microscopic examination of the various sections of the mandible demonstrated invasive well-differentiated squamous cell carcinoma adjacent to the root surface and extending into bone (Fig. 3). The soft tissue and bony margins were free of neoplastic involvement. All 47 cervical lymph nodes were negative for metastatic cancer as was the left spinal accessory lymph node. Both the partially resected parotid gland and the submandibular gland were free of tumor. The patient has remained free of disease for 18 m o n t h s after surgery. Case 2

A 56-year-old edentulous m a n presented to an oral and maxillofacial surgeon with a large mass on the left buccal mucosa. The clinical impression was a fibroma and the surgeon removed the lesion and submitted it for histological examination. The patient also complained of a bad taste in his mouth, which became increasinlgy noticeable during the last 6 months. U p o n examination, drainage was noted in the left maxillary canine area. A panoramic X-ray revealed a large, poorly circumscribed radiolucency (Fig. 4). U n d e r local anesthesia, the lesion was totally removed and submitted for examination. Erosion of the buccal and palatal bone was noted intraoperatively. Microscopic examination of the lesion from the buccal mucosa revealed a fibroma. The intraosseous lesion grossly appeared as a cyst measuring 3 × 2 x 1 cm. The cyst was partially lined by hyperplastic stratified squamous epithelium which was altered by the intense inflammatory cell infiltrate (Fig. 5A). In areas the epithelium exhibited prominent dysplastic changes characterized by pleomorphic and hyperchromatic cells, and an abnormal epithelial maturation sequence (Fig. 5B). In some

Fig. 5. (A) Cyst lumen lined by keratinized stratified squamous epithelium H & E, x 40. (B) A portion of the residual cyst lining with prominent dysplastic changes (H & E, x 100).

Fig. 6. C o m p u t e d tomographic scan reveals a large osteolytic lesion in the left maxilla.

364

Miiller and Waldron

areas superfical invasion of the malignant cells was noted. A computed tomographic scan of the head and neck revealed a large osteolytic area in the left maxilla (Fig. 6). A left hemimaxillectomy was performed and the specimen sent for histological examination. The premaxilla demonstrated granulation tissue with marked inflammation, necrosis and benign squamous epithelium showing reactive atypia; however, no carcinoma was identified. The hemimaxilla showed a large cystic lesion with extensive necrosis, granulation tissue and reactive bone formation. No residual carcinoma was identified. During a post-operative examination 9 weeks later a soft mass in the left submandibular area was found. A fine needle aspiration of the mass was performed under local anesthesia. Approximately 2 ml of a cloudy fluid was obtained which was positive for metastatic squamous cell carcinoma. A left modified radical neck dissection was performed and submitted for microscopic examination. One of 48 cervical nodes was positive for squamous cell carcinoma. The submandibular gland exhibited chronic sialadenitis but there was no evidence of tumor. Consultation from the Department of Radiation Oncology was obtained and treatment was decided upon. Radiation therapy consisted of 5760 cGy to the maxilla and 5080 cGy to the subclavicular area delivered in 32 treatments. The patient experienced transient mucositis, monilia and lymphedema.

Discussion The majority of P I O C appear to arise from the epithelial lining of pre-existing odontogenic cysts. A review of the literature revealed 119 documented cases of intraosseous squamous cell carcinoma, including the present case reports, of which 81 (70%) appeared to have originated in odontogenic cysts 1 13, 15-19. Although other forms of odontogenic cysts such as dentigerous cyst, odontogenic keratocyst, calcifying odontogenic cyst, and lateral periodontal cysts, have demonstrated malignant transformation, the residual cyst represents the source of most PIOC. Most P I O C occur in the posterior mandible, although cases occurring in the anterior maxilla along the line of fusion of the fronto-nasal and maxillary processes have also been reported. The estimated incidence of P I O C with origins in an odotogenic cyst has been projected to range from 1-2% of all oral cancers 15. VAN DER WAAL et al. reported 5 cases of squamous cell carcinoma with origins in odontogenic cysts among 292 oral cancers (1.8%) 17" OTTEN and colleagues found 5 cases of PIOSC in their series of 371 oral carcinomas (1.6%);

however, only 2 cases are probably associated with cysts 1°. STOELINGAet al. in a review of 677 cysts over a 13-year period diagnosed carcinoma in one 15. This is similar to KREIDLER et al. who reported one squamous cell carcinoma in 758 cysts 6. Combining the data from previously reported cases of PIOSC arising de novo, the mean age of occurrence is estimated to be 50, with a male to female predominance of 3:1. These data correlate suprisingly well with earlier reviews of ELZAY 3, GARDNER 5 and a more recent review of To et alJ 6. In PIOSC with a presumed origin in a cyst the age of occurrence ranged from 22 to 85 years with a mean age of 59. The male to female ratio is estimated to be 2:1. OHTAKE et al. noted a male to female ratio of 2:3 in their review of 28 central carcinomas of the jaws which included central mucoepidermoid carcinoma as well as P I O C arising de novo or from a cyst 9, Whether the female predominance in this study reflects a regional difference remains to be determined. The most c o m m o n signs and symptoms noted in cases associated with P I O C of the jaws are pain and swelling, although P I O C may mimic routine dental disorders resulting in delayed diagnosis. To et al. cited a delay in correct diagnosis ranging from a few weeks to as long as 18 months 16. Obviously this delay can contribute to a p o o r prognosis. The 2-year survival rate of patients with P I O C arising from odontogenic cysts (Type I PIOC) has been reported at 53% in the 36 cases presented by EVERSOLEet al. 4. Metastases to regional lymph nodes occurred in 3 previously reported cases and in our case (5%) 1'2'11. Local recurrences were encountered in some patients that did not undergo primary radical excision. In the 12 cases of de novo (Type 3 PIOC) lesions reported by ELZAY, a 40% 2-year survival was noted. Sixty-six percent of these patients had regional metastasis 3. Similarly, in the review of 28 cases of de novo carcinoma reported by To et al., 46% of the patients survived for a period varying from 6 months to 5 years. Whether or not Type 1 and Type 3 have a significantly different prognosis cannot be determined at present; however, the above data would suggest that P I O C arising from odontogenic cysts have a more favorable prognosis. Follow-up information on previously documented cases of PIOC, as well as the reporting of additional new cases is needed to better define both prognosis and management of these carcinomas.

References 1. CHRETIENPB, CARPENTERDF, WHITE NS,

HARRAHJD, LIGHTBODYPM. Squamous carcinoma arising in a dentigerous cyst: presentation of a fatal case and review of four previously reported cases. Oral Surg 1970: 30: 387-8. 2. EL-MOETY SK, SHANNON MT, MUSTOE TA. Lymph node metastasis in spindle cell carcinoma arising in odontogenic cyst. Oral Surg 1991: 71: 209-13. 3. ELZAY RP. Primary intraosseous carcinoma of the jaws. Oral Surg 1982: 52: 299 303. 4. EVERSOLELR, SABESWR, ROVINS. Agressive growth and neoplastic potential of odontogenic cysts. Cancer 1975: 35: 270 82. 5, GARDNERAF. The odontogenic cyst as a potential carcinoma: a clinicopathologic appraisal, J Am Dent Assoc 1969: 78: 746-55. 6, KREIDLERJ, HAASES,. KEMP W, Karzinomagenese in kieferzysten. Dtsch Zahnarztl Z 1985: 40: 548-50. 7, LINDQVISTC, TEPPO g. Primary intraosseous carcinoma of the mandible. Int J Oral Maxillofac Surg 1986: 15:209 14. 8. Loos D. Central epidermoid carcinoma of the jaws. Dtsch Monatschr Zahnheilk 1913: 31: 308. 9. OHTAKE K, YOKOBAYASHIY, SHINGAKI S, NAKAJIMA T, ISHIKI T, KAWASAKIZ. Central carcinoma of the jaws. J Craniomaxil-

lofac Surg 1989: 17: 155-61. 10. OTTEN JE, Joos U, SCmLLI W. Karzinomentstehung auf dem boden des zystenbildenden odontogenen epithels. Dtsch Zahnarztl A 1985: 40: 544-7. t 1. PEARCEYRG. Squamous cell carcinoma arising in dental cysts. Clin Radiol 1985: 36:387 8. 12. PETRI WH, AUCLAIRPA, BRANHAMGB, KELLY MJ, ASH HL. Case 56: Intraosseous tumor of the maxilla. J Oral Maxillofac Surg 1985: 43:726 34. 13. RUSKIN JD, COHEN DM, DAVISLF. Primary intraosseous carcinoma: report of two cases. J Oral Maxillofac Surg 1988: 46: 425 32. 14. SLOOTVCZGPJ, M~LLER H. Malignant ameloblastoma or ameloblastic carcinoma. Oral Surg 1984: 57: 168-76. 15. STOELINGAPJW, BRONKHORSTFB. The incidence, multiple presentation and recurrence of aggressive cysts of the jaws. J Craniomaxillofac Surg 1988: 16: 18595. 16. To EHW, BROWNJS, AVERYBS, WARDBOOTH RE Primary intraosseous carcinoma of the jaws, Three new cases and a review of the literature. Br J Oral Surg 1991: 29: 19-25. 17, VAN DER WAAL I, RAUHAMAAR, VAN DER KWASTWAM, SNOWGB. Squamous cell carcinoma arising in the lining of odontogenie cysts. Int J Oral Surg 1985: 14: 146-52.

Primary intraosseous squamous carcinoma 18. VANWYK CW, PADAYCHEEA, NORTJE CJ, VONDERHEYDENU. Primary intraosseous carcinoma involving the anterior mandible. Br J Oral Surg 1987: 25: 427-32.

19. WALDRON CA, MUSTOETA. Primary intraosseous carcinoma of the mandible with probable origin in an odontogenic cyst. Oral Surg 1989: 67: 716-24.

Address:

D~ Susan Miiller Emory University Department of Oral Pathology 1462 Clifton Road, N.E. Atlanta, GA 30322 USA

365

Primary intraosseous squamous carcinoma. Report of two cases.

Two cases of primary intraosseous squamous carcinoma (PIOSC) are reported. One PIOSC is in the mandible of a 24-year-old man and appears to be a kerat...
3MB Sizes 0 Downloads 0 Views