Sarcomatoid carcinoma of the mandible: report of a case

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DOI:10.5125/jkaoms.2010.36.3.228

228

Ⅰ. Introduction

Sarcomatoid carcinoma is a rare and occurs mainly in the upper aerodigestive tract such as the oral cavity, esophagus and vocal cords. It is a unique variant of squamous cell carcinoma consisting of sarcomatoid proliferation of plemorphic spindle-shaped cells sometimes with a biphasic appearance presenting as a part of frank squamous cell carcinoma^1,2. This type of carcinoma has been described using various terms, including spindle cell squamous carcinoma, carcinosarcoma, pseudosarcoma, and pleomorphic carcinoma1.

We report the case of a patient with spindle cell squamous cell carcinoma involving the mandible.

Ⅱ. Report of the case

An 80-year-old male visited to our hospital because of pain- less submandibular swelling. The swelling appeared 2 weeks ago and decreased after lower teeth extraction. His medical history included surgical treatment for cerebral abscess after admission one month ago. The patient didn’t take any medicine. He was not a smoker.

On intraoral examination, tooth extracted area was complete-

ly healed state. Oral mucosa and gingiva appeared normal col- or and texture but a hard mass was palpable on right lower vestibular area. The tongue and floor of mouth was not elevat- ed. On extraoral examination, a hard submandibular mass was palpable but overlying skin was normal appearance. On panoramic view, any pathologic findings were not showed. We recommended further radiographic examinations to him and his family but that was refused by him.

One week later, he was referred from the department of neurosurgery to evaluate the right lower gingival mass after admission. He was admitted to the department of neurosurgery for seizure and abnormal behavior two days before. Clinical examination revealed the swelling of right lower edentulous area with hematoma and elevation of the tongue and mouth floor.(Fig. 1. A) The patient complained of continuous bleed- ing and foul odor. He could not seal the lips because of a pro- trusive mass.(Fig. 1. B)

Computerized tomography showed that 5.3×3.2 cm sized, relatively well marginated?heterogeneous mass was noted in anterosuperior aspect of the right mandibular body and focal cortical destruction was seen in right parasymphysis of the mandible.(Fig. 2) A biopsy of the hematoma-like lesion was performed and showed a sarcomatoid carcinoma.(Fig. 3. A) The microscopic examination of biopsy showed an infiltrative mass consisting of diffuse tumor cells. The tumor cells had round to spindle cytoplasms with hyperchromatic, bizarre nuclei.(Fig. 3. B) The immunohistochemical stainings were performed and the tumor cells were immunoreactive for pan- cytokeratin and vimentin, supporting the diagnosis of sarcomatoid carcinoma.(Figs 3. C, D)

윤 경 인

155-755 서울시 동작구 흑석동224-1 중앙대학교병원 치과센터 구강악안면외과 Kyoung-In Yun

Department of Oral and Maxillofacial Surgery, Chung-Ang University Hospital

224-1 Hukseok-dong, Dongjak-gu, Seoul, 155-755, Korea TEL: +82-2-6299-2875 FAX: +82-2-6299-2880 E-mail: [email protected]

Sarcomatoid carcinoma of the mandible: report of a case

Gui-Young Kwon¹, Young-Jun Choi², Min-Seok Song², Kyoung-In Yun²

Departments of ¹Pathology, and ²Oral and Maxillofacial Surgery, College of Medicine, Chung-Ang University, Seoul, Korea

Sarcomatoid carcinoma is a rare and occurs mainly in the upper aerodigestive tract such as the oral cavity, esophagus and vocal cords. It is a unique variant of squamous cell carcinoma. We report the case of a patient with spindle cell squamous cell carcinoma involving the mandible. At initial examination, overlying mucosa of that lesion was normal appearance. One week later, that lesion showed ulcerative and bloody change and rapid growth in size. This case showed unpredictable rapid growth although rapid growth in size was suspected of undergoing malignancy.

Key words:Sarcomatoid carcinoma, Mandible, Mandibular neoplasms

[원고접수일 2010.3.15 / 1차수정일 2010.4.15 / 2차수정일 2010.4.29 / 게재확정일 2010.5.13]

Abstract (J Korean Assoc Oral Maxillofac Surg 2010;36:228-30)

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229 Unfortunately, he expired due to multiple metastatic carcino-

mas from the lung or stomach.

Ⅲ. Discussion

Sarcomatoid carcinoma is a subgroup of malignant mixed tumors and is extremely rare in maxilla. The initial description of this type of malignancy was reported in 1,864 by Virchow, who labeled it as carcinosarcoma³. Later on, Saphir and Vass analyzed 153 cases of carcinosarcomas of various sites. They concluded that the sarcomatous component represented a vari- ation in the squamous portion of the carcinoma or an inflam- matory reaction of the underlying stroma³.

Numerous hypotheses regarding the histogenesis of this type of tumor have been proposed. Three dominant pathogenetic theories have been proposed: the tumor (1) represents a “colli- sion tumor”(carcinosarcoma), (2) is a squamous cell carcoma with an atypical reactive stroma (pseudosarcoma), or (3) is of epithelial origin, with “de-differentiation”or transformation to a spindle cell morphology (sarcomatoid carcinoma)⁴. Recently, the third hypothesis has been supported by following evi-

dences: their occurrence in the exact sites that normally have squamous epithelium and a preponderance of carcinomas rather than sarcomas; a superficial location; a polypoid appearance; the direct continuity and smooth transition of the spindle cells with areas of squamous epithelium; immunoreactivity with epithelial antigens; a dual expression of epithelial and mesenchymal differentiation with double labeling techniques in some neoplastic spindle cells; and the presence of epithelial only, sarcomatous only, or a duality of expression in metastatic deposits from laryngeal sarcomatoid carcinoma⁵. Furthermore, recent molecular studies have shown evidence of a monoclonal origin from a stem cell capable of divergent differentiation^6,7.

When the malignant surface epithelium is histologically evi- dent, the diagnosis of a sarcomatoid carcinoma is made with confidence. However, when the surface epithelium is ulcerated or denuded, the correct diagnosis is more difficult.

Furthermore, the biopsies from the squamous cell component tend to be misdiagnosed as squamous cell carcinoma and biopsies from spindle cell component tend to be diagnosed as sarcoma. In our case, the lesion looked like an ecchymosis on initial clinical examination.

Fig. 2.CT findings.

A. CT revealed that the anterior border of the mandible was partially resorbed.

B. The mass occupied the lower anterior vestibular area.

(CT: computed tomography)

Fig. 3.Histopathologic examination.

A. Lower power views shows diffuse sheet of neoplastic cells with no organoid structure.(H&E staining, original magnification x40)

B. Highly anaplastic epitheloid tumor cells are present with prominent nucleus and mitosis.(H&E staining, original magnification x400)

C. Immunopositivity for cytokeratin.

D. Immunopositivity for vimentin.

Fig. 1.Clinical examination.

Sarcomatoid carcinoma of the mandible: report of a case

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대구외지 2010;36:228-30

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Sarcomatoid carcinoma of the oral cavity presents a male predominance at a mean age of 57 years and site predilection for the lower lip, tongue and alveolar ridge or gingiva although most tumors in head and neck region occur in the larynx^2,4. Growth configuration is often exophytic polypoid, but sessile, nodular or endophytic configuration has also been described.

The lesion usually has an extensive surface ulceration with fri- able, fibrinoid necrosis of variable thickness or shaggy exu- dates^5,8. Radiation, trauma, tobacco use or alcohol consumption seemed to play a role in etiological factors⁸.

The differential diagnosis includes a number of benign and malignant tumors, such as squamous cell carcinoma, fibrosar- coma, malignant fibrous histiocytoma, leiomyosarcoma, rhab- domyosarcoma, malignant peripheral nerve sheath tumor, osteosarcoma, mesenchymal chondrosarcoma, Kaposi’s sarcoma, angiosarcoma, synovial sarcoma, malignant melanoma, fibromatosis, leiomyoma, nodular fasciitis and reactive epithelial proliferations⁵.

Wide surgical excision is the treatment of choice. Most authors agree that irradiation is ineffective. Radiation therapy is considered an acceptable alternative for inoperable patients.

Furthermore, adjuvant irradiation might be of benefit in cases in which the surgical margins are positive or in patients with nodal metastasis at the time of diagnosis².

Prognosis is related to location, tumor size, depth of inva- sion, stage of disease and with the presence of any keratin staining in the spindle cells⁸. A tumor of the oral cavity and oropharynx is potentially aggressive and seems recur easily

and to metastasize easily⁹. The incidence of metastases was 36% and the 2-year survival rate was 55% in tumors involving the oral cavity¹.

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