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Year : 2015  |  Volume : 11  |  Issue : 3  |  Page : 656

Histopathological, immunohistochemical and special stain unraveling the enigmatic carcinosarcoma - A case report

1 Department of Oral Pathology and Microbiology, Yenepoya Dental College, Yenepoya University, Mangalore, Karnataka, India
2 Department of Oral Pathology and Microbiology, Yenepoya Dental College, Yenepoya University, Mangalore, KarnatakaDepartment of Oral Pathology and Microbiology, Yenepoya Dental College, Yenepoya University, Mangalore, Karnataka, India

Date of Web Publication9-Oct-2015

Correspondence Address:
Imran Mohtesham
Assistant Professor, Department of Oral Pathology and Microbiology, Yenepoya Dental College, Yenepoya University, Nithyananda Nagar, Mangalore - 575 018, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0973-1482.140812

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 > Abstract 

Carcinosarcoma is an extremely rare, highly malignant tumor having a characteristic of both epithelial and mesenchymal components; the epithelial component exhibiting focal squamous cell carcinoma and mesenchymal component having sarcomatoid stroma. The occurrence of carcinosarcoma in the oral cavity is very rare and only few cases have been reported in the literature, although its occurrence in other parts of the body has been widely reported.

Keywords: Carcinosarcoma, pseudosarcoma, spindle cell carcinoma

How to cite this article:
Shakil M, Mohtesham I, Jose M. Histopathological, immunohistochemical and special stain unraveling the enigmatic carcinosarcoma - A case report. J Can Res Ther 2015;11:656

How to cite this URL:
Shakil M, Mohtesham I, Jose M. Histopathological, immunohistochemical and special stain unraveling the enigmatic carcinosarcoma - A case report. J Can Res Ther [serial online] 2015 [cited 2019 Nov 14];11:656. Available from: http://www.cancerjournal.net/text.asp?2015/11/3/656/140812

 > Introduction Top

Carcinosarcoma is a highly malignant tumor characterized by dual malignant histologic differentiation of the epithelial component consisting of a focally squamous cell carcinoma and a mesenchymal component having a sarcomatoid stroma. [1],[2] Carcinosarcoma remains an extremely rare and aggressive entity. A range of terms have been used to describe this perplexing neoplasm such as spindle cell carcinoma, pseudosarcoma, squamous cell carcinoma with pseudosarcoma and Lane tumors. The tumor has been extensively reported in the squamous epithelium-lined areas of the body such as the lungs, urinary tract, breast and uterus. In the head and neck region, the pharynx and larynx are most frequently involved, followed by the esophagus, the oral cavity and the nasal area. [1] Its occurrence in oral cavity is very rare and only few cases have been reported in the literature. We report a case of carcinosarcoma of buccal mucosa.

 > Case report Top

A 61-year-old female patient reported to the outpatient department of our dental college and hospital with soft tissue mass in the right buccal mucosa, reddish white in color and soft in consistency. Excisional biopsy of the growth was carried out and sent for histopathological examination. The lesional tissue was sent for routine H and E staining, reticulin special staining and immunostaining for Delta Np63.

On histological examination, the lesion consisted of malignant epithelial and mesenchymal components. The two components were distinctly demarcated without a transition zone. The epithelial element was a well-differentiated squamous cell carcinoma [Figure 1]. It presented a well-differentiated and keratinizing area, with the formation of epithelial pearls, but also presented poorly differentiated and non-keratinizing squamous cell carcinoma areas, with cells forming sheets, cords, and bundles separated by varying amounts of vascular connective tissue. The mesenchymal component also showed sarcoma-like, malignant features, including hypercellularity, marked pleomorphism, and enlarged nuclei. Reticulin stain showed the arrangement of fibers as shown in [Figure 2]. Reticulin staining was positive for vascular connective tissue [Figure 2]. Malignant epithelial cells present in connective tissue showed positivity for p63 immunostaining [Figure 3]. It must be noted that the Delta Np63 is a wild form in the p63 tumor marker belonging to the molecular policeman P53 family.
Figure 1: Histological feature of carcinosarcoma exhibitng both epithelial and mesenchymal components

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Figure 2: Tumor cells positive for immunohistochemical marker P63

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Figure 3: Malignant epithelial cells showing positivity for delta NP63

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The lesion was surgically removed and the patient was advised for regular follow-up.

 > Discussion Top

The initial description of this type of malignancy was reported in 1864 by Virchow, who labelled it as 'carcinosarcoma'. [3] Later on, Saphir and Vass analyzed 153 cases of carcinosarcomas of various sites. They concluded that the sarcomatous component represented a variation in the squamous portion of the carcinoma or an inflammatory reaction of the underlying stroma. Various hypotheses have been proposed in the histogenesis of this type of tumor. Three dominant pathogenetic theories have been proposed: The tumor [1] represents a "collision 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 evidence: 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 carcinoma. [5] In short, its histogenesis is explained based on two antithetical hypotheses: One proposes a multiclonal origin arising from two or more stem cells; the other a monoclonal origin arising from a single totipotential stem cell that differentiates in separate epithelial and mesenchymal directions. [6]

Carcinosarcoma accounts for less than 1% of all tumors of oral regions. [7] In the oral cavity, it presents a male predominance at a mean age of 57 years and the most common site being the lower lip, tongue and alveolar ridge or gingiva, while most tumors in head and neck region occur in the larynx. [4],[8] Growth configuration of carcinosarcoma is often described as exophytic polypoid, but sessile, nodular or endophytic configuration has also been illustrated in the literature. The lesion usually presents as an extensive surface ulceration with friable, fibrinoid necrosis of variable thickness or unshorn exudates. [5],[9] Radiation, trauma, tobacco use or alcohol consumption have been implicated to play a role in etiological factors. [9]

The differential diagnosis of carcinosarcoma includes various benign and malignant tumors such as squamous cell carcinoma, malignant fibrous histiocytoma, fibrosarcoma, rhabdomyosarcoma, leiomyosarcoma, malignant peripheral nerve sheath tumor, mesenchymal chondrosarcoma, osteosarcoma Kaposi's sarcoma, synovial sarcoma, angiosarcoma, malignant melanoma, fibromatosis, nodular fasciitis, leiomyoma and reactive epithelial proliferations. [5]

Wide surgical excision is the treatment of choice. Some authors are of opinion that irradiation is ineffective; while others consider 'radiation therapy' is an acceptable alternative for inoperable patients. Besides, 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 diagnosis. [8] Prognosis is related to location, tumor size, depth of invasion and stage of disease. Tumor epithelial component namely the malignant epithelial cells are immunostained for Delta Np63 a wild form oncoprotein in the connective tissue. Delta Np63 is a wild isoform amongst the six isoforms of P63 belonging to the genome guardian P53 family.

 > Conclusion Top

Carcinomasarcoma is a rare biphasic neoplasm and a variant of squamous cell carcinoma. Its perplexing pathogenesis and aggressive nature poses a challenge to the clinician. Special staining and immunohistochemistry can give us an insight into the pathogenesis of the lesion. The lesion has a tendency to recur easily and metastasize. It is difficult to predict the biologic behavior, but tumors with deep invasion are of poor prognosis.

 > References Top

Middlehurst RJ, Blackburn CW, Sloan P. Spinde-cell carcinoma: A case report. Br J Oral Maxillofac Surg 1990;28:114-6.  Back to cited text no. 1
Goellner JR, Devine KD, Weiland LH. Pseudosarcoma of the larynx. Am J Clin Pathol 1973;59:312-26.  Back to cited text no. 2
Berthelet E, Shenouda G, Black MJ, Picariello M, Rochon L. Sarcomatoid carcinoma of the head and neck. Am J Surg 1994;168:455-8.  Back to cited text no. 3
Huang SF, Chen IH, Liao CT, Chen TM, Lee KF. Sarcomatoid carcinoma of the parotid gland with apparent metastasis of epidermoid elements to cervical lymph nodes. Acta Otolaryngol 2006;126:667-71.  Back to cited text no. 4
Thompson LD, Wieneke JA, Miettinen M, Heffner DK. Spindle cell (sarcomatoid) carcinomas of the larynx: A clinic pathologic study of 187 cases. Am J Surg Pathol 2002;26:153-70.  Back to cited text no. 5
Thompson L, Chang B, Barsky SH. Monoclonal origins of malignant mixed tumors (carcinosarcomas): Evidence for a divergent histogenesis. Am J Surg Pathol 1996;20:277-85.  Back to cited text no. 6
Jordan RC, Regezi JA. Oral spindle cell neoplasms: A review of 307 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003;95:717-24.  Back to cited text no. 7
Rizzardi C, Frezzini C, Maglione M, Tirelli G, Melato M. A look at the biology of spindle cell squamous carcinoma of the oral cavity: Report of a case. J Oral Maxillofac Surg 2003;61:264-8.  Back to cited text no. 8
Minami SB, Shinden S, Yamashita T. Spindle cell carcinoma of the palatine tonsil: Report of a diagnostic pitfall and literature review. Am J Otolaryngol 2008;29:123-5.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3]


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