Initial report of a case of carcinosarcoma of the supraglottis.
Minor salivary gland minor salivary gland
Any of the small salivary glands of the oral cavity, including the labial, buccal, molar, lingual, and palatine glands. carcinosarcoma is a rare malignancy with an ominous prognosis. This report recounts a single case of sup raglottic minor salivary gland carcinosarcoma that was treated aggressively. We discuss the peculiarities of the histology, with special mention of the distinction that must be made between mucosal-origin and salivary-gland-origin carcinosarcoma. Carcinosarcomas are aggressive regardless of their origin, but differentiating their origin is important because the prognosis is worse for those that arise in salivary glands than for those that originate in the surface epitheliam. This report adds to the literature a new case of minor salivary-gland-origin carcinosarcoma involving the upper airway. It is the first case we could identify in which the supraglottis was involved.
Carcinosarcoma is a rare neoplasm neoplasm or tumor, tissue composed of cells that grow in an abnormal way. Normal tissue is growth-limited, i.e., cell reproduction is equal to cell death. with both malignant epithelial and malignant mesenchymal components. A distinction can, and should, be made between lesions of mucosal origin and those that begin in minor or major salivary glands. Mucosal-origin lesions typically occur in the larynges la·ryn·ges
A plural of larynx. of men during the seventh decade of life. [1-4] Salivary-gland-origin lesions are rare, occur in a broader age range, and are more equally distributed between men and women. [5-7]
Our patient was a 23-year-old white woman with a 12-month history of left-sided otalgia otalgia /otal·gia/ (o-tal´jah) pain in the ear; earache.
Pain in the ear; earache.
o·tal , an alteration in voice quality, and a 17-lb weight loss. She was a nonsmoker, and she did not abuse alcohol. Examination revealed the presence of an exophytic lesion that involved the left epiglottis epiglottis (ĕp'əglŏt`ĭs): see larynx. and extended along the left aryepiglottic fold. No lymphadenopathy lymphadenopathy /lym·phad·e·nop·a·thy/ (-op´ah-the) disease of the lymph nodes.
angioimmunoblastic lymphadenopathy , angioimmunoblastic lymphadenopathy with dysproteinemia was present.
The patient was taken to the operating room for biopsy and airway management. We observed a 3 x 3 x 4-cm supraglottic mass, with no involvement of the false vocal folds and no pre-epiglottic space extension. For safe airway management, a tracheostomy was performed. Biopsy revealed a poorly differentiated malignancy that was most consistent with a high-grade mucoepidermoid carcinoma. The patient balked balk
v. balked, balk·ing, balks
1. To stop short and refuse to go on: The horse balked at the jump.
2. at further management, and as a result, treatment was delayed for 3 weeks. During this delay, a 2 x 2-cm node became palpable in the left midjugular chain.
The patient returned for treatment and underwent a supraglottic laryngectomy Laryngectomy Definition
Laryngectomy is the partial or complete surgical removal of the larynx, usually as a treatment for cancer of the larynx.
Normally a laryngectomy is performed to remove tumors or cancerous tissue. with negative surgical margins and a left modified neck dissection modified neck dissection Surgery A subtotal resection of the neck region, usually for CA of the floor of the mouth; most MNDs preserve the spinal accessory nerve, internal jugular vein, and sternocleidomastoid muscle. See Commando operation. Cf Radical neck dissection. . Seven of 15 nodes in the left side of the neck had been invaded by metastatic tumor. Postoperative radiation therapy was delivered to the primary site and to both sides of the neck.
A percutaneous esophagogastrostomy tube was placed at the time of the laryngectomy, and the patient was decannulated 12 weeks after surgery. Initially her weight began to increase, but a progressive deterioration ensued within 6 months, and radiography detected mediastinal mediastinal /me·di·as·ti·nal/ (-as-ti´n'l) of or pertaining to the mediastinum.
of or pertaining to the mediastinum. and pulmonary metastases. Palliative radiation therapy was delivered to the chest. The woman died 10 months after her initial diagnosis and 22 months after she had first noticed her symptoms.
Radiologic findings. Magnetic resonance imaging magnetic resonance imaging (MRI), noninvasive diagnostic technique that uses nuclear magnetic resonance to produce cross-sectional images of organs and other internal body structures. (MRI 1. (application) MRI - Magnetic Resonance Imaging.
2. MRI - Measurement Requirements and Interface. ) performed at the initial presentation revealed the presence of an irregular, sharply defined, supraglottic, oval-shaped mass that measured 2.9 cm in its maximum dimension (figure 1). Compared with muscle, the mass was isointense on spin-echo T1-weighted (TR 800/TE 22) MRI and hyperintense on spin-echo T2-weighted (TR 2700/TE 90) MRI. Moderate diffuse enhancement was seen on gadodiamide-enhanced images.
Unenhanced computed tomography (CT) showed that the mass was slightly hypodense compared with muscle. The mass was centered in the supraglottis, and it involved the entire suprahyoid epiglottis, with pre-epiglottic space extension. The mass obscured the vallecula vallecula /val·lec·u·la/ (vah-lek´u-lah) pl. valle´culae [L.] a depression or furrow.vallec´ular
vallecula cerebel´li .
Pathologic findings. The laryngectomy specimen contained a highly variegated, poorly differentiated malignancy that was centered in the subepithelial tissue. Much of the tumor was carcinomatous and made up of nests of basaloid cells that had small, densely hyperchromatic nuclei and scant amounts of cytoplasm. Other areas featured trabecular and cribriform cribriform /crib·ri·form/ (krib´ri-form) perforated like a sieve.
Perforated like a sieve.
perforated like a sieve. patterns with a distinct gland formation (figure 2). Mucin mucin: see glycoprotein. stains were focally positive. Although initial sections did not show a sarcomatous sarcomatous /sar·co·ma·tous/ (sahr-ko´mah-tus) pertaining to or of the nature of a sarcoma.
pertaining to or of the nature of a sarcoma. component, additional sections did reveal chondrosarcomatous foci that were characterized by a chondroid matrix surrounding numerous atypical nuclei (figure 3). These hypercellular chondroid areas were distinct from trapped remnants of normal cartilage. Fascicles of undifferentiated malignant spindle cells were also identified.
The combination of carcinomatous and sarcomatous elements was diagnostic of carcinosarcoma. We concluded that this tumor most likely originated in the minor salivary gland based on its morphologic resemblance to carcinosarcomas of major salivary glands, the lack of a distinct in situ component in the surface epithelium, and the fact that the tumor's epicenter was in the submucosal submucosal /sub·mu·co·sal/ (-mu-ko´sal)
1. pertaining to the submucosa.
2. beneath a mucous membrane. tissue.
Discussion Controversy has been a hallmark of our understanding of carcinosarcoma for nearly a century. The debate stems from our uncertainty over the pathogenesis of these rare malignant tumors. There are four proposed etiologic factors: (1) a carcinoma (usually squamous) with a reactive mesenchymal proliferation (pseudosarcoma), (2) a so-called collision tumor, in which carcinoma and sarcoma sarcoma (särkō`mə), highly malignant tumor arising in connective- and muscle-cell tissue. It is the result of oncogenes (the cancer causing genes of some viruses) and proto-oncogenes (cancer causing genes in human cells). arise simultaneously in a specific anatomic site, (3) a malignancy arising in an embryonic rest of epithelial and mesenchymal tissues, and (4) a divergent differentiation of a carcinoma into sarcomatous components, or sarcomatoid carcinoma.
Each of the first three theories has a significant weakness. In fact, the first theory is no longer in favor because the spindle cell component can metastasize me·tas·ta·size
To be transmitted or transferred by or as if by metastasis.
Spread of cells from the original site of the cancer to other parts of the body where secondary tumors are formed. independently of the carcinoma. For the collision tumor theory to hold would require an unlikely occurrence-namely, that multiple, sequential carcinogenic events in two or more independent cell types would result in a malignant transformation of each. The third theory, that malignancy arises in an embryonic rest of epithelial and mesenchymal tissues, is simply a variation of the collision tumor hypothesis and has the same shortcomings.
The fourth theory, that there is a divergent differentiation of a carcinoma into sarcomatous components, appears to be the most likely explanation. This model requires the malignant transformation of only a single cell line, which can then undergo clonal evolution and differentiation into a variety of carcinomatous and sarcomatous components. These tumors can originate in stem (totipotential) cells in a manner somewhat analogous to the formation of teratomas. Immunohistochemical and ultra-structural studies support this theory. Wick and Swanson favor the term sarcomatoid carcinoma for these complex neoplasms, but carcinosarcoma and spindle cell carcinoma spindle cell carcinoma
A carcinoma composed of elongated cells, frequently a poorly differentiated squamous cell carcinoma.
spindle cell carcinoma continue to appear in the literature and remain commonly accepted terms.
Hyams reported 39 cases of laryngeal laryngeal /lar·yn·ge·al/ (lah-rin´je-al) pertaining to the larynx.
la·ryn·geal or la·ryn·gal
Of, relating to, affecting, or near the larynx. sarcomatoid carcinoma. The supraglottis was the site of involvement in only two of these patients. Most of the 39 patients were men (mean age: 68 years) who had a history of smoking. The spindle cell component predominated. The squamous cell component was often at the base or stalk of polypoid tumors. Cervical metastases featured both carcinomatous and sarcomatous components. These lesions were presumed to be mucosal in origin. Eight of 20 patients available for followup bad died of their disease between 4 months and 2 years after diagnosis.
Olsen et al reported 34 patients with laryngeal carcinosarcoma.  The clinical features of these patients were remarkably similar to those reported by Hyams; smoking men in the seventh decade of life predominated. Survival rates were similar to those of patients who had pure squamous cell carcinoma squamous cell carcinoma
A carcinoma that arises from squamous epithelium and is the most common form of skin cancer. Also called cancroid, epidermoid carcinoma. of the larynx or hypopharyax. Olsen et al recommended that intervention be based on the tumor's stage, location, and size, just as if these patients had conventional squamous cell carcinoma. This clinical behavior and therapeutic approach support the concept that these tumors are fundamentally carcinomas that simply contain areas of divergent differentiation.
Leventon and Evans analyzed the clinical outcomes of 20 patients with head and neck sarcomatoid carcinoma, including three laryngeal tumors.  Nine of 10 patients whose tumors had invaded muscle or bone died of disease. Conversely, all 10 patients who had only superficially invasive tumors survived.
Carcinosarcoma of salivary gland origin (so-called true malignant mixed tumor) is rare. To our knowledge, ours is the first reported case of a laryngeal minor salivary gland carcinosarcoma. Twenty-six salivary gland carcinosarcomas have been identified in the literature.[5-7, 9-13] Three of these cases arose in the minor salivary glands of the palate, and the remainder occurred in the parotid parotid /pa·rot·id/ (pah-rot´id) near the ear.
1. Situated near the ear.
2. Of or relating to a parotid gland.
A parotid gland. and submandibular glands. None has been described in the minor salivary glands of the supraglottis. Analysis of these 27 cases (including ours) reveals a wide age range (23 to 88 years) and a slight predominance of women over men. Clinical followup information was available for 19 of the 27 patients. Fifteen died of disease, two were alive with disease, and two were alive without disease. The two patients without disease were long-term survivors ([greater than]4 yr post-treatment), and in both of them, the tumor had originated in the parotid gland.
The authors thank Stacey Mills, MD, Anthony Mancuso, MD, and Randal Weber, MD, who participated in the 1996 Penrose Cancer Conference and whose comments are incorporated into this paper.
From the Department of Otolaryngology--Head and Neck Surgery (Dr. Ernster), the Department of Pathology (Dr. Franquemont), and the Department of Radiology (Dr. Sweeney), Penrose--St. Francis Healthcare System, Colorado Springs, Colo.
Reprint requests: Joel A. Ernster, MD, 715 N. Cascade Ave., Colorado Springs, CO 80903-3246. Phone: (719) 633-3803; fax: (719)4718378; e-mail: firstname.lastname@example.org
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