Supracricoid partial laryngectomy as a primary treatment for carcinosarcoma of the larynx.

Abstract

Carcinosarcoma of the larynx is uncommon. When it does occur, its clinical features resemble those of sarcomatoid carcinoma, and its biologic behavior is similar to that of malignant mesenchymal neoplasms. We describe 2 cases of carcinosarcoma of the glottis. The tumors were staged as T3N0M0 and T2N0M0. Both patients were treated with supracricoid partial laryngectomy with cricohyoidoepiglottopexy. Eight months postoperatively, 1 of the 2 patients experienced a recurrence of the sarcomatous component of the tumor, and he underwent a total laryngectomy. The other patient remained free of disease at 12 months of follow-up. We conclude that supracricoid partial laryngectomy may be offered as an organ-preserving measure even in patients with sarcomatous disease; total laryngectomy can be held in reserve as a rescue measure. Patients must be closely monitored for early detection of recurrence. The role of adjuvant therapy for sarcomatous neoplasms in this area has not yet been clearly established.

Introduction

Carcinosarcoma of the head and neck is uncommon. Most reported cases have occurred in the major salivary glands; other sites include the larynx and pharynx and, less frequently, the oral and nasal cavities and the esophagus. (1-6) True carcinosarcomas of the larynx and the hypopharynx represent less than 1% of all malignant neoplasms in this area. (3,4,6-8) The mean age of carcinosarcoma patients is 60 years, and there is a male predominance (3.5 to 1). (4,6-8) Risk factors are similar to those of squamous cell carcinoma (e.g., smoking and alcoholism), and there is a higher risk in patients who are exposed to radiation. The classic clinical presentation features dysphonia and dyspnea secondary to laryngeal obstruction. (5-7,9)

The histogenesis of carcinosarcoma is controversial. The most widely accepted theory ascribes its origin to the differentiation of primitive blastic mesenchymal cells that can mature and produce malignant neoplasms of multiple differentiation. At diagnosis, a true carcinosarcoma exhibits a malignant mesenchymal component (sarcoma), as well as an epithelial component (carcinoma or adenocarcinoma). Histologic study will reveal the presence of pleomorphic cells and atypical mitosis. (1-4,7) In cases when histologic lineages that are uncommon in the larynx have been found, treatment has almost invariably involved total laryngectomy. Only a few reports exist of cases of nonsquamous (nonepidermoid) histology in which organ preservation was attempted. (6,10) To our knowledge, no case of laryngeal carcinosarcoma treated with supracricoid partial laryngectomy and cricohyoidoepiglottopexy has been previously reported. In this article, we describe 2 such cases, and we discuss their salient clinical and histologic features.

Case reports

Patient 1. A 55-year-old man sought medical attention for a 4-month history of progressive dysphonia and recent-onset dyspnea. He had been a heavy smoker and heavy drinker since the age of 15 years. Another physician had previously performed a biopsy of the larynx, which revealed the presence of a carcinosarcoma.

Our clinical and endoscopic examination revealed that a tumor had ari sen from the left true vocal fold and obstructed 90% of the endolaryngeal lumen. The affected vocal fold was fixed, and the arytenoids were mobile. The tumor was staged as T3N0M0.

The patient underwent a supracricoid partial laryngectomy with cricohyoidoepiglottopexy, which preserved the arytenoids, and a selective bilateral dissection from levels II through V, which preserved both jugular veins and the XIth cranial nerve. Transoperative and definitive studies confirmed the diagnosis of carcinosarcoma and revealed that the surgical margins were lesion-free. No complications occurred during the postoperative period. On postoperative day 6, the tracheotomy tube was removed and the patient was able to speak. He was discharged the following day with a nasogastric feeding catheter in place. The catheter was removed on postoperative day 21. The patient was not considered for postoperative radiotherapy.

Eight months later, the patient reported respiratory difficulties and odynophagia. Physical exploration and endoscopy detected a 4-cm tumor in the neoglottic area at the site of the left arytenoids. The patient underwent a total laryngectomy. He was monitored with periodic physical examinations, fiberoptic nasolaryngoscopy, chest x-rays, and computed tomography (CT) of the head and neck. At the 12-month examination, he exhibited no evidence of tumoral activity.

Macroscopic evaluation. The specimen excised during the first surgery included thyroid cartilage and newly formed red-violet endopharyngeal tissue, which had arisen from the left true vocal fold. The mass had a polypoid appearance, a pedunculated base, and small areas of surface ulceration (figure 1, A). It measured 3 x 2.5 x 2 cm. The base of the tumor was continuous, with an irregular plaque-like white lesion that extended along the vocal process of the arytenoids. The right false and true vocal folds were without alteration. Sagittal sectioning revealed that the newly formed tissue was solid and featured alternating white and violaceous areas.

[FIGURE 1 OMITTED]

Microscopic evaluation. At the histologic level, the newly formed tissue contained two types of cells. The major component was pleomorphic fusiform cells; these cells had ovoid nuclei and eosinophilic cytoplasm, and they exhibited numerous mitotic figures (figure 1, B). The cells were continuous, with areas of osteoid material. In some areas, epithelial ducts that contained carcinoma were present within the sarcomatous neoplasm (figure 1, C). Immunohistochemical study revealed intense immunoreactivity to cytokeratin in the carcinomatous areas and to vimentin in the sarcomatous areas. The final diagnosis was carcinosarcoma of the left true vocal fold that extended to the vocal process of the arytenoids and infiltrated Reinke's space and the skeletal muscle. Dissection of both sides of the neck turned up no evidence of metastasis. The recurrent tumor was made up of only the sarcomatous component of carcinosarcoma (osteosarcoma).

Patient 2. A 54-year-old man with progressive dysphonia of 4 months' evolution and a 32-year history of smoking 15 cigarettes per day was referred to our hospital for management of a malignant laryngeal tumor. Another physician had biopsied the larynx and rendered a diagnosis of squamous cell carcinoma.

Our physical and endoscopic examinations revealed that a 3-cm tumor located on the right true vocal fold had obstructed approximately 80% of the endolaryngeal lumen. Vocal fold and arytenoid mobility was normal, and the tumor was staged as T2N0M0.

We performed a supracricoid partial laryngectomy with cricohyoidoepiglottopexy and bilateral dissection of the neck from levels II through V. Intraoperative histopathology confirmed that the surgical margins were clear. During the postoperative period, the patient developed edema of the arytenoids, which was alleviated by steroid therapy. He was decannulated on postoperative day 10 and was able to speak. He was discharged on postoperative day 11 with a nasogastric catheter. The catheter was removed on postoperative day 23. Radiotherapy was not considered. At 12 months of follow-up, no evidence of tumoral activity was observed.

Macroscopic evaluation. The surgical specimen was made up of newly formed white, multinodular, polypoid, pedunculated endolaryngeal tissue that was located on the right true vocal fold. The mass measured 2.7 x 2.1 x 1.4 cm and contained areas of hemorrhage. The false and true left vocal folds were devoid of tumor. Sagittal section identified a solid, firm neoplasm with alternating white and violaceous areas. Thyroid cartilage was clearly evident at the periphery of the specimen.

Microscopic evaluation. Again, histology revealed two cellular types and, again, the major components were round and fusiform pleomorphic cells with abundant eosinophilic cytoplasm exhibiting a high mitotic index (figure 2, A). Other areas contained foci of in situ carcinoma and grade II dysplasia (figure 2, B). No continuity existed between the two neoplastic components. Immunohistochemical study revealed intense immunoreactivity to vimentin and actin in the sarcomatous areas and to cytokeratin in the carcinornatous areas. The definitive histopathologic diagnosis was a carcinosarcoma made up of areas of in situ carcinoma and leiomyosarcoma of the right true vocal fold that involved the vocal process of the arytenoid and infiltrated into Reinke's space without affecting the vocal muscle or thyroid cartilage. Dissection of both sides of the neck revealed no metastasis.

[FIGURE 2 OMITTED]

Discussion

Carcinosarcoma is sometimes called by several other names (pseudosarcoma, sarcomatoid carcinoma, pleomorphic carcinoma, and pseudocarcinosarcoma), not all of which are correct. (6,8,11,12) The variety of terms, as well as the tumor's variable clinical presentation and morphologic characteristics, has the potential to cause some confusion.

Carcinosarcoma vs. sarcomatoid carcinoma. There is a difference between carcinosarcoma and sarcomatoid carcinoma; the latter is a squamous cell carcinoma with spindle-cell changes that simulate a sarcoma. Also, a sarcomatoid carcinoma grows more slowly. Clinically, dysphonia is present in patients with both neoplasms, but only carcinosarcoma is generally associated with dysphagia, dyspnea, and obstruction as an initial symptom. Most carcinosarcomas are longer than 2 cm; sarcomatoid carcinomas are usually smaller. Regional metastasis of the epithelial component of carcinosarcoma occurs in only 10 to 20% of cases, but distant metastasis of the mesenchymal component is more common. (1,2) Differences also exist with respect to the histogenic, histopathologic, and immunohistochemical features of the two tumors (table). (1,3,4,6-9,11-13)

The sarcomatous components of carcinosarcoma are, in decreasing order of frequency, chondrosarcoma, osteosarcoma, fibrosarcoma, and malignant fibrous histiocytoma. (2-4) Carcinosarcoma has an aggressive biologic behavior, and it is associated with high rates of recurrence and distant metastasis despite radical primary treatment. (1,3,5,7,9) The diagnosis is usually established early on the basis of the clinical presentation, which is generally characterized by the presence of an exophytic tumor that quickly causes obstruction as a result of its rapid growth, especially the growth of the mesenchymalcomponent. When ulceration is present, the affected area can become overinfected, which can lead to a false-negative biopsy and delay diagnosis. Therefore, patients with suspected carcinosarcoma should undergo a deep biopsy of a nonulcerated area.

Treatment of carcinosarcoma. There is no consensus as to the most appropriate therapy for carcinosarcoma. (6) The most common treatment is total laryngectomy, complemented in some cases by adjuvant chemotherapy and/or radiotherapy because the prognosis is dependent on the type of malignant mesenchymal component. (1-5,13) We agree with Ianniello et al, (6) who suggested that the therapeutic approach to and prognosis for carcinosarcoma is similar to that of squamous cell carcinoma. Because conservative surgery has been shown to provide just as much local control as radical surgery, supracricoid partial laryngectomy is indicated for selected cases of squamous cell carcinoma and nonsquamous cell carcinoma. (10,14-16) The quality of life and phonation in patients who have undergone this type of surgical procedure at our institution has been previously reported. (17) In our experience, these patients were able to achieve an almost normal biopsychosocial integration.

Supracricoid partial laryngectomy has an advantage over other partial surgeries with respect to surgical margins. (10) In 2001, 2 reports of mesenchymal tumors treated with conservative surgery were published; Ianniello et al (6) reported using vertical hemilaryngectomy, and Veivers et al (10) used supracricoid partial laryngectomy with cricohyoidoepiglottopexy. The case reported by Ianniello et al (6) was diagnosed as a carcinosarcoma, and the case from Veivers et al (10) was diagnosed as a leiomyosarcoma; in retrospect, however, the latter might also have been a carcinosarcoma because the patient developed metastatic squamous cell carcinoma from an unknown primary 6 months after the initial treatment. Because carcinosarcoma is a high-grade malignant neoplasm that can metastasize, and because neck dissections are associated with low mortality, (1) we agree with Veivers et al (10) that elective neck dissection must be performed at the time of the initial treatment of a nonepithelial neoplasm.

Our 2 patients were not considered for adjuvant treatment with radiotherapy because their tumors were confined to the endolarynx and both necks were negative for metastasis. The recurrence in patient 1 involved an osteosarcoma, which is not an especially radiosensitive neoplasm; therefore, the patient was rescued by a total laryngectomy. It is interesting to note that in most cases of nonsquamous cell cancer of the larynx, the most common cause of death is a distant (lung) metastasis. (7,10)

In conclusion, we believe laryngeal carcinosarcoma can be managed with conservative organ-preserving surgery, with total laryngectomy reserved for recurrences.

Reprint requests: Kuauhyama Luna-Ortiz, MD, Departamento de Cabeza y Cuello, Av. San Fernando #22, Tlalpan D.F. 14080, Mexico, Phone: [phone], ext. 285; fax: [phone]; e-mail: [e-mail] or [e-mail]

Originally presented at the Sixth International Conference on Head and Neck Cancer; Aug. 11, 2004; Washington, D.C.

References

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(14.) Laccourreye H, Laccourreye O, Weinstein G, et al. Supracricoid laryngectomy with cricohyoidoepiglottopexy: A partial laryngeal procedure for glottic carcinoma. Ann Otol Rhinol Laryngol 1990;99:421-6.

(15.) Laccourreye H, Laccourreye O, Weinstein G, et al. Supracricoid laryngectomy with cricohyoidopexy: Apartial laryngeal procedure for selected supraglottic and transglottic carcinomas. Laryngoscope 1990; 100:735-41.

(16.) Luna-Ortiz K, Granados Garcia M, Veivers D, et al. [Supracricoid laryngectomy with cricohyoidoepiglottopexy (CHEP). Preliminary report of the National Institute of Cancer]. Rev Invest Clin 2002;54:515-20.

(17.) Luna-Ortiz K, Nunz-Valencia ER, Tamez-Velarde M, GranadosGarcia M. Quality of life and functional evaluation after supracricoid partial laryngectomy with cricohyoidoepiglottopexy in Mexican patients. J Laryngol Otol 2004;118:284-8.

Kuauhyama Luna-Ortiz, MD; Adalberto Mosqueda-Taylor, DDS, MSc

From the Department of Head and Neck Surgery, Instituto Nacional de Cancerologia, Tlalpan (Dr. Luna-Ortiz), and the Oral Pathology Laboratory, Universidad Autonoma Metropolitana Xochimilco, Col. Villa Quietud (Dr. Mosqueda-Taylor), Mexico.

Table. Comparison of histogenesis and the histologic and
immunohistochemical features of sarcomatoidcarcinoma and
carcinosarcoma

                       Sarcomatoid carcinoma

Histogenesis           Squamous cell carcinoma with an atypical
                       pseudosarcomatous stromal reaction;
                       epithelial with transformation to fusiform
                       morphology

Histology              Dysplasia to invasive carcinoma;
                       mesenchymal carcinoma with pleomorphic
                       cell component or fusiform cells, scarce or
                       no mitoses, and bizarre cells; areas of
                       transition are observed

Immunohistochemistry   Epithelial component is positive to
                       cytokeratin; sarcomatoid component is
                       positive to cytokeratin and occasionally
                       positive to vimentin with coexpression to
                       cytokeratin

                       Carcinosarcoma

Histogenesis           Primitive mesenchymal blastic cell
                       differentiation that can mature and
                       produce malignant neoplasms of
                       multiple differentiation

Histology              Dysplasia to invasive carcinoma or
                       adenocarcinoma; true sarcoma is the
                       major component with high mitotic index
                       and cell pleomorphism; no areas of
                       transition are observed

Immunohistochemistry   Epithelial component is positive to
                       cytokeratin; mesenchymal component
                       is positive to vimentin and other markers,
                       (S-100, actin, desmin, etc.) depending on
                       the tumor's origin