The Journal of Laryngology and Otology October 1979. Vol. 93. pp. 1031-1035
True carcinosarcoma of the larynx: a case report By USHA SRINIVASAN and G. V. TALVALKAR
TUMOURS of mixed mesenchymal and epithelial origin are very rare, as is evident from the very infrequent reports of such tumours in the literature. These are known to occur in the uterus, breast (Harris and Persaud, 1974), bladder (Holtz et ah, 1972), stomach, and rarely in other sites (Ashley, 1978). The larynx appears to be a highly unusual site, only two authentic cases having been described. Not only is carcinosarcoma rare in the larynx, but other mesenchymal tumours are also uncommon. We report here a case of a true 'collision' tumour of the larynx showing rhabdomyosarcomatous and squamous components.
Case report A 55-year-old male was admitted to our hospital with complaints of hoarseness, dysphagia and a burning sensation in the throat for two months. He was a heavy smoker. He was seen by an otolaryngologist before he came to our hospital. Biopsy of the tumour was performed and the patient was referred to our centre for further treatment. Indirect laryngoscopic examination in our hospital revealed a bulky polypoidal tumour of the left arytenoid and aryepiglottic fold. No lymph nodes were detected in the neck. In view of the histopathological report of a rhabdomyosarcoma, laryngectomy was planned. However, unfortunately the patient developed acute laryngeal obstruction and in spite of an emergency tracheostomy, he died within two hours of developing the obstruction. An autopsy was performed. Pathology Autopsy findings: A bulky, grey-pink, polypoidal, finely lobulated tumour was found to be blocking the glottic area completely (Fig. 1). The tumour was attached to the left pyriform fossa by a thin pedicle and it had produced a ballvalve obstruction of the airway. The tumour measured 3-5x3 cm, was soft and had a pink tan, glistening, lobulated cut surface. Ther*; were no other significant findings, except for marked oedema and congestion of the lungs. The cervical lymph nodes were not involved. Microscopic findings The original biopsy referred by the original surgeon revealed a small fragment of tumour lined by a thin epithelium without any evidence even of mild dysplasia (Fig. 2). The tumour itself was pleomorphic (Fig. 3) and was made up of spindle, strap-like, ovoid and polygonal cells, with a fair amount of acidophilic cytoplasm which was granular or homogeneous. The nuclei were hyperchromatic and showed considerable variations in size. Numerous bizarre mitoses were 1031
1032
U. SRINIVASAN AND G. V. TALVALKAR
FIG. 1 Showing a bulky polypoidal tumour blocking the glottic airway. The tumour is attached to the pyriform fossa by a thin pedicle.
noted, and scattered bizarre giant cells were seen. The most striking feature was the presence of striations, both transverse and longitudinal, demonstrated clearly even in the haematoxylin and eosin stain (Fig. 4). This was reported as a pleomorphic rhabdomyosarcoma. The microscopic features of the tumour seen at the autopsy were interesting. In addition to the rhabdomyosarcomatous component, which was predominant, areas of in-situ and infiltrating squamous cell carcinoma were also detected (Fig. 5). The squamous cell carcinoma was grade II in nature. PTAH staining showed cross striations of rhabdomyoblasts, even more prominently than were seen in the haematoxylin and eosin stain. The cervical lymph nodes did not show metastasis. Discussion
Carcinosarcomas are very rare neoplasms which may occur in several organs. The diagnosis implies a mixed neoplasm containing both mesenchymal and epithelial elements, each of which displays the histologic and biologic criteria of malignancy. In the larynx, the diagnosis of an apparently mixed tumour is often controversial. According to Goellner, Devine and Weiland (1973), the sarcomatous pattern in these tumours is a unique non-malignant connective tissue
CLINICAL RECORDS
1033
FIG. 2 Photomicrographs of original biopsy of the tumour showing a submucous pleomorphic tumour. Overlying epithelium is almost normal (H. and E., x 320).
FIG. 3 Higher magnification of the same tumour showing marked pleomorphism, atypical mitosis and giant cells (H. and E., x800).
1034
U. SRINIVASAN AND G. V. TALVALKAR
£?4
**
-
'N
FIG. 4 Showing cross and longitudinal striations of the rhabdomyoblasts (H. and E., x Oil immersion).
FIG. 5 Showing squamous cell carcinoma grade II with adjoining rhabdomyosarcomatous elements (H. andE., x320).
CLINICAL RECORDS
1035
reaction in association with moderately differentiated squamous cell carcinoma; but others (Hyams, 1974) believe that the sarcomatous component is nothing but a spindle-cell anaplasia of the squamous component. Nevertheless, true carcinosarcomas do occur in the larynx. Batsakis (1974) mentions one case of true carcinosarcoma with a mixture of rhabdomyosarcoma and squamous cell carcinoma. Another authentic case has been reported by Minkler et al. (1970). They described a lesion which metastasized both as carcinoma and as sarcoma and resulted in the death of the patient 15 months after its detection. Mesenchymal tumours of the larynx are by themselves very unusual, accounting for not more than 2 per cent of all laryngeal neoplasms (Batsakis, 1974). Frugoni and Ferlito (1976) reviewed the literature comprehensively and quoted only 7 well-documented cases of rhabdomyosarcoma, including their own case. Only 3 of these cases belonged to the category of pleomorphic rhabdomyosarcoma, the remaining four cases being embryonal variants occurring in children. Because of the paucity of reported cases of carcinosarcoma and pleomorphic rhabdomyosarcoma, it is not possible to make any valid generalizations about their clinical and histological behaviour, and the symptoms of these rare neoplasms do not differ from those of other neoplasms. However, these tumours are usually polypoidal, with intact overlying mucosa; hence, acute respiratory obstruction is common. Carcinosarcoma appears to be a highly malignant tumour, whereas laryngeal rhabdomyosarcoma appears to be slightly less aggressive than rhabdomyosarcoma of the head and neck (Frugoni and Fertito, 1976). Summary
A case of true carcinosarcoma of the larynx occurring in a 55-year-old male is reported. The two components of this very rare tumour are pleomorphic rhabdomyosarcoma and squamous cell carcinoma. A review of the literature has revealed only two authentic cases of true carcinosarcomas. REFERENCES ASHLEY, D. J. B. (1978) Evans' histologic appearances of tumours. 3rd Edition, Published by Churchill Livingstone. BATSAKIS, J. G. (1974) Tumours of head and neck—clinical and pathological considerations. Published by Williams and Wilkins, Co., Baltimore. FRUGONI, P., and FERLITO, A. (1976) Journal of Laryngology and Otology, 90. 687. GOELLNER, J. R., DEVINE, K. D., and WEILAND, L. H. (1973) American Journal of Clinical
Pathology, 59, 312. HARRIS, M., and PERSAUD, (1974) Journal of Pathology, 112, 99. HOLTZ, F., Fox, J. E., and ABELL, M. R. (1972) Cancer, 29, 294.
HYAMS, V. J. (1974) Centennial Conference on Laryngeal Cancer. Published by AppletonCentury-Crofts, New York, p. 489. MINCKLER, D. S., MELIGRO, C. H., and NORRIS, H. T. (1970) Cancer, 26, 195.
Department of Pathology, Tata Memorial Hospital, Parel, Bombay 400 012, India.
True carcinosarcoma of the larynx: a case report By USHA SRINIVASAN and G. V. TALVALKAR
TUMOURS of mixed mesenchymal and epithelial origin are very rare, as is evident from the very infrequent reports of such tumours in the literature. These are known to occur in the uterus, breast (Harris and Persaud, 1974), bladder (Holtz et ah, 1972), stomach, and rarely in other sites (Ashley, 1978). The larynx appears to be a highly unusual site, only two authentic cases having been described. Not only is carcinosarcoma rare in the larynx, but other mesenchymal tumours are also uncommon. We report here a case of a true 'collision' tumour of the larynx showing rhabdomyosarcomatous and squamous components.
Case report A 55-year-old male was admitted to our hospital with complaints of hoarseness, dysphagia and a burning sensation in the throat for two months. He was a heavy smoker. He was seen by an otolaryngologist before he came to our hospital. Biopsy of the tumour was performed and the patient was referred to our centre for further treatment. Indirect laryngoscopic examination in our hospital revealed a bulky polypoidal tumour of the left arytenoid and aryepiglottic fold. No lymph nodes were detected in the neck. In view of the histopathological report of a rhabdomyosarcoma, laryngectomy was planned. However, unfortunately the patient developed acute laryngeal obstruction and in spite of an emergency tracheostomy, he died within two hours of developing the obstruction. An autopsy was performed. Pathology Autopsy findings: A bulky, grey-pink, polypoidal, finely lobulated tumour was found to be blocking the glottic area completely (Fig. 1). The tumour was attached to the left pyriform fossa by a thin pedicle and it had produced a ballvalve obstruction of the airway. The tumour measured 3-5x3 cm, was soft and had a pink tan, glistening, lobulated cut surface. Ther*; were no other significant findings, except for marked oedema and congestion of the lungs. The cervical lymph nodes were not involved. Microscopic findings The original biopsy referred by the original surgeon revealed a small fragment of tumour lined by a thin epithelium without any evidence even of mild dysplasia (Fig. 2). The tumour itself was pleomorphic (Fig. 3) and was made up of spindle, strap-like, ovoid and polygonal cells, with a fair amount of acidophilic cytoplasm which was granular or homogeneous. The nuclei were hyperchromatic and showed considerable variations in size. Numerous bizarre mitoses were 1031
1032
U. SRINIVASAN AND G. V. TALVALKAR
FIG. 1 Showing a bulky polypoidal tumour blocking the glottic airway. The tumour is attached to the pyriform fossa by a thin pedicle.
noted, and scattered bizarre giant cells were seen. The most striking feature was the presence of striations, both transverse and longitudinal, demonstrated clearly even in the haematoxylin and eosin stain (Fig. 4). This was reported as a pleomorphic rhabdomyosarcoma. The microscopic features of the tumour seen at the autopsy were interesting. In addition to the rhabdomyosarcomatous component, which was predominant, areas of in-situ and infiltrating squamous cell carcinoma were also detected (Fig. 5). The squamous cell carcinoma was grade II in nature. PTAH staining showed cross striations of rhabdomyoblasts, even more prominently than were seen in the haematoxylin and eosin stain. The cervical lymph nodes did not show metastasis. Discussion
Carcinosarcomas are very rare neoplasms which may occur in several organs. The diagnosis implies a mixed neoplasm containing both mesenchymal and epithelial elements, each of which displays the histologic and biologic criteria of malignancy. In the larynx, the diagnosis of an apparently mixed tumour is often controversial. According to Goellner, Devine and Weiland (1973), the sarcomatous pattern in these tumours is a unique non-malignant connective tissue
CLINICAL RECORDS
1033
FIG. 2 Photomicrographs of original biopsy of the tumour showing a submucous pleomorphic tumour. Overlying epithelium is almost normal (H. and E., x 320).
FIG. 3 Higher magnification of the same tumour showing marked pleomorphism, atypical mitosis and giant cells (H. and E., x800).
1034
U. SRINIVASAN AND G. V. TALVALKAR
£?4
**
-
'N
FIG. 4 Showing cross and longitudinal striations of the rhabdomyoblasts (H. and E., x Oil immersion).
FIG. 5 Showing squamous cell carcinoma grade II with adjoining rhabdomyosarcomatous elements (H. andE., x320).
CLINICAL RECORDS
1035
reaction in association with moderately differentiated squamous cell carcinoma; but others (Hyams, 1974) believe that the sarcomatous component is nothing but a spindle-cell anaplasia of the squamous component. Nevertheless, true carcinosarcomas do occur in the larynx. Batsakis (1974) mentions one case of true carcinosarcoma with a mixture of rhabdomyosarcoma and squamous cell carcinoma. Another authentic case has been reported by Minkler et al. (1970). They described a lesion which metastasized both as carcinoma and as sarcoma and resulted in the death of the patient 15 months after its detection. Mesenchymal tumours of the larynx are by themselves very unusual, accounting for not more than 2 per cent of all laryngeal neoplasms (Batsakis, 1974). Frugoni and Ferlito (1976) reviewed the literature comprehensively and quoted only 7 well-documented cases of rhabdomyosarcoma, including their own case. Only 3 of these cases belonged to the category of pleomorphic rhabdomyosarcoma, the remaining four cases being embryonal variants occurring in children. Because of the paucity of reported cases of carcinosarcoma and pleomorphic rhabdomyosarcoma, it is not possible to make any valid generalizations about their clinical and histological behaviour, and the symptoms of these rare neoplasms do not differ from those of other neoplasms. However, these tumours are usually polypoidal, with intact overlying mucosa; hence, acute respiratory obstruction is common. Carcinosarcoma appears to be a highly malignant tumour, whereas laryngeal rhabdomyosarcoma appears to be slightly less aggressive than rhabdomyosarcoma of the head and neck (Frugoni and Fertito, 1976). Summary
A case of true carcinosarcoma of the larynx occurring in a 55-year-old male is reported. The two components of this very rare tumour are pleomorphic rhabdomyosarcoma and squamous cell carcinoma. A review of the literature has revealed only two authentic cases of true carcinosarcomas. REFERENCES ASHLEY, D. J. B. (1978) Evans' histologic appearances of tumours. 3rd Edition, Published by Churchill Livingstone. BATSAKIS, J. G. (1974) Tumours of head and neck—clinical and pathological considerations. Published by Williams and Wilkins, Co., Baltimore. FRUGONI, P., and FERLITO, A. (1976) Journal of Laryngology and Otology, 90. 687. GOELLNER, J. R., DEVINE, K. D., and WEILAND, L. H. (1973) American Journal of Clinical
Pathology, 59, 312. HARRIS, M., and PERSAUD, (1974) Journal of Pathology, 112, 99. HOLTZ, F., Fox, J. E., and ABELL, M. R. (1972) Cancer, 29, 294.
HYAMS, V. J. (1974) Centennial Conference on Laryngeal Cancer. Published by AppletonCentury-Crofts, New York, p. 489. MINCKLER, D. S., MELIGRO, C. H., and NORRIS, H. T. (1970) Cancer, 26, 195.
Department of Pathology, Tata Memorial Hospital, Parel, Bombay 400 012, India.
