Diagnostic Role of Intraoperative Imprint Cytology in an Unusual Case of Chondrosarcoma Presenting as Acute Onset Stridor.

Sir,

Mesenchymal tumors of the larynx are extremely rare, and chondrosarcomas of the larynx account for less than 0.5% of all primary tumors.[1234]. We report a rare case of a chondrosarcoma of the larynx who presented with acute onset stridor, wherein a definitive preoperative diagnosis could not be established on clinical examination and imaging. Intraoperative touch imprint cytology was suggestive of a chondrosarcoma, which was confirmed on histopathological examination.

A 52-year-old male presented to the emergency department with acute onset stridor since morning and associated hoarseness of voice for six weeks. The stridor was aggravated on exertion and relieved on lying down. An emergency tracheostomy was performed. Direct laryngoscopy showed a posterior cricoid mass. The vocal cords were not visualized and endoscope was not negotiable beyond the larynx. Barium swallow showed a normal study. Contrast-enhanced magnetic resonance (CEMRI) showed a well-defined enhancing growth epicentered in the right true vocal cord with supraglottic and subglottic extensions, thyroid cartilage spread, and subglottic extralaryngeal spread suggestive of glottic carcinoma [Figure 1a]. Positron emission tomography scan revealed metabolically active disease in larynx measuring 3.6 × 3.1 × 3.5 cm which was suggestive of carcinoma larynx. Repeated laryngoscopic biopsies were inconclusive. A surgical exploration with total laryngectomy and bilateral neck dissection was performed. Intraoperatively, touch preparations were moderately cellular with clusters of polygonal cells, including many binucleate cells, having well-defined cytoplasm, rounded nuclei showing moderate nuclear pleomorphism embedded in a chondromyxoid background, suggestive of chondrosarcoma [Figure 1b]. Total laryngectomy specimen revealed a well-circumscribed right posterior cricoid mass extending to the left side and inferiorly up to the upper tracheal ring. Cut surface showed cavitary lesion measuring 3.7 × 2.9 × 2.5 cm filled with abundant gelatinous material [Figure 1c]. Histologic sections [Figure 1d] showed a multilobular growth pattern with clusters of malignant chondrocytes exhibiting moderately pleomorphic, round to oval nuclei showing occasional mitosis in a chondroid matrix. No extension into vocal cords, thyroid cartilage, epiglottis, surgical margins, or resected neck nodes was seen. Final opinion of “grade II chondrosarcoma, cricoid cartilage” was given.

Figure 1

(a) Contrast-enhanced MRI image (sagittal view) showing laryngeal mass (arrow). (b) Imprint smears showing clusters of neoplastic chondrocytes having well-defined cytoplasm and moderate nuclear pleomorphism along with binucleate cells in a chondromyxoid matrix (Leishman Giemsa stain, ×400). (c) Laryngectomy specimen showing cavitary tumor measuring 3.7 × 2.9 × 2.5 cm within cricoid cartilage. (d) Histopathologic section showing grade-2 chondrosarcoma with fibrous capsule (H and E, ×100)

Chondrosarcomas of larynx are slow growing tumors occurring predominantly in the sixth decade.[123] The most common presenting symptom is hoarseness of voice; others being stridor, neck mass, dyspnea, dysphagia, and dysphonia.[3] Our patient presented to the emergency with stridor, which is a late uncommon presentation.[23] Many authors suggest that CT is the imaging examination of choice, as it detects bone destruction and intratumoral calcification.[123] CEMRI did not help in reaching a conclusive diagnosis in this case. Repeated biopsies also did not help as the lesion was located posteriorly and was covered by a thick fibrous capsule. Cytology of chondrosarcoma of head and neck has rarely been reported in literature.[5] To our knowledge, this is the first report from the Indian literature.

To conclude, obtaining a biopsy in rare cases of laryngeal chondrosarcoma is extremely difficult due to its thick fibrous capsule and its posterior location in the subglottic area, wherein an intraoperative touch imprint proves to be of immense diagnostic help.

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