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| LETTER TO THE EDITOR |
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| Year : 2015 | Volume
: 52
| Issue : 1 | Page : 125-126 |
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A rare and unusual occurrence of rhabdomyosarcoma arising from the larynx
GK Chiramel1, BR Chacko1, R Thomas2, D Jebakumar3
1 Department of Radiology, Christian Medical College, Vellore, Tamil Nadu, India
2 Department of ENT, Christian Medical College, Vellore, Tamil Nadu, India
3 Department of Pathology, Christian Medical College, Vellore, Tamil Nadu, India
| Date of Web Publication | 3-Feb-2016 |
Correspondence Address:
G K Chiramel
Department of Radiology, Christian Medical College, Vellore, Tamil Nadu
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0019-509X.175575
How to cite this article:
Chiramel G K, Chacko B R, Thomas R, Jebakumar D. A rare and unusual occurrence of rhabdomyosarcoma arising from the larynx. Indian J Cancer 2015;52:125-6 |
Sir,
Laryngeal cancer accounts for 25% of head and neck cancers and is an important cause of morbidity and mortality in smokers.[1] More than 95% of the laryngeal malignancies are squamous call carcinomas. Non-squamous malignant neoplasms like sarcomas and salivary gland tumors are very rare. While mesenchymal tumors which originate from the larynx have been reported, rhabdomyosarcoma, a malignant tumor of striated muscle, is a very rare occurrence in the larynx.[1]
A seventy-year-old man presented with two years of gradually worsening hoarseness of voice and obstructive sleep apnoea with the sensation of a lump in the throat but did not have pain, dysphagia or dyspnoea. There were no symptoms associated with the ear or nose. Flexible laryngoscopy showed a smooth pink swelling involving the right aryepiglottic fold and both arytenoids showing decreased mobility. The right pyriform fossa could not be visualised because of the bulk of the tumour. Computed Tomography scan (CT scan) of the neck showed a well-defined lobulated enhancing mass measuring 2.8 × 2.6 cm arising from the right aryepiglottic fold and narrowing the supraglottic airway and involving the posterior pharyngeal wall [Figure 1]. Microlaryngoscopy and biopsy of the lesion were performed under general anaesthesia. Biopsy showed fibrocollagenous tissue covered by stratified squamous epithelium and fragments of a tumour composed of sheets of polygonal to spindle cells with moderately pleomorphic vesicular to hyperchromatic mitotically active nuclei, a few prominent nucleoli and moderate amounts of granular eosinophilic cytoplasm [Figure 2]. Many bizzare tumour cells were also present. On immunostaining, the cells showed strong and diffuse cytoplasmic positivity for Desmin and Vimentin [Figure 3], focal membrane positivity for cytokeratin and were negative for S-100, MYF-4 and EMA. These findings suggested a malignant pleomorphic neoplasm with predominantly rhabdomyosarcomatous component. Since the patient had malignant involvement of the posterior pharyngeal wall at presentation (stage T2N0M0), he was advised chemotherapy and irradiation followed by surgery - total laryngopharyngoesophagectomy with gastric pull through. The patient was not keen on surgery andhe did not return for treatment after the initial dose of chemotherapy. | Figure 1: (a and b) Axial images of the contrast enhanced CT scan of the neck showing the malignant lesion as a polypoid enhancing soft tissue component projecting into the supraglottic lumen from the posterior aspect
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 | Figure 2: Low power magnification (H and E, ×200) shows fragments of tumour composed of sheets of undifferentiated round, polygonal and spindle shaped cells with nuclear pleomorphism and granular eosinophilic cytoplasm. Inset (H and E, ×400) highlights striking nuclear pleomorphism
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 | Figure 3: High power magnification (×400) shows strong and diffuse cytoplasmic positivity of tumour cells for Vimentin (left half) and Desmin (right half)
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Rhabdomyosarcoma is a mesenchymal tumour predominantly seen in the pediatric age group [1] and is classified into pleomorphic and embryonal histological types. The larynx is a very rare site. The presentation is similar to squamous cell carcinoma and could vary from a voice alteration to a lump-like sensation in the throat. They grow rapidly and have a short history.[2] Both benign and malignant lesions can appear polypoid. Diagnosis is made from the histopathological examination of a biopsy taken during direct laryngoscopy. Imaging studies such as contrast enhanced computed tomography would help to determine the tumour extent. Although surgical treatment and adjuvant radiotherapy is currently the treatment of choice for laryngeal malignancies,[3] a combination of adjuvant chemotherapy with radiotherapy is preferred since it reduces the need for surgery, which can be very mutilating. It is still unclear if this approach has any significant effect on the outcomes.[4] Prognostic groups and treatment plans were first suggested by the Intergroup Rhabdomyosarcoma Study Group which then merged with other groups to form the Children's Oncology Group. The Soft Tissue Sarcoma Committee of this group uses the TNM-based staging system to prognosticate and develop treatment protocols for rhabdomyosarcoma.[5] The prognosis depends on the primary tumor site, presence or absence of tumor invasion of surrounding tissues, tumor size, regional lymph node status, and the presence or absence of metastases.[6] The treatment plans prescribed by the IRS-I, IRS-II and IRS-III studies were based on surgery and pathology where groups were defined by the disease extent and by the completeness of initial surgical resection after pathologic review of the tumor specimen.[7],[8]
This is an unusual occurrence of a pleomorphic rhabdomyosarcoma in the larynx of an elderly man who was a non-smoker and highlights the fact that laryngeal rhabdomyosarcoma is a possibility that should be considered even in the older age group even though it is extremely rare. Appropriate diagnosis and treatment should be performed before the tumour spreads to involve the surrounding structures, which would render it inoperable.
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| 2. | Newman AN, Rice DH. Rhabdomyosarcoma of the head and neck. Laryngoscope 1984;94:234-9. [ PUBMED] |
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| 4. | Shayah A, Agada FO, Karsai L, Stafford N. Adult laryngeal rhabdomyosarcoma: Report of a case and literature review. Ann Afr Med 2007;6:190-3. [ PUBMED]  |
| 5. | Lawrence W Jr, Gehan EA, Hays DM, Beltangady M, Maurer HM. Prognostic significance of staging factors of the UICC staging system in childhood rhabdomyosarcoma: A report from the Intergroup Rhabdomyosarcoma Study (IRS-II). J Clin Oncol 1987;5:46-54. [ PUBMED] |
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| 8. | Crist W, Gehan EA, Ragab AH, Dickman PS, Donaldson SS, Fryer C, et al. The third intergroup rhabdomyosarcoma study. J Clin Oncol 1995;13:610-30. |
[Figure 1], [Figure 2], [Figure 3]
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