A Rare Case of Malignant Peripheral Nerve Sheath Tumor of Larynx
- PDF / 556,847 Bytes
- 3 Pages / 595.276 x 790.866 pts Page_size
- 86 Downloads / 169 Views
CLINICAL REPORT
A Rare Case of Malignant Peripheral Nerve Sheath Tumor of Larynx U. V. Akshay Viswanath1 • V. P. Salim2 • Salahudheen Thottiyen2 M. Lilly3
•
Received: 1 September 2020 / Accepted: 16 October 2020 Ó Association of Otolaryngologists of India 2020
Abstract Malignant peripheral nerve sheath tumors are highly aggressive tumors and they arise either de novo or from preexisting benign schwannoma or neurofibromatosis. Malignant peripheral nerve sheath tumor of the larynx is an extremely rare neoplasm, for which accurate diagnosis is difficult and the therapy is usually delayed. Although wide surgical excision is the mainstay of treatment, radiation therapy also plays an important role in the control and treatment of locally recurrent disease and non resectable cases. Here we are reporting a case of malignant peripheral nerve sheath tumor of the larynx without definite evidences of von Recklinghausen’s disease or preexisting benign peripheral nerve sheath tumor. Keywords Malignant peripheral nerve sheath tumor Larynx
Case Summary This 75 year old gentleman, with no history of any comorbidites, presented with complaints of hoarseness of voice, which he noticed for 2 month duration. General physical examination and systemic examinations were
& U. V. Akshay Viswanath [email protected] 1
Department of General Surgery, Aster Malabar Institute of Medical Sciences (MIMS), Govindapuram P.O., Calicut, Kerala 673016, India
2
Department of Oncosurgery, Aster Malabar Institute of Medical Sciences (MIMS), Calicut, India
3
Department of Pathology, Aster Malabar Institute of Medical Sciences (MIMS), Calicut, India
nothing contributory. He was evaluated and laryngoscopy was done which showed an ulcerated fleshy mass in the subglottic region, more towards the left side. CT of the neck (Fig. 1a) was taken for further evaluation, in which a minimally enhancing soft tissue thickening of size 1.5 9 1 cm was noted in the infraglottic region, more to the left side and extending to the anterior commissure with enlarged bilateral level 1b, 2 and level 5 lymphnodes. No luminal narrowing was noted. Biopsy was taken with help of VLS and tracheostomy was performed in view of possible stridor. Morphology and immunohistochemistry features (elevated S100) of the specimen was suggestive of Malignant peripheral nerve sheath tumor. As the metastatic workup was negative, decided to proceed with total laryngectomy. Intraoperatively, a greyish white ulceroproliferative mass of size 3 9 1.8 9 1 cm was noted, extending from supraglottic region to subglottic region (Fig. 1b). Laryngectomy was done and an end tracheostomy was created. Pathological examination showed neoplasm in the larynx, composed of spindle shaped cells arranged diffusely and in fascicles (Fig. 2a). Cells showed fusiform and ovoid vesicular hyperchromatic nuclei and distinct nucleoli with moderate cytoplasm. Tumor cells were showing positivity on S100 staining (Fig. 2b). Tumor was invading the thyroid and cricoids cartilage. There were of necrosis present within the
Data Loading...
