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Introduction
Rhabdomyosarcoma is one of the most commonly seen soft-tissue sarcomas in children and teenagers, approximately accounting for half of all soft-tissue sarcomas[1]. The tumor is usually found in head and neck, four limbs and urogenital system. However, the tumor originating from the laryngohypopharynx is rare. In 2008, a patient with alveolar rhabdomyosarcoma of the laryngohypopharynx was admitted to our hospital. Clinical information of the disease was reported as follows, and clinicopathologic features and current therapeutic principle were discussed and summarized in combination with the review of literature, to facilitate the diagnosis and treatment of the patients with rhabdomyosarcoma of the laryngohypopharynx in future.
Case Report
A 26 year-old man was admitted to the First Affiliated Hospital of Shanghai Jiaotong University in July, 2008 because of his cough accompanied by hoarseness for more than a year. The result of the physical examination showed that no lymphadenectasis was palpable in the central trachea and bilateral necks. An elliptical neoplasm was found in the epiglottis (-) and supra-glottic region and covered the postmedian part of hibateral vocal cords, with a size of about 2.0 cm × 3.0 cm × 3.0 cm and a rough surface. The results of CT scan and MRI imaging on the neck showed that there was an orbicular-ovate soft-tissue tumor in the postcricoid area, which had rough surface and clear margin, with the size of approximate 22 mm × 31 mm × 33 mm and a uniform density. The left arytenoid swelling and vocal cord were found to be possibly involved. The preliminary diagnosis was laryngopharyngeal neoplasm. After preoperative preparation, the surgery was performed in which the laryngo-pharyngeal tumor was removed via the pathway of left neck under the general anesthesia. It was found during the surgery that the tumor was situated close to the postcricoid area of the left arytenoid swelling, protruding towards the laryngo-pharyngeal cavity and the supraglottic region and presented an exogenous cauliflower-like shape with the size of about 2.0 cm × 3.0 cm × 3.0 cm, an unconspicuous deep infiltration, and crispness. A complete excision of the tumor was performed during the surgery. The postoperative pathologic examination showed that rhabdomyosarcoma (RMS) cells presented a roundish or orbicular-ovate shape, with a little transparent cytoplasm. The cytoplasm was rich in part of the RMS cells and acidophilic, with dyssymmetry of the cell nucleus, a lamellate or alveolar cellular arrangement, or sporadic cellular infiltration. The cell arrangement was sparse in the center of the cell nests, forming an adenoid structure (H&E stained, 200 ×) (Fig. 1). The result of the pathologic diagnosis was a (post-cricoid area) alveolar rhabdomyosarcoma.
The tumor cells of RMS are round to polygonal in shape, with sparse, transparent cytoplasm. The cytoplasm was rich in part of the RMS cells, which is acidophilic, with a asymmetrical nucleus, a lamellar or alveolar cellular arrangement with sporadic cellular infiltration. The cell arrangement was sparse in the center of the cell nests, forming an adenomatoid structure (H&E stained, 200 ×).
Discussion
The rhabdomyosarcoma originates from the primitive mesenchymal cell which differentiates to the striated muscle, and is compose of the striated muscle cells with various levels of differentiation. The pathological classification of RMS mainly includes the embryo, the alveolus and the multiformity types. The embryo type contains 2 subtypes, i.e. the botryoid and fusiform cell types. The alveolus type approximately accounted for 20% of all types of RMS, and this type is usually found in children and teenagers[1]. However, the patient treated in our hospital was an adult, which was rarely seen before. The clinical manifestation of RMS depends on the primary focus of the tumor. In this case, the tumor was situated close to the post-cricoid area of the arytenoid swelling, causing paroxysmal cough and hoarseness. This clinical manifestation is similar to that of the laryngeal RMS[2,3], therefore, it may lead misdiagnosis. In this case, the imaging methods such as CT, MRI, and endoscopic examination should be taken to precisely distinguish the tumor location. The immunohistochemical stain is of important value in diagnosis of RMS, and the indicators of the assay mainly include the following: intermediate filament proteins, cytoplasm proteins and the contractile proteins[4,5]. At present, the modality of the treatment includes chemotherapy plus surgery or radiotherapy or plus both for treating RMS. The studies of Ogilvie et al.[6] showed that the 2-year overall survival rate and 2-year disease-free survival rate could respectively achieve 55% and 64% in the patients treated with the therapeutic alliance of surgery combined with chemo-radiotherapy. It was reported by Zhang et al.[7] that the combined therapy of the surgery plus chemo-radiotherapy could apparently raised the survival rate of the patients with head and neck RMS, especially the survival rate of the early RMS patients. The VAC therapeutic regimen (Vincristine, actinomycin D, cyclophosphamide) was the main modality of chemotherapy recognized by the international organization of RMS research[8]. The recommended radiation dose was in an range from 36 Gy and 50.4 Gy, based on the age and tumor site of the patients[9]. Since there was the tendency of a partial excision of tumor in the surgery of RMS, usually combining with other modalities of treatment, a severe impairment of the organs and tissues and the functional incapacitation could both be avoided as the survival rate rose[10,11]. In regard to this case, his tumor was completely excised, without the procedure of laryngectomy. A boost of the radiation and chemotherapy were given to the patient after the surgery, and no signs of RMS recurrence have been found till now. The correct early diagnosis, standard treatment and regular follow-up are the key to improve the survival rate of the patients.
Conflict of interest statement
No potential conflicts of interest were disclosed.
- Received January 5, 2010.
- Accepted February 12, 2010.
- Copyright © 2010 by Tianjin Medical University Cancer Institute & Hospital and Springer
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