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Year : 2011  |  Volume : 54  |  Issue : 3  |  Page : 581-583
Alveolar soft-part sarcoma of tongue

1 Department of Oral medicine and Radiology, Faculty of Dental Sciences, Porur, Chennai, Tamil Nadu, India
2 Department of Pathology, Sri Ramachandra University, Porur, Chennai, Tamil Nadu, India

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Date of Web Publication20-Sep-2011


Alveolar soft-part sarcoma is a clinically and morphologically distinct soft-tissue sarcoma of adolescent and young adult patients. Though immunohistochemical stains implicate a myogenic origin, the histogenesis of this tumor has not yet been established. Its high vascular nature leads to dissemination of the tumor cells into the bloodstream and metastasis. It comprises less than 1% of all soft-tissue sarcomas and less than 0.1% of sarcomas of the head and neck, preferably involving the orbit (48%) and tongue (25%). Lingual involvement is very rare and only about 31 cases have been reported in English literature. Their deceivingly indolent clinical courses often lead to misdiagnosis and delayed treatment. The reported case indicates its asymptomatic nature and microscopic similarity to granular cell tumor, which is the common benign tumor of the tongue.

Keywords: Alveolar soft-part sarcoma, granular cell tumor

How to cite this article:
Anbarasi K, Sathasivasubramanian S, Kuruvilla S, Susruthan. Alveolar soft-part sarcoma of tongue. Indian J Pathol Microbiol 2011;54:581-3

How to cite this URL:
Anbarasi K, Sathasivasubramanian S, Kuruvilla S, Susruthan. Alveolar soft-part sarcoma of tongue. Indian J Pathol Microbiol [serial online] 2011 [cited 2022 Aug 7];54:581-3. Available from: https://www.ijpmonline.org/text.asp?2011/54/3/581/85099

   Case Report Top

A 25-year-old man reported with a complaint of swelling on the tongue of three years' duration. It was smaller in size and asymptomatic initially. Gradual enlargement associated with discomfort on tongue movement made him consult an oral physician. The patient denied any other medical problems. On examination there was an ovoid, 3 Χ 2 cm, firm, non-tender, non-fluctuant, fixed swelling evident on the right dorsum of the anterior tongue [Figure 1]. The mucosa overlying the swelling was normal in color with no evidence of blanching and pulsations. He had no cervical lymphadenopathy.
Figure 1: Nodular swelling on the right dorsum of the tongue

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Benign tumor of the tongue was considered as the clinical diagnosis and incisional biopsy was done. The report revealed solitary cellular lesion composed of sheets of polypoidal cells with abundant granular eosinophilic cytoplasm and oval vesicular nuclei arranged in organoid pattern with many intervening thin-walled blood vessels. Skeletal muscle was seen at the periphery with no evidence of mitosis. Features were compatible with those of granular cell myoblastoma. Immunohistochemical studies and special staining procedures were carried out for confirmation of the diagnosis. The results were negative for S-100 [Figure 2], vimentin, desmin, cytokeratin, smooth-muscle actin, Human Melanoma Black-45, Neuron-Specific Enolase and synaptophysin. Periodic acid Schiff- Diastase staining revealed intracytoplasmic needle-shaped crystals consistent with alveolar soft-part sarcoma (ASPS).
Figure 2: Immunohistochemical staining showing negative result for S 100

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Subsequently, computerized tomographic examination of the chest and abdomen was performed and metastasis of lung and liver ruled out.

The lesion was completely excised under general anesthesia. Microscopically, the lesion showed a poorly circumscribed tumor of 4 mm thickness consisting of polygonal cells with abundant granular cytoplasm and vesicular nuclei arranged in pseudo alveolar pattern [Figure 3]. Some of the nests showed loss of cohesion and central necrosis. Lymphovascular invasions were evident with little mitosis. All the margins were free of tumor. On periodic acid-schiff (PAS) stains, characteristic PAS-positive, diastase-resistant granular material was found [Figure 4]. The patient had an uneventful postoperative course and remains well with no recurrence three years after his surgery and is still under regular follow-up.
Figure 3: Cells arranged in alveolar pattern with abundant granular cytoplasm (Hematoxylin and eosin, x100)

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Figure 4: Reveling PAS-positive, diastase-resistant granules (Periodic acid- Schiff , x200)

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   Discussion Top

ASPS is a rare variety of sarcoma affecting individuals in the first two decades of life and has female preponderance. [1] Christopherson et al, in 1952 first named it as alveolar soft-part sarcoma due to its histological appearances and distinguished it from other bony sarcomas. [2] Various terminologies like malignant organoid granular cell myoblastoma, angioendothelioma and liposarcoma were used to represent the same condition. ASPS was originally considered as a malignant variant of granular cell myoblastoma and subsequently has been disproved by several ultra-structural and immunohistochemical studies by denoting the absence of neuroendocrine differentiation in ASPS. [3] Genetic analysis of ASPS revealed unbalanced chromosomal translocation der (17) t(x; 17) (p11.2; q25) and so, it is included in the family of translation associated sarcomas. [4] The presence of two X chromosomes in female enhances the chance of translocation on this chromosome, thereby showing female predilection. [5] The transcription factor gene (TFE3) of X chromosome fuses with Chromosome 17 and the aberrant fusion protein aspl-tfe3 formed in this event is largely distributed on tumor cells.

Metastasis is common for lung (42-65%), brain and bone. Lymph node involvement is unusual. Patients with localized form of disease are in the mean age of 22 years and have favorable prognosis with prolonged survival. [4] Metastatic lesion alone or combination of local recurrence with metastatic lesion is the frequent complication. Solid pattern of ASPS varies from sheet-like appearance in very young children to solid organoid nests in adolescent patients. The characteristic alveolar type is seen in young adult patients. [6] Antibody against the C-terminus of the TFE3 is a highly sensitive diagnostic marker for ASPS but strong TFE3 positivity of granular cell tumor is potentially confusing. [7] Computerized tomography (CT) and magnetic resonance imaging (MRI) mainly help to decide the tumor-free resection margins. Ashley et al., reported a case of ASPS on the tongue, which mimicked features of hemangioma on both CT and MR images. [6] They suggested that the presence of a hyperintense lesion with central necrosis and flow voids within the lesion on T1- and T2- weighted MRI will support the diagnosis of ASPS.

Aggressive surgical excision of the primary tumor is the recommended treatment option to prevent local recurrence. [8] The role of adjuvant chemotherapy (doxorubicin plus ifosfamide) is inconclusive. Radiotherapy remains to be established for primary tumor management but is recommended for residual lesions after surgical excision and for metastatic conditions. [9]

ASPS reveals no atypical mitotic figures and resembles hamartomatous granular cell tumor (GCT). GCT is an asymptomatic nodular growth, measuring less than 3 cm in size and covered by normal mucosa. [10] The microscopic picture of GCT shows strands of cells with granular eosinophilic cytoplasm and absence of mitotic figures. But GCTs show strong positive S-100 protein expression in their cells. [11] Surgical excision is the treatment of choice with reports of low to negligible rate of recurrence. PAS-positive and diastase-resistant rhomboid crystals are present in almost every tumor cell cytoplasm and act as a diagnostic marker for ASPS. In addition, tumor cells exhibit characteristic round, regular, eccentrically placed nuclei with vesicular chromatin and prominent nucleolus. Confirmatory diagnosis is largely based on immunohistochemical investigations like Positive result for C - terminus of ASPL - TFE3 fusion protein and negative results for epithelial markers (like cytokeratin and epithelial membrane antigen), specific endocrine markers (chromogranin A and synaptophysin), and specific melanocyte markers (HMB45 and Melan A). [5] The prognostic parameters of ASPS include age at diagnosis, tumor size and presence of metastasis.

To conclude, the clinical nature and microscopic appearance of ASPSs are variable and resemble benign lesions on many occasions, similar to our case. In contrast to other sites, the primary tongue tumor has a good prognosis and long tumor-free survival period, if diagnosed appropriately and treated early. Hence investigations should extend beyond the routine histopathological examination for differentiation from benign lesions.

   References Top

1.Portera CA Jr, Ho V, Patel SR, Hunt KK, Feig BW, Respondek PM, et al. Alveolar soft part sarcoma: Clinical course and patterns of metastasis in 70 patients treated at a single institution. Cancer 2001;91:585-91.  Back to cited text no. 1
2.Christopherson WM, Foote FW, Stewart FW. Alveolar soft- part sarcomas: Structurally characteristic tumors of uncertain histogenesis. Cancer 1952;5:100-11.  Back to cited text no. 2
3.Ordonez NG. Alveolar soft part sarcoma; a review and update. Adv Anat Pathol 1999;6:125-39.  Back to cited text no. 3
4.Fanburg-Smith JC, Miettinen M, Folpe AL, Weiss SW, Childers EL. Lingual alveolar soft part sarcoma; 14 cases: Novel clinical and morphological observations. Histopathology 2004;45:526-37.  Back to cited text no. 4
5.Folpe AL Deyrup AT. Alveolar soft part sarcoma: A review and update. J Clin Pathol 2006;59:1127-32.  Back to cited text no. 5
6.Aiken AH, Stone JA. Alveolar soft part sarcoma of the tongue. Am J Neuroradiol 2003;24:1156-8.  Back to cited text no. 6
7.Sreekantaiah C, Li FP, Weidner N, Sandberg AA. Multiple and complex abnormalities in a case of alveolar soft- part sarcoma. Cancer Genet Cytogenet 1991;55:167-71.  Back to cited text no. 7
8.Ryu J, kwon Y, Park BK, Jung YS. Lingual alveolar sarcoma treated only by conservative resection. Int J Pediatr Otorhinolaryngol Extra 2006;1:243-8.  Back to cited text no. 8
9.Donald PJ. Alveolar soft part sarcoma of the tongue. Head Neck Surg 1987;9:172-8.  Back to cited text no. 9
10.Goodstein ML, Eiselle DW, Hyams JV, Kashima HK. Multiple synchronous granular cell tumors of the upper aerodigestive tract. Otolaryngol Head Neck Surg 1990;103:664-8.  Back to cited text no. 10
11.Nekazato Y, Ishizeki J, Takahashi K. Immunohistochemical localization of S-100 protein in granular cell myoblastoma. Cancer 1982;49:1624-5.  Back to cited text no. 11

Correspondence Address:
K Anbarasi
Department of Oral Medicine and Radiology, Faculty of Dental Sciences, Sri Ramachandra University, Chennai, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.85099

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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