A Rare Presentation of Extra Pulmonary Small Cell Carcinoma at Tongue

Case Report

Austin J Radiat Oncol & Cancer. 2016; 2(1): 1017.

A Rare Presentation of Extra Pulmonary Small Cell Carcinoma at Tongue

Soni A¹*, Dhull AK¹, Verma R², Kaushal V¹ and Verma M³

1Department of Radiotherapy, University of Health and Sciences, India

2Department of Pathology, University of Health and Sciences, India

3Department of Biochemistry, University of Health and Sciences, India

*Corresponding author: Soni A, Department of Radiotherapy, University of Health and Sciences, 236G Model Town, Rohtak, India

Received: May 16, 2016; Accepted: June 01, 2016; Published: June 03, 2016


Context: To report a case of extrapulmonary small cell carcinoma at a rare site of tongue with excellent prognosis with standard therapy.

Case: A 65-year-old previously healthy male presented initially with difficulty in swallowing and later, with a solitary neck lump and he was diagnosed as extrapulmonary small cell carcinoma of tongue. Unlike small cell carcinoma lung, extrapulmonary small cell carcinoma is uncommon and in base of tongue, it is extremely rare. Cisplatin (75 mg/m2) and Etoposide (100 mg/m2) were administered in combination at 3 weeks interval as neoadjuvant chemotherapy for 3 cycles, as well as concomitant chemotherapy for 3 cycles with external beam radiation in a dose of 64 Gy. The patient tolerated well the treatment.

Conclusion: The prognosis of extrapulmonary small cell carcinoma is felt to be extremely poor. Contrary to other reports, this case demonstrates that a good response with standard therapy is possible.

Keywords: Cisplatin; Poor; Tongue; Extrapulmonary; Radiation


Extra Pulmonary Small Cell Carcinoma (EPSCC) was first described by Duguid and Kennedy in 1930, as a clinic pathological entity different from Small Cell Carcinoma Lung (SCLC) [1,2,3]. The small cell carcinoma usually develops from lung and approximate 2.5- 4% of it arises in extrapulmonary sites [4,5]. However, EPSCC is still often confused with metastatic SCLC [6]. Unlike SCLC, the natural history of EPSCC remains undiscovered and hence, optimal therapy determination is complicated [7]. The most commonly reported sites with EPSCC are aero-digestive tract including paranasal sinuses, nasal cavity, salivary glands, thyroid gland, larynx and trachea [8]. It has also been found rarely in ovaries, prostate gland, urinary bladder, cervix and breast [9]. In the head and neck region, oral cavity and oropharynx are the rarest sites for EPSCC. The EPSCC shares the similar histopathological features as of SCLC. The clinical course of EPSCC is more aggressive than SCLC and being more recurrent, it usually demonstrates a poorer prognosis [6,7]. The treatment of EPSCC patients has been very likely to the protocols used for treating SCLC. Due of its chemo sensitive nature, most of the EPSCC patients have been treated with Cis-platinum based chemotherapy [10]. As it is a systemic disease, localized treatment as a sole modality produces only limited survival; so, multimodality therapy is preferred even at early stage [10]. Present case is a primary extrapulmonary small cell carcinoma with nodal metastasis in ipsilateral upper cervical node without paraneoplastic feature and good response to treatment suggesting a different clinic pathophysiological behaviour than typical pulmonary small cell carcinoma.

Case Presentation

A 65 years old male with 30 pack year history of smoking presented to radiotherapy department with difficulty in swallowing for the past 3 months. Initially the patient reported difficulty in swallowing to solid food which later progressed to semisolid food and eventually patient got dependent upon liquid diet only. The patient also reported associated history of pain in head and neck region since the past 2 months, which was continuous, progressive and relieved only after medication. The patient also reported lump in left side of the upper neck which was gradually progressive in nature and was associated with pain. There was no history of chewing betel nuts or tobacco. The patient was non-alcoholic. There was no significant past history related to hypertension, diabetes mellitus, tuberculosis or any other chronic illness. No significant family history was reported. Oral cavity examination showed a large, proliferative growth at base of tongue associated with left sided upper deep cervical lymphadenopathy of neck level II, 3.5 × 2 cm firm, mobile palpable mass. Rest of the systemic examination was within normal limits.


Routine blood biochemistry parameters were within normal limits. The fiber optic endoscopy showed a large, proliferative growth at left base of tongue, involving vallecula and left tonsil. On indirect laryngoscopy, both vocal cords were mobile and free of any growth. The fine needle aspiration cytology of the left sided neck node revealed small round cells with scant cytoplasm. As primary small cell carcinoma of the tongue is a rare entity, biopsy of the tongue was recommended for definitive diagnosis. The biopsy from growth base of tongue was consistent with small cell carcinoma (Figure 1a and 1b). The immunehistochemical staining was positive for CK, synaptophysin and chromogranin A, and was negative for LCA and p 63 (Figure 2a, 2b and 2c).