|Year : 2022 | Volume
| Issue : 1 | Page : 36-38
Small cell carcinoma base of tongue
Isha Shah, U Suryanarayana, Aastha Shah, Dinesh Anand, Monik Patel
Department of Radiotherapy, GCRI, Ahmedabad, Gujarat, India
|Date of Submission||17-Sep-2021|
|Date of Acceptance||23-Sep-2021|
|Date of Web Publication||21-Oct-2021|
Dr. Isha Shah
Department of Radiotherapy, GCRI, Ahmedabad, Gujarat
Source of Support: None, Conflict of Interest: None
Neuroendocrine small cell carcinomas (SCCs) are most commonly seen in the lung, and they rarely may arise in the extra pulmonary sites. Small cell neuroendocrine carcinoma of the tongue is an extremely rare entity with only seven cases reported in literature till date. Herein, we present a case of a 59-year-old male with a limited-stage small cell neuroendocrine carcinoma of the base of the tongue. We discuss clinical, pathological, radiological findings, and management of SCC of the base of tongue. Histopathological examination using immunohistochemistry, clinical features, and radiologic findings are all important for the correct diagnosis of extrapulmonary SCCs. As no standard therapeutic regimen exists, it is important to be aware of and to know how to deal with such rare cases.
Keywords: Base of tongue small cell carcinoma, case report, extrapulmonary small cell carcinoma, head-and-neck small cell carcinoma, small cell carcinoma
|How to cite this article:|
Shah I, Suryanarayana U, Shah A, Anand D, Patel M. Small cell carcinoma base of tongue. J Radiat Cancer Res 2022;13:36-8
| Introduction|| |
Extrapulmonary small cell carcinoma (EPSCC) was first described by Duguid and Kennedy in 1930, as a different entity from small cell carcinoma lung (SCLC). The small cell carcinoma (SCC) usually arises from the lung and approximately 2.5%–4% of it arises in extrapulmonary sites. EPSCC is still often confused with metastatic SCLC. Unlike SCLC, the natural history of EPSCC remains unknown so optimal therapy determination is complicated. Commonly reported sites of EPSCC are aerodigestive tract including paranasal sinuses, nasal cavity, salivary glands, thyroid gland, larynx, and trachea. It has been found rarely in ovaries, prostate, urinary bladder, cervix, and breast. In the head and neck, oral cavity and oropharynx are rare sites for EPSCC. The EPSCC shares quite similar histopathological features as of SCLC. The clinical course of EPSCC is highly aggressive, being more recurrent and most of the times, it demonstrates a poorer prognosis. The treatment of EPSCC patients has been very similar to the treatment of SCLC. Due to its chemosensitive nature, most of the EPSCC patients have been treated with cisplatin-based chemotherapy. As it is a systemic disease, multimodality therapy is preferred at an early stage. The present case is a primary extrapulmonary SCC of a base of tongue with nodal metastasis in ipsilateral upper jugulodigastric node and good response to treatment suggesting a different clinicopathophysiological behavior than typical pulmonary SCC.
| Case Presentation|| |
A 59-year-old smoker presented with difficulty in swallowing both solids and liquids for 3 months. It is associated with a lump in the left side of his neck, which was initially treated by antibiotics without any response by his physician. Fine needle aspiration of the left neck mass was performed at an outside hospital which was in favor of metastatic carcinoma. He was referred to our institute for further management. He had a smoking history of 10 pack years with abstinence from the past 6 months. No other comorbidity was associated.
| Clinical Findings|| |
On physical examination, there was induration present on the left side of base of tongue. It was associated with a solitary, 2 cm × 1 cm in size, firm left Level II neck mass which was not fixed to underlying tissue or overlying skin. Indirect laryngoscopic examination was suggestive of ulcerative lesion on the left side of the base of tongue and extending minimally into the adjacent tonsillar fossa and tonsillo lingular sulcus. Lingual surface of epiglottis and bilateral vocal cords appear within normal limits. There is no abnormality detected on the rest of the head-and-neck examination.
| Diagnostic Assessment|| |
A computed tomography (CT) scan of the paranasal sinus, neck, and thorax showed mildly enhancing soft-tissue thickening in the left side of base of tongue with maximum thickness of about 7 mm. Lesion involved left tonsillar fossa and tonsillo lingular sulcus and adjacent oropharyngeal wall on the left side. It was also suggestive of 26 mm × 12 mm necrotic lesion on left level II lymph node possibility of lymphnodal metastasis as shown in [Figure 1].
|Figure 1: Computed tomography scan of paranasal sinuses and neck showing lesion in left side base of tongue and enlarged left Level II lymph node|
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Examination direct laryngoscopic examination under local anesthesia was done which was suggestive of lesion in left side base of tongue, tonsillar fossa, tonsillo lingual sulcus and left lateral oropharyngeal wall. Punch biopsy was taken from the left side of the base of tongue lesion. Histopathological review of the lesion showed squamous mucosa infiltrated by a tumor composed of sheets and nests of small to medium-sized cells with hyperchromatic nuclei, granular chromatin, nuclear molding, high nuclear/chromatin ratio, scanty cytoplasm, increased mitosis, and apoptosis suggestive of poorly differentiated carcinoma consistent with small cell neuroendocrine carcinoma or small cell variant of squamous cell carcinoma. Immunohistochemistry of the tumor was positive for chromogranin, TTF1, and AE1 as shown in [Figure 2]. It was weakly positive for P63 and negative for synaptophysin and P40. The cytology of fine-needle aspiration of left neck mass was also consistent with metastatic SCC. Thus, the patient was diagnosed with poorly differentiated small cell neuroendocrine carcinoma of the oropharynx with metastasis to neck, Stage III, T1N1.
|Figure 2: Histopathologic slides showing small cell carcinoma base of tongue|
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| Therapeutic Intervention|| |
The Pretreatment dental check-up, nutrition counseling, chest physiotherapy and swallowing and speech exercises were taught. He was planned for concurrent chemoradiation with cisplatin at 50 mg/m2 weekly with daily radiotherapy with five fractions per week. He received a total dose of 66 Gy in 30 fraction intensity modulated radiotherapy with simultaneous integrated boost technique with 6MV photons. The gross tumor volume (GTV) for primary disease included the entire oropharynx, base of tongue and bilateral tonsillar fossa and tonsillo lingual sulcus. Left level II lymph node was taken as GTV for the nodal. Clinical target volume drawn separately for primary and left Level II gross node with adequate margin. Furthermore, the other nodal volumes from Level I to V lymph nodes are drawn separately on both sides. Moreover, ultimately planning target volume was given as per institute protocol. With simultaneous integrated boost technique, the primary tumor and nodal regions were devised in three risk group areas such as high risk, intermediate risk, and low risk. High-risk areas included GTV primary and bilateral Level I, II and left Level III lymph nodal groups which received 66 Gy in 30 fractions. Intermediate risk areas such as right Level III and bilateral Level IV and left Level V received 60 Gy in 30 fractions. Low-risk area involved contralateral Level V radiated with 54 Gy in 30 fraction.
| Follow-up and Outcomes|| |
On follow-up, every monthly indirect laryngoscopic examination done for 3 months and CT scan of the paranasal sinuses, neck, and thorax done at 3 months which showed complete response in primary as well as neck nodal disease. Then, the patient has been followed for 3 years without any evidence of recurrence.
| Discussion|| |
SCC is seen in the lungs and gastrointestinal tract. 20% of lung carcinomas are SCCs. They uncommonly arise in extrapulmonary sites. SCC of the base of tongue is characterized by a solid tumoral mass composed of sheets, cords, or irregular nests of anaplastic cells. Tumor cells are usually 2–3 times larger than mature small lymphocytes and have round to oval nuclei with scant cytoplasm, frequent necrosis, and abundant mitoses. Immunohistochemically, the tumor shows positive for chromogranin, TTF1, and AE1. The prognosis of SCC of the base of tongue is better than that of SCC carcinoma of lung or larynx. Local recurrence and distant metastases develop in many cases over a period of time. SCCs of the base of the tongue are treated the same as other oropharyngeal cancers and concurrent chemoradiotherapy remains the mainstay of treatment. No particular treatment approach appeared to improve the prognosis although SCC of the base of tongue has good prognosis compared to SCC of lung and gastrointestinal tract. Prophylactic cranial irradiation is not recommended routinely in extrapulmonary SCCs. The incidence of brain metastases is low, and there are differences in disease biology and metastatic spread between extrapulmonary SCCs and small cell lung cancer.
To summarize this, the management of localized SCC of the head and neck includes a combination of surgery, radiation, and chemotherapy although the recurrence rate is high and distant metastases occur often, especially when the disease involves the cervical lymph nodes. In nonmetastatic disease, chemotherapy can be used in the neoadjuvant, concurrent, or adjuvant setting to reduce tumor burden and decrease the risk of distant metastases. Combined chemotherapy and radiotherapy is the most common treatment in both early and locally advanced disease. Surgical resection has a very limited role in the SCCs of head and neck. In the metastatic setting, chemotherapy has been shown to prolong survival. Chemotherapy regimens used in SCC of the head and neck are similar to the regimens used in the management of SCC of the lung.
| Conclusion|| |
It is important to remember that although neuroendocrine SCCs are commonly seen in the lung, they rarely may arise in the extrapulmonary sites as well. Histopathological examination using immunohistochemistry, clinical features, and radiologic findings are all important for the correct diagnosis of EPSCCs. As no standard therapeutic regimen exists, it is important to be aware of and to know how to deal with such rare cases.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]