CC BY-NC-ND
0Journal of the Egyptian National Cancer Institute (2015) xxx, xxx-xxx
Cairo University Journal of the Egyptian National Cancer Institute
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Case Report
Small cell carcinoma of the lung in a treated case of Myoepithelial carcinoma of the tongue - Report of a rare case with illustrated review of the literature
Bhanuprasad Venkatesulu, Supriya Mallick *, Archana George, Suman Bhasker
Department of Radiation Oncology, All India Institute of Medical Sciences, New Delhi, India Received 16 March 2015; revised 5 May 2015; accepted 3 June 2015
KEYWORDS
Myoepithelial carcinoma; Tongue;
Small cell carcinoma lung
Abstract Myoepithelial carcinoma has rarely been reported in the oral cavity and oropharynx. We found only 6 cases of myoepithelioma of the tongue reported till date. Two cases had a benign myoepithelioma; four had epithelial-Myoepithelial carcinoma. The present case had malignant myoepithelioma, a distinct entity from other histologies.
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nd/4.0/).
Introduction
Myoepithelial cells are ectodermal origin cells found in secretary organs like salivary glands, breast, skin, and kidney [1,2]. Sheldon was the first to identify Myoepithelial tumours as a distinct salivary gland tumour entity [3]. Myoepitheliomas are unusual and uncommon tumours and constitute only 1-1.5% of all salivary gland tumours [4]. In the literature till now only one case of Myoepithelial carcinoma of the base of the tongue has been described. Rationale in reporting this 22 year old patient is to illustrate the diagnostic and therapeutic dilemma encountered.
* Corresponding author. Tel.: +91 9899448450; fax: +91 11 26589243.
E-mail address: drsupriyamallick@gmail.com (S. Mallick).
Peer review under responsibility of The National Cancer Institute,
Cairo University.
Case report
A 22 year old male patient presented to our multidisciplinary head and neck cancer clinic with complaints of non healing ulcer over the posterior part of left side of the tongue for 6 months, difficulty in swallowing for 4 months, dull aching pain, non radiating for 4 months. The patient did not give any history of smoking, alcoholism, sharp teeth or oral sexual habits. He had no history of malignancy in the family. He had a performance status of ECOG-One [European cooperative oncology group]. Oral examination and laryngoscopy revealed a 4 * 3 cm sized ulcer-proliferative growth on the posterior 1/3rd of the tongue on the left side and extending to the base of the tongue. Neck node examination was found normal. A biopsy from the edge of the ulcer with normal tissue was taken and was reported as poorly differentiated carcinoma and positive for cytokeratin. Hence, a final diagnosis was achieved as carcinoma tongue, CT2 N0 M0. The patient was planned for
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1110-0362 © 2015 Production and hosting by Elsevier B.V. on behalf of National Cancer Institute, Cairo University. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
wide local excision and ipsilateral selective neck dissection followed by adjuvant radiotherapy. The patient underwent transoral laser excision of the left posterior 1/3rd and base of tongue tumour with supra-omohyoid neck dissection and tracheostomy under general anaesthesia. Post operative recovery was uneventful.
The histopathology revealed a mass size of 4 * 3 cm. Microscopic examination revealed a poorly differentiated malignant tumour. Tumour cells were arranged in diffuse sheets. There was evidence of perineural invasion. Tumour cells were focally immune positive for cytokeratin (CK), epithelial membrane antigen (EMA), smooth muscle actin (SMA) and vimentin and negative for synaptophysin, Neu-IV chromogranin leucocyte common antigen (LCA), desmin, Myogenin, and MIC-2, HMB-45, CD54, GFAP and S-100 protein (Fig. 1). Features were in favour of Myoepithelial carcinoma. None of the nine dissected lymph nodes revealed evidence of metastasis. Subsequently, the patient received postoperative radiotherapy 50 Gray in 25 fractions over 5 weeks with 5 fractions per week by the 3DCRT technique. The radiotherapy was planned in Eclipse treatment planning system with a 6 MV photon beam. The patient tolerated the treatment well with no treatment interruptions and no major toxicity reported. The patient was kept on regular follow up with clinical examination every 3 months and CECT face and neck repeated every 6 months.
After three and half years of symptom free interval he presented with chest pain and breathlessness for 1 month. Chest X-ray was ordered and opacity in left upper and middle zone was found. Contract enhanced computed tomography (CECT chest and face and neck) was done which revealed postoperative changes with surgical drops at the base of the tongue and moderate pleural effusion on the left side with a partial collapse of the underlying lung showing patchy consolidation fibrosis without any evidence of mediastinal lymphadenopathy.
Patient was evaluated in an pulmonary medicine clinic and advised CT guided biopsy of the lung mass. Microscopy revealed small cell carcinoma of the lung with areas of necrosis. Tumour cells were immune-positive for synaptophysin, chromogranin and focally for cytokeratin, MIB1 labelling index is approximately 90%.
Subsequently he was started on chemotherapy with cis-platin 30 mg/m2 and etoposide 100mg/m2 intravenous (IV)
B. Venkatesulu et al.
day one through day three repeated every 3 weekly for six cycles. At the time of writing this case report the patient was being planned for thoracic radiation and prophylactic cranial irradiation.
Discussion
Squamous cell carcinomas are the most common histology of the base of the tongue with >90% of patients diagnosed with SCC. Human papilloma virus has been found to be an aetio-logical factor for tumours of the oropharynx. Myoepithelial carcinoma may arise from minor salivary glands of the base of the tongue. According to World Health Organization histo-logical classification of salivary gland tumours Myoepithelial carcinoma has been recognized as a distinct entity from 1991 and accorded a morphology code of 8982/3 of the International classification of disease for oncology (ICO-O) [5]. The very low historic incidence is probably due to their recent recognition as a separate tumour entity. Myoepithelial carcinoma is known to occur in partial, submandibular, sublingual, minor salivary gland, hard palate, breast, lung, soft tissue, kidney, paranasal sinus, trachea, nasal cavity and lacrimal gland [6-8].
Myoepithelial cells exhibit 4 main cell morphologies: spindle (most common), epithelioid, plasmacytoid and clear cells (least common). Myoepithelial cells are found usually to be positive for cytokeratin (AEI/AE3, CK 5/6, Cam 52, CK-7 and CK-14) and vimentin (positive in neoplastic Myoepithelial cell and negative in normal Myoepithelial cells. Neoplastic transformation of Myoepithelial cells results in loss or modification of their smooth muscle phenotype resulting in positivity of S100 calponin, smooth muscle actin (SMA), muscle specific actin (MSA), smooth muscle myosin, p53 protein and glial fibrillary acidic protein (GFAP). Myoepithelial cells are typically negative for carcino-embryonic antigen (CEA), signifying no tubular differentiation and E-cadherin expression also detected in myoepithelioma [9-11]. Our patient was focally immune positive for cytokeratin, epithelial membrane antigen, smooth muscle actin and vimentin. Malignant myoep-ithelioma are a distinct entity from epithelial Myoepithelial neoplasms and should not be confused to entertain a diagnosis of malignant myoepithelioma. Cytokeratin and other Myoepithelial markers including smooth muscle actin,
Figure 1 (A) Photomicrograph showing tumour arranged in diffuse sheets and infiltrating into the skeletal muscle (10x). (B) Tumour cells show scant rim of cytoplasm, vesicular nucleus, prominent eosinophilic nucleus and moderate degree of anisonucleosis (40x) (both H&E stain).
Small cell carcinoma of lung in a treated 3
Table 1 Pathological and immunochemistry details of reported cases.
S. No. Author Histopathology Immunohistochemistry Diagnostic imaging used
1 Casas et al. (2001) N21 Myoepithelioma S-100 + , GFAP + Muscle specific actin + No imaging stage NA
2 L12J Puri et al. (2004) Epithelial Myoepithelial Cytokeratin (AE1/3) negative smooth muscle Stage CT3 N0M0 (only clinical
[131 tumour actin + , CK + examination)
3 Kei Woo et al. Myoepithelioma S-100 + , vimentin + Leu7, p63 + Imaging used stage NA
(2005) [14]
4 Kumai et al. (2006) Epithelial Myoepithelial Cytokeratin + , Smooth muscle actin+, S- Stage* CT2 N0M0 (MRI** used)
[151 tumour 100 +
5 DeMatos et al. Epithelial Myoepithelial Cytokeratin + Smooth muscle actin + CT1 N0M0 (MRI used)
(2009) [16] tumour
6 Peters et al. (2010) Epithelial Myoepithelial P53 + CK 5/6 + CK34 + SMA + AEI/ CT2 N0M0 (MRI used)
[171 carcinoma AE3 +
7 Present case (2014) Malignant Cytokeratin + EMA + SMA + Vimentin + CT2 N0M0 no imaging
myoepithelioma
* Staging done according to the American Joint Committee on Cancer Research, AJCC 2008 (TNM staging). MRI - magnetic resonance imaging.
Table 2 Clinical features, treatment details and end status details of reported cases.
S.No. Author Site of lesion Age/sex Treatment modality Follow up Recurrence
1 Casas et al. Midline cystic 65/M Complete resection 10 months No recurrence at last follow up
(2001) [12] tongue lesion
2 Puri et al. Base of 48/M Neo-adjuvant Chemotherapy cisplatin, 14 months No recurrence at last follow up
(2004) [13] tongue 5 fluorouracil x 2 cycles fi 66 Gy RT
3 Kei Woo Midline 19/F EL excision 14 months No recurrence at last follow up
et al. (2005) dorsal lesion
4 Kumai et al. Base of 76/M Surgery (subtotal glossectomy, b/l 19 months No recurrence at last follow up
(2006) [15] tongue neck dissection, and reconstruction)
5 de Matos Ventral 48/F 1st surgery Excision biopsy 7 years Recurrence at 4 years, No further
et al. (2009) surface of recurrence at 7 years
[16] tongue
6 Peters et al. Base of 60/F End surgery WLE RT 60 Gy/30#/ NR No recurrence at last follow up
(2010) [17] tongue 6 weeks
7 Present case Base of 22/M Transoral laser 42 months No recurrence but second new
(2014) tongue surgery + supraondyoid neck primary in the lung (small cell
dissection fi RT 50 GY/25# carcinoma)
GFAP, CD10 calponin and smooth muscle myosin hearing heavy chain are required for diagnosis.
We found only 6 cases of myoepithelioma of the tongue reported till date. Two cases had a benign myoepithelioma; four had epithelial-Myoepithelial carcinoma. The present case had malignant myoepithelioma, a distinct entity from other histology. The pathological and immuno-histochemistry details of the reported cases have been summarized in Table 1 [12-17]. Table 2 summarizes the clinical, treatment details and end results of the reported cases [12-17].
In the management of tumour due to its enigmatic presentation and rarity different modalities of surgery and radio-chemotherapy have been applied. There was a concurrence for surgery in the form of wide local excision for 4 patients and radiotherapy was used in 3 patients. Since the present patient had the largest follow up of 42 months and in head and neck cancer maximum recurrence occured within 6 months of treatment completion, we may claim that initial
surgery followed by a radiation dose of 50 Gy could be sufficient for adequate local control.
Another unusual aspect of our case was after 3 and [half] -years of symptom free interval, patients developed a second new primary of small cell carcinoma of the lung [18,19]. There have been case reports of myoepithelioma of the lung. But in the present case immunohistochemistry was positive for synaptophysin, chromogranin and cytokeratin, and does not favour myoepithelioma. Whether Myoepithelial malignancy of the tongue and small cell carcinoma of the lung have really any cytogenetic or molecular association or was it just a coincidence is not known.
Conclusion
What is the rationale in reporting those rare cases? In a high volume tertiary referral hospital like ours, unknown cases
are seen more frequently with a catalogue of individual experiences and a database could be built in respect to behaviour and treatment options, so a standard surgical approach and radiation dose could be devised for this uncommon and rarely diagnosed neoplasm.
Disclosures
The authors have nothing to disclose. Conflict of interest
The authors have no conflict of interest. References
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