|Year : 2020 | Volume
| Issue : 3 | Page : 134-136
Clear-cell carcinoma of the larynx: A report of an unusual case
Anirban Halder1, Rituparna Biswas2, Sujit Shukla1, Geetika Khanna3, Vikas Yadav1, Jaspreet Kaur1
1 Department of Radiation Oncology, VMMC and Safdarjung Hospital, New Delhi, India
2 Department of Radiation Oncology, Dr. B.R.A. IRCH, All India Institute of Medical Sciences, New Delhi, India
3 Department of Pathology, VMMC and Safdarjung Hospital, New Delhi, India
|Date of Submission||09-Jun-2019|
|Date of Decision||26-Sep-2019|
|Date of Acceptance||26-Nov-2019|
|Date of Web Publication||31-Dec-2019|
Dr. Rituparna Biswas
Department of Radiation Oncology, Dr. B.R.A. IRCH, All India Institute of Medical Sciences, New Delhi
Source of Support: None, Conflict of Interest: None
Clear-cell carcinoma (CCC) of the larynx is an extremely rare neoplasm with high malignancy potential and poor outcome. We herein report the 11th case of CCC of the larynx in a 42-year-old female who was managed with definitive radiochemotherapy. Due to a paucity of this entity, treatment option is not clearly defined, however, considering its grave nature, surgery should be offered primarily along with adjuvant radiotherapy with or without chemotherapy.
Keywords: Clear-cell carcinoma, larynx, radiotherapy
|How to cite this article:|
Halder A, Biswas R, Shukla S, Khanna G, Yadav V, Kaur J. Clear-cell carcinoma of the larynx: A report of an unusual case. J Med Sci 2020;40:134-6
| Introduction|| |
Clear-cell carcinoma (CCC) of the larynx is an unusual diagnosis, with only ten previously reported cases in literature. Although it is much more common in the kidneys, ovaries, uterus, and lungs, this neoplasm typically presents in the head and neck as a salivary tumor (1% of salivary neoplasms). CCC typically consists of clusters of large cells with clear cytoplasm, with high mitotic activity. It has been described as a variant of either mucoepidermoid or adenocarcinoma, or of squamous cell origin. In the larynx, CCC has been shown to have a predilection for the supraglottis and typically presents as a large, exophytic mass with cartilage erosion. It is an aggressive neoplasm, with a high recurrence rate and poor long-term survival. In previous reports, four laryngeal CCC were classified as adenocarcinoma variant, and six were a variant of squamous cell carcinoma. We present the seventh case of CCC of the larynx of squamous origin.
| Case Report|| |
An otherwise healthy, 42 -year-old female, a known hypertensive on medications for 2 years with a history of tobacco smoking for 25 years presented with cough for 4 months. General and systemic examination was unremarkable. Contrast-enhanced computed tomography scan of the face and neck showed diffuse asymmetric thickening with homogeneous enhancement of the epiglottis extending to pre- and para-epiglottic space and involving the aryepiglottic folds. Multiple subcentimetric to enlarged cervical lymph nodes are observed with the largest node measuring 16 mm ×7.8 mm in level II on the right side [Figure 1]. Direct laryngoscopy revealed an ulceroproliferative growth over the laryngeal surface of the epiglottis without vocal cord involvement [Figure 2]. Biopsy from the growth showed tumor cells with clear cytoplasm lying in the solid nest and surrounded by hyalinizing fibrocollagenous stroma; focal areas in the tumor show squamous differentiation, suggesting clear-cell variant of squamous cell carcinoma of the larynx [Figure 3]. The chest radiograph was normal. Hence, she was staged as cT3 N2c M0 as per the American Joint Committee on Cancer staging manual, eighth edition. She was treated with definitive radiochemotherapy with external beam radiation dose of 66 Gy in conventional fractionation to the primary site and bilateral necks and weekly cisplatin 40 mg/m concurrently. She was planned by two-dimensional radiotherapy with source-to-surface distance technique on telecobalt. Although the option of total laryngectectomy with bilateral neck dissection was offered to the patient for which she denied. She tolerated treatment well and was evaluated after 6 weeks of treatment completion with direct laryngoscopy which showed complete remission. She is disease-free after 6 months follow-up till date.
|Figure 1: Contrast-enhanced computed tomography showing diffuse asymmetrical thickening with homogeneous enhancement in the epiglottis and aryepiglottic folds (a) sagittal view, (b) axial view|
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|Figure 2: Direct laryngoscopy showing an ulcero proliferative growth over the laryngeal surface of the epiglottis|
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|Figure 3: Histopathological examination showing tumor cells with clear cytoplasm lying in a solid nest and surrounded by hyalinizing fibrocollagenous stroma, and focal areas in the tumor show squamous differentiation, suggesting clear-cell variant of squamous cell carcinoma of the larynx|
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| Discussion|| |
CCC frequently arises in the kidney or lung and has also been reported in the ovary and uterus., CCC of the larynx is a rare entity, with only ten previously reported cases. We report the seventh case of squamous type. These tumors are characterized by large cells with abundant clear cytoplasm and frequent mitoses. Based on histological staining, these can be divided into squamous type or adenocarcinoma type, both of which are considered poorly differentiated. Squamous type will stain positive for cytokeratin, p63, periodic acid-Schiff (PAS), and carcinoembryonic antigen but negative for mucin and show squamous differentiation, glycogen deposition, and cytoplasmatic lipid absence. Adenocarcinoma will stain negative for PAS and show no squamous differentiation, whereas clear-cell variant of mucoepidermoid will stain PAS and mucin positive. CCC of the larynx has a predilection for the supraglottis (50%) and tends to present in elderly males (80%), with a median age of 65 years [Table 1]. Similarly, in our index case also, there was massive supraglottic involvement, but contradicting literature presented case was a middle-aged female. Like other laryngeal neoplasms, smoking and alcohol are the major risk factors, and dysphonia and dysphagia are the most common complaints. The tumor typically presents at an advanced stage with laryngeal cartilage erosion (80%) and positive neck lymphadenopathy (70%), as was also found in our case. Because of the small number of cases and uncertain histogenetic classification of this tumor, the biological behavior of the neoplasm is not well understood. However, CCC of the larynx is considered a highly aggressive neoplasm with a high locoregional recurrence rate (87.5%) and a mean survival time of 12 months (range 3–24 months). Distant metastases also arose in the two patients treated with postoperative radiotherapy, further indicating aggressiveness of this tumor. Surgical resection has been the treatment of choice for this neoplasm. Given the aggressive nature of CCC of the larynx, total laryngectomy with bilateral neck dissections, with consideration for postoperative chemotherapy and radiation therapy should be offered. Frequent follow-up is necessary to detect recurrences, as a similar morphology in the salivary glands has a high recurrence rate. However, our index case received definitive chemoradiation in keeping with her wish to preserve the larynx.
|Table 1: Characteristics of clear-cell carcinoma of the larynx, as described in the literature|
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| Conclusion|| |
CCC of the larynx is a rare but aggressive neoplasm. We report the seventh case of CCC of the larynx of squamous origin. Although natural history of these rare tumors is not well understood because of the paucity of cases encountered, this malignant lesion seems to be associated with a poor prognosis, hence to be treated aggressively.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understand that her name 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], [Figure 3]