|Year : 2015 | Volume
| Issue : 3 | Page : 647
Primary laryngeal peripheral T-cell lymphoma-An autopsy report with review of literature
Preithy Uthamalingam1, Amanjit Bal1, Pankaj Malhotra2, E Rabindranath2
1 Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India
|Date of Web Publication||9-Oct-2015|
Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012
Source of Support: None, Conflict of Interest: None
Primary laryngeal non-Hodgkin's lymphomas are very rare constituting 1% of all laryngeal tumors. Of these, the peripheral T-cell lymphomas are extremely rare with <10 case reports in English literature. Laryngeal lymphomas are clinically confused with the more common laryngeal malignancies like squamous cell carcinoma, thus, histopathological examination is mandatory for diagnosing, sub-typing and guiding therapy for these lymphomas. We report a case of rare primary laryngeal peripheral T-cell lymphoma with autopsy findings.
Keywords: Larynx, non-Hodgkin′s lymphoma, peripheral T-cell lymphoma
|How to cite this article:|
Uthamalingam P, Bal A, Malhotra P, Rabindranath E. Primary laryngeal peripheral T-cell lymphoma-An autopsy report with review of literature. J Can Res Ther 2015;11:647
|How to cite this URL:|
Uthamalingam P, Bal A, Malhotra P, Rabindranath E. Primary laryngeal peripheral T-cell lymphoma-An autopsy report with review of literature. J Can Res Ther [serial online] 2015 [cited 2019 Nov 14];11:647. Available from: http://www.cancerjournal.net/text.asp?2015/11/3/647/140789
| > Introduction|| |
Primary non-Hodgkin lymphomas (NHL) of the hypopharynx, larynx or trachea are very rare accounting for 1% of all primary extranodal NHL. Especially rare are the peripheral T-cell lymphomas of the larynx.  If the relatively commoner T/natural killer (NK) cell lymphomas are excluded, only around five cases are documented in the English literature with the diagnosis of primary NHL of the peripheral T-cell type. , We present such a case with autopsy findings, to demonstrate the diagnostic difficulties, treatment dilemmas and gross and microscopic features of a rare primary laryngeal T-cell lymphoma.
| > Case report|| |
A 58-year-old Indian male patient presented with progressively increasing neck swelling for 40 days in the month of May 2012. It was noticed more on the left side, which progressed on to cause dysphagia for solid foods. He did not have any history of stridor or hoarseness of voice. No history of systemic symptoms was forthcoming. The patient was a known heavy smoker and alcoholic for 30 years. There were no other co-morbid illnesses.
Clinically, the ill-defined mass measured 16 cm × 12 cm extending from mandible to the clavicle having a vague nodular surface. Computed tomography showed infiltrative soft tissue mass on the left side of the neck extending superiorly from the level of nasopharynx and inferiorly reaching thoracic inlet [Figure 1]. Based on radiological findings, a possibility of high-grade lymphoma versus a soft tissue sarcoma was suggested. No enlarged lymph nodes were seen.
The patient underwent fine-needle aspiration cytology (FNAC), and diagnosis of high grade NHL was given based on the large atypical lymphoid cells. Subsequently, the patient underwent an incisional biopsy from the mass. The biopsy showed diffuse infiltration by atypical lymphoid cells, which were 2-3 times the size of a mature lymphocyte with some showing irregular nuclear contours, coarse chromatin and inconspicuous nucleoli with scant to moderate amount of cytoplasm. The background showed capillary proliferation, a few eosinophils and neutrophils. Immunohistochemically, these cells were strongly and diffusely positive for CD3 and negative for CD20, CD10, CD15, CD30 and CD56. Ki-67 index was 40%. T-cell receptor rearrangement studies were not performed. Based on the radiological findings, morphology and the immunohistochemical patterns, the diagnosis of primary laryngeal NHL of peripheral T-cell type, not otherwise specified (NOS) was given [Figure 2].
|Figure 1: Computed tomography showing infiltrative soft tissue mass on the left side of the neck extending superiorly from the level of nasopharynx and inferiorly reaching thoracic inlet|
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|Figure 2: Photomicrographs showing. (a and b) Diffuse infiltration by atypical lymphoid cells in the sub-epithelial region at places infiltrating the overlying epithelium. Tumor cells are two to three times the size of a mature lymphocyte with some showing irregular nuclear contours, coarse chromatin and inconspicuous nucleoli and scanty to a moderate cytoplasm (H and E, ×100 and ×200), (c and d) the tumor cells were strongly and diffusely positive for CD3, and negative for CD20 (CD3 and CD20 Immunostain, ×200)|
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Following diagnosis, he was given three cycles of cyclophosphamide, Hydroxydaunorubicin (doxorubicin), oncovin (vincristine), and prednisone (CHOP) chemotherapy during which there was about 30% reduction in the size of the tumor. After 2 months of treatment with CHOP regimen, he presented with stridor in the month of July for which he underwent a tracheostomy. A month after tracheostomy, he was brought dead to the hospital. The course of events is summarized in [Table 1].
A partial autopsy with removal of abdomino-thoracic organs was done. The pharynx appeared normal while on the superior aspect the airway was almost occluded by the grey white mass measuring 16 cm × 8 cm × 5 cm, in the supraglottis [Figure 3]a. On slicing, the mass was seen involving the whole of the supraglottis, glottis (both true and false cords) and extending into the subglottis [Figure 3]b. There was no ulceration of the mucosa. The tumor appeared to extend into the para-laryngeal regions and beyond the thyroid cartilage. The tumor was bulging into the hypopharynx, but not ulcerating it.
Microscopically, the morphology was similar to the incision biopsy. The infiltration was extending into the surrounding fat, skeletal muscles and entrapping large nerves. The lympho-reticular organs such as lymph nodes, liver, spleen and the bone marrow did not show involvement. The lung showed negative pressure pulmonary edema and hemorrhage, which suggested the possibility of tracheostomy tubal block as a cause for the sudden demise of the patient.
|Figure 3: Gross photographs (a) showing larynx occluded with a grey-white polypoidal mass, (b) on slicing, a grey-white fleshy mass was seen involving the whole of the supra glottis and the glottis and extending into the subglottis|
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| > Discussion|| |
In the head and neck region, the majority of extra nodal lymphomas are NHLs, with Waldeyer's ring being the most common site of involvement. Extra nodal NHLs limited to the larynx are rare. In a retrospective analysis done by Markou et al.,  the most common site of development of primary laryngeal lymphomas is the supraglottic region, followed by the glottic area, and the rest are either subglottic or transglottic. B-cell lymphomas are reported to be strikingly more common than T-cell lymphomas and of T-cell lymphomas; NK/T-cell lymphomas are more common. Until date as archived from PubMed, a total of 16 cases of T-cell NHL have been reported in the literature [Table 2].
|Table 2: Characteristics of the reported cases of laryngeal T-cell lymphomas till date (as archived from PubMed)|
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The symptoms of the laryngeal lymphomas are largely nonspecific and overlap considerably with the more common squamous cell carcinoma. Radiologically however, the primary laryngeal lymphomas are described as smooth submucosal masses, and ulceration is rarely seen. , The index patient also showed a large mass involving the pharynx and the larynx on the left side of the neck without mucosal ulceration and a possibility of the NHL could only be suggested. Though imaging techniques may be helpful, histopathological examination is essential for the final diagnosis. In FNAC, exact sub-typing of NHL can be erroneous, due to sampling error and heterogeneous involvement by the atypical cells.  Thus, incision biopsy is important from the most representative area as guided by radiology.
The few cases of peripheral T-cell lymphomas (PTCLs) reported in the literature were treated either with radiotherapy or combined radio and chemotherapy however the prognosis was uniformly bad. , The patient was planned for CHOP regimen chemotherapy like the PTCLs elsewhere as the data for treatment of primary laryngeal PTCL, NOS is sparse.  The laryngeal lymphomas are usually confined to the larynx, thus remaining in the lower stage for long. However, primary laryngeal peripheral T-cell type lymphomas appear to do worse than the nodal counterparts. This might be due to an unrelated cause of death, like in the present case the cause of death was primarily an acute airway obstruction. The patient initially did show improvement with CHOP therapy after which he had a worsening of symptoms. Whether or not the patient should have been given radiotherapy and/or a debulking surgery is a matter of debate. 
| > Conclusion|| |
Although primary laryngeal lymphoma is rare, it should be considered in the differential diagnosis of a mass in the neck region, especially in the supraglottic area with suggestive radiological features. The review of the literature reveals that the small number of patients with primary laryngeal PTCL is uniformly associated with bad prognosis. More of these types of cases should be documented in the literature for improving the insights regarding the ideal treatment for these patients.
| > References|| |
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[Figure 1], [Figure 2], [Figure 3]
[Table 1], [Table 2]