Larynx lymphoepithelial carcinoma: surgical management

Abstract

Lymphoepithelial carcinoma (LEC) is an entity mostly frequent in the nasopharynx, which represents 40% of all neoplasms. The incidence’s not exclusive of a geographic area; however, it has an endemic distribution in Southeast Asia and Eskimos. LEC is not exclusive of the nasopharynx, has also been reported in other anatomical areas, such as the sinonasal tract, nasolacrimal duct, oral cavity, oropharynx, salivary glands, thymus, hypopharynx, oesophagus, stomach, trachea, lung and others. Non-nasopharyngeal and nasopharyngeal LEC have the same microscopic features, but the nasopharyngeal is more likely associated with Epstein-Barr virus. LEC has been approved by the WHO. LEC located in the larynx is quite rare and worthy of attention for its implication in the treatment and prognosis. We present a case of LEC treated in our ENT department in a middle-aged man.

Background

Lymphoepithelial carcinoma (LEC) of the larynx affects frequent median age men.^1–3 Smoking is considered a risk factor, our patient is a severe ex-smoker. The most frequent clinical presentation is dysphonia, dysphagia, hoarseness and odynophagia. This particular subtype is an extremely rare and aggressive form of tumour, which accounts for 0.2% of all laryngeal cancers.

Outcome and follow-up

The patient presented a favourable outcome and is discharged from our unit after 20 days. A first control follow-up visit is carried out at the end of the radiotherapy cycle and a CT head and neck and Positron emission tomography (PET) studies are performed at 6 months with no signs of recurrence. At the last follow-up visit in our ENT department, at 12 months from the discharge, the patient is free from disease.

Case presentation

We present a case of a 59-year-old man, a severe ex-smoker (44 smoking packper year), who consulted our otorhinolaryngology department for important dyspnoea, with 2 months history of progressive dysphonia and odynophagia, no dysphagia. Familiar history was positive for breast cancer and gastric cancer. At nasopharyngeal and laryngeal endoscopy, laryngeal aditus and vestibule are free of pathology, a nonulcerated exofitic neoformation that extended from false right vocal fold to ipsilateral glottis preventing vision of ipsilateral vocal fold. Arytenoid motility was preserved with reduced glottic fold (figure 1). Neck examination was negative. Contrasted CT scan of the neck is performed showing on the right chordal fold a pseudonodular tissue that originates from the middle third of the false vocal cord and protrudes into the laryngeal lumen. This tissue, which shows contrasting impregnation, extends ventrally to the anterior commissure, infiltrating the ventral third of the contralateral false vocal chord, caudally it infiltrates the ventral half of the true ipsilateral vocal cord and commissure. No signs of thyroid infiltration. Another pseudonodular component in the right paramedian site with partial obliteration of the pre-epiglottic extended to the epiglottic ligament. A 3 cm laryngocele is present on the Morgagni’s ventricle. Several lymphadenopathies <1 cm are present (figure 2). The day after the admission, direct preoperative microlaryngoscopy biopsy under general anaesthesia is performed. The histopathological study of the surgical specimen revealed a poorly differentiated carcinoma, exhibiting an important lymphocitary infiltrated: LEC (figure 3). Total laryngectomy extended to hyoid bone with bilateral functional neck dissection is carried out, stapler 100 mm suture of the pharynx after larynx resection is performed (figure 4). Histological examination of the operative specimen confirmed a poorly differentiated squamous cell carcinoma with intense lymphocyte reaction, also we performed on the final specimen EA early antigen, EBNA, VCA IgM, and IgG for Epstein Barr-virus (EBV) tests, which resulted negative. Twenty-four lymphnodes are extracted and analysed from right cervical dissection, with no neoplastic infiltration. Fourteen lymphnodes are extracted and analysed from the left cervical dissection, with no neoplastic infiltration. The patient presented good clinical conditions and is discharged after 20 days from admission. In the second month after the discharge, we performed a PET–CT study that did not show lymphnodes metastases, radiotherapist consulting indicates an external bean radiotherapy cycle due to the high radiosensitivity, a 30-session cycle is performed. A first follow-up control visit was performed at the end of the radiotherapy, and at 12 months, no signs of recurrence at PET–CT study.

Figure 1.

Rhinofibrolaryngoscopy shows nonulcerated exofitic neoformation that extended from false right vocal fold to ipsilateral glottis preventing vision of ipsilateral vocal fold.

Figure 2.

CT with MDC head and neck: shows contrasting impregnation, extends ventrally to the anterior commissure, infiltrating the ventral third of the contralateral false vocal cord, caudally it infiltrates the ventral half of the true ipsilateral vocal cord and commissure. No signs of thyroid infiltration. Another pseudonodular component in the right paramedian site with partial obliteration of the pre-epiglottic extended to the epiglottic ligament. A 3 cm laryngocele is present on the Morgagni’s ventricle. Several aspecifics lymphadenopathies<1 cm are presented.

Figure 3.

(A) Important lymphocitary infiltrated that is suggested lymphoepithelial carcinoma. (B) Important CD4 infiltrated.

Figure 4.

Larynx resection.

Discussion

LEC was first described in 1921 by Schminke and Regaud independently. Ewing, after, in 1929 noted that LE should be considered as a separate category of nasopharyngeal carcinoma.⁴ LEC of the larynx usually affects median age men, smoking is considered a risk factor,1–3 5 6 our patient is a severe ex-smoker. The most frequent clinical presentation is dysphonia, dysphagia, hoarseness and odynophagia.^{2 3 7} This particular subtype is extremely rare in the larynx, which accounts for 0.2% of all laryngeal cancers having an aggressive behaviour.2 5 6 8 Lymphnode invasion at the first visit is documented in 75% of cases and metastases are described in almost one-third of the cases.^{2 3} In our case, the tumour was not accompanied by specific cervical lymphadenopathies. Hammas et al⁵ postulated that this lymphoid-lined structure is probably the site of origin of laryngeal LEC. On the other hand, Toker and Peterson⁹ have suggested that the origin’s site of these lesions may be the active basal epithelium of the larynx, which is similar to the epithelium of tonsillar crypts. As we say in the background, LEC is an entity mostly frequent in the nasopharynx, which represents 40% of all neoplasms.2 5 7 10 11 Both have an important predisposition for early regional and distant metastases. Diagnosis in the initial stage is the primary factor of a good prognosis.¹²

Microscopically and morphologically, laryngeal LEC is similar to the nasopharyngeal one, but nasopharyngeal is most associated with the infection by EBV, the association into laryngeal type remains controversial.2 5 10 11 MacMillan et al¹ showed how in his cases none was positive for EBV, Marioni et al² confirmed the low prevalence of EBV infection, only 25% of his cases are positive. In our case, the patient is negative to the EBV confirming the low incidence and the not important correlation to the development of laryngeal LEC. Histologically, it is an undifferentiated carcinoma with an intermixed reactive lymphoplasmacytic infiltrate as we noted on the histopathological examination. The literature indicates that laryngeal LEC is a very radiosensitive disease and suggests that radiotherapy should be considered like election treatment in early tumours providing an excellent local control rate,^{9 11–16} in our case, the patient prefers the surgery as first therapy and adjuvant radiotherapy. However, in the advanced cases of the disease, surgical treatment is the first-line treatment with adjuvant radiotherapy.^{4 5 17} Neoadjuvant chemotherapy may be recommended in patients who present early regional adenopathy to decrease the possibility to develop metastatic disease.^{12 13}

Learning points.

• Lymphoepithelial carcinoma (LEC) of the larynx affects more frequent median age men. Smoking is considered a risk factor. The most frequent clinical presentation is dysphonia, dysphagia, hoarseness and odynophagia. This particular subtype is an extremely rare and aggressive form of tumour, which accounts for 0.2% of all laryngeal cancers.

• Microscopically and morphologically, laryngeal LEC is similar to the nasopharyngeal one, but nasopharyngeal is most associated with the infection by Epstein-Barr virus, the association to laryngeal one remains controversial.

• Histologically, it is an undifferentiated carcinoma with an intermixed reactive lymphoplasmacytic infiltrate as we noted on the histopathological examination.

• The literature indicates that laryngeal LEC is a very radiosensitive disease and suggests that radiotherapy should be considered like election treatment in early tumours providing an excellent local control rate. In the advanced cases of the disease, surgical treatment is the first-line treatment with adjuvant radiotherapy; neoadjuvant chemotherapy may be recommended in that patient who presents early regional adenopathy to decrease the possibility to develop metastatic disease.

Ethics statements

Patient consent for publication

Obtained.