Primary non-Hodgkin lymphoma of the larynx.

Abstract: A rare case of primary large B-cell non-Hodgkin lymphoma of the larynx is reported. The patient was an 80-year-old female who presented with pharyngeal discomfort and dysphagia. Radiotherapy was instituted with complete remission of the tumor. Nine months afterward, she presented with aspiration pneumonia and dysphagia. Indirect laryngoscopy disclosed laryngeal and esophageal edema, which was probably related to radiotherapy. No signs of tumor recurrence were observed. Three months later, she was entirely asymptomatic. Although cases of aggressive course and poor response to therapy exist, primary large B-cell non-Hodgkin lymphoma of the larynx is generally considered a relatively benign and radiosensitive tumor.

Key Words: laryngeal neoplasms, primary large B-cell non-Hodgkin lymphoma of the larynx, radiation therapy

**********

Non-Hodgkin lymphoma (NHL), a predominantly lymph node tumor, presents in extranodal sites in about one third of cases. (1) The condition usually occurs in late adult life, with a mild male predominance. Primary involvement of the larynx is extremely rare in this lymph node malignancy, with only a few cases reported in the English medical literature so far. (1-3) We describe a case of primary NHL of the larynx, which presented a successful response to radiotherapy, one of the accepted modalities of treatment of the disease. (3)

Case Report

An 80-year-old female presented with a 3-month history of foreign body sensation in her throat, mild swallowing difficulty, and anorexia with weight loss (8 kg). She had not smoked at any time, and her medical record was remarkable only because of longstanding severe left hearing loss. Indirect laryngoscopy revealed the presence of a submucosal glottic and supraglottic mass, with partial occlusion of the laryngeal lumen. A unique lymph node (1 cm in diameter) was palpated in the right side of her neck, but otherwise the systemic physical examination was normal.

Blood analysis showed total cholesterol of 246 mg/dL, with hemoglobin, leukocyte count, liver function tests, and all other results normal. An electrocardiogram, chest radiography, and an abdominal ultrasound examination revealed no abnormalities. Magnetic resonance imaging (MRI) of the neck disclosed an enhancing diffuse laryngeal mass that extended from epiglottis to glottis and was partially obstructing the laryngeal lumen (Figure). Endoscopic findings of the nasopharynx and oropharynx were normal. A biopsy of the laryngeal tumor revealed large B-cell NHL. The patient did not give consent for a bone marrow biopsy or other studies, and she rejected chemotherapy as a possible treatment option. Local radiotherapy was applied in the laryngeal area, with resolution of all symptoms and disappearance of the cervical lymph node.

The next 9 months, the patient remained in good condition, but she began to have progressive swallowing difficulty and cough episodes provoked by ingestion of food. A chest radiograph showed several infiltrates in both lung fields. Indirect laryngoscopy and MRI of the neck demonstrated edema in the larynx and upper esophagus, with no recurrence of the tumor. Antibiotic treatment was initiated. Within a few days, all symptoms subsided, and lung infiltrates disappeared. Laryngeal and esophageal edema spontaneously resolved. Three months afterward, she was entirely asymptomatic.

Discussion

Squamous cell carcinoma is the most common malignant tumor of the larynx, with NHL accounting for less than 1% of laryngeal neoplasms. (3-5) NHL of the larynx is one example of lymphoma arising from mucosa-associated lymphoid tissue (MALT) and, as any NHL originating in extranodal sites, usually has a diffuse histology. (1,6)

[ILLUSTRATION OMITTED]

As with other NHL, NHL of the larynx generally occurs in elderly people, with predominance in males. Common symptoms at presentation include dysphagia, dysphonia, hoarseness, cervical lymphadenopathy, stridor, cough, and weight loss. (7) The lymphoma may be localized to one side of the larynx or may affect the entire organ, as occurred in our patient. On macroscopic examination, it appears as a smooth submucosal swelling or a polypoid mass. (8) Computed tomography scan and MRI were very helpful for evaluation of laryngeal neoplasms, (9) although histopathology is necessary to establish the diagnosis.

Cases of death shortly after diagnosis have been reported, usually caused by severe local complications such as laryngeal obstruction or massive hemorrhage. However, NHL of the larynx usually tends to remain localized for a long time and then slowly disseminates to other extranodal and nodal sites. (1)

Radiotherapy has been reported to be the initial treatment of choice in this process because the tumor is thought to be highly radiosensitive. A favorable response is common, not only in localized but also in disseminated disease. (3) Combined radiotherapy and chemotherapy has also achieved good results in localized NHL of several organs. (10) Radiotherapy alone was effective in our patient, with a disease-free survival of at least 1 year.

Laryngeal and esophageal edema, a relatively common early and late complication of radiotherapy, (11) was the presumed cause of aspiration pneumonia in our patient.

Conclusion

Although NHL of the larynx is rare, it should be considered in the differential diagnosis of a mass in the neck region. The tumor tends to remain localized for a long time and generally responds to radiotherapy.

Accepted April 7, 2004.

References

1. Cavalot AL, Preti G, Vione N, et al. Isolated primary non-Hodgkin's malignant lymphoma of the larynx. J Laryngol Otol 2001;115:324-326.

2. Ohta N, Suzuki H, Fukase S, et al. Primary non-Hodgkin's lymphoma of the larynx (stage IE) diagnosed by gene rearrangement. J Laryngol Otol 2001;115:596-599.

3. Ansell SM, Habermann TM, Hoyer JD, et al. Primary laryngeal lymphoma. Laryngoscope 1997;107:1502-1506.

4. Puig P, Martinez P, Piles A, et al. Subglottic MALT-type lymphoma, a rare location. Acta Otorrinolaringol Esp 2002;53:693-696.

5. Kato S, Sakura M, Takooda S, et al. Primary non-Hodgkin's lymphoma of the larynx. J Laryngol Otol 1997;111:571-574.

6. Cheng CJ, Chen PR, Liu MC, et al. Primary malignant lymphoma of mucosa-associated lymphoid tissue of larynx. Otolaryngol Head Neck Surg 1999;121:661-662.

7. Kundu SS, Kundu AK, Pal NK. Primary non-Hodgkin's lymphoma of the larynx presenting with protracted cough. J Assoc Physicians India 2000;48:748-749.

8. Pak MW, Woo JK, Van Hasselt CA. T-cell non-Hodgkin's lymphoma of the larynx and hypopharynx. Otolaryngol Head Neck Surg 1999;121:335-336.

9. Takayama F, Takashima S, Momose M, et al. MR imaging of primary malignant lymphoma in the larynx. Eur Radiol 2001;11:1079-1082.

10. Miller TP, Dahlberg S, Cassady JR, et al. Chemotherapy alone compared with chemotherapy plus radiotherapy for localized intermediate- and high-grade non-Hodgkin's lymphoma. N Engl J Med 1998;339:21-26.

11. Ichimura K, Sugasawa M, Nibu K, et al. The significance of arytenoid edema following radiotherapy of laryngeal carcinoma with respect to residual and recurrent tumour. Auris Nasus Larynx 1997;24:391-397.

RELATED ARTICLE: Key Points

* Primary large B-cell non-Hodgkin lymphoma of the larynx accounts for less than 1% of laryngeal neoplasms.

* The tumor is generally considered relatively benign.

* Radiation therapy is effective and considered treatment of choice in that neoplasm.

Bernardino Roca, MD, PHD, Barbara Vidal-Tegedor, MD, and Magin Moya, MD

From the Medicine and Otolaryngology Departments, Hospital General of Castellon, Spain.

Reprint requests to Dr. Bernardino Roca, Catalunya, 33-A, 4, 12004 Castellon, Spain. Email: [e-mail]