Angiomyomatous hamartoma in a submandibular lymph node: a case report.

Abstract

Angiomyomatous hamartomas have been found almost exclusively in the inguinal and femoral lymph nodes; few reports of these lesions in the head and neck region have been published. We present a case of angiomyomatous hamartoma in the submandibular area, a site that has not been previously reported in the literature. The mass was initially diagnosed as an enlarged lymph node. When it did not regress following broad-spectrum antibiotic treatment, the patient, a M-year-old woman, underwent an excisional biopsy. Histopathology identified the mass as an angiomyomatous hamartoma. At follow-up 3 years and 9 months postoperatively, the patient exhibited no evidence of recurrence on physical examination and computed tomography. Even though angiomyomatous hamartoma of the head and neck is rare, we suggest that otolaryngologists include it in the differential diagnosis of head and neck masses.

Introduction

Lymph node architecture can be partially replaced in conditions such as lymph node infarction, vascular transformation of the sinuses, bacillary angiomatosis, and metastatic angiosarcoma. (1-3) In 1992, Chan et al were the first to report an angiomyomatous hamartoma as an independent condition characterized by replacement of the nodal parenchyma with smooth-muscle cells and fibrous tissue arranged in a sclerotic stroma. (4) Angiomyomatous hamartomas have been described almost exclusively in the inguinal and femoral lymph nodes. (4) Only a few cases in the head and neck region have been documented in the literature. (5,6) Because these lesions are benign, the treatment of choice is surgical excision.

We present a case of angiomyomatous hamartoma in the submandibular area, a site that to the best of our knowledge has not been previously reported.

Case report

A 51-year-old woman was admitted to our department for evaluation of a left submandibular mass that had been present for 3 years. She reported that the mass had grown significantly over the previous year. The mass was painless and had not disturbed the patient until shortly before presentation, when it had become large and visible.

On physical examination, a 3-cm elastic submandibular mass was palpated. No skin involvement or fluctuation was noted. No lymph nodes were palpated, and no evidence of facial nerve palsy was noted. Ultrasonography demonstrated a hypoechoic, nonhomogeneous mass that involved most of the submandibular salivary gland and two adjacent small lymph nodes. Fine-needle aspiration biopsy was performed twice, but neither cellular nor fluid components were noted. Computed tomography (CT) of the neck demonstrated bilateral cervical lymphadenopathy. A 23-mm mass was noted in the left submandibular region, pushing the submandibular salivary gland and platysma muscle (figure 1). The mass was diagnosed as an enlarged lymph node.

The patient was prescribed a broad-spectrum antibiotic, but the mass did not regress. She then underwent an excisional biopsy. The mass was located deep to the platysma and adjacent to the submandibular salivary gland. Grossly, the 25-mm mass was irregular, elastic, and purple. Macroscopically, its cut surface was mottled gray and red. Microscopically, the specimen showed prominent proliferation of irregularly distributed thick-walled hilar blood vessels with smooth-muscle cells that were sparsely disposed in a dense collagenous stroma that extended focally into the medulla (figure 2). The smooth-muscle component of the blood vessel walls and the stroma exhibited immunoreactivity for muscle-specific actin, but not for HMB-45. The remaining lymph node parenchyma showed reactive changes. The morphologic and immunophenotypical features were compatible with an angiomyomatous hamartoma. (7,8)

[FIGURE 1 OMITTED]

At follow-up 3 years and 9 months postoperatively, the patient exhibited no evidence of recurrence on physical examination and CT.

Discussion

Albrecht coined the term hamartoma in 1904, when he became the first to distinguish true neoplasms from tumor-like lesions. (9) Hamartomas may occur in any organ because they represent a focal overgrowth of mature normal cells and tissues at sites of identical cellular composition. They may arise from any germ layers. They do not metastasize.

[FIGURE 2 OMITTED]

In their original description of angiomyomatous hamartomas, Chan et al noted that they are characterized by a proliferation of thick-walled hilar blood vessels that extend into the nodal parenchyma. (4) Although these lesions have been described as occurring throughout the body, they are rare in the head and neck region. While Fine et al (10) reported 60 cases of laryngeal hamartoma, there are only isolated reports of these lesions in other head and neck sites, such as the ethmoid sinus, (5) hard palate, (11) nasopharynx, (12) neck, (5,13) and parotid gland. (5,14) As far as we know, a case of angiomyomatous hamartoma in the submandibular area has not been previously reported in the literature.

The diagnosis of angiomyomatous hamartoma is based on histologic examination. Once the diagnosis has been established, complete excision of the lesion is the treatment of choice. Although this entity is rare, we believe that otolaryngologists should include it in the differential diagnosis of head and neck masses.

References

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(10.) Fine ED, Dahmas B, Arnold JE. Laryngeal hamartoma: A rare congenital abnormality. Ann Otol Rhinol Laryngol 1995;104(2):87-9.

(11.) Harada H, Morimatsu M, Kusukawa J, Kameyama T. A hamartoma-like mass on the palate? A possible discussion regarding the components of a pigmented naevus and hyperplastic salivary gland. J Laryngol Otol 1997;111(3):296-9.

(12.) Majumder NK, Venkataramaniah NK, Gupta KR, Gopalakrishnan S. Hamartoma of nasopharynx. J Laryngol Otol 1977;91(8):723-7.

(13.) Laeng RH, Hotz MA, Borisch B. Angiomyomatous hamartoma of a cervical lymph node combined with haemangiomatoids and vascular transformation of sinuses. Histopathology 1996;29(1):80-4.

(14.) Tsuda H, Morinaga S, Mukai K, et al. Hamartoma of the parotid gland: A case report with immunohistochemical and electron microscopic study. Virchows Arch A Pathol Anat Histopathol 1987;411(5):473-8.

Geva Barzilai, MD; Yaakov Schindler, MD; Raanan Cohen-Kerem, MD

From the Department of Otolaryngology-Head and Neck Surgery (Dr. Barzilai and Dr. Cohen-Kerem) and the Department of Pathology (Dr. Schindler), Carmel Medical Center, Haifa, Israel.

Corresponding author: Dr. Geva Barzilai, Department of Otolaryngology-Head and Neck Surgery, Carmel Medical Center, 7 Michal St., Haifa, 34362 Israel. Phone/fax: [phone]; e-mail: [e-mail]