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Clinical Problem Solving: Radiology
November 2002

Diagnosis Radiology Quiz Case 1

Arch Otolaryngol Head Neck Surg. 2002;128(11):1332-1333. doi:
Diagnosis: Vascular leiomyoma of the larynx

Angiomyoma and angioleiomyoma are synonyms for vascular leiomyoma, but the latter is a more accepted term, because it is more accurately descriptive and widely used.1 Vascular leiomyoma is an uncommon type of smooth muscle tumor and is rarely seen in the head and neck. In a review of 562 cases by Hachisuga et al2 over a 17-year period, only 48 were seen in the head and neck. Most commonly, vascular leiomyomas arise in the female genital tract, gastrointestinal system, and pilar arrector muscles of the skin. Their exact pathogenesis is not known, but most authors agree that they probably originate in smooth muscle cells within blood vessel walls.3,4 In the head and neck, vascular leiomyomas are found in the lips, auricle, nostrils, skin of the occipital region, and cheeks. They very rarely occur in the larynx.5

Most vascular leiomyomas present in middle-aged and elderly men; laryngeal subsites seem evenly represented. When the lesions occur in the larynx, they result in hoarseness or dyspnea. Pain and dysphagia are uncommon symptoms. The tumors are slow growing, and the duration of symptoms is reported to range from 2 to 16 years.6 The most common symptom before admission is a rapidly progressive dyspnea, for which tracheostomy is often a necessary life-saving measure. Sometimes, however, a globus sensation, cough, or laryngeal pain is the only presenting symptom.5

Histopathologic classification of the vascular leiomyoma is complicated by its similarity to another benign laryngeal lesion, the simple leiomyoma. The World Health Organization recognizes 3 types of benign smooth muscle tumors: leiomyoma (simple leiomyoma), angiomyoma (vascular leiomyoma), and epithelioid leiomyoma (bizarre leiomyoma or leiomyoblastoma). The majority (7) of the 9 reported cases of laryngeal leiomyoma reported in the recent literature were vascular leiomyoma.7

Vascular leiomyomas are spindle cell neoplasms with numerous blood vessels but lacking mitoses. They have been reported to be encapsulated and made up of smooth muscle bundles among numerous vascular spaces.6 The spindled fascicles stain strongly with actin, supporting the diagnosis of vascular leiomyoma. Degenerative changes such as fibrosis, calcifications, and giant cell reactions can also be present. The list of histopathologic differential diagnoses includes hemangioma, leiomyoblastoma, angiomyolipoma, and vascular leiomyosarcoma.

The pathogenesis of benign smooth muscle tumors is unknown. Duhig and Ayer8 proposed that these lesions develop sequentially: smooth muscle proliferation within a vascular hamartoma leads to the formation of an angiomyoma, and continued smooth muscle proliferation results in a simple leiomyoma. Subsequent studies have refuted this theory of histogenesis, leaving the relationship between simple and vascular leiomyomas unclear.9 An etiologic role for estrogen has been considered, but estrogens appear to have little influence on leiomyomas outside the uterus, in that such tumors have shown no female predominance.7

Morimoto10 classified vascular leiomyomas into 3 histologic subtypes in 1973: solid (capillary), cavernous, and venous. Venous is the most common type found in the head and neck area. The solid type has smooth muscle bundles that surround the vascular slitlike channels. The cavernous type has dilated vascular channels with lesser amounts of smooth muscle. The venous type has vascular channels with thick muscular walls that are easily distinguished from smooth muscle bundles. In some lesions, features of all 3 types may be found.10 Malignant variants of vascular leiomyomas are rare. The absence of mitosis is the most useful indicator of a benign lesion.11

Surgical extirpation is the treatment of choice for vascular leiomyomas. There are 3 reports of recurrent vascular leiomyoma of the head and neck, to our knowledge.1 One was of a recurrent vascular leiomyoma of the larynx in which some mitotic figures had been noted histologically.3 This suggests that complete resection of such lesions is important and that the patients involved should be monitored for recurrence on a regular basis after surgery. Some small tumors have been resected through microlaryngoscopy; larger tumors may require external approaches. If laryngeal vascular leiomyomas are treated endoscopically, Nuutinen and Syrjanen6 recommend preliminary tracheostomy, general anesthesia, and, in the case of profuse bleeding, an external laryngeal approach.

Because of the tremendous vasculartity of these tumors, there is a high risk of significant bleeding. Shibata and Komune5 excised 2 small tumors through microdirect laryngoscopy. The patients involved lost a total of 600 to 1000 mL of blood during the primary procedure and from postoperative hemorrhage. Nuutinen and Syrjanen6 used cryotherapy after partial resection to control profuse bleeding. Anderson and Weinstein3 reported that the use of the carbon dioxide laser in the initial resection provided adequate hemostasis.

As surgical options are clearly not without risk, patients should be selected carefully. If there is evidence of malignancy, or compromise of the patient's airway or vocal quality, complete surgical excision is the recommended therapy. Because our patient was satisfied with the quality of his voice, and there were no findings to suggest a malignancy, we elected not to perform surgery. Instead, the patient will be followed up regularly over the upcoming years.

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