Neurofibroma

Abstract

Spindle-cell hemangloendothelioma is an uncommon vascular lesion that exhibits a predilection for the extremities. Very few reports have been published describing this lesion in the head and neck, and to the best of our knowledge, its occurrence in the oropharynx has not been previously reported. In addition to reporting an unusual site of this lesion, out rationale for publishing this case is to comment on the diagnostic dilemma that arose in view of an unclear clinicohistopathologic pattern and to discuss this lesion's similarity to other aggressive tumors.

Introduction

In 1988, Enzinger and Weiss described a "new" vascular tumor that resembled a cavernous hemangioma and Kaposi's sarcoma; they called it a spindle-cell hemangioendothelioma. (1) It was considered to be a low-grade angiosarcoma. The most common locations are the superficial soft tissues of the distal portions of the extremities. The tumor is slowly progressive but locally aggressive. It has a tendency to recur, but no metastasis has been documented. (2)

We report the case of a young man who presented with a mass in the posterior pharyngeal wall that was classified on histopathology as a spindle-cell hemangioendothelioma. After reviewing the literature, we determined to the best of out knowledge that no other case of this tumor in the oropharynx has been previously reported, although there have been a few isolated reports of spindle-cell angiosarcoma and Kaposi's sarcoma affecting the oropharynx. (3,4)

Case report

A 25-year-old man presented with a 6-month history of slowly progressive swallowing difficulty, a change in voice, and breathing difficulty at night. On oropharyngeal examination, a hemispherical, mucosa-covered, pinkish mass was noted to have arisen from the posterior pharyngeal wall. The mass measured approximately 6 x 6 cm. On indirect laryngoscopy, the cystic mass extended from the posterior pharyngeal wall up to the epiglottis, and it obscured the view of the endolarynx. No lymph nodes were palpable on examination of the neck.

Fine-needle aspiration yielded a straw-colored fluid. Cytologic examination of the fluid identified only neutrophils and lymphocytes and was nondiagnostic. Contrast-enhanced computed tomography (CT) demonstrated a mildly enhancing globular swelling arising from the posterior pharyngeal wall; the mass measured approximately 4 x 3.5 cm (figure 1). The anatomy of the laryngeal structures was normal. Scout film revealed the entire vertical extent of the lesion (figure 2).

[FIGURES 1-2 OMITTED]

Following elective tracheotomy, the patient was kept in the supine position with the atlantooccipital joint extended. A Boyle Davis mouth gag was placed, and a 4-cm submucosal vertical incision was made. The tumor was excised completely via blunt dissection. The incision was sutured after complete hemostasis was ensured. On gross morphology, the tumor was a well-encapsulated spherical mass; after the lesion was cut, irregular areas of empty spaces were evident (figure 3).

[FIGURE 3 OMITTED]

Histopathologic examination identified cavernous spaces with organizing thrombi interspersed with spindle cells. The spindle cells were arranged in fascicles, and they exhibited bland-appearing nuclei with irregular cytoplasmic borders (figure 4).

[FIGURE 4 OMITTED]

The patient was decannulated after 5 days. He recovered well and remained free of recurrence 4 years postoperatively.

Discussion

Spindle-cell hemangioendothelioma occurs almost equally in males and females. The age distribution is wide, although most reported cases have occurred between the second and fourth decades of life. (2) Our patient's age (25 yr) coincided very well with that typically reported for spindle-cell hemangioendothelioma.

These tumors can appear as solitary or multiple cutaneous and subcutaneous nodules. They are either vascular and hemorrhagic or firm and pale with foci of hemorrhage. The most common sites are the superficial soft tissues of the lower and upper limbs, followed by the trunk, vulva, penis, and ear. The first reported case of a spindle-cell hemangioendothelioma in the oral cavity occurred in a 12-year-old girl whose lesion was located in the mandibular-buccal fold. (2)

Etiology. The etiopathogenesis of spindle-cell hemangioendothelioma is unknown. Originally considered to be a vascular neoplasm with malignant potential, the facts that argue against it being malignant are the absence of reported metastases, its long clinical course, and its tendency toward multifocality. Moreover, microscopic examination reveals a low degree of proliferative cell activity and a lack of cellular atypia and mitosis. The idea that spindle-cell hemangioendotheliomas are nonneoplastic was advocated by Fletcher et al, who reported that these tumors arise in association with a local acquired or congenital focal abnormality of blood flow at the affected site. (4)

Other authors have postulated that injury is a possible etiology, pointing out that the lesions arise at sites of repeated injections or surgical excisions and that they frequently arise on the distal extremities. (4,5) No such history was elicited from out patient, although a possible etiology in this case might have been the repeated impact of food boluses on the posterior pharyngeal wall during the pharyngeal stage of swallowing.

Imayama et al described spindle-cell hemangioendothelioma as a reactive vascular proliferation caused by successive episodes of angioformation occurring during the recanalization process after thrombosis. (5) Occasional cases of intramuscular spindle-cell hemangioendothelioma have been reported; one of them occurred in the extensor muscle of the little finger and another in the gluteus maximus. (6)

Cellular characteristics. In addition to spindle-cell hemangioendothelioma, other vascular lesions that have a prominent spindle-cell component include Kaposi's sarcoma and angiosarcoma. (3) Both Kaposi's sarcoma and spindle-cell hemangioendothelioma contain spindle-cell stroma, but only the latter contains dilated blood vessels and epithelioid endothelial cells. (4) Histopathologic examination of a spindle-cell hemangioendothelioma reveals cavernous spaces, which may contain organizing thrombi at different stages of organization. Between the cavernous spaces is a proliferation of bland-appearing spindle cells with elongated nuclei; these cells are arranged either in interlacing short fascicles or randomly. Unlike the case with Kaposi's sarcoma, these areas also contain occasional rounded or epithelioid endothelial cells, similar to those seen in epithelioid hemangioendothelioma. (1) Spindle-cell hemangioendothelioma and epithelioid hemangioendothelioma share many features, but the latter exhibits a more solid growth pattern and an absence of cavernous spaces, thrombi, phleboliths, and discrete spindle-cell areas.

Diagnosis. Although our patient reported a 6-month history of symptoms, his lesion may have been present for a longer time, as evidenced by the size of the swelling and the predominance of cavernous spaces on histopathologic examination.

A mass on the posterior pharyngeal wall could be caused by a chronic tubercular retropharyngeal abscess, a lipoma, a neurofibroma, a synovial sarcoma, or some other type of tumor. A synovial sarcoma might be suspected when a circumscribed mass posterior to the pharynx exhibits hemorrhage and fluid-fluid levels. (7) Most such tumors, however, are variable in appearance, and a diagnosis based solely on radiologic investigations cannot be made with confidence. Other lesions that should be considered in the differential diagnosis include a cavernous hemangioma, a papillary endothelial hyperplasia, a disseminated lobular capillary hemangioma, and epithelioid anglomatosis. (2)

Based on CT findings, the lesion in our patient was reported as a benign cystic mass with well-circumscribed margins. However, we were not able to establish a diagnosis via aspiration cytology, so we planned an excisional blopsy. Because the lesion was reported as benign, we were able to easily excise it via a peroral route. No vascular malformations or abnormal vessels were seen in the vicinity of the lesion.

Treatment. Wide local excision is usually adequate for removing solitary or multiple adjacent lesions, although angiographic examination is advised to assess the possibility of a clinically occult vascular abnormality.

Recurrence. Perkins and Weiss evaluated 78 patients with spindle-cell hemangioendothelioma and found that approximately 60% of them experienced a local recurrence. (8) Recurrences are known to arise within the immediate area of a previous surgical site. According to Fletcher et al, it is highly likely that patients with multiple lesions that have developed over a prolonged period are manifesting a "field-change" effect rather than a true recurrence or biological progression. (4) They recommended a thorough angiographic examination for all such patients. Reported recurrences most likely represent separate primaries because they develop in previously uninvolved adjacent tissue and not at the site of a formal excision. After 4 years of follow-up, out patient remained free of any local recurrence and any new lesion.

Metastasis. To the best of our knowledge, no case of regional or distant metastasis has been documented.

In conclusion, pathologists and clinicians alike should be aware of the existence of spindle-cell hemangioendotheliomas. An early and accurate diagnosis is particularly important in view of the increase in the incidence of Kaposi's sarcoma among the same young adult population that is most often affected by spindle-cell hemangioendothelioma. The lesion in our patient posed a diagnostic challenge, and it was definitively identified as a spindle-cell hemangioendothelioma only after excision.

References

(1.) Enzinger FM, Weiss SW. Spindle cell hemangioendothelioma. In: Enzinger FM, Weiss SW, eds. Soft Tissue Tumors. 2nd ed. St. Louis: Mosby, 1988:632-5.

(2.) Toslos K, Koutlas IG, Kapranos N, Papanicolaou SI. Spindle-cell hemangioendothelioma of the oral cavity. A case report. J Oral Pathol Med 1995;24:379-82.

(3.) Eltorky M, McC Chesney T, Sebes J, Hall JC. Spindle cell hemangioendothelioma. Report of three cases and review of the literature. J Dermatol Surg Oncol 1994;20:196-202.

(4.) Fletcher CD, Beham A, Schmid C. Spindle cell haemangioendothelioma: A clinicopathological and immunohistochemical study indicative of a non-neoplastic lesion. Histopathology 1991;18: 291-301.

(5.) Imayama S, Murakamai Y, Hashimoto H, Hori Y. Spindle cell hemangioendothelioma exhibits the ultrastructural features of reactive vascular proliferation rather than of angiosarcoma. Am J Clin Pathol 1992;97:279-87.

(6.) Isayama T, Iwasaki H, Ogata K, Naito M. Intramuscular spindle cell hemangioendothelioma. Skeletal Radiol 1999;28:477-80.

(7.) Eskey CJ, Robson CD, Weber AL. Imaging of benign and malignant soft tissue tumors of the neck. Radiol Clin North Am 2000;38: 1091-1104.

(8.) Perkins P, Weiss SW. Spindle cell hemangioendothelioma. An analysis of 78 cases with reassessment of its pathogenesis and biologic behavior. Am J Surg Pathol 1996;20:1196-1204.

From the Department of Otolaryngology, University College of Medical Sciences and Guru Teg Bahadur Hospital, Shahdara, Delhi, India.

Reprint requests: Dr. Neelima Gupta, A-304 Abhyant Apartments. Plot No. 2, Vasundhara Enclave, Delhi 110096, India. Phone: 91-98-1074-5370; fax: 91-11-2658-9325; e-mail: write2neelima@hotmail. com

Originally presented as a poster during the 54th annual conference of the Association of Otolaryngologists and Head and Neck Surgeons of India; Jan. 10-13, 2002; Bangalore, India.

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