Primary Orbital Leiomyoma and Leiomyosarcoma

Abstract

A case of an extremely rare primary orbital leiomyoma in a 25-year-old male patient is presented who had a lifelong history of deviation of the left eye globe with slight enophthalmos and reduced motility. Because of pain and increasing deviation of the eye the tumor was totally resected. On histologic examination the tumor showed ossification which is extremely rare so that a calcifying fibroma had to be ruled out. In immunohistochemistry, however, this tumor stained with smooth muscle antigen. Less than 2% of cells stained positive for Ki-S1, a proliferation marker. The second case is a rare primary orbital leiomyosarcoma in an 84-year-old female patient that showed massive growth. After exenteration histologic examination showed a dedifferentiated highly malignant soft tissue tumor which expressed desmin and smooth muscle actin but was negative for myoglobin, S-100 and HMB-45.

Leiomyoma of hard palate: A rare case report

“…Highest prevalence is seen in the 4th and 5th decades of life with slight male predilection [13] (1.43:1). However, few reports suggest slight female predominance of leiomyomas in the head and neck region and the authors attribute it to hormonal variation, i.e., progesterone receptor positive and estrogen receptor negative on immunochemical studies [14,15]. Although most of these lesions are asymptomatic, few authors reported symptomatic lesions that are associated with pain, tooth mobility, difficulty in chewing and swallowing.…”

Orbital metastasis of retroperitoneal leiomyosarcoma

“…A search of MEDLINE (1966-2009) using the key words ''retroperitoneal leiomyosarcoma'' and ''orbit'' produced 28 citations; however, no report of orbital metastasis of a retroperitoneal leiomyosarcoma was found. In nine cases, leiomyosarcomas originated from the orbit [2][3][4][5][6][7]. In six cases, orbital leiomyosarcomas occurred after high-dose radiation therapy for retinoblastoma [8][9][10][11].…”

Leiomyoma of the Orbit and Periocular Region

“…Since then, only 15 well-documented cases of orbital leiomyoma have been reported in the English-language medical literature. [2][3][4][5][6][7][8][9][10][11][12][13][14][15] The diagnosis of orbital leiomyoma was confirmed by immunohistochemistry in only 4 of these 22 reported cases. We reviewed 4 cases of orbital leiomyoma.…”

“…Although rare, intracranial extension 5,14 and extension in paranasal sinuses have been reported. 12 Orbital CT usually shows a well-circumscribed orbital mass. Although MRI was used previously in 2 cases, 12,13 the imaging characteristics have not been well documented.…”

“…Only 22 well-documented cases have been previously reported in the literature. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15] Leiomyoma and vascular leiomyoma (angiomyoma) are tumors of smooth muscle origin that are composed of interlacing fascicles of spindle-shape cells with prominent cytoplasmic acidophilia. Some tumors show prominent vascular spaces, whereas others are predominantly solid spindle cell proliferations that do not exhibit a conspicuous vascular component.…”