Unusual Progression of Pleomorphic Adenoma of the Lacrimal Gland-Case Report-

Abstract

A 72-year-old female complained of acute pain on left eye movement followed by progressive exophthalmos. Neuroimaging revealed a large well-demarcated lesion consisting of solid and cystic parts, as well as bone destruction and hemorrhage, within the left orbital cavity. The preoperative diagnosis was pleomorphic adenoma with or without malignant transformation, or cavernous angioma. En bloc excision including adjacent tissues was planned to resolve the progressive symptoms and to obtain a histological diagnosis. The transcranial route was chosen since tumor invasion to the cranial base was possible. The histological diagnosis was pleomorphic adenoma. Pathological and preoperative radiological examinations indicated that repeated intratumoral hemorrhage had caused the orbital bone destruction and acute orbital pain. Neoplasms should be differentiated from a wide spectrum of other possible pathologies. Accurate clinical diagnosis of neoplasm in the orbital cavity is important for correct therapeutic management. Malignancy is generally suspected if painful and progressive signs and symptoms are associated with an orbital mass lesion. The present case suggests that pleomorphic adenoma should also be considered in the differential diagnosis. The therapeutic strategy for lacrimal gland tumors remains controversial, so a flexible management approach is required.

An unusual presentation of lacrimal gland pleomorphic adenoma

“…Although not usual, there may be intratumoral hemorrhage (7) , bone destruction (4,9) and calcification (4,9,10) . Intracranial extension, as happened in our case, is very rare.…”

“…It is believed that preoperative biopsy and incomplete resection could lead to the tumor recurrence (even after years), as well as to malignant transformation (2)(3)(4)(5)(6)(7) .…”

“…A recurrence rate of 3% within five years has been reported in complete excisions and a recurrence rate of 32% over 15 years in incomplete ones (2,3,7) . It is estimated that 10% of pleomorphic adenomas undergo malignant change within 20 years after first treatment and 20% by the end of 30 years.…”

“…Radiological investigation may be done either by computerized tomography (CT) or magnetic resonance imaging (MRI). The treatment is the complete excision of the tumor and adjacent tissues (2)(3)(4)(5)(6)(7) , and the prognosis is good when the lesion is completely excised with an intact capsule (3)(4)(5) .…”

Pleomorphic adenoma of the nasopharyngeal mucosal space with locally aggressive appearance (2010: 11b)

“…In this case, bone changes were atypical, with a destructive appearance on CT more suggestive of an aggressive lesion. An aggressive pattern of bone destruction has been described in recurrent pleomorphic adenoma following presumed incomplete surgical excision [6] and there are single case reports of bone destruction associated with pleomorphic adenoma of the lacrimal gland [7] and middle ear/mastoid [8]. However, this appearance is unusual and should be considered a feature suggestive of malignancy in routine clinical practice.…”