RESUMEN
INTRODUCTION: Apocrine cystadenomas are rare, benign tumors that might arise in the periocular region from glands of Moll. They characteristically demonstrate proliferative features on histopathological examination, which differentiate them from simple hidrocystomas. PRESENTATION OF CASES: We retrospectively identified 4 consecutive cases of apocrine cystadenomas in male patients with a mean age of 48.5 years (range 20-62). One of the cases was a recurrent lesion. The Preoperative clinical diagnosis was mostly hidrocystoma. All patients agreed on complete surgical excision of their cystic lesions. Histopathological review of the excised cysts confirmed the diagnosis of apocrine cystadenoma based on the presence of proliferative features with no atypia or infiltrative behavior. Cases are summarized in Table 1 and the histopathological appearance is demonstrated in the included figure. DISCUSSION: Apocrine cystadenoma is rare. It occurs in the areas of skin with hair follicles, such as the axilla, neck, and trunk, and may be mistaken for other skin lesions, such as nevi or syringomas. In the eyelid region, they are commonly missed and frequently diagnosed as simple hidrocystoma due to the presence of bluish hue such as in our series. Recurrence is rare but was found in one of our patients at initial presentation. Our series included unique locations of this lesion in the medial canthus in one patient and near the eyelid tarsus in another. CONCLUSION: Ophthalmologist should be aware of the rare occurrence of this lesion in the periocular region. Further studies to explain the etiology of such proliferative nature in apocrine cysts would be interesting.
RESUMEN
INTRODUCTION AND IMPORTANCE: Adenoid cystic carcinoma (ACC) is an uncommon malignant epithelial tumor of the salivary and lacrimal glands. Orbital ACC is rare, originating commonly from the lacrimal gland, with only a few cases reported without lacrimal gland involvement. Deep orbital ACC may be associated with extension into skull base structures, and further intracranial invasion. CASE PRESENTATION: We report a 47-year-old gentleman who presented with insidious onset of bilateral proptosis, left ophthalmoplegia, and loss of vision. Imaging revealed left orbital infiltrative mass with intracranial invasion and bilateral cavernous sinus extension. The lacrimal gland was not involved clinically nor radiologically. Histopathology showed ACC with classical cribriform pattern. There was no evidence of primary source of tumor or metastasis. DISCUSSION: ACC of the orbit commonly originates from the lacrimal gland. Only a few cases of orbital ACC without lacrimal gland involvement were found in English literature. Workup for our patient did not reveal a primary source of tumor. Tumor may have risen from ectopic orbital lacrimal gland tissue, extension from non-orbital sites, or through perineural or hematogenous spread. Only one case of bilateral cavernous sinus extension has been previously reported. Treatment for advanced orbital ACC is exenteration in most cases. However, due to the advanced nature of disease in our patient, palliative radiotherapy was the treatment of choice. CONCLUSION: Orbital ACC of non-lacrimal origin is rare and is associated with high morbidity and mortality. Early recognition and treatment are key for preventing organ and life-threatening complications such as advanced intracranial spread.
RESUMEN
PURPOSE: We present a rare case of intramuscular alveolar soft part sarcoma (ASPS) of the lateral rectus (LR) muscle and the surgical technique used to maintain orthotropia after complete resection of the mass. OBSERVATIONS: A 5-year-old boy presented with progressive proptosis of the left eye due to an orbital tumor. The patient was previously diagnosed with ASPS of the left LR muscle from an incisional biopsy, and the tumor size increased despite 5 cycles of chemotherapy prior to presenting to our center. Magnetic resonance imaging showed a 28x19x15mm-sized contrast-enhancing intramuscular mass of the left LR muscle, and there was no evidence of nodal or distant metastasis. The mass was excised en bloc, along with the insertion and the posterior normal part of LR muscle. To maintain proper eye alignment after resecting LR muscle, a 4-0 Prolene® hang-back suture was placed between the scleral insertion and the periorbita of the posterior orbit and the left medial rectus muscle was injected with botulinum toxin. During the follow-up of 51 months after surgery, the patient had no evidence of recurrence or metastasis and remained orthotropic in primary gaze, with a good cosmetic result. CONCLUSIONS AND IMPORTANCE: ASPS of extraocular muscles is a rare tumor occurring mainly in children and young adults, and treatment may cause significant sequelae such as orbital exenteration, radiation-induced complications, and large-angle strabismus. Complete resection of tumor including the extraocular muscle is essential for treatment, and a subsequent reconstruction using a hang-back suture technique is useful to achieve proper eye alignment as well as a good cosmetic outcome.
