Sino-orbital osteochondroma with malignant transformation to osteosarcoma.

Purpose: Osteosarcoma is an aggressive malignant osteogenic tumor that commonly arises in long bones of pediatric populations. Primary osteosarcomas of the head and neck are rare, comprising less than 0.5% of malignancies in this region, usually affecting the mandible or maxilla. Here we present an extraordinary case of a rare benign osteochondroma of the ethmoid sinus and bilateral orbits evolving to an intermediate grade osteosarcoma. Observations: An 80-year-old woman with a history of right orbital tumor resection 20 years ago presented to our clinic with right eye proptosis and palpable bony prominence of the right orbit and nasal bridge. Partial resection demonstrated sino-orbital osteochondroma. Relapse a year later prompted repeat partial resection with unchanged histology. The patient was followed clinically until an abrupt relapse four years after initial presentation. Imaging demonstrated a large bony mass involving the right orbit, ethmoid and frontal sinuses, and anterior cranial fossa. Repeat debulking confirmed transformation to intermediate grade osteosarcoma. Conclusions: Osteochondroma is an extremely rare tumor in the orbit with only three cases previously reported. This patient is the first known case of benign osteochondroma of the orbit undergoing malignant transformation to osteosarcoma. Rapid progression of orbital osteochondroma should raise the suspicion of malignant transformation to osteosarcoma and prompt biopsy. Our patient subsequently underwent palliative radiation treatment and is stable with no gross progression.

Unusual Case of a Caruncular Oncocytoma.

ABSTRACT: Oncocytomas are benign neoplasms that are most often found in the salivary glands and kidneys. Ocular oncocytoma typically present as an asymptomatic dark blue papule on the lacrimal caruncle. Histologically, the tumor is composed of oncocytes, cells notable for eosinophilic cytoplasmic granules representing large quantities of dysfunctional mitochondria. The neoplastic proliferation may be partially or totally encapsulated and may be surrounded by myoepithelial cells. Discussed is the pathophysiology and histopathology of an ocular oncocytoma from the right lacrimal caruncle of a 68-year-old man. The patient complained of a foreign body sensation and tearing associated with a purpuric 1 × 1-mm papule of the right caruncle that had been present for 6 months. An excisional biopsy showed an oxyntic neoplasm with small cystic glandular spaces associated with goblet cells and mucin, confirmed by mucicarmine stain. Given the rarity of ocular oncocytomas and presence of mucin, exclusion of an endocrine mucin-producing sweat gland carcinoma was necessary. Peripheral myoepithelial cells noted on p63 immunostaining are seen in both endocrine mucin-producing sweat gland carcinoma and oncocytomas. However, endocrine mucin-producing sweat gland carcinoma is excluded by the presence of goblet cells, the uniform oxyphilic cytoplasm of the epithelial cells, and the negative immunohistochemical staining for neuron-specific enolase, chromogranin, synaptophysin, estrogen receptor, and progesterone receptor. Complete surgical excision is the treatment of choice for oncocytomas. This case highlights the clinical and histopathological presentation of ocular oncocytomas and raises awareness of this rare entity for both the practicing dermatologist and dermatopathologist.

Caruncular Dacryops After Cataract Surgery With Histopathological Characterization.

ABSTRACT: Caruncular dacryops is a rare cyst of lacrimal tissue most commonly found in the palpebral lobe of the lacrimal gland. The exact cause of dacryops is unclear, although it may be congenital or associated with trauma, infection, or inflammation. The pathophysiology and histology of a caruncular dacryops of the left lacrimal gland in a 68-year-old woman is reported. The patient presented 3 months after a cataract surgery with symptoms of irritation and pruritus of the left eye. A 6- × 6-mm clear cyst of the lacrimal gland prolapsing into the lateral fornix was noted on physical exam. Histological examination of the cyst showed a double layer of flat cuboidal epithelium surrounded by fibrous tissue with minor lacrimal glands present. Periodic acid Schiff-diastase and mucicarmine staining showed the presence of goblet cells dispersed in the epithelial cells. Complete excision of the cyst is the treatment of choice for most caruncular dacryops, and our case remains without recurrence after excision. This case highlights the clinical and histological presentation of caruncular dacryops and raises awareness of their incidence following presumed trauma during cataract surgery.