Aim: To describe and review the clinical, radiological, and histopathological characteristics of an orbital perivascular epithelioid cell tumor (PEComa). Methods: A systematic review of clinical records, radiological investigations, microscopic features, and immunohistochemical characteristics was done. Results: A 9-year-old female child presented with a year-long history of a large orbital mass associated with painless, progressive proptosis of the right eye. Radiologically, a well-defined orbital mass was seen with no intracranial extension. Excision was performed and histopathological examination showed uniform epithelioid cells in nests separated by thin fibrovascular septae. The tumor cells stained positively for Human Melanoma Black-45, but negatively for desmin, S-100, smooth muscle actin, MyoD1, microphthalmia-associated transcription factor, vimentin, CD10, CD31, and CD34 with a low proliferation index of 5–7%. Based on the tumor’s morphological and immunohistochemical characteristics, a diagnosis of giant orbital PEComa was made. No recurrence was seen at the last follow-up. Conclusions: PEComas are uncommon mesenchymal neoplasms that have typical histological features, with an immunohistochemical profile of negativity for epithelial markers and positivity for melanocytic markers. For benign PEComas, complete excision is advised. However, since PEComas elsewhere in the body have been known to be malignant, a close follow-up of such cases is recommended.
Background: Choroidal metastases being the sole presenting feature of lung cancer is rare. Erlotinib, a tyrosine kinase inhibitor (TKI), is used in the treatment of lung adenocarcinoma where tumor cells exhibit epidermal growth factor receptor (EGFR) mutations. We report a case of metastatic non-small-cell lung cancer (NSCLC) with choroidal metastasis, which was the sole presenting feature and which responded to erlotinib. Methods: We performed a retrospective case review. Case: A 78-year-old man presented with a choroidal mass which was found to be the presenting feature of metastatic NSCLC. Our patient, a nonsmoker, had disseminated bony metastases, and therefore was advised to undergo palliative chemotherapy, which he refused. He was therefore instituted on oral erlotinib. Results: Tumor cells expressing EGFR mutations are known to be susceptible to TKIs. Even though the tumor in our case showed no mutation, i.e. was classified as ‘wild-type', our patient showed a dramatic response to erlotinib. At 1 year, the choroidal lesion had regressed and visual acuity had recovered. Conclusions: TKIs may be beneficial in patients with choroidal metastases from NSCLC, especially those in which an EGFR mutation is noted. Even in the absence of such mutations, choroidal metastases may show a favorable effect in response to TKIs, such as erlotinib.
We describe the clinicopathological features of a solid variant of orbital angioleiomyoma. A review of clinical records, diagnostic, and radiographic studies combined with histopathological evaluation with standard histochemical staining and immunohistochemistry was conducted. A 22-year-old male patient presented with a mass in the region of the left lacrimal gland that was gradually increasing over the past 2 years. Radiological and clinical examinations showed no signs suspicious of a malignancy and fine needle aspiration cytology was inconclusive. Therefore, an excision biopsy was performed. On histopathological examination, the picture was consistent with a benign spindle cell tumor. Immunohistochemistry showed positivity for CD 34 and CD 31 (markers for vascular endothelium). The tumor also showed positivity for smooth muscle actin and Ki-67 proliferative index was low. Angioleiomyomas are rarely encountered in the orbit and has features seen in leiomyoma as well as some vascular tumor elements. In most cases, surgical excision is usually curative.
Introduction. Fibrocystic disease of the male breast is uncommon. The presence of a spectrum of changes ranging from fibrocystic disease to duct papilloma to papillary carcinoma in the same patient renders the case a rarity and therefore reportable. Case Report. A case of intracystic papillary carcinoma of the male breast is presented. Discussion. The pathological, clinical, diagnostic, and therapeutic options are discussed after reviewing the literature. Conclusion. Modified radical mastectomy with axillary clearance is the safest option for established cases.
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