Huw Oliphant BSc MBBCh Mark Taylor FRCPath Saul Rajak PhD FRCOphth
Cutaneous pleomorphic rhabdomyosarcoma (PRMS) is a rare malignant mesenchymal tumour which can affect all age ranges, though subcutaneous forms of this disease are uncommonly described. We present a case of a 77‐year‐old lady with a rapidly growing periocular lesion subsequently demonstrated on histopathological and immunohistochemical examination to be a PRMS. No orbital involvement was demonstrated and no distant metastases were evident. PRMSs represent highly aggressive and infiltrative tumours and must be recognized in a timely fashion to allow safe and adequate treatment. Alongside traditional histopathological examination, immunohistochemistry is invaluable in the diagnosis of this condition…..
Maftuhim Addenan, Choo May May, Teoh Kean Hooi, Fazliana Ismail, Tengku Ain Kamalden
Langerhans cell histiocytosis (LCH) is rarely encountered in ophthalmology practice. It is a spectrum of disorder characterized by accumulation of histiocytes in various tissues. Diagnosis is challenging as it may simulate periorbital hematoma, rhabdomyosarcoma, and neuroblastoma. We report a case of unifocal LCH with orbital extension. Diagnosis was obtained from incisional biopsy, and histopathological examination showed numerous histiocytes with eosinophilic infiltrations. The presence of Langerhans cells was confirmed by the presence of protein S-100, CD1a, and/or Langerin (CD207). Treatment depends on the degree of organ involvement. She responded well to cytotoxic drugs and steroids. This emphasized that prompt tissue diagnosis is crucial for early management.
Justin D Pennington, R Joel Welch, Sara E Lally, Jerry A Shields, Ralph C Eagle, Carol L Shields
No abstract available
Morten Jørgensen, Steffen Heegaard
Tumors that invade the orbit are uncommon. The majority are meningiomas arising from the sphenoid ridge (66%). Others are bone and cartilage tumors arising from the surrounding bones of the orbit, pituitary adenomas, and epithelial tumors arising from the paranasal sinuses and nasal cavity. Meningiomas occur more often in women, whereas epithelial tumors have a predilection for men. Meningiomas and epithelial tumors typically present in the sixth decade of life, whereas bone tumors tend to affect individuals in their third decade of life. Patients often present with a combination of ophthalmological and otorhinolaryngological symptoms, including proptosis, pain, decreased visual acuity, restrictions in motility of the eye, epistaxis, and nasal obstruction. Sarcomas and benign bone and cartilage tumors arise from surrounding structures, whereas carcinomas usually arise from the paranasal sinuses. Surgery is the mainstay of treatment. Depending on the aggressiveness and histology of the tumor, surgery may be combined with radiation and chemotherapy. The prognosis is generally poor, but varies depending on histology and cell origin, size of the tumor, and degree of invasion. Meningiomas and benign bone tumors have the best prognoses. Sinonasal undifferentiated carcinomas, small-cell neuroendocrine carcinomas, osteosarcomas, and rhabdomyosarcomas have poorer prognoses.
Ang Li, Alexander Blandford, Rao V. Chundury, Elias I. Traboulsi, Peter Anderson, Erin Murphy, Sumit Parikh, Julian Perry
We present the case of a 6-year old girl with Leigh syndrome and an orbital rhabdomyosarcoma and describe her clinical course and our multidisciplinary approach to treatment. To our knowledge, this is the first report of rhabdomyosarcoma in a patient with Leigh syndrome.