Distal Tibiofibular Joint Reconstruction Using Autograft in a Rare Case of Lower Limb Sclerosing/Spindle Cell Rhabdomyosarcoma: A Two-Year Follow-Up.

Sclerosing/spindle cell rhabdomyosarcoma (s-scRMS) is a rare variant of striated muscle tumours. It has been recognised as an individual entity, the fourth subtype of rhabdomyosarcoma in the latest WHO classification. In the paediatric population, it occurs more commonly in the paratesticular area, whereas in adults, it occurs more commonly in the head and neck region. It has distinctive characteristics in terms of its histopathological and immunochemistry findings, which help in accurate diagnosis. The mainstay of treatment is a multimodal approach, i.e., surgery, chemotherapy, and radiation therapy. However, no standard care is still being established internationally for adult cases. In adults, this tumour has a poorer prognosis as compared to children. We describe a patient with s-scRMS of the lower limb who has undergone wide local resection of the tumour with surgical reconstruction of the distal tibiofibular joint with autograft and its two-year outcome.

Unusual Presentation of Giant Upper Limb Malignant Melanoma.

Melanoma is rare among the Asian population. In Malaysia, there is little public awareness of melanoma compared to other types of cancer. Giant melanomas measuring more than 10 cm are rarely encountered and there are limited data available regarding this disease. We would like to report an unusual presentation of giant malignant melanoma of upper limb in a 62-year-old patient who presented with a five-month history of a progressively enlarging, painless mass on his left arm. This mass which measures 10 x 15 cm turned out to be a giant malignant melanoma of the left arm. To our knowledge, this is the largest melanoma of arm reported in Malaysia. Radiological findings were suggestive of soft tissue sarcoma with lung metastasis. Patient underwent wide local excision of left-arm mass and upper limb reconstruction. The diagnosis of malignant melanoma was made based on the final histopathology report. Despite aggressive treatment involving multidisciplinary units, it did not prevent the disease progression and patient succumbed five months after his presentation. Here, we present our experience in the management of this large malignant melanoma of the arm and to stress the sinister nature of melanoma.

Metastatic Vascular Pleomorphic Leiomyosarcoma in a Previously Treated Cervical Carcinoma Patient: A Diagnostic Dilemma.

Vascular leiomyosarcoma is a rare malignant tumour of the smooth muscle connective tissue. Patients are usually asymptomatic in the early stages and only present when the lesion causes compressive or obstructive effects or has metastasized. We report a case of vascular pleomorphic leiomyosarcoma in a 70-year-old lady with a background history of squamous cell carcinoma of the cervix. She presented with a three-month history of low back pain, which radiated to the anterior bilateral thigh. Initial radiological findings revealed metastatic lesions involving the spine and lungs. Two spinal biopsies done were inconclusive. Increasing severity of pain over the right thigh prompted further imaging, which revealed bilateral femoral lesions. The patient underwent surgery which involved excision of the tumour in the right proximal femur with skeletal reconstruction using megaprosthesis. Proximal femoral nail was performed for the left femur. Intra-operatively, tumour was noted at the anteromedial aspect of the proximal right thigh surrounding the superficial femoral vein. Histopathological report of the right thigh mass finally confirmed a diagnosis of vascular pleomorphic leiomyosarcoma. The patient presented four months later with bilateral pulmonary embolism with deep vein thrombosis in addition to progression of the disease.