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Benign tumors
Published in Archana Singal, Shekhar Neema, Piyush Kumar, Nail Disorders, 2019
Enchondroma is the most frequent of the subungual chondromas. It is a solitary intraosseous proliferation of cartilage. Multiple enchondromas are observed in enchondromatosis (chondrodysplasia of Ollier, OMIM 166000) and chondrodysplasia with multiple soft tissue and intraosseous hemangiomas (Maffucci–Kast syndrome, OMIM 614569); both syndromes are due to cartilage that fails to undergo normal ossification and both tend to progress to chondrosarcoma—almost 25%—and have a higher malignancy rate in general.223 Solitary enchondromas of the distal phalanx with enlargement of the tip of the digit is rare.224 About 20% of the lesions remain asymptomatic, but paronychia, clubbing, and secondary nail changes with longitudinal ridging are seen.225 Pathologic fractures are the result of progressive thinning of the cortical bone. Radiography demonstrates well-defined to cloudy radiolucent defects with expansion of the terminal phalanx, in most cases close to the articular surface of the distal interphalangeal joint.5
Orthopaedic oncology
Published in Pankaj Sharma, Nicola Maffulli, Practice Questions in Trauma and Orthopaedics for the FRCS, 2017
Pankaj Sharma, Nicola Maffulli
Chondrosarcoma most commonly occurs in the fifth to sixth decade of life, and the male:female ratio is 1.5:1. A better prognosis is seen with clear-cell chondrosarcoma, and only a 10% survival rate is reported for dedifferentiated chondrosarcoma. Patients with Ollier’s disease (multiple enchondromatosis) or Maffucci’s syndrome (multiple enchondromas and haemangiomas) are at much higher risk of chondrosarcoma than the normal population, and often present in their third or fourth decade.
Test Paper 3
Published in Teck Yew Chin, Susan Cheng Shelmerdine, Akash Ganguly, Chinedum Anosike, Get Through, 2017
Teck Yew Chin, Susan Cheng Shelmerdine, Akash Ganguly, Chinedum Anosike
The patient has developmental dysplasia of the hip, which has resulted in subluxation of the femoral head. The centre edge angle is useful in older toddlers up to adulthood to estimate the degree of acetabular over or under coverage. It is the angle between a vertical line drawn up from the centre of the femoral head and the outer edge of the acetabular roof. In an adult, an angle less than 20 degrees indicates underlying dysplasia. Perthes disease is a paediatric condition characterised by avascular necrosis of the femoral head. Ollier’s disease is the condition of multiple enchondromas (enchondromatosis). Isolated traumatic subluxation requires significant trauma in the absence of an underlying dysplasia.
IDH1 mutated acute myeloid leukemia in a child with metaphyseal chondromatosis with D-2-hydroxyglutaric aciduria
Published in Pediatric Hematology and Oncology, 2020
Anand Srinivasan, Yaolin Zhou, Teresa Scordino, Sandeep Prabhu, Andrea Wierenga, Garfield Simon, Klaas J. Wierenga, Joel Thompson, Rikin Shah, Arpan A. Sinha
A six-month-old boy, born at term to non-consanguineous parents, presented with unilateral congenital cataract, short stature and developmental delay. He had abnormal skin pigmentation – predominantly abdominal streaky and whorly hyperpigmented papules and plaques; large lop ears; bumpy irregularities of femur and tibia and brachydactyly. Dermatology felt his skin lesions most likely represented angiokeratoma circumscriptum due to their well-demarcated, plaque-like appearance. Imaging showed skeletal enchondromatosis as evidenced by benign hyaline cartilage forming tumors in the medulla of the metaphyseal bone14 (Figure 1). Workup revealed elevated urine D-2-HGA levels, consistent with the diagnosis MC-HGA.
Malignant transformation of a phalangeal enchondroma into a recurrent grade II chondrosarcoma requiring successive transcarpal amputations: a case report
Published in Case Reports in Plastic Surgery and Hand Surgery, 2022
Ceyran Hamoudi, Benjamin Bouillet, Antoine Martins
Recurrence of enchondromas is uncommon, and malignant transformation of a benign enchondroma or multiple enchondromatosis to secondary chondrosarcomas in the hand is extremely rare [6]. Some conditions, such as Ollier disease or Maffucci syndrome, characterized by multiple enchondromas, are associated with a high malignant transformation rate, varying from 20% to 57% [10]. Nevertheless, in our case, a malignant transformation of an enchondroma to a grade II chondrosarcoma was observed.