Adamantinoma of the Right Tibia Case Study

Ada gaytinoma of the Right Tibia Case StudyINTRODUCTIONAdamantinoma is base grade malignant tumour of fibroblast origin. in like manner called as primary epidermoid carcinoma of bone, malignant angioblastoma or epithelial tumor of bone. It was first described by Fischer in 1913.1 It represents less than 0.4% of all malignant bone tumors.2 The exact origin of adamantinoma is unknown, the classical variant is composed of epithelial cells and osteofibrous components. It is more prevalent in work force than in women, ratio being 54. The common come a capacious of innovation is in the second decade, though it can vary greatly mingled with the second to fifth decades.3 The common site of occurrence is the ramus of the mandible. The otherwise unmatched locations entangle shaft of long bones (97%), mid-shaft of tibia (80%-85%).4 Other archaic locations includes the humerus, ulna, femur, fibula and radius but ribs, spine, metatarsal and carpal bones.5 The characteristic features o f adamantinoma is s woeful, dawdling development with high chances of local restitutions and as well as metastases to lungs.6 In this case study, we absorb presented a r atomic number 18 histological acanthomatous variant of adamantinoma of the even off tibia with metastases to the respectable inguinal region.CASE REPORTA 45 year experienced male forbearing presented with history of pain in sound forking since 3-4 months, insidious onset, gradually progressive, aggravated on exercise, relieved on tarry and medication. Later persevering had no relief on medication. on that point was no history of trauma or fall preceeding to onset of symptoms. The patient too complained of swelling in the slump groin since 1 month, without pain. On examination, the patient was stable. Local examination try outed, a swelling of about 5X3cm in the amphetamine end serious leg, tender, well defined, hard in consistency, smooth surface, involving the right knee joint line. There was restr iction of movement, but range of movements was present. interrogation of right inguinal region revealed a 7X6cm firm, non-matted mass, immobile, non-tender, non-reducible, non-pulsatile mass. Local examination of penis, scrotum, anal canal, hernia orifices was normal. Baseline investigations were done, all the haematological parameters were normal, ultrasound abdomen and chest radiograph was normal. Plain radiograph of right tibia showed large rally lytic lesion with sclerotic margins, involving the upper end and shaft of tibia without involvement of right knee joint. Supra-patellar amputation of right leg with right inguinal and right outer iliac nodal dissection was done. Histopathological examination of the mass was done. The reported was suggestive of acanthomatous adamantionoma. The features ar as described, central area of squamous cell nests which were well differentiated with keratinization border by a rim of myxoid cells. Further immunohistochemistry(IHC) study was d one to back the diagnosing. IHC was also legitimate with the diagnosis and confirmed the diagnosis. Positivity was found for cytokeratin(CK), EMA, CK-19, CK-5, CK-6, P-63, and Ki-67 and vimentin. Also metastases to right inguinal lymph node was confirmed.DISCUSSIONThe first bone tumor with epithelial characteristic was reported by Maier in 1900, later in 1913 Fischer termed it as adamantinoma. It is more common in men than in women. The age of presentation varies between the second to fifth decade. The uncommon sites includes the shaft of long bones(97%), of which the mid-shaft of tibia being the about common site(80-85%). The symptoms at presentation include pain with localised swelling. Swelling is the most common symptom. Pain whitethorn be present due to history of repetitive trauma associated with adamantinoma.7 Adamantinoma is low grade malignant tumor, and can metastasize to loco-regional nodal areas and to lungs at presentation. The patient in the case report was also a m iddle aged man in the fifth decade with pain and swelling in the right tibia since 3-4 months and swelling in the right groin.Three theories have been proposed for the bloodline of cell of origin, 1) epithelial, 2) angioblasts, and 3) synovial tumors from uncommon ectopic sites. This was done because the tumor thread is composed of twain epithelial and osteofibrous components.8 In different combinations, different conceptions are formed. On microscopy, the commonest form is the classical variant, followed by the osteofibrous pattern. In the classic variant, the types are basaloid, spindle cell, tubular, and squamous patterns. In the classical variant, central epithelial component with border zone of fibrous tissue containing few immature trabecuated bone components are seen. In the osteofibrous variant, cental area of fibrous tissue, surrounded by off-base zone of epithelial components are seen. Spindle cell variant is most commonly seen with recurrent lesion and metastatic le sions. The typical findings of classical variant were consistent in our case as well, which showed a well differentiated keratinizing pattern of squamous cell nests surrounded by a rim of myxoid cells.Immunohistochemistry exhibits positivity for both epithelial and osseous elements. Fibrous components are positive for vimentin. Epithelial components show positivity for cytokeratin(CK) 5, 14, 19 rarely positive for CK 1,13,17. It is ordinarily negative for CK 8 and 18. Also shows positivity for epithelial growth fixings, fibroblast growth factor, fibroblast growth factor receptor. Our case had features as described, also positivity was found for p-63, Ki-67 and CK-6, which favour our diagnosis of acantomatous adamantinoma.Genetic studies reveal chromosomal abberations on chr 7,8,12,19.9 Also aneuploidy has been associated with epithelial components.10 There is loss of heterozygosity of p53 gene. Rarely,(1122) translocations have been reported in case of adamantinoma, and is termed adamantinoma-like ewings sarcoma.11 1122 translocation is not seen is in adamantinoma.Radiological investigations include radiographs, computer tomography(CT), magnetised resonace imaging(MRI). All the modalities are necessary not only to confirm the diagnosis, also to define the extent of involve and the aggressiveness of the tumor. On plain radiographs usually eccentric, lobulated, cental lytic lesions with a peripheral sclerosis is present in the diaphyseal or metaphyseal ends of long bones. These lesion tend to appear on tibia with a typical picture of soap-bubble appearance.12 CT shows osteolytic lesion in the bone. MRI is the investigation of choice. It helps not only in defining the extent of cortical involvement of bone, docile tissue involvement, but also helps in ruling out other etiologies such as ewings sarcoma, adamantinoma, osteosarcoma, metastatic disease, fibrous dysplasia of bone.13 The typical features as described in the literature was true in our case as well. Treatment of adamantinoma has no definitive guidelines. Excisional biopsy is preferred rather than curratage of bone. Curratage of bone is associated with recurrences, upto 30% recurrence has been reported with curettage.14 Radical excision of the bone is associated with intermit outcome and prognosis. Limb salvage procedures include en-block resection, followed by reconstruction of the scraggy defect which includes vascularised or non-vascularised bone grafts, custom made protheses, intercalary allografts and embarrassment osteogenesis.15 When limb salvage is not possible, amputation is the definitive treatment of choice. The incidence of mortality rate with adamantinoma is about 17%, and increases to about 20% when pulmonary metastasis is present. In our patient work up was done and right supra patellar amputation was done, with right inguinal and right external iliac lymph nodal dissection was done and histopathological examination set ahead showed metastases to right inguin al region.