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  • Aggressive osteoblastoma is characterized by

    2019-05-21

    Aggressive osteoblastoma is characterized by epithelioid osteoblasts that are, two to three times larger than conventional osteoblasts, and have abundant eosinophilic cytoplasm, large vesicular eccentric round to oval nuclei with prominent nucleoli. Usually there are rare or absence of mitoses and if present, are normal in appearance [11]. The neoplastic bone may be trabecular or coarse and lace-like in appearance. Phenotypic markers that can help to discern an osteoblastoma over an osteosarcoma are sharp margin of the tumor and presence of osteoblastic rimming [2]. In the case of aggressive osteoblastoma, the tumor border shows peripheral maturation without sharp demarcation [12]. The border between pre-existing Cy3-dUTP or marrow trabeculae must be examined histologically as osteoblastomas do not infiltrate and isolate pre-existing lamellar bone structures as does osteosarcoma [3]. Moreover, significant nuclear atypia, altered nuclear-to-cytoplasmic ratios, and profuse mitoses together with atypical forms are characteristics of osteosarcoma and absent in osteoblastoma [2]. Osteoblastomas are surgically treated as the tumor may exhibit aggressive radiological appearance, despite its benign nature, as in the present case. Extended intralesional curettage is usually done in most locations while resection with wide margins is done in expendable bones like ribs and fibula [13]. Reconstruction may be required in cases where surgical excision or curettage creates a sizable defect. The present case treated with a ray amputation as cortical involvement prevented intralesional curette. Postoperative radiation or chemotherapy is not required in such tumor. The various histologic subtypes of osteoblastoma have a similar clinical behavior, although there are some disagreements regarding the aggressive type. In most cases, osteoblastoma behaves as a benign tumor, having no metasizising capacity. However they can grow, become large, and produce bone destruction and pain [2]. Although prognosis of osteoblastoma is excellent, both relapse and malignant tumor transformation has been described, thus requiring thorough postoperative follow-up [14]. Recurrences are likely to happen in such a location which has difficult surgical access resulting in inadequate curette [3].
    Conflict of Interest Statement
    Discussion Trichorhinophalangeal syndrome (TRPS) was first described in 1966 by Giedon who delineated a syndrome characterized by sparse and slowly growing hair, a long pair shaped nose with a bulbous tip, and finger deformities [1]. Since Geidon\'s first descriptions of TRPS, only subsequent small cases series have been published detailing further phenotypical characteristics along with attempts to specify the genetic abnormalities. TRPS can be subdivided into three sub-types with the above key features expressed in all three [2,3]. The syndrome can have multi-system involvement with associated endocrine disorders, renal alterations, heart anomalies and bone dysplasia [1–4]. TRPS I and III are inherited in an autosomal dominant fashion, while TRPS II are often sporadic cases [4]. Deletions, insertions and translocations of chromosome 8, specifically from 8q24.11 to 8q24.13 resulting in haploinsufficiency of a specific zinc finger protein that is a putative transcription factor have been indicated as the causative genetic abnormality [5–7]. In addition to the genetic anomalies for TRPS I, TRPS II is caused by a further mutation in the EXT1 gene that spans the TRPS I locus resulting in multiple cartilaginous exostoses [8–10]. No further cytogenetic abnormalities have been described in TRPS III; supporting the theory that TRPS III is on the severe end of the same spectrum as TRPS I [2]. The diagnosis and sub classification of TRPS is, however, based on clinical and radiological findings, supported by genetic analysis in equivocal cases [4]. Orthopedic surgeons are likely to come into contact with these patients due to their bony deformities of the hands, femoral head and presence of multiple exostoses that may cause nerve impingement, joint mobility problems or extreme discomfort [11–13].