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ABSTRACT Calcifying fibrous tumor is a rare benign mesenchymal neoplasm. The etiology and pathogenesis of this tumor are uncertain. It has wide anatomical distribution. The tumor is most commonly found in the soft tissues of the extremities in younger individuals. However, in middle-aged patients, it tends to affect the visceral locations more commonly. In visceral location, it can mimic aggressive lesions clinically. The purpose of this report is to describe a case of calcifying fibrous tumor in a 71-year-old female with a history of breast carcinoma who was found to have an incidental small bowel mass on her follow-up. Clinically and radiologically, the mass was suspicious for either metastatic disease or gastrointestinal stromal tumor. The patient underwent open small bowel resection, and a 6.5 cm segment of the small bowel was sent to pathology. Grossly, a 2.0 cm tan-pink smooth round submucosal polyploid mass protruding into the lumen, mimicking a gastrointestinal stromal tumor, was identified. The tumor was hard and serially sectioned to reveal a white, calcified cut surface. Microscopically, the tumor appeared hypocellular and composed of scant spindle cells embedded in a dense, hyalinized and calcified collagenous stroma. Immunohistochemical stains for pan-cytokeratin, DOG1, desmin, S100, CD34, and MUC4 were negative, and a diagnosis of the calcifying fibrous tumor was rendered. This case provides a rare gross specimen image of calcifying fibrous tumor and highlights the importance of knowledge of rare entities in providing an accurate diagnosis for entities that can mimic other lesions.
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ABSTRACT Background Fibrous hamartoma of infancy (FHI) is a rare soft tissue lesion arising as a subcutaneous mass involving the axilla, trunk, and upper arm in infants and children <2yrs. Sarcomatous transformation in FHI is described in anecdotal cases in the literature. Case Report We describe one such example arising as a mass in the lower back in a 3-month-old infant. On histology, the tumor contained classic triphasic morphology; however, brisk mitotic activity noted at multiple foci was diagnostically challenging to categorize. The tumor was evaluated for ETV6-NTRK3 fusion to exclude other common differentials. Conclusion While FHI may be frequently encountered in infants, rare sarcomatous transformation are known to occur and merits special attention as it can be misdiagnosed. Also, a close follow-up is warranted as the lesion is known to recur locally.
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ABSTRACT Objective: To compare the agreement of clinical and radiographic diagnosis with the histopathological diagnosis in fibro-osseous lesions of the jaws. Material and Methods: An analytical and exploratory study was made based on systematic collected data, carried out in the laboratory of surgical pathology of a public Dental School. There were evaluated cases of fibrous dysplasia (FD), cemento-osseous dysplasia (COD) and ossifyng fibroma (OF), diagnosed by clinical, radiographic (panoramic and periapical radiography), and histopathological analysis, in a period of 12 years (from March 2001 to June 2013). Descriptive and inferential statistics (Fisher's exact test) were obtained. Results: Ninety-six cases of FOLs were evaluated. The radiographic aspects of the FOLs studied did not differ significantly (p=0.09). Radiolucent lesions were the least frequent, corresponding to approximately 13.5% of radiographic findings. Mixed lesions and radiopaques were more present, how they were COD and FD, respectively. The more aggressive variation of OF (Juvenile Ossifying Fibroma - JOF) was less frequent among the pathologies evaluated. In approximately 61.46% of the cases clinical and radiographic diagnosis were confirmed by histopathological diagnosis of FOLs. The highest agreement and the highest disagreement were observed in COD cases (40.7% and 62.2%, respectively). Conclusion: FOLs of the maxillaries represent a group of lesions in which the establishment of the clinical and radiographic diagnosis supported by the histopathological confirmation is critical and challenging.
Subject(s)
Pathology, Oral , Pathology, Surgical , Neoplasms, Fibrous Tissue/pathology , Fibrous Dysplasia of Bone/pathology , Schools, Dental , Brazil , Radiography, Dental/instrumentation , Data Interpretation, Statistical , Fibroma, OssifyingABSTRACT
O dermatofibrossarcoma protuberante é um sarcoma localmente agressivo, de malignidade intermediária, que predomina na faixa de adultos jovens à meia-idade. Lesões congênitas ou na infância são raras, mas foram relatadas. Nos estágios precoces, pode ser mal diagnosticado e ser incompletamente excisado, o que aumenta o risco de recorrências. Relata-se o caso de paciente do sexo masculino, de 24 anos, com história de mácula hipercrômica em região abdominal desde o nascimento que evoluiu para tumoração após duas abordagens cirúrgicas. Biópsia e imuno-histoquímica confirmaram o diagnóstico de dermatofibrossarcoma protuberante, e a lesão foi excisada com margem de 3cm
Dermatofibrosarcoma protuberans is a locally aggressive sarcoma of intermediate malignancy that predominates in the range of young adults to middle age. Congenital or childhood lesions are rare but have been reported. It can be misdiagnosed and incompletely excised in the early stages, which increases the risk of recurrence. The present article reports the case of a 24-year-old male patient presenting a hyperchromic macula in the abdominal region from birth that evolved into a tumor after several surgical approaches. Biopsy and immunohistochemistry confirmed the diagnosis of dermatofibrosarcoma protuberans, and the lesion was excised with a 3 cm margin
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El tumor fibroso solitario es una neoplasia fuso celular, poco común de localización pleural frecuentemente. Presentamos un caso de presentación crónica evidenciándose masa palpable identificando tumoración de 35 x 20 x 15 cm. Diagnosticándose por anatomía patológica tumor fibroso solitario gigante (TFSG). En la revisión bibliográfica sobre los tumores diagnosticados como TFSG es muy infrecuente, siendo el primero reportado en nuestro medio.
Giant solitary fibrous tumor (TFSG) is a spindle cell neoplasm, uncommon, being pleural a common site. We report a case of chronic presentation of 4 months duration with a palpable mass. We found, a tumor 35 x 20 x 15 cm .In the literature tumors diagnosed as TFSG are very rare, the first reported in our country.
Subject(s)
Humans , Male , Middle Aged , Retroperitoneal Neoplasms/diagnosis , Solitary Fibrous Tumors/diagnosisABSTRACT
A 78-year-old male patient presented with an inguinoscrotal nodule that had lasted for 3 years. The patient reported a past medical history of hydrocele formation. The final diagnosis was fibrous pseudotumor of the paratesticular region, which was based on a histopathological examination and immunohistochemical findings. The aim of this case report is to spread awareness and recognition of neoplastic and non-neoplastic entities that occur in the paratesticular region.
Subject(s)
Aged , Humans , Male , Cell Proliferation , Diagnosis , Neoplasms, Fibrous TissueABSTRACT
Objective To observe CT characteristics of primary retroperitoneal malignant fibrous histiocytoma (MFH). Methods CT images and clinical data of 25 patients with primary retroperitoneal MFH proved pathologically were reviewed and analyzed retrospectively. Results A total of 37 lesions were identified in 25 patients, in which 17 had single lesion and 8 had multiple lesions, with mean diameter of the tumor of 12.85 cm, including 32 light lobulated and 5 round-shaped lesions. Among all 37 lesions, 28 were well-defined and the others had unclear border, while 7 masses were homogenous in density and the other 30 were inhomogenous with necrosis, cysts, bleeding or calcification. All lesions enhanced in various degrees with slight enhancement in arterial phase and moderate enhancement in venous phase. All the patients underwent immunohistochemistry examination, and the positive rate of Vimentin, CD68, AACT, S-100, CKpan and EMA were 94.74%, 90.48%, 88.24%, 0, 0 and 15.00%, respectively. Conclusion Primary retroperitoneal MFH have some specific CT manifestations that being helpful to the diagnosis, but final diagnosis depends on the cytopathology and immunohistochemistry.
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Objective To investigate the diagnosis and treatment of malignant fibrous histiocytoma (MFH) in abdominal cavity. Methods The clinical manifestations, imaging examination, pathology, methodology, effect of treatment and prognosis of 28 MFH patients were retrospectively analyzed. Results Tumors were in the retroperitoneal space in 17 cases, within abdominal cavity in 6, in mesentery in 3 cases, and in the abdominal wall in 2 cases. The clinical symptoms were body weight loss, abdominal pain and mass. B ultrasonography and CT scanning showed mass in the abdomen. 24 cases were with multiplefoci,4 cases were with a single mass. Seventeen cases underwent complete tumor resection,11 cases with partial resection, 16 cases received postoperative chemotherapy and 14 cases with postoperative radiotherapy. The overall survival rate of 1-, 3- and 5- year was 76.9%,26.9% and 3.9%,respectively. Conclusion MFH is most often located in retroperitoneal space, surgical resection was the treatment of choice. The prognosis in patients with MFH might be improved by complete resection combined with chemotherapy or/ and radiotherapy.