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Background: Liposarcomas including atypical lipomatous tumors (ALT)/well-differentiated liposarcomas (WDLPS) and dedifferentiated liposarcomas (DDLPSs) display a histomorphological spectrum with their several diagnostic mimics. Murine double minute 2(MDM2) gene amplification characterizes ALT/WDLPS and DDLPS. Presently, there is no documented study from our subcontinent on the validation of MDM2 gene testing in these tumors. Material and Methods: Twenty-eight cases, diagnosed as ALT/WDLPS (n = 5) and DDLPSs (n = 23), along with 10 other tumors were tested for MDM2 gene amplification, using fluorescence in situ hybridization (FISH) on tissue microarrays (TMAs). Fourteen cases, diagnosed as ALT/WDLPS and DDLPS, along with 49 other tumors were tested for MDM2 immunostaining. Twenty tumors were tested for p16INK4a immunostaining. Results: FISH was interpretable in 25 (89.2%) cases. Among the 20 cases diagnosed as DDLPSs, 19 displayed MDM2 gene amplification. Among the 5 cases diagnosed as ALT/WDLPS, four showed MDM2 gene amplification. Finally, 19 cases were confirmed as DDLPS and 4 as ALT/WDLPS. Furthermore, 7/19 cases confirmed as DDLPS and all 4 cases as ALT/WDLPS tested for MDM2 immunostaining, displayed its diffuse immunoexpression, while a single case of DDLPS showed its focal immunostaining. None of the 49 control cases displayed diffuse MDM2 immunoexpression. ALL 16 DDLPSs and 4 cases of ALT/WDLPS displayed p16INK4a immunostaining. The sensitivity for diffuse MDM2 immunostaining was 87.5% in cases of DDLPS, 100% in ALT/WDLPS, and specificity was 100%. The sensitivity for MDM2 gene amplification was 94.7% in cases of DDLPS and 100% in cases of ALT/WDLPS. The sensitivity for p16INK4a was 100%. Conclusion: This constitutes the first sizable study on MDM2 testing in ALT/WDLPS and DDLPS from our subcontinent using TMAs. MDM2 gene amplification testing continues as the diagnostic gold standard for ALTs/WDLPSs and DDLPSs and is useful in cases of diagnostic dilemmas. Diffuse MDM2 (IF2 clone) and p16INK4a immunostaining, together seem useful for triaging cases for FISH.
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Resumen ANTECEDENTES: Los liposarcomas son un tipo de tumor de partes blandas. El subtipo desdiferenciado es una de las variantes más comunes junto con el liposarcoma bien diferenciado. CASO CLÍNICO: Paciente de 68 años que acudió a consulta por aparición de una masa en la zona vaginal que clínicamente se diagnosticó como hernia inguinal directa. Se procedió a la resección quirúrgica de la masa. El reporte histopatológico fue de miofibrolipoma, sin atipias y con bordes libres de neoplasia. Tres años después, la paciente retornó a la consulta por reaparición de la masa y se negó a una nueva biopsia. Posteriormente, en el 2020, la paciente aceptó la resección de la masa; el reporte histopatológico fue: liposarcoma desdiferenciado con diferenciación lipoblástica homóloga a liposarcoma pleomórfico. CONCLUSIÓN: Los estudios histopatológicos son necesarios para la confirmación del diagnóstico y la resección quirúrgica es el tratamiento de elección.
Abstract BACKGROUND: Liposarcomas are a type of soft tissue tumor. The dedifferentiated subtype is one of the most common variants along with well-differentiated liposarcoma. CLINICAL CASE: A 68-year-old female patient presented for consultation due to the appearance of a mass in the vaginal area which was clinically diagnosed as a direct inguinal hernia. Surgical resection of the mass was performed. The histopathological report was myofibrolipoma, without atypia and with borders free of neoplasia. Three years later, the patient returned to the office for recurrence of the mass and refused a new biopsy. Subsequently, in 2020, the patient accepted the resection of the mass; the histopathologic report was: dedifferentiated liposarcoma with lipoblastic differentiation homologous to pleomorphic liposarcoma. CONCLUSION: Histopathological studies are necessary for confirmation of the diagnosis and surgical resection is the treatment of choice.
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@#<p style="text-align: justify;">An adrenal mass can be a diagnostic challenge as it is not easy to differentiate the adrenal glands from other adrenal pseudotumours with only radio-imaging. We report a 28-year-old patient who was diagnosed radiologically as an adrenal cortical carcinoma after he presented with abdominal pain and fullness. Biochemically, he demonstrated secondary hyperaldosteronism. Intra-operatively there was a huge mass, inferior to a normal right adrenal, which was histopathologically proven to be a dedifferentiated liposarcoma.</p>
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Objective@#To investigate the imaging appearance of CT and MRI in retroperitoneal dedifferentiated liposarcoma (DDL) based on pathological findings.@*Methods@#Twelve patients with retroperitoneal DDL (13 lesions) who were surgically and pathologically confirmed were retrospectively collected in the Cancer Hospital of Chinese Academy of Medical Sciences. The correlation of CT and MRI features with histopathologic findings was analyzed.@*Results@#The CT and MRI images of retroperitoneal DDLs were large, heterogeneous soft-tissue masses, mostly lobulated (30.8%, 4/13) or multinodular (46.2%, 6/13), invading adjacent anatomic structures (46.2%, 6/13). The lesions contained different proportions of fatty and non-fatty components, and usually with clear boundaries. The CT images of dedifferentiated components showed non-fatty masses of soft tissue density or mixed density, among which ground-glass nodules may be related to mucinous components. Occasionally calcification or ossification was seen (45.5%, 5/11). The contrast-enhanced CT and MRI images of non-fatty components commonly showed intense heterogeneous enhancement (84.6%, 11/13), central cystic changes and necrosis (61.5%, 8/13), pathologically corresponding to multiple types of soft tissue sarcomas without significant specificity. The well-differentiated components were fatty masses with irregular fibrous septa or soft tissue nodules, which is pathologically corresponding to well differentiated liposarcoma. Lymph node or distant metastasis was rare.@*Conclusions@#The imaging manifestations of retroperitoneal DDLs are diverse and closely related to the proportion and distribution of different components. CT, MRI and contrast-enhanced imaging has a certain diagnostic value for retroperitoneal DDLs.
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Objective To investigate the CT findings of primary abdominal dedifferentiated liposarcoma(DDL),and to improve the diagnostic accuracy.Methods CT images of 23 cases with primary abdominal DDL confirmed by pathologically were analyzed retrospectively,and the CT findings were compared with pathological results.Results The masses in 20 cases out of 23 cases were located in retroperitoneal region,2 in abdominal cavity and 1 in the pelvic extraperitoneal space.The mean diameter of the masses was 26.5 cm.CT showed the fatty and non-fatty regions in the masses.In 2 1 cases,the non-fatty region was manifested as a single mass and was located at the edge or on the surface of fatty region.In 2 cases,the non-fatty region was manifested as multiple well-defined masses and was located within the fatty region.In 2 1 cases,the fatty region showed misty density with strip and reticular septa.In 2 other cases,the fatty region showed slightly low density,mingled with some fat tissue density,which showed no enhancement with mild enhanced internal septa.In 20 cases,the non-fatty region showed soft tissue density,with mild to moderate heterogeneous enhancement.In 3 other cases,the density of non-fatty region varied between liquid and soft tissue with patchy enhancement on delayed phase.In addition,stippled calcification was found in 4 cases.Conclusion The primary abdominal DDL has relatively specific CT findings,which different dedifferentiated components pro-duce different CT characterizes.Finding well-differentiated fat components around non-fatty mass may help to improve the accuracy of diagnosis and reduce the misdiagnosis.
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Background: De-differentiated liposarcomas (DDLSs) are uncommon tumors with a wide histopathological spectrum. Materials and Methods: Over an 8-year period (2003-2011), 25 DDLSs, after review, were critically analyzed for histopathological features. Results: Twenty-five tumors, in 14 men and 11 women (M: F = 1.2:1), with age range of 22-88 years (mean, 53.9), occurred in retroperitoneum (14) (56%), thigh (3) (12%), paratesticular region (3) (12%), chest wall (2) (8%), leg (1) (4%), shoulder (1) (4%), and groin (1) (4%). Tumor size (21 tumors) varied from 7.5 to 25 cm (mean, 17.5). Histopathologically, DD component was high grade in 19 (76%) and low grade in 6 (24%) tumors. Whereas the most common WD component was adipocytic type; the most common DD component was pleomorphic sarcomatous (13) (52%), followed by myxofibrosarcomatous (MFS)-type (6) (24%). Low-grade DD components included MFS (2), fibrosarcoma (2), myogenic/myofibroblastic type (1), and IMFT-type (1). Three tumors displayed meningothelial-like whorls and metaplastic bone formation. Heterologous elements, noted in 11 (44%) tumors, included bone (8) and rhabdomyoblastic differentiation (2). Two tumors displayed homologous differentiation, reinforced with MDM2 staining. S100-P was diffusely positive in WD components (5/7) and focally in DD components (2/9). All patients were treated with surgery, including 10, who underwent adjuvant radiotherapy. Outcomes (16 patients, 64%), over 1-48 months included 10 patients free of disease, 4 died of disease, and 2 patients alive with disease. Conclusions: This study forms the largest documentation of DDLSs, including its wide histopathological spectrum, from our country. Rare cases overlap with pleomorphic liposarcoma. S100-P and MDM2 are useful in substantiating adipocytic differentiation, especially in selected cases. Analysis of adequate tumor sections is vital for correct identification of a DDLS. Surgical excision with adjuvant RT forms optimal treatment.
Subject(s)
Adult , Aged , Aged, 80 and over , Diagnosis, Differential , Female , Histocytochemistry , Humans , Immunohistochemistry , India , Liposarcoma/diagnosis , Liposarcoma/pathology , Male , Middle Aged , Proto-Oncogene Proteins c-mdm2/analysis , S100 Proteins/analysis , Tertiary Care CentersABSTRACT
A dedifferentiated liposarcoma of the retro-peritoneum of a 45-year-old female is described in this case report. It showed dedifferentiation into a histological low-grade type and thus defies the traditional definition of dedifferentiated liposarcoma. The excised specimen was a huge, multi-nodular encapsulated mass surrounding the kidney and infiltrating into it. The mass showed a dedifferentiated focus different in color from the surrounding tumor and containing areas of necrosis and hemorrhage. The sections from the dedifferentiated part of the tumor appeared predominantly as benign spindle cell component on histology but the tumor was infiltrating into the kidney. Hence, a close and long-term follow-up is expected in such cases though they look benign. In the retro-peritoneum, a lipoma-like well-differentiated liposarcoma with spindle cell component, like the present tumor, which shows dedifferentiation, should not be overlooked.
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Dedifferentiated liposarcoma is a variant of liposarcoma, and this is characterized by the coexistence of well-differentiated liposarcoma with areas of poorly differentiated, non-lipogenic tumor and this is also known to be associated with more aggressive behavior. Dedifferentiated liposarcoma occurs principally in the retroperitoneum or the deep soft tissue of limbs, but it can also occur in subcutaneous locations. We report here on a peculiar case of dedifferentiated liposarcoma that developed in the dermis, which is an exceedingly rare location for this type of tumor. The occurrence of this tumor in the dermis made it easy to surgically remove and monitor for recurrence, and we expect this patient to have a better prognosis than that of a patient with dedifferentiated liposarcoma located in the retroperitoneum or deep soft tissue.
Subject(s)
Humans , Dermis , Extremities , Liposarcoma , Organothiophosphorus Compounds , Prognosis , Recurrence , SkinABSTRACT
A liposarcoma is the most common soft tissue sarcoma in adults with an incidence of betwen 9.8 and 16% of all soft tissue sarcomas. Among the various histological types, a dedifferentiated liposarcoma has rarely been reported. We experienced a case of a giant 7 kg dedifferentiated liposarcoma arising in the mesentery of a 57-year old male patient. Abdominal computed tomography showed a huge lipoid mass occupying the whole abdomen. A surgical excision was carried out. Histologically, the tumor was composed of a well-differentiated liposarcomatous area and a leiomyosarcoma-like dedifferentiated area. The authors reviewed the prognostic factors and treatments for a liposarcoma.
Subject(s)
Adult , Humans , Male , Abdomen , Incidence , Liposarcoma , Mesentery , SarcomaABSTRACT
A liposarcoma is the most common soft tissue sarcoma in adults with an incidence of betwen 9.8 and 16% of all soft tissue sarcomas. Among the various histological types, a dedifferentiated liposarcoma has rarely been reported. We experienced a case of a giant 7 kg dedifferentiated liposarcoma arising in the mesentery of a 57-year old male patient. Abdominal computed tomography showed a huge lipoid mass occupying the whole abdomen. A surgical excision was carried out. Histologically, the tumor was composed of a well-differentiated liposarcomatous area and a leiomyosarcoma-like dedifferentiated area. The authors reviewed the prognostic factors and treatments for a liposarcoma.
Subject(s)
Adult , Humans , Male , Abdomen , Incidence , Liposarcoma , Mesentery , SarcomaABSTRACT
We report a case of dedifferentiated liposarcoma of retroperitoneum as a recurrent form in a 41 year old male. The patient received a extirpation for retroperitoneal mass and diagnosed as myxoid liosarcoma 4 years ago. The patient experienced 3 recurrences over a period of 4 years and diagnosed as myxoid liposarcoma in the second, third recurrence also. Histologically, the mass was composed of several clearly distinct elements : well differentiated liposarcoma, myxoid liposarcoma, myxoid malignant fibrous histiocytoma, poorly differntiated sarcoma, and fibrosarcoma. Immunohistochemically, S-100 protein was expressed in the area of spindle cell sarcoma, well differentiated liposarcoma, and malignant fibrous histiocytoma but alpha-1-antichymotrypsin was only expressed in the area of myxoid malignant fibrous histiocytoma.