Applied Immunohistochemistry & Molecular Morphology, Aug 1, 2015
Plasmablastic lymphoma is a rare, highly aggressive lymphoma characterized by large lymphoid cell... more Plasmablastic lymphoma is a rare, highly aggressive lymphoma characterized by large lymphoid cells with immunoblastic or plasmablastic features, absent expression of CD45 and CD20, positivity for CD138, and monoclonal rearrangement of the immunoglobulin heavy chain gene. It was originally reported in oral cavity in the setting of underlying human immunodeficiency viral infection but may occur also in lymph nodes or extranodal sites after transplantation and, more rarely, immunocompetent patients. Herein, we report a case of PBL presenting as an ulcerated lesion of the tongue in an HIV-negative patient, 6 years after renal transplantation. To date, only rare cases of plasmablastic lymphoma presenting after solid organ transplantation have been reported. Although a reduction of immunosuppression and an aggressive chemotherapy were performed, the patient died after a few months because of septic and cardiovascular complications.
Collecting duct carcinoma is an uncommon variant of renal cell carcinoma that usually occurs at a... more Collecting duct carcinoma is an uncommon variant of renal cell carcinoma that usually occurs at an earlier age compared to conventional renal cell carcinoma. It is characterised by an aggressive, often fatal, course. Renal cell carcinoma rarely occurs in paediatric patients, and is almost always in association with specific genetic alterations; the most common histotypes are the clear cell and chromophobe cell variants. Collecting duct carcinoma is rare, and only 8 cases have been reported in the literature. The authors describe the clinico-pathological features of a fatal collecting duct carcinoma in an 11-year-old boy.
ABSTRACT We report a case of diffuse and extreme cytoplasmic vacuolization of tumour cells in a r... more ABSTRACT We report a case of diffuse and extreme cytoplasmic vacuolization of tumour cells in a rectal adenocarcinoma after neoadjuvant treatment. A 64-year-old man with a moderately differentiated rectal adenocarcinoma, diagnosed by endoscopic rectal biopsy, underwent surgical treatment after chemoradiotherapy. Residual tumour mass was represented by foci of neoplastic cells with the morphological features of conventional type adenocarcinoma, and surprisingly, by numerous areas consisting of several giant vacuoles, variable in size, merging to form multilocular spaces separated by a rim of cell membrane with a "plant-like" appearance. Cytoplasmic vacuolization may represent a distinct form of cell death, and pathologists should carefully consider this unusual and potentially alarming morphological change among the chemoradiotherapy-induced effects on tumour mass.
Fibrous hamartoma of infancy is a soft tissue subdermal fibromatous tumour that characteristicall... more Fibrous hamartoma of infancy is a soft tissue subdermal fibromatous tumour that characteristically occurs in the first years of life. It is histologically composed of three different components that are intimately admixed: well-defined bundles of fibro-myofibroblastic spindle-shaped cells, nodular proliferations of immature-looking mesenchymal cells set in a myxoid stroma, and mature adipose tissue. A wide intralesional and interlesional cellular composition is commonly observed. Fibrous hamartoma of infancy usually arises from subcutaneous tissue of the trunk, axilla, upper extremities and inguinal region. Only rarely has fibrous hamartoma of infancy been reported in genital organs, with only one case described in the labium majus. We report a rare case of fibrous hamartoma of infancy in the labium majus of a 1-year old female child. Ultrasonography revealed the presence of a mass-like lesion involving subcutaneous tissue, with ill-defined margins. We emphasize that fibrous hamartoma of infancy should be included in the differential diagnosis of soft tissue tumour-like and tumour lesions of the vulva in children. Awareness that fibrous hamartoma of infancy occurs at this site with irregular margins is important to avoid confusion with other lesions exhibiting a more aggressive behaviour.
Hamartoma of the breast is a pseudotumoural lesion that does not usually pose diagnostic problems... more Hamartoma of the breast is a pseudotumoural lesion that does not usually pose diagnostic problems for the pathologist. Although atypical stromal cell (ASCs) can be encountered in several benign and malignant breast lesions, their occurrence in hamartoma has not been reported to date. The authors report a case of breast hamartoma containing numerous atypical mono- or multinucleated stromal cells within the fibro-fatty component. This unusual feature raised differential diagnostic problems with pleomorphic lipoma, well-differentiated liposarcoma and malignant phylloid tumour with a lipomatous heterologous component. Immunohistochemistry, showing positivity to vimentin and CD34, revealed that ASCs are fibroblastic in nature, and thus are likely to represent a morphological variant of the fibroblasts of the native mammary stroma.
Atypical and epithelioid cell variants of dermatofibroma may represent a potential diagnostic pit... more Atypical and epithelioid cell variants of dermatofibroma may represent a potential diagnostic pitfall. Only rarely atypical dermatofibroma may show focal epithelioid cell features. We herein report a rare case of dermatofibroma composed of a predominant (> 90%) epithelioid/deciduoid-like cell component, in which rare multinucleated bizarre cells and atypical mitoses were additional findings. Tumour was classified as "atypical dermatofibroma with predominant epithelioid/deciduoid-like cell component". The coexistence of at least two different variants, i.e. epithelioid and atypical variants, in the same dermatofibroma suggests that dermal fibrohistiocytic tumours belong to a continuous morphological spectrum. Accordingly, the morphological variants of dermatofibroma should be regarded as variations on a common basic theme. Differential diagnosis with other epitheliod cell dermal tumour- and tumour-like lesions is discussed.
BackgroundPrimary intracranial ependymomas (IE) are rare brain tumors rarely metastasizing outsid... more BackgroundPrimary intracranial ependymomas (IE) are rare brain tumors rarely metastasizing outside the central nervous system. We systematically reviewed the literature on extra-neural metastases from primary IEs.MethodsPubMed, Scopus, Web-of-Science, and Cochrane were searched following the PRISMA guidelines to include studies of extra-neural metastases from primary IEs. Clinical features, management strategies, and survival were analyzed.ResultsWe collected 48 patients from 43 studies. Median age was 13 years (range, 2-65). Primary IEs were frequently located in the parietal (22.9%) and frontal (16.7%) lobes, and mostly treated with resection (95.8%) and/or radiotherapy (62.5%). Most IEs were of grade-III (79.1%), and few of grade-I (6.3%) or grade-II (14.6%). 45 patients experienced intracranial recurrences, mostly treated with resection (86.7%), radiotherapy (60%), and/or chemotherapy (24.4%). Median time-interval from primary IEs was 28 months (range, 0-140). Most extra-neural ...
Applied Immunohistochemistry & Molecular Morphology, Aug 1, 2015
Plasmablastic lymphoma is a rare, highly aggressive lymphoma characterized by large lymphoid cell... more Plasmablastic lymphoma is a rare, highly aggressive lymphoma characterized by large lymphoid cells with immunoblastic or plasmablastic features, absent expression of CD45 and CD20, positivity for CD138, and monoclonal rearrangement of the immunoglobulin heavy chain gene. It was originally reported in oral cavity in the setting of underlying human immunodeficiency viral infection but may occur also in lymph nodes or extranodal sites after transplantation and, more rarely, immunocompetent patients. Herein, we report a case of PBL presenting as an ulcerated lesion of the tongue in an HIV-negative patient, 6 years after renal transplantation. To date, only rare cases of plasmablastic lymphoma presenting after solid organ transplantation have been reported. Although a reduction of immunosuppression and an aggressive chemotherapy were performed, the patient died after a few months because of septic and cardiovascular complications.
Collecting duct carcinoma is an uncommon variant of renal cell carcinoma that usually occurs at a... more Collecting duct carcinoma is an uncommon variant of renal cell carcinoma that usually occurs at an earlier age compared to conventional renal cell carcinoma. It is characterised by an aggressive, often fatal, course. Renal cell carcinoma rarely occurs in paediatric patients, and is almost always in association with specific genetic alterations; the most common histotypes are the clear cell and chromophobe cell variants. Collecting duct carcinoma is rare, and only 8 cases have been reported in the literature. The authors describe the clinico-pathological features of a fatal collecting duct carcinoma in an 11-year-old boy.
ABSTRACT We report a case of diffuse and extreme cytoplasmic vacuolization of tumour cells in a r... more ABSTRACT We report a case of diffuse and extreme cytoplasmic vacuolization of tumour cells in a rectal adenocarcinoma after neoadjuvant treatment. A 64-year-old man with a moderately differentiated rectal adenocarcinoma, diagnosed by endoscopic rectal biopsy, underwent surgical treatment after chemoradiotherapy. Residual tumour mass was represented by foci of neoplastic cells with the morphological features of conventional type adenocarcinoma, and surprisingly, by numerous areas consisting of several giant vacuoles, variable in size, merging to form multilocular spaces separated by a rim of cell membrane with a "plant-like" appearance. Cytoplasmic vacuolization may represent a distinct form of cell death, and pathologists should carefully consider this unusual and potentially alarming morphological change among the chemoradiotherapy-induced effects on tumour mass.
Fibrous hamartoma of infancy is a soft tissue subdermal fibromatous tumour that characteristicall... more Fibrous hamartoma of infancy is a soft tissue subdermal fibromatous tumour that characteristically occurs in the first years of life. It is histologically composed of three different components that are intimately admixed: well-defined bundles of fibro-myofibroblastic spindle-shaped cells, nodular proliferations of immature-looking mesenchymal cells set in a myxoid stroma, and mature adipose tissue. A wide intralesional and interlesional cellular composition is commonly observed. Fibrous hamartoma of infancy usually arises from subcutaneous tissue of the trunk, axilla, upper extremities and inguinal region. Only rarely has fibrous hamartoma of infancy been reported in genital organs, with only one case described in the labium majus. We report a rare case of fibrous hamartoma of infancy in the labium majus of a 1-year old female child. Ultrasonography revealed the presence of a mass-like lesion involving subcutaneous tissue, with ill-defined margins. We emphasize that fibrous hamartoma of infancy should be included in the differential diagnosis of soft tissue tumour-like and tumour lesions of the vulva in children. Awareness that fibrous hamartoma of infancy occurs at this site with irregular margins is important to avoid confusion with other lesions exhibiting a more aggressive behaviour.
Hamartoma of the breast is a pseudotumoural lesion that does not usually pose diagnostic problems... more Hamartoma of the breast is a pseudotumoural lesion that does not usually pose diagnostic problems for the pathologist. Although atypical stromal cell (ASCs) can be encountered in several benign and malignant breast lesions, their occurrence in hamartoma has not been reported to date. The authors report a case of breast hamartoma containing numerous atypical mono- or multinucleated stromal cells within the fibro-fatty component. This unusual feature raised differential diagnostic problems with pleomorphic lipoma, well-differentiated liposarcoma and malignant phylloid tumour with a lipomatous heterologous component. Immunohistochemistry, showing positivity to vimentin and CD34, revealed that ASCs are fibroblastic in nature, and thus are likely to represent a morphological variant of the fibroblasts of the native mammary stroma.
Atypical and epithelioid cell variants of dermatofibroma may represent a potential diagnostic pit... more Atypical and epithelioid cell variants of dermatofibroma may represent a potential diagnostic pitfall. Only rarely atypical dermatofibroma may show focal epithelioid cell features. We herein report a rare case of dermatofibroma composed of a predominant (> 90%) epithelioid/deciduoid-like cell component, in which rare multinucleated bizarre cells and atypical mitoses were additional findings. Tumour was classified as "atypical dermatofibroma with predominant epithelioid/deciduoid-like cell component". The coexistence of at least two different variants, i.e. epithelioid and atypical variants, in the same dermatofibroma suggests that dermal fibrohistiocytic tumours belong to a continuous morphological spectrum. Accordingly, the morphological variants of dermatofibroma should be regarded as variations on a common basic theme. Differential diagnosis with other epitheliod cell dermal tumour- and tumour-like lesions is discussed.
BackgroundPrimary intracranial ependymomas (IE) are rare brain tumors rarely metastasizing outsid... more BackgroundPrimary intracranial ependymomas (IE) are rare brain tumors rarely metastasizing outside the central nervous system. We systematically reviewed the literature on extra-neural metastases from primary IEs.MethodsPubMed, Scopus, Web-of-Science, and Cochrane were searched following the PRISMA guidelines to include studies of extra-neural metastases from primary IEs. Clinical features, management strategies, and survival were analyzed.ResultsWe collected 48 patients from 43 studies. Median age was 13 years (range, 2-65). Primary IEs were frequently located in the parietal (22.9%) and frontal (16.7%) lobes, and mostly treated with resection (95.8%) and/or radiotherapy (62.5%). Most IEs were of grade-III (79.1%), and few of grade-I (6.3%) or grade-II (14.6%). 45 patients experienced intracranial recurrences, mostly treated with resection (86.7%), radiotherapy (60%), and/or chemotherapy (24.4%). Median time-interval from primary IEs was 28 months (range, 0-140). Most extra-neural ...
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