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Angiofibroma of soft tissue
Pathology (2014) 46(S2), pp. S5–S44
Main Program
Autopsy Pathology: SY01-1
Bone and Soft Tissue Pathology: SC02-3
WE STILL HAVE MANY THINGS TO LEARN FROM AUTOPSY
ANGIOFIBROMA OF SOFT TISSUE
Koichi Honma Department of Pathology, Dokkoy Medical University, Japan Autopsy not only provides a final diagnosis for a) sudden/acute death or rapidly deteriorating illnesses, where a sufficient clinical investigation was not available, or b) difficult cases, where biopsy and/or surgical pathology could not establish the diagnosis, but also: 1) helps us grow new insights/viewpoints for already well-known disorders, making clear of their intrinsic problems; 2) provides a correct understanding of a given pathologic finding, which had once been described in the old archival literature but may since have been neglected/overlooked by modern textbooks; 3) establishes a new disease entity by providing full-blown pathologic descriptions especially regarding its end stage, and taking a whole range of evolution of the disorder into account; 4) provides comprehensive pathologic descriptions of an altered nature, i.e., consequences of newly explored therapeutic modalities; and 5) helps us develop our own hands-on pathology for a sophisticated understanding of the human body as a whole.
Bone and Soft Tissue Pathology: SC02-1 CARTILAGE TUMORS 1
Yoshinao Oda Department of Anatomic Pathology, Kyushu University, Fukuoka, Japan Angiofibroma of soft tissue arising in the thigh of a 54-year-old female will be demonstrated. Grossly, well circumscribed tumor showed grayish white color with focal gelatinous appearance on its cut surface. Histologically, the tumor is composed of oval to short spindle-shaped cells arranged in patternless pattern, accompanied by slit-like or hemangiopericytomatous vessels, fibrocollagenous or myxoid stroma. Immunohistochemically, tumor cells are negative for CD34, myogenic, myoepithelial or epithelial markers. INI1 expression is preserved. Characteristic AHRRNCOA2 fusion transcript was detected by RT-PCR. This tumor is characterized by two components: uniformly proliferating bland spindle shaped cells in fibro-collagenous or myxoid stroma, and prominent vascular network composed of small, branching hemangiopericytomatous, and thin walled blood vessels. In this slide seminar detailed clinicopathological features of angiofibroma of soft tissue will be demonstrated. Moreover, the differential diagnosis of this peculiar tumor including cellular angiofibroma, solitary fibrous tumor, low-grade fibromyxoid sarcoma and low-grade myxofibrosarcoma or myxoid/round cell liposarcoma will be explained.
Bone and Soft Tissue Pathology: SC02-3 2
Franco Bertoni and Patrizia Bacchini 1University of Bologna, and 2Villa Erbosa Hospital Bologna, Italy Bone-forming lesions and cartilage-forming lesions are the most common primary tumors of the bone. They are classified according to their biological potential as benign (chondroblastoma, chondromyxoid fibroma, enchondroma, osteochondroma, periosteal chondroma) and malignant (chondrosarcoma, clear cell chondorsarcoma, mesenchymal chondrosarcoma, dedifferentiated chondrosarcoma) and as to their anatomic location (central/intramedullary, or peripheral and periosteal). Radiographic and histologic features are the cornerstone reaching a diagnosis. Problems: poor inter-observer reproducibility in: 1) Transformation and distinction between benign (enchondroma) and malignant (low grade chondrosarcoma). 2) Different results in the treatment of low grade chondrosarcoma according to the anatomic locations (axial vs appendicular skeleton). Genetic findings: molecular markers increasingly used in the diagnosis of cartilaginous neoplasms: 1) IDH 1–2 mutations are frequent events in cartilaginous neoplasms (negative in osteosarcomas). 2) Mutation of COL2A1 in chondrosarcomas.
Print ISSN 0031-3025/Online ISSN 1465-3931
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UNUSUAL SOFT TISSUE TUMORS Yoshinao Oda Department of Anatomic Pathology, Kyushu University, Fukuoka, Japan In this session, the detailed clinicopathological and genetic findings, and the differential diagnosis of the following five unusual soft tissue tumors will be demonstrated. 1) Inflammatory myofibroblastic tumor occurs in abdominal soft tissue, GI tract, and lung of children and young adult. The prediction of biological behavior is difficult. Cytogenetic study demonstrates ALK rearrangement in more than half cases. 2) Pseudomyogenic hemangioendothelioma is rarely metastasizing endothelial neoplasm. It was initially named as epithelioid sarcoma-like hemangioendothelioma and mimicking myoid tumor or epithelioid sarcoma. FLI1 and ERG are useful markers for this tumor. 3) Myoepithelioma predominantly involve lower or upper limb of young to middle aged adult. This intermediate tumor have broad spectrum of biological behavior, according to cytological atypia. 45% of the tumor shows EWSR1 rearrangement. 4) Malignant rhabdoid tumor is very aggressive tumor and it arises in the deep axial location, such as neck and
2014 Royal College of Pathologists of Australasia
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
Main Program
Autopsy Pathology: SY01-1
Bone and Soft Tissue Pathology: SC02-3
WE STILL HAVE MANY THINGS TO LEARN FROM AUTOPSY
ANGIOFIBROMA OF SOFT TISSUE
Koichi Honma Department of Pathology, Dokkoy Medical University, Japan Autopsy not only provides a final diagnosis for a) sudden/acute death or rapidly deteriorating illnesses, where a sufficient clinical investigation was not available, or b) difficult cases, where biopsy and/or surgical pathology could not establish the diagnosis, but also: 1) helps us grow new insights/viewpoints for already well-known disorders, making clear of their intrinsic problems; 2) provides a correct understanding of a given pathologic finding, which had once been described in the old archival literature but may since have been neglected/overlooked by modern textbooks; 3) establishes a new disease entity by providing full-blown pathologic descriptions especially regarding its end stage, and taking a whole range of evolution of the disorder into account; 4) provides comprehensive pathologic descriptions of an altered nature, i.e., consequences of newly explored therapeutic modalities; and 5) helps us develop our own hands-on pathology for a sophisticated understanding of the human body as a whole.
Bone and Soft Tissue Pathology: SC02-1 CARTILAGE TUMORS 1
Yoshinao Oda Department of Anatomic Pathology, Kyushu University, Fukuoka, Japan Angiofibroma of soft tissue arising in the thigh of a 54-year-old female will be demonstrated. Grossly, well circumscribed tumor showed grayish white color with focal gelatinous appearance on its cut surface. Histologically, the tumor is composed of oval to short spindle-shaped cells arranged in patternless pattern, accompanied by slit-like or hemangiopericytomatous vessels, fibrocollagenous or myxoid stroma. Immunohistochemically, tumor cells are negative for CD34, myogenic, myoepithelial or epithelial markers. INI1 expression is preserved. Characteristic AHRRNCOA2 fusion transcript was detected by RT-PCR. This tumor is characterized by two components: uniformly proliferating bland spindle shaped cells in fibro-collagenous or myxoid stroma, and prominent vascular network composed of small, branching hemangiopericytomatous, and thin walled blood vessels. In this slide seminar detailed clinicopathological features of angiofibroma of soft tissue will be demonstrated. Moreover, the differential diagnosis of this peculiar tumor including cellular angiofibroma, solitary fibrous tumor, low-grade fibromyxoid sarcoma and low-grade myxofibrosarcoma or myxoid/round cell liposarcoma will be explained.
Bone and Soft Tissue Pathology: SC02-3 2
Franco Bertoni and Patrizia Bacchini 1University of Bologna, and 2Villa Erbosa Hospital Bologna, Italy Bone-forming lesions and cartilage-forming lesions are the most common primary tumors of the bone. They are classified according to their biological potential as benign (chondroblastoma, chondromyxoid fibroma, enchondroma, osteochondroma, periosteal chondroma) and malignant (chondrosarcoma, clear cell chondorsarcoma, mesenchymal chondrosarcoma, dedifferentiated chondrosarcoma) and as to their anatomic location (central/intramedullary, or peripheral and periosteal). Radiographic and histologic features are the cornerstone reaching a diagnosis. Problems: poor inter-observer reproducibility in: 1) Transformation and distinction between benign (enchondroma) and malignant (low grade chondrosarcoma). 2) Different results in the treatment of low grade chondrosarcoma according to the anatomic locations (axial vs appendicular skeleton). Genetic findings: molecular markers increasingly used in the diagnosis of cartilaginous neoplasms: 1) IDH 1–2 mutations are frequent events in cartilaginous neoplasms (negative in osteosarcomas). 2) Mutation of COL2A1 in chondrosarcomas.
Print ISSN 0031-3025/Online ISSN 1465-3931
#
UNUSUAL SOFT TISSUE TUMORS Yoshinao Oda Department of Anatomic Pathology, Kyushu University, Fukuoka, Japan In this session, the detailed clinicopathological and genetic findings, and the differential diagnosis of the following five unusual soft tissue tumors will be demonstrated. 1) Inflammatory myofibroblastic tumor occurs in abdominal soft tissue, GI tract, and lung of children and young adult. The prediction of biological behavior is difficult. Cytogenetic study demonstrates ALK rearrangement in more than half cases. 2) Pseudomyogenic hemangioendothelioma is rarely metastasizing endothelial neoplasm. It was initially named as epithelioid sarcoma-like hemangioendothelioma and mimicking myoid tumor or epithelioid sarcoma. FLI1 and ERG are useful markers for this tumor. 3) Myoepithelioma predominantly involve lower or upper limb of young to middle aged adult. This intermediate tumor have broad spectrum of biological behavior, according to cytological atypia. 45% of the tumor shows EWSR1 rearrangement. 4) Malignant rhabdoid tumor is very aggressive tumor and it arises in the deep axial location, such as neck and
2014 Royal College of Pathologists of Australasia
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.