Recently characterized soft tissue tumors that bring biologic insight

Fletcher, Christopher D.�M.

doi:10.1038/modpathol.2013.172

Cited by 27 publications

(8 citation statements)

References 56 publications

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“…The glycogen-rich clear cells are periodic acid-Schiff stain (PAS) positive and diastase sensitive. Immunohistochemistry demonstrates co-expression of smooth muscle and melanocytic markers including SMA, HMB-45, and HMSA-1, as well as melan-A in greater than 50% of cases (25,28,30). Epithelial markers are not expressed (25,28).…”

Section: Perivascular Epithelioid Cell Tumormentioning

confidence: 96%

Nonepithelial Neoplasms of the Pancreas: Radiologic-Pathologic Correlation, Part 1—Benign Tumors:From the Radiologic Pathology Archives

Manning¹,

Srivastava²,

Paal³

et al. 2016

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Solid and cystic pancreatic neoplasms are being recognized more frequently with increasing utilization and spatial resolution of modern imaging techniques. In addition to the more common primary pancreatic solid (ductal adenocarcinoma) and cystic neoplasms of epithelial origin, nonepithelial neoplasms of the pancreas may appear as well-defined solid or cystic neoplasms. Most of these lesions have characteristic imaging features, such as a well-defined border, which allows differentiation from ductal adenocarcinoma. Solid masses include neurofibroma, ganglioneuroma, leiomyoma, lipoma, and perivascular epithelioid cell tumor (PEComa). Schwannomas and desmoid tumors can be solid or cystic. Cystic tumors include mature cystic teratoma and lymphangioma. Lipoma, PEComa, and mature cystic teratoma can contain fat, and ganglioneuroma and mature cystic teratoma may contain calcification. Although these unusual benign neoplasms are rare, the radiologist should at least consider them in the differential diagnosis of well-defined lesions of the pancreas. The goal of this comprehensive review is to improve understanding of these rare primary pancreatic mesenchymal tumors.

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Section: Perivascular Epithelioid Cell Tumormentioning

confidence: 96%

Nonepithelial Neoplasms of the Pancreas: Radiologic-Pathologic Correlation, Part 1—Benign Tumors:From the Radiologic Pathology Archives

Manning¹,

Srivastava²,

Paal³

et al. 2016

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“…Scattered inflammatory cells are usually present, and osteoclast‐like giant cells may also be seen. Mitoses and necrosis are rare . The immunohistochemical profile of HFLT is non‐specific, and IHC plays a limited, if any, role in diagnosis; the spindle cell component is positive for CD34 and negative for SMA, desmin, and S100.…”

Section: Newly Described Cutaneous Mesenchymal Neoplasmsmentioning

confidence: 99%

Advances in the clinicopathological and molecular classification of cutaneous mesenchymal neoplasms

Costigan

Doyle

2016

Histopathology

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In recent years, there have been several important refinements in the classification of cutaneous mesenchymal neoplasms, including the description of new tumour types, along with the identification of novel and recurrent molecular genetic findings. In addition to providing new insights into tumour biology, many of these advances have had significant clinical consequences with regard to diagnostics, management, and prognostication. Newly described entities include pseudomyogenic haemangioendothelioma, haemosiderotic fibrolipomatous tumour, and fibroblastic connective tissue naevus, which are reviewed in the context of the principal differential diagnoses and significant clinical implications. Genetic characterization of several soft tissue tumour types that occur in the skin has resulted in the identification of diagnostically useful markers: ALK gene rearrangement with corresponding ALK protein expression by immunohistochemistry in epithelioid fibrous histiocytoma; the WWTR1-CAMTA1 fusion gene with CAMTA1 protein expression in epithelioid haemangioendothelioma; MYC amplification and overexpression in radiation-associated angiosarcoma; and EWSR1 gene rearrangement in cutaneous myoepithelial tumours. Finally, the classification of intradermal smooth muscle tumours and unclassified/pleomorphic dermal sarcoma has been refined, resulting in both improved classification and improved prognostication. Many of the tumour types listed above are encountered not only by specialist dermatopathologists, but also by practising general surgical pathologists, and this review should therefore provide a widely applicable update on the histological and molecular classification of cutaneous mesenchymal neoplasms, along with the appropriate use of ancillary diagnostic tests, in particular immunohistochemistry, in the evaluation of such lesions and their histological mimics.

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“…Perivascular epithelioid cell tumors (PEComas) constitute a family of mesenchymal tumors composed of cells co‐expressing melanocytic and myogenic markers, with the cells showing focal association with the walls of small blood vessels . Initially proposed by Bonetti et al, the PEComa family now includes the entities angiomyolipoma (AML), clear cell (“sugar”) tumor of the lung (CCST), and lymphangioleiomyomatosis (LAM) . In addition, histopathologically and immunohistochemically similar non‐AML, non‐CCST, non‐LAM lesions in various other sites are simply called PEComas .…”

Section: Introductionmentioning

confidence: 99%

“…PEComas have a wide range of morphologic characteristics, from nests of epithelioid cells to spindled cells to very pleomorphic cells, which may pose difficulties in diagnosis. Sometimes, the stroma may become very collagenized, such as in a histopathologic variant called sclerosing PEComa .…”

Section: Introductionmentioning

confidence: 99%

Cutaneous “fibroma‐like” perivascular epithelioid cell tumor: A case report and review of literature

et al. 2020

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Perivascular epithelioid cell tumors (PEComas) are a group of lesions sharing the common features of co‐expression of melanocytic and myogenic markers, with focal association of the cells with vascular walls. The PEComa group exhibits a wide range of morphologies. A “fibroma‐like” variant of PEComa has been recently described. The case reported herein is that of an infant with tuberous sclerosis complex (TSC) presenting with a lip mass. Excisional biopsy showed a moderately cellular tumor composed of spindled to stellate cells embedded within a collagenized stroma. The cells showed focal perivascular accumulation and positivity for both melanocytic (HMB‐45) and myogenic (desmin) markers. This is the fifth reported case of “fibroma‐like” PEComa in literature and the youngest patient to date. All of the “fibroma‐like” PEComas were found in patients with tuberous sclerosis—hence, the diagnosis of this entity should prompt a workup for TSC; conversely, a fibroma‐like lesion in a patient with TSC or with TSC‐related conditions should be evaluated using melanocytic and myogenic markers. Melanocytic and myogenic markers are also useful in differentiating “fibroma‐like” PEComa from other differential diagnoses such as fibroma and benign fibrous histiocytoma.

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Recently characterized soft tissue tumors that bring biologic insight

Cited by 27 publications

References 56 publications

Nonepithelial Neoplasms of the Pancreas: Radiologic-Pathologic Correlation, Part 1—Benign Tumors:From the Radiologic Pathology Archives

Nonepithelial Neoplasms of the Pancreas: Radiologic-Pathologic Correlation, Part 1—Benign Tumors:From the Radiologic Pathology Archives

Advances in the clinicopathological and molecular classification of cutaneous mesenchymal neoplasms

Cutaneous “fibroma‐like” perivascular epithelioid cell tumor: A case report and review of literature

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