PEC and Sugar

Bonetti, Franco; Pea, Maurizio; Martignoni, Guido; Zamboni, Giuseppe

doi:10.1097/00000478-199203000-00013

Cited by 388 publications

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“…The descriptive term perivascular epithelioid cell (PEC) was proposed by Bonetti et al [1,20] to describe a novel cell type demonstrating HMB45 immunoreactivity and the presence of premelanosomes ultrastructurally, and which morphologically resembled the CCST of lung, AML of the kidney and liver, and LAM [21][22][23][24][25][26]. They hypothesized that the PEC may originate from the walls of blood vessels, given the perivascular association and the fact that there is no known normal cellular counterpart to the PEC.…”

Section: Discussionmentioning

confidence: 99%

“…The term perivascular epithelioid cell tumor (PEComa), first coined by Bonetti et al [1], is currently defined as a mesenchymal tumor composed of distinctive perivascular myoid cells. PEComas are a family of neoplastic lesions that share overlapping morphology, immunohistochemistry, and ultrastructure and includes angiomyolipoma (AML), lymphangioleiomyomatosis (LAM), clear cell ''sugar'' tumor (CCST) of the lung, as well as similar tumors occurring in a variety of visceral, cutaneous, and soft tissue sites throughout the body.…”

Section: Introductionmentioning

confidence: 99%

“…PEComas are a family of neoplastic lesions that share overlapping morphology, immunohistochemistry, and ultrastructure and includes angiomyolipoma (AML), lymphangioleiomyomatosis (LAM), clear cell ''sugar'' tumor (CCST) of the lung, as well as similar tumors occurring in a variety of visceral, cutaneous, and soft tissue sites throughout the body. PEComas may be associated with the tuberous sclerosis complex (TSC), particularly AML and LAM [2][3][4], or represent a forme fruste of the disease [1]. Notably, non-AML and non-LAM PEComas are only rarely associated with TSC.…”

Section: Introductionmentioning

confidence: 99%

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Perivascular Epithelioid Cell Tumors (PEComas) of the Head and Neck: Report of Three Cases and Review of the Literature

Bandhlish

Barnes

Rabban

et al. 2011

Head and Neck Pathol

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PEComas are a family of neoplastic lesions that share overlapping morphology, immunohistochemistry, and ultrastructure that include angiomyolipoma, lymphangioleiomyomatosis, clear cell ''sugar'' tumor of the lung as well as similar tumors occurring in a variety of visceral, cutaneous and soft tissue sites throughout the body. The defining histopathological features are epithelioid cells with a perivascular distribution containing clear to pale eosinophilic granular cytoplasm and a round-to-oval centrally located nucleus with an inconspicuous nucleolus. Immunohistochemically, coexpression of melanocytic (HMB-45 and/or Melan-A) and myoid markers are characteristic. In the present study, we describe three PEComas occurring in the head and neck (nasal cavity and larynx) and discuss the behavior of these distinctive tumors and review the literature of head and neck PEComas. The importance of recognizing this entity will ensure its consideration in the differential diagnosis of tumors of the head and neck with a similar morphology. The histogenesis of PEComas still remains elusive and additional cases with a prolonged follow up remain important to accurately determine the behavior of these distinctive tumors. Complete surgical excision still remains the treatment of choice for histologically benign PEComas.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Perivascular Epithelioid Cell Tumors (PEComas) of the Head and Neck: Report of Three Cases and Review of the Literature

Bandhlish

Barnes

Rabban

et al. 2011

Head and Neck Pathol

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“…The World Health Organization (WHO) defines PEComa as a "mesenchymal tumor that contains perivascular epithelioid cells presenting histological and immunohistochemical differences". While classical AML is benign, epithelioid AMLs are defined as potentially malign mesenchymal lesions for their capacity to develop local recurrence and metastasis (1,2,3). Surgical treatment may be required in AML for symptoms such as hemorrhage and pain, or for the risk of malignancy in tumors that cannot be differentiated from AML in the case of PEComa and certain variants of AML (3,4,5).…”

Section: Introductionmentioning

confidence: 99%

Perivascular Epithelioid Cell Tumor of the Kidney: A Rare Case Report

Karakoç¹,

Çelik²,

Bozkurt³

et al. 2017

jus

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IntroductionPerivascular epithelioid cell tumors (PEComas) of the kidney are a group of tumor family covering classical angiomyolipoma (AML), microscopic AML (microhamartoma), intraglomerular lesions, cystic AML, epithelioid AML, oncocytoma-like AML and lymphangiomyomatosis of the renal sinus. The World Health Organization (WHO) defines PEComa as a "mesenchymal tumor that contains perivascular epithelioid cells presenting histological and immunohistochemical differences". While classical AML is benign, epithelioid AMLs are defined as potentially malign mesenchymal lesions for their capacity to develop local recurrence and metastasis (1,2,3). Surgical treatment may be required in AML for symptoms such as hemorrhage and pain, or for the risk of malignancy in tumors that cannot be differentiated from AML in the case of PEComa and certain variants of AML (3,4,5).This case report aimed to present a patient who undergone retroperitoneoscopic partial nephrectomy due to a renal mass and diagnosed with PEComa after pathological examination. Case PresentationWe planned magnetic resonance imaging (MRI) in a 25-year-old male patient, who was admitted to our clinic due to left-sided flank pain, with no pathological finding during the examination and laboratory tests, however, with a suspicious mass lesion located at the inferior pole of the left kidney detected during ultrasonographic examination. It was decided to perform surgical excision after detection of a 4 cm tumoral lesion in the inferior pole of the left kidney on MRI (Figure 1). We performed left retroperitoneoscopic partial nephrectomy in our clinic. The total operative time was 90 minutes with the warm ischemia time Böbreğin perivasküler epiteloid hücre tümörleri nadir görülen histolojik ve immünohistokimyasal olarak farklı perivasküler epiteloid hücrelerden oluşan mezenkimal tümörlerdir. Çoğunluğu benign tümörlerdir; fakat malign olanları agresif seyredebilir ve metastaza yol açabilir. Standart tedavi yöntemi ise cerrahi eksizyondur. Olgumuzda 25 yaşında sol böbrek alt polünde 4 cm kitle saptanan ve cerrahi olarak eksize ettiğimiz hastayı sunmayı amaçladık. Anahtar Kelimeler: Böbrek tümörü, perivasküler epiteloid hücre tümörü, parsiyel nefrektomi Perivascular epithelioid cell tumors are rare mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. They are mostly benign, however, malignant tumors with aggressive behavior and distant metastasis can also occur. The standard treatment is surgical excision. Here, we report a case of a 25-year-old male with a 4 cm-mass located in the inferior pole of the left kidney, treated by surgical excision.

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“…The tumor cells are epithelioid or spindle-shaped and tend to be distributed around blood vessels (1)(2)(3). The cells uniquely show immunoactivity for melanocytic markers such as HMB-45 or myogenic markers such as α-smooth muscle actin (α-SMA) (2).…”

Section: Introductionmentioning

confidence: 99%

A Resected Perivascular Epithelioid Cell Tumor (PEComa) of the Pancreas Diagnosed Using Endoscopic Ultrasound-guided Fine-needle Aspiration

et al. 2013

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Primary perivascular epithelioid cell tumors (PEComas) of the pancreas are extremely rare. We herein report our experience with a patient who had a primary PEComa of the pancreas that was diagnosed by the preoperative histopathological examination of a biopsy specimen obtained by endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA). The patient was a 43-year-old woman whose chief complaint was abdominal pain. Imaging studies revealed a pancreatic tumor. Gastrointestinal stromal tumor (GIST), solid pseudopapillary tumor and neuroendocrine tumor were considered in the differential diagnosis. A histopathological examination of a specimen of the tumor obtained using EUS-FNA showed spindle-shaped tumor cells with enlarged nuclei and eosinophilic cytoplasm. The tumor cells proliferated in a sheet-like fashion and stained positive for the melanoma-associated antigen HMB-45. A PEComa was thus diagnosed. If an adequate tumor specimen can be obtained using EUS-FNA, immunostaining may facilitate the diagnosis of extremely rare diseases and therefore assist in deciding the treatment policy.

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PEC and Sugar

Cited by 388 publications

References 0 publications

Perivascular Epithelioid Cell Tumors (PEComas) of the Head and Neck: Report of Three Cases and Review of the Literature

Perivascular Epithelioid Cell Tumors (PEComas) of the Head and Neck: Report of Three Cases and Review of the Literature

Perivascular Epithelioid Cell Tumor of the Kidney: A Rare Case Report

A Resected Perivascular Epithelioid Cell Tumor (PEComa) of the Pancreas Diagnosed Using Endoscopic Ultrasound-guided Fine-needle Aspiration

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