A Case Report of Multifocal PEComa Affecting the Pancreas and Liver and a Literature Review

Sugar Tumor ◽

Head Of The Pancreas

Abstract Background: Perivascular epithelioid cell tumor (PEComa) is a rare tumor derived from soft tissues and organs. Its morphology includes several types such as angiomyolipoma, lymphangiomyomatosis and pulmonary clear-cell “sugar” tumor with similar immunophenotype. Multifocal PEComa is a rarer lesion, which would cause confusion in imaging examination and clinical management. We reported a case of multifocal angiomyolipoma in pancreas and liver. At the same time, we reviewed the literature to explore the clinicopathological characteristics and possible pathogenesis of multifocal PEComa.Case presentation: A 28-year-old woman presented with a lesion in the head of the pancreas and liver 5/6 segment respectively, initially suspected of pancreatic tumor with liver metastasis. In the liver mass resection and pancreatic mass biopsy, it was found that the two lesions had similar pathological morphology. The tumor cells were mainly epithelioid and spindle cells arranged in nests pattern and partially radially distributed around blood vessels. The tumors had no malignant manifestations in morphology. Immunohistochemical assay showed that the tumor cells expressed melanocytic and smooth muscle markers. A diagnosis of pancreatic and liver angiomyolipoma was rendered.Conclusion: Based on the case we reported here and the other 12 cases we reviewed for the study, multifocal PEComa with no indications of malignancy based on morphology is biologically benign and has a good prognosis. The lesion may be caused by the blood dissemination of tumor cells rather than metastasis. Radical treatment should not be used in clinical management. In addition, follow-up for PEComa would be long-term, because a second PEComa lesion may appear many years later for the “benign” dissemination of tumor cells.

Multifocal Pancreatic and Liver PEComas Mimic Metastatic Tumors: Case Report and Literature Review

10.21203/rs.3.rs-692687/v1 ◽

2021 ◽

Author(s):

Jian Guan ◽

Guihua Shen ◽

Wenting Huang

Keyword(s):

Soft Tissues ◽

Epithelioid Cell ◽

Clinicopathological Characteristics ◽

Good Prognosis ◽

Metastatic Tumors ◽

Epithelioid Angiomyolipoma ◽

Sugar Tumor ◽

Head Of The Pancreas

Abstract Background: Perivascular epithelioid cell tumor (PEComa) is a mesenchyme tumor derived from soft tissues and organs. Its morphology includes several types such as angiomyolipoma, lymphangiomyomatosis and pulmonary clear-cell “sugar” tumor with similar immunophenotype. Multifocal PEComas are rare lesions, which would confuse imaging examination and clinical management. We reported a case of multifocal angiomyolipomas in the pancreas and liver in a young patient. And we reviewed the literature to explore the clinicopathological characteristics and possible pathogenesis of multifocal PEComas.Case presentation: A 28-year-old woman presented with a lesion in the head of the pancreas and liver 5/6 segment respectively, initially suspected of a pancreatic tumor with liver metastasis. To explore the nature of the lesion, a liver mass resection and a pancreatic mass biopsy were performed at the first operation. After the postoperative pathology was confirmed as an epithelioid angiomyolipoma, the patient underwent a pancreatic segmental resection. After the operation, the patient did not receive any adjuvant treatment and was followed up for 25 months without tumor recurrence or metastasis.Conclusion: Multifocal PEComas present a chronic clinical course and have a good prognosis. Surgical resection is the main treatment. In the diagnosis, in addition to metastatic tumors, multifocal lesions also need to be differentiated with multifocal PEComa; in the treatment, radical treatment should not be performed for this type of disease; in the long-term monitoring of the disease, it is necessary to consider the possibility of a second or a greater number of PEComa lesions even many years later.

Malignant Perivascular Epithelioid Cell Tumor Involving the Prostate

10.5858/2003-127-e96-mpecti ◽

2003 ◽

Vol 127 (2) ◽

pp. e96-e98 ◽

Cited By ~ 6

Author(s):

Chin-Chen Pan ◽

An-Hang Yang ◽

Hung Chiang

Keyword(s):

Seminal Vesicle ◽

Tumor Cells ◽

S100 Protein ◽

Epithelioid Cell ◽

Cell Tumor ◽

Epithelioid Cells ◽

Granular Cytoplasm ◽

Hmb 45

Abstract Perivascular epithelioid cell tumor (PEComa) is a neoplasm chiefly composed of HMB-45–positive epithelioid cells with clear-to-granular cytoplasm and a perivascular distribution. We describe such a tumor involving the prostate and seminal vesicle in a 46-year-old man. The tumor had characteristic histologic features of PEComa. Immunohistochemically, the tumor cells were positive for HMB-45 but negative for epithelial markers, Melan-A, and S100 protein. The tumor behaved in a malignant fashion, and the patient died of the disease 4 years after diagnosis.

An Inguinal Perivascular Epithelioid Cell Tumor Metastatic to the Orbit

Case Reports in Pathology ◽

10.1155/2018/5749421 ◽

2018 ◽

Vol 2018 ◽

pp. 1-5

Author(s):

Zofia Tynski ◽

Way Chiang ◽

Albert Barrett

Keyword(s):

Clear Cell ◽

Middle Age ◽

Epithelioid Cell ◽

Ligamentum Teres ◽

Cell Tumor ◽

The Third ◽

Mesenchymal Neoplasms ◽

First Case ◽

Sugar Tumor

Malignant PEComas are rare mesenchymal neoplasms. These tumors harbor distinct myomelanocytic phenotype. The PEComa family of tumors includes lymphangioleiomyomatosis, angiomyolipoma, clear cell sugar tumor of the lung, and myomelanocytic tumor of the falciparum ligament/ligamentum teres. PEComas have no known normal cell counterpart. Majority of PEComas are benign and occur predominantly in the middle-age women. These tumors are commonly encountered in the uterus. Herein, we report a 20-year-old woman with a left inguinal mass metastatic to orbit, brain, lumbar spine, and skin at presentation. To our knowledge, this is the first case of metastatic PEComa to the orbit. This is the third case of primary PEComa of the inguinal area.

Adrenal Perivascular Epithelioid Cell Tumor: A Case Report With Discussion of Differential Diagnoses

10.5858/2010-0202-cr.1 ◽

2011 ◽

Vol 135 (4) ◽

pp. 499-502

Author(s):

Alireza Zarineh ◽

Jan F Silverman

Keyword(s):

Soft Tissues ◽

S100 Protein ◽

Female Genital Tract ◽

Epithelioid Cell ◽

Cell Tumor ◽

Differential Diagnoses ◽

Falciform Ligament ◽

Eosinophilic Cytoplasm

Abstract A 50-year-old man presented with nausea and abdominal bloating sensation of a few months' duration and was found to have a large adrenal mass that was resected after computed tomography scan evaluation. The histopathologic examination showed epithelioid and spindle cells having clear to granular eosinophilic cytoplasm, and small, central, round to oval nuclei with small nucleoli. The tumor cells demonstrated reactivity for smooth muscle actin, HMB-45, Melan-A, and vimentin, with focal positivity for desmin and S100 protein and negative staining for inhibin, synaptophysin, and chromogranin. A diagnosis of perivascular epithelioid cell tumor was made. Perivascular epithelioid cell tumors can arise in a variety of locations, including omentum/mesentery, female genital tract, pelvic soft tissues, skin, and falciform ligament, but an adrenal primary tumor has not been previously reported, to the best of our knowledge. The differential diagnosis is broad and site-dependant and in our case includes several neoplasms with clear cells that can involve the adrenal gland, including a primary adrenocortical neoplasm. A review of the related literature and discussion of the differential diagnoses are presented.

Perivascular Epithelioid Cell Tumor of the Ileum Presenting as Diverticulitis

Case Reports in Pathology ◽

10.1155/2012/476941 ◽

2012 ◽

Vol 2012 ◽

pp. 1-4 ◽

Cited By ~ 1

Author(s):

Saime Unluoglu ◽

Umit Bayol ◽

Nilay Korkmaz ◽

Bekir Ozenen ◽

Fuat Ipekci ◽

...

Keyword(s):

Tumor Cells ◽

Case Reports ◽

Smooth Muscle Actin ◽

Epithelioid Cell ◽

Epithelioid Cells ◽

Mesenchymal Neoplasms ◽

Granular Cytoplasm ◽

Diagnostic Difficulties ◽

Gross Examination

Perivascular epithelioid cell tumors (PEComas) are a group of rare mesenchymal neoplasms. Gastrointestinal PEComas are exceptionally rare, there being only a few case reports in the literature involving the colon and small intestine. Nearly all PEComas show immunoreactivity for both melanocytic (HMB45 and/or Melan-A) and smooth muscle (actin and/or desmin) markers. A 36-year-old male was admitted to the hospital with acut- abdomen. At laparatomy, a nodular mass protruding from the ileum which clinically simulated a diverticulitis was noticed. Gross examination of the specimen revealed a2×1,5×1 cm secondarily ulcerated, solid, nodular, gray white tumor mass in the ileal wall. Histologically, tumor cells were composed of nests of round-polygonal epithelioid cells with abundant clear to slightly eosinophilic granular cytoplasm and round vesicular nuclei. The nests were separated by thin fibrovascular septa. Minimal necrosis and low mitotic activity were noticed in the tumor. Immunohistochemically, tumor cells were positive for SMA, HMB45, and Melan-A and negative for CD10, RCC, CD45, CD117, CD34, EMA, and Desmin. Diagnosis was PEComa of the ileum. We report the case of ileal PEComa to remind the unusual presentation (diverticulitis) of these tumors, besides rarity and diagnostic difficulties.

Malignant Perivascular Epithelioid Cell Tumor of the Gallbladder: A Case Report and Review of Literature

10.5858/arpa.2013-0092-cr ◽

2014 ◽

Vol 138 (9) ◽

pp. 1238-1241 ◽

Cited By ~ 3

Author(s):

Liena Zhao ◽

Karl H. Anders

Keyword(s):

Clear Cell ◽

Epithelioid Cell ◽

Ligamentum Teres ◽

Cell Tumor ◽

Falciform Ligament ◽

Perivascular Epithelioid Cell Tumors

Mesenchymal Neoplasms ◽

Sugar Tumor ◽

Perivascular epithelioid cell tumors are rare mesenchymal neoplasms composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. The perivascular epithelioid cell tumor family includes angiomyolipoma, clear cell sugar tumor of the lung, lymphangioleiomyomatosis, clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres, and rare clear cell tumors of other anatomic sites. Perivascular epithelioid cell tumors have been reported previously in various sites, but to our knowledge not in the gallbladder. We report here, for the first time, a malignant perivascular epithelioid cell tumor arising in the gallbladder.

Pigmented Perivascular Epithelioid Cell Tumor of the Kidney

10.5858/133.12.1981 ◽

2009 ◽

Vol 133 (12) ◽

pp. 1981-1984 ◽

Cited By ~ 1

Author(s):

Masaharu Fukunaga ◽

Tohru Harada

Keyword(s):

Tumor Cells ◽

Spindle Cell ◽

S100 Protein ◽

Epithelioid Cell ◽

Cell Tumor ◽

Cell Renal Cell Carcinoma ◽

Spindle Cell Proliferation ◽

Clear Cytoplasm

Abstract A case of pigmented perivascular epithelioid cell tumor of the kidney in a 57-year-old woman with a clinically indicated tuberous sclerosis is presented. The lesion was a 3.0-cm, well-demarcated, black-colored mass. The tumor was histologically characterized by an epithelioid arrangement of round to polygonal cells with round nuclei and clear cytoplasm containing abundant melanin. Tumor cells showed mild atypia and low mitotic activity. A spindle cell proliferation was focally observed. There were no adipose elements or thick-walled vascular vessels. The stroma demonstrated intervening, thin, fibrovascular septa. Immunohistochemically, the tumor cells were strongly positive for HMB-45 but negative for epithelial and muscle markers, vimentin, and S100 protein. The patient had no evidence of disease 3 months after surgery. Pathologists and clinicians should know about the existence of pigmented perivascular epithelioid cell tumor of the kidney. This type of tumor should be differentiated from clear cell renal cell carcinoma or malignant melanoma.

Pancreatic Perivascular Epithelioid Cell Tumour Presenting with Upper Gastrointestinal Bleeding

Case Reports in Oncological Medicine ◽

10.1155/2015/431215 ◽

2015 ◽

Vol 2015 ◽

pp. 1-4 ◽

Cited By ~ 4

Author(s):

Christos Petrides ◽

Kyriakos Neofytou ◽

Aamir Z. Khan

Keyword(s):

Gastrointestinal Bleeding ◽

Epithelioid Cell ◽

Maximum Diameter ◽

Granulation Tissue Formation ◽

Multiple Biopsies ◽

Rare Tumours ◽

Massive Gastrointestinal Bleeding ◽

Head Of The Pancreas ◽

First Time

PEComa is a family of rare mesenchymal tumours which can occur in any part of the human body. Primary PEComas of the pancreas are extremely rare tumours with uncertain malignant potential. A 17-year-old female was admitted to the hospital due to melena. She required several transfusions. CT scan demonstrated a mass at the head of the pancreas measuring 4.2 cm in maximum diameter. An endoscopic ultrasound showed an ulcerating malignant looking mass infiltrating 50% of the wall of the second part of the duodenum in the region of the ampulla. Multiple biopsies taken showed extensive ulceration with granulation tissue formation and underlying large macrophages without being able to establish a definite diagnosis. We proceeded with pylorus-preserving pancreaticoduodenectomy. The postoperative course of the patient was unremarkable, and she was discharged on the 8th postoperative day. Histology examination of the specimen showed a PEComa of pancreas. Eighteen months after resection the patient is disease free. To the best of our knowledge this is the first time we describe a case of a pancreatic PEComa presenting with massive gastrointestinal bleeding.

Perivascular Epithelioid Cell Tumor of Urinary Bladder

American Journal of Clinical Pathology ◽

10.1093/ajcp/aqaa204 ◽

2020 ◽

Author(s):

You-li Wu ◽

Lang Lang ◽

Qiang Ma ◽

Feng Wu ◽

Yu Zhang ◽

...

Keyword(s):

Urinary Bladder ◽

Tumor Cells ◽

Clinical Symptoms ◽

Epithelioid Cell ◽

Cell Tumor ◽

Diagnostic Challenge ◽

Female Ratio ◽

Eosinophilic Cytoplasm

Abstract Objectives To review the clinicopathologic features of perivascular epithelioid cell tumor (PEComa) of the urinary bladder. Methods Seven cases of bladder PEComa were studied by light microscopy, immunohistochemistry, and fluorescence in situ hybridization (FISH). Results In our 7 cases, 5 patients were female and 2 were male, with ages between 26 and 78 years. Patients presented with hematuria and recurrent abdominal discomfort as the main clinical symptoms. Microscopically, the epithelioid and spindle-shaped tumor cells with clear to granular eosinophilic cytoplasm were arranged in fascicular, acinar, or nested patterns. The tumor cells were positive for HMB45, melan-A, and SMA, but no TFE3 gene rearrangement was detected in any of the 7 samples by FISH. The analysis of all 35 cases from the literature and ours showed a patient age range from 16 to 78 years (mean age, 39 years), a male-to-female ratio of 1:1.3, maximal tumor diameters from 0.6 to 18.8 cm (mean, 4.5 cm). With a mean follow-up of 27 months, the recurrence, metastasis, and mortality rates were 10.7%, 10.7%, and 7.1%, respectively. Conclusions Bladder PEComa is extremely rare, remains a diagnostic challenge, and needs more attention. Strengthening the understanding of this tumor will improve diagnostic accuracy.