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Perivascular Epithelioid Cell Tumor of Gastrointestinal Tract Case Report and Review of the Literature

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Perivascular Epithelioid Cell Tumor of Gastrointestinal Tract Case Report and Review of the Literature Perivascular Epithelioid Cell Tumor of Gastrointestinal Tract Case Report and Review of the Literature Biyan Lu, MD, PhD, Chenliang Wang, MD, Junxiao Zhang, MD, Roland P. Kuiper, PhD, Minmin Song, MD, Xiaoli Zhang, MD, Shunxin Song, MD, Ad Geurts van Kessel, PhD, Aikichi Iwamoto, MD, PhD, Jianping Wang, MD, PhD, and Huanliang Liu, MD, PhD Perivascular epithelioid cell tumors of gastrointestinal tract (GI PECo- markers. Molecular pathological assays confirmed a PSF-TFE3 gene mas) are exceedingly rare, with only a limited number of published fusion in one of our cases. Furthermore, in this case microphthalmia- reports worldwide. Given the scarcity of GI PEComas and their associated transcription factor and its downstream genes were found to relatively short follow-up periods, our current knowledge of their exhibit elevated transcript levels. biologic behavior, molecular genetic alterations, diagnostic criteria, Knowledge about the molecular genetic alterations in GI PEComas and prognostic factors continues to be very limited. is still limited and warrants further study. We present 2 cases of GI PEComas, one of which showed an (Medicine 94(3):e393) aggressive histologic behavior that underwent multiple combined che- motherapies. We also review the available English-language medical Abbreviations: AML = angiomyolipoma, CCMMT = clear cell literature on GI PEComas-not otherwise specified (PEComas-NOS) and myomelanocytic tumor of the falciform ligament/ligamentum teres, discuss their clinicopathological and molecular genetic features. CCST = clear cell ‘‘sugar’’ tumor of the lung, CDK2 = cyclin- Pathologic analyses including histomorphologic, immunohisto- dependent kinase 2, C-MET = met proto-oncogene, CRP = C- chemical, and ultrastructural studies were performed to evaluate the reactive protein, CT = computed tomography, DCT = dopachrome clinicopathological features of GI PEComas, their diagnosis, and differ- tautomerase, GAPDH = glyceraldehyde-phosphate dehydrogenase, ential diagnosis. Immunohistochemistry, semiquantitative reverse tran- G-CSF = granulocyte colony-stimulating factor, GI PEComas = scriptase polymerase chain reaction, and DNA sequencing assays were perivascular epithelioid cell tumors of gastrointestinal tract, HIF-1a carried out to detect the potential molecular genetic alterations in our = hypoxia inducible factor 1, alpha subunit. HMB45 = human cases melanoma black 45, HPF = high power field, LAM = lym- Microscopically, the tumors showed distinctive histologic features phangioleiomyomatosis, MiTF = microphthalmia-associated of PEComas-NOS, including fascicular or nested architecture, epithe- transcription factor, NBI = narrow-band imaging, PEComas = lioid or spindled cell type, and clear to eosinophilic cytoplasm. The perivascular epithelioid cell tumors, PEComas-NOS = PEComas- tumor cells were immunohistochemically positive for melanocytic not otherwise specified, PECs = perivascular epithelioid cells, PR = pathogenesis related protein, pSTAT3 = phospho-signal transducers Editor: Chun-xia Cao. and activators of transcription 3, Q3W = once every 3 weeks, RT- Received: August 3, 2014; revised and accepted: December 3, 2014. PCR = semiquantitative reverse transcriptase polymerase chain From the Guangdong Institute of Gastroenterology and the Sixth Affiliated Hospital, (BL, CW, JZ, MS, XZ, SS, JW, HL); Guangdong Key Laboratory reaction, SMA = smooth muscle actin, TBX2 = T-box 2, TFE3 = of Colorectal and Pelvic Floor Diseases, (BL, CW, JZ, MS, XZ, JW, HL); Transcription factor E3, TSC = tuberous sclerosis complex, TYR = Institute of Human Virology, (BL, CW, JZ, MS, XZ, HL) Key Laboratory tyrosinase, WBC = white blood cell. of Tropical Disease Control (Ministry of Education); Sun Yat-sen University, Guangzhou (BL, CW, JZ, MS, XZ, HL); Dongguan Health School, Dongguan, China (BL); Department of Human Genetics, Radboud University Medical Center, Nijmegen, The Netherlands (JZ, RPK, AGK); INTRODUCTION and Advanced Clinical Research Center, Institute of Medical Science, erivascular epithelioid cell tumors (PEComas) are a family University of Tokyo, Tokyo, Japan (AI). Correspondence: Huanliang Liu, Guangdong Institute of Gastroenterology, P of rare mesenchymal neoplasms histologically and immu- Sun Yat-sen University, 26 Yuan Cun Er Heng Road, Guangzhou, nohistochemically characterized by perivascular epithelioid cell Guangdong 510655, China (e-mail: [email protected]); Jianp- (PEC) differentiation.1 The PECs have variable morphologic ing Wang, The Sixth Affiliated Hospital, Sun Yat-sen University, 26 features, with an epithelioid to spindled cell type resembling Yuan Cun Er Heng Road, Guangzhou, Guangdong 510655, China (email: [email protected]). smooth muscle, clear to granular lightly eosinophilic cytoplasm, Grant Support: This study was supported by Science and Information and round to oval nuclei with small nucleoli. The PECs also Technology Bureau of Guangzhou, Guangdong (2011J5200009); exhibit a distinct immunophenotype with a coexpression of Guangdong Provincial Department of Science and Technology melanocytic and myogenic markers, such as HMB45, Melan-A, (2012B050500004); Guangdong Innovative Research Team Program 2 (2009010058); Overseas Excellent Professor Project, Ministry of Edu- MiTF, smooth muscle actin (SMA), and calponin. The cation; ‘‘985 Project’’ of Sun Yat-sen University, Guangdong Transla- PEComa family includes angiomyolipoma (AML), clear cell tional Medicine Public Platform (4202037); National Key Clinical ‘‘sugar’’ tumor of the lung (CCST), lymphangioleiomyomato- Discipline; and Japan Ministry of Education, Culture, Sports, Science sis (LAM), clear cell myomelanocytic tumor of the falciform and Technology (MEXT) for Program of Japan Initiative for Global Research Network on Infectious Diseases (J-GRID). ligament/ligamentum teres (CCMMT), and unusual clear cell The authors do not have any possible conflicts of interest. tumors in other locations. PEComas have been reported in Copyright # 2015 Wolters Kluwer Health, Inc. All rights reserved. various anatomic sites, with a marked female predominance. This is an open access article distributed under the Creative Commons Due to their relative rarity, the diagnostic criteria, optimal Attribution License 4.0, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. treatment strategies, and prognostic factors for PEComas have ISSN: 0025-7974 not yet been confirmed at this time. We report 2 cases of DOI: 10.1097/MD.0000000000000393 PEComas arising in the gastrointestinal tract, including the Medicine Volume 94, Number 3, January 2015 www.md-journal.com | 1 Lu et al Medicine Volume 94, Number 3, January 2015 clinicopathological features and potential molecular genetic abdomen (Figure 1). During the surgical operation, a large alterations of this rare tumor. tumor was found in the terminal ileum about 12 cm from ileocecal valve adhering tightly to the mesentery of the ileum and the right pelvic wall. Surgical resection of the tumor and the MATERIALS AND METHODS affected segment of the intestine was carried out. After surgery, the patient received 5 courses of multiple combined che- Case Presentation motherapies including ifosfamide 2000 mg/m2 day 1 to 4, 2 2 Case 1 epidoxorubicin 30 mg/m day 1 to 3, dacarbazine 350 mg/m day 1 to 4, and mesna 4800 mg/m2 day 1 to 4 once every 3 weeks A 29-year-old Chinese woman was admitted to our hospi- (Q3W). In addition, granulocyte colony-stimulating factor (G- tal because of gradual onset of abdominal pain, nausea, vomit- 3 CSF; Filgrastim) was given at a dose of 5 pg/kg/day subcu- ing and weight loss for 6 months. The patient did not have a taneously from day 5 to day 12 of each cycle. Follow-up CT medical history of gastrointestinal tumors, inflammatory bowel scans were performed every 6 months after chemotherapy. The disease, or tuberous sclerosis complex. Her family history was patient was alive and well with no signs of recurrence or unremarkable. Physical examination revealed a large mass at metastasis for 28 months of follow-up. the right lower abdomen. All blood and biochemical tests were within the normal ranges, apart from a hemoglobin reading of 85 mg/dL and a C-reactive protein (CRP) reading of 15 mg/dL. Case 2 An intravenous contrast-enhanced computed tomography (CT) A 41-year-old Chinese woman with a history of hyster- scan showed an ill-defined multilocular soft tissue tumor ectomy for benign leiomyoma presented with progressive epi- measuring 13 cm  8cm 7 cm in the pelvis and lower gastric pain and dark stools. The patient denied any family AB CD FIGURE 1. Abdominal computed tomography images (case 1): coronal (A), sagittal (B), and axial (C and D) reconstructions. Computed tomography of the abdomen showed an ill-defined multilocular low-density mass measuring 13  8  7 cm in the pelvis and lower abdomen. 2 | www.md-journal.com Copyright # 2015 Wolters Kluwer Health, Inc. All rights reserved. Medicine Volume 94, Number 3, January 2015 Perivascular Epitheloid Cell Tumor of Gastrointestinal Tract AB FIGURE 2. Endoscopy appearance of GI PEComa (case 2). White light (A) and narrow band imaging (B) endoscopy showed a 2-cm diameter, polypoid tumor protruding into the lumen of the terminal ileum. GI PEComa ¼ perivascular epithelioid cell tumors of gastrointestinal tract. history of gastrointestinal (GI) cancer or inflammatory bowel Histologic and Immunohistochemical Analyses disease. Laboratory investigations showed a white blood cell
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