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 ¤  Abstract
 ¤ Introduction
 ¤ Case Report
 ¤ Discussion
 ¤ Conclusion
 ¤  References
 ¤  Article Figures

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 Table of Contents     
UNUSUAL CASE
Year : 2015  |  Volume : 11  |  Issue : 4  |  Page : 282-284
 

Laparoscopic excision of infra-renal PEComa


Department of Surgery and Minimal Access surgery, Kaushalya Hospital, Thane, Maharashtra, India

Date of Web Publication1-Oct-2015

Correspondence Address:
Abhay N Dalvi
257 Walkeshwar Road, Mumbai - 400 006, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-9941.166439

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 ¤ Abstract 

Perivascular epitheloid cell tumors (PEComas) are rare tumors of malignant potential. There is no normal cell variant to these cells. The family is large and includes angiomyolipoma, clear cell "sugar" tumor amongst others. Imaging modalities are not very diagnostic. The diagnosis hence is often postoperative. A 55-year old female presented to us with ultrasonographic diagnosis of solid mass in the right infrarenal region. Contrast-enhanced computerized sonography (CECT) suggested paraganglioma or a soft tissue retroperitoneal tumor. Laparoscopic excision was successful. The rarity of this pathology and laparoscopic modality of excision prompted us to publish this report.


Keywords: Laparoscopy, PEComa, surgery


How to cite this article:
Bhanushali AH, Dalvi AN, Bhanushali HS. Laparoscopic excision of infra-renal PEComa. J Min Access Surg 2015;11:282-4

How to cite this URL:
Bhanushali AH, Dalvi AN, Bhanushali HS. Laparoscopic excision of infra-renal PEComa. J Min Access Surg [serial online] 2015 [cited 2019 Nov 11];11:282-4. Available from: http://www.journalofmas.com/text.asp?2015/11/4/282/166439



 ¤ Introduction Top


PEComas are rare soft tissue tumors of variable malignant potential. Available investigative modalities like CECT scan are often not diagnostic. The differential diagnosis usually depends upon the organ of origin. The final diagnosis is often obtained after histopathological examination. Laparosscopic excision has been sparsely reported. We report one such case of a patient having been diagnosed with an infra-renal mass that was excised laparoscopically and found to be PEComa.


 ¤ Case Report Top


A 55-year old postmenopausal woman presented with nonspecific abdominal pain. There were no obvious gastrointestinal or urinary complaints. She had visited a physician who had ordered an ultrasonography of the abdomen which revealed an 8 cm mass situated inferior and medial to the right kidney. The patient was referred to us for further treatment.

On investigations, biochemistry was normal with no hematuria. Contrast-enhanced computerized tomography (CECT) revealed a well-defined, heterogeneously enhancing soft tissue lesion, 7 × 5 × 4 cm anterior to the right psoas muscle and inferior to the right kidney. Possibilities of paraganglioma, soft tissue neoplasm, or a renal capsular tumor were raised. The fat planes with surrounding structures were maintained [Figure 1]. The patient was investigated with paraganglioma in mind. All the markers for functioning tumor were negative. The patient was scheduled for laparoscopic excision.
Figure 1: CECT showing the mass (marked in red arrow) around the lower pole of right kidney

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The patient was placed in lateral position. One 10 mm (telescope) and two 5 mm ports were placed to get a triangulation with the mass. Laparoscopy revealed a solid mass possibly arising from the capsule of the right kidney. Clean and clear planes with the kidney and rest of the tissues with clipping of single major vascular pedicle arising from right renal artery allowed complete laparoscopic resection. The tumor was retrieved in a bag by extending the telescopic incision. Recovery was uneventful.

The histopathology revealed a vascular tumor without adipolytic component. Microscopy revealed polygonal and elongated cells with whorled appearance. Immunohistochemistry studies revealed dual myomelanocytic differentiation with expression of SMA, Desmin, Caldesmon and focal expression of HMB-45, and Melan-A. Screening with c-kit, DOG-1, Synaptophysin, Chromoganin A, Cytokeratin, CK-8/18, S-100 protein, and CD 34 yielded negative results.

[Figure 2] shows intraoperative photograph, gross and microscopic nature of the tumor [Figure 2].
Figure 2: Intraoperative photograph, gross and microscopy of PEComa

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 ¤ Discussion Top


Perivascular epitheloid cell tumor (PEComa) is a conglomerate of rare tumors comprising perivascular epitheloid cells. These can arise from various organs common being kidney and liver. [1],[2],[3],[4],[5],[6],[7] The diagnosis is established only after microscopy.

Bonetti (1992) first noted this cell type - epitheloid features, clear cytoplasm, perivascular distribution, and immunoreactive to melanocytic markers. There was no normal corresponding cell in this pathology. [8] World Health Organization (2002) recommended PEComas to be separate pathology. [9] Angiomyolipomas, clear cell "sugar" cell tumors have been included in this family. The family also includes lymphangioleiomyomatosis (LAM), primary extrapulmonary sugar cell tumor (PEST), and clear cell myomelanocytic tumor (CCMMT). [8],[10],[11]

The tumors usually present as a mass symptomatic or otherwise as in our case. Due to heterogeneous composition of the mass, CECT imaging is usually inhomogeneous and hence exact pathological diagnosis is often not possible. In our case, fat component of angiomyolipoma being absent, PEComa was not suspected. The differential diagnosis includes a plethora of benign and malignant epithelial and mesenchymal tumors including retroperitoneal sarcomas. However, PEComas should come in the ambit of differential diagnosis if the tumor is well circumscribed as in our case. [12]

As there is no normal cell counterpart, it is proposed that tumors > 5 cm and showing infiltrative growth, necrosis, vascular invasion, hypercellularity, high nucleo-cytoplasmic ratio, and mitosis > 1 per 50 hpf (any two or more) should be considered at potentially malignant. Smaller tumors without mitosis are considered benign. [13] The group in between is considered as uncertain malignant potential. Our patient has a large tumor with positive focal HMB-45 but low atypia and hence considered as that with uncertain malignant potential.

Surgical excision is the treatment of choice. Laparoscopic resection of PEComas though reported at other sites; [14],[15] is not reported in this location.

Genetic abnormalities have found to be responsible for cell proliferation and sirolimus (and derivatives) have been shown to inhibit this activation especially in angiomyolipomas [16] which is not so in this case. These studies are unavailable in our place and hence were not done. The patient is 4 months postop, negative for tuberous sclerosis, and is under continued surveillance.


 ¤ Conclusion Top


PEComas are a family of rare tumors comprising perivascular epitheloid cells. Since there is no normal cell to this tumor cell, all are considered malignant of different aggressive potential. Surgical excision offers best chance for cure. Laparoscopic excision provides the benefits of early recovery and less pain to these patients.

 
 ¤ References Top

1.
Zhao LJ, Yang YJ, Wu H, Huang SM, Liu K. Perivascular epithelioid cell tumor of the liver: A case report and literature review. Eur Rev Med Pharmacol Sci 2013;17:1665-8.   Back to cited text no. 1
    
2.
Neri S, Ishii G, Aokage K, Hishida T, Yoshida J, Nishimura M, et al. Multiple perivascular epithelioid cell tumors: Clear cell tumor of the lung accompanied by angiomyolipoma of the liver. Ann Thorac Cardiovasc Surg 2013 Apr 20 [In Press].   Back to cited text no. 2
    
3.
Fu X, Jiang JH, Gu X, Li Z. Malignant perivascular epithelioid cell tumor of mesentery with lymph node involvement: A case report and review of literature. Diagn Pathol 2013;8:60.   Back to cited text no. 3
    
4.
Im S, Yoo C, Jung JH, Choi HJ, Yoo J, Kang CS. Primary perivascular epithelioid cell tumor in the rectum: A case report and review of the literature. Pathol Res Pract 2013;209:244-8.   Back to cited text no. 4
    
5.
Mourra N, Lazure T, Colas C, Arrive L, de Gramont A. Perivascular epithelioid cell tumor: The first malignant case report in the pancreas. Appl Immunohistochem Mol Morphol 2013;21:e1-4.   Back to cited text no. 5
    
6.
Yin L, Bu H, Chen M, Yu J, Zhuang H, Chen J, et al. Perivascular epithelioid cell neoplasm of the urinary bladder in an adolescent: A case report and review of the literature. Diagn Pathol 2012;7:183.   Back to cited text no. 6
    
7.
Desy NM, Bernstein M, Nahal A, Aziz M, Kenan S, Turcotte RE, et al. Primary perivascular epithelioid cell neoplasm (PEComa) of bone: Report of two cases and review of the literature. Skeletal Radiol 2012;41:1469-74.   Back to cited text no. 7
    
8.
Bonetti F, Pea M, Martignoni G, Doglioni C, Zamboni G, Capelli P, et al. Clear cell ('sugar') tumor of the lung is a lesion strictly related to angiomyolipoma -the concept of a family of lesions characterized by the presence of the perivascular epithelioid cells (PEC). Pathology 1994;26:230-6.  Back to cited text no. 8
    
9.
Folpe A. Neoplasma with perivascular epitheloid cell differentiation (PEComas). In World Health Organization classification of Tumors: Pathology and Genetics of tumors of Soft tissue and bone. In: Fletcher CD, Unni KK, Mertens F, editors. Lyon: IARC Press; 2002. p. 221-2.  Back to cited text no. 9
    
10.
Tazelaar HD, Batts KP, Srigley JR. Primary Extrapulmonary Sugar tumor (PEST): A report of four cases. Mod Pathol 2001;14:615-22.  Back to cited text no. 10
    
11.
Folpe AL, Goodman ZD, Ishak KG, Paulino AF, Taboada EM, Meehan SA, et al. Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres: A novel member of the perivascular epitheloid clear cell family of tumors with predilection for children and young adults. Am J Surg Pathol 2000;24:1239-46.  Back to cited text no. 11
    
12.
Tan Y, Zhang H, Xiao EH. Perivascular epithelioid cell tumor: Dynamic CT, MRI and clinicopathological characteristics - Analysis of 32 cases and review of the literature. Clin Radiol 2013;68:555-61.  Back to cited text no. 12
    
13.
Bleeker JS, Quevedo JF, Folpe AL. 'Malignant' perivascular epithelioid cell neoplasm: Risk stratification and treatment strategies. Sarcoma 2012;2012:541626.  Back to cited text no. 13
    
14.
Yang B, Wang L, Wu Z, Li M, Wang H, Sheng J, et al: Synchronous transperitoneal laparoscopic resection of right retroperitoneal schwannoma and left kidney monotypic PEComa in the presence of a duplicated inferior vena cava (IVC). Urology 2012;80:e7-8.  Back to cited text no. 14
    
15.
Phongnarisom C, Khunamornpong S, Pattamapaspong N, Srisomboon J. Laparoscopic radical excision of primary round ligament perivascular epitheloid cell tumor mimicking leiomyoma. J Minim Invasive Gynecol 2009;16:626-9.  Back to cited text no. 15
    
16.
Wolff N, Kabbani W, Bradley T, Raj G, Watumull L, Brugarolas J. Sirolimus and temsirolimus for epitheloid angiomyolipoma. J Clin Oncol 2010;28:e65-8.  Back to cited text no. 16
    


    Figures

  [Figure 1], [Figure 2]



 

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