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J Chest Surg 2023; 56(5): 367-370

Published online September 5, 2023 https://doi.org/10.5090/jcs.22.139

Copyright © Journal of Chest Surgery.

Primary Perivascular Epithelioid Cell Tumor of the Lung: A Case Report

Hye Rim Na , M.D.1, Jong Hui Suh , M.D., Ph.D.2, Jiyun Lee , M.D.2

1Department of Thoracic and Cardiovascular Surgery, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul; 2Department of Thoracic and Cardiovascular Surgery, Incheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Incheon, Korea

Correspondence to:Jiyun Lee
Tel 82-32-280-5441
Fax 82-32-280-5556
E-mail jiyunlee.303@catholic.ac.kr
ORCID
https://orcid.org/0000-0002-4554-9676

Received: November 10, 2022; Revised: January 20, 2023; Accepted: January 26, 2023

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Perivascular epithelioid cell tumors (PEComas) are very rare mesenchymal neoplasms, composed of histologically and immunohistochemically distinctive cells that form a sheet-like appearance around vessel lumens. Although most are benign, a malignant subset does exist, complicating clinical diagnostic efforts. Proper evaluation and management are thus essential for long-term patient survival. Herein, we present a rare case of a benign pulmonary PEComa that was diagnosed postoperatively in a 45-year-old woman.

Keywords: Perivascular epithelioid cell neoplasms, Pulmonary neoplasms, Case report

A 45-year-old woman with a round lung nodule discovered on a simple chest radiograph (Fig. 1A), was referred to Incheon St. Mary’s Hospital. At the time, she was an active smoker (10 pack-years) and reported no symptoms. The physical examination and laboratory results were unremarkable. Non-enhanced computed tomography (CT) of the lung revealed a round and smoothly contoured nodule (16 mm) of low radiodensity (30–60 Hounsfield units [HU]) at the base of the right lower lobe, showing no pre-contrast calcification (Fig. 1B). On contrast imaging, substantial enhancement (110–180 HU) was noted, suggesting intense vascularity (Fig. 1C), but no enlarged lymph nodes were visible. The preoperative differential diagnosis included Castleman’s disease, hypervascular metastasis, and primary lung cancer.

Figure 1.Pulmonary perivascular epithelioid cell tumor imaging studies. (A) Initial plain chest film showing a round, nodular shadow in the right lower lung field (arrow). (B) Pre-contrast computed tomography (CT) view of a round, low-density (30–60 Hounsfield units [HU]) nodule (16 mm) in the right lower lobe (arrow). (C) Post-contrast CT showing a strongly enhanced (110–180 HU), heterogeneous nodule, reflecting intense vascularity (arrow). L-PA, left posterior-anterior.

The patient underwent video-assisted thoracoscopic wedge resection for the initial purpose of frozen-section diagnosis. During exploration, the visceral surface of the right lower lobe demonstrated a round and reddish-tan mass that was removed using an endostapler (Fig. 2A). Once excised, its cut surface exposed a grayish-red lesion well demarcated from the normal parenchyma (Fig. 2B). A frozen section examination favored a benign pathology, pending further immunohistochemical studies. The operation ended without a further procedure and was well tolerated.

Figure 2.Surgical approach to perivascular epithelioid cell tumor of the lung. (A) Ring-shaped forceps were used to ensnare the dark-red, round nodule in the lung, using an endostapler for wedge resection. (B) Incised red-tan tumor, well-demarcated and devoid of necrosis or calcification.

Microscopically, a proliferation of uniform, round-to- cuboidal epithelioid cells was evident in a sheet-like arrangement, with thin-walled vascular networks interposed. The intracellular cytoplasm appeared granular and eosinophilic or variably clear, indicative of high glycogen content. Most importantly, the immunostaining results were positive for melanocytic and myogenic markers, specifically HMB45 and actin (Fig. 3). Other markers, such as CD10, TTF-1, and cytokeratin, proved negative. Hallmarks of malignancy (i.e., infiltration, necrosis, and extreme cellularity) were also lacking. Tumor resection was complete, with no margin involvement.

Figure 3.Histologic attributes of pulmonary perivascular epithelioid cell tumor. (A) Low-power (H&E, ×10) perspective of round and well-circumscribed mass, distinct from normal lung parenchyma. (B) High- power (H&E, ×200) view showing an abundance of round-to-cuboidal cells in a sheet-like arrangement around vessels, with a cytoplasm clear or eosinophilic cytoplasm showing a granular appearance. (C) Positive staining of the tumor marker actin (×40), typically expressed by myocytes, in thin-walled sinusoidal vessels. (D) Focal positivity of HMB-45 (×200), typically expressed by melanocytes, in perivascular epithelioid cells.

The patient recovered uneventfully. On postoperative day 2, the chest tube was removed, and the patient was discharged on postoperative day 5, free of complications. Given the rarity of perivascular epithelioid cell tumors (PEComas), a multidisciplinary conference was held for discussion. Abdominal and pelvic CT studies were also done as a precaution to check for other tumors or metastases. Fortunately, we found no signs of malignant disease, only a diffusely enlarged uterus and multiple myomas, prompting a gynecologic referral.

The Institutional Review Board of Incheon St. Mary’s Hospital approved the study (IRB approval no., OC22ZASI0128). The patient provided written informed consent to publish the clinical details and images.

PEComas comprise a family of rare mesenchymal tumors, few of which have been documented worldwide. According to Korea Education and Research Information Service, only 26 cases of PEComa have been reported in Korea, and just 2 have originated in the lung. We thus present this case as another rarity in the medical literature.

The PEComa tumor family is known for its heterogeneity, incorporating a number of entities that share unique pathological and immunohistochemical features. The cellular constituents are described as round and epithelioid, with eosinophilic or clear cytoplasm, forming nests along vessel lumina. This nomenclature was first introduced by Leibow and Castleman [1] in 1971 and currently includes lymphangioleiomyomatosis, angiomyolipoma, pulmonary clear cell “sugar tumor” (CCST), extrapulmonary sugar tumor, clear cell melanocytic tumor of the falciform ligament or ligamentum teres, and others that defy classification [2].

These growths may originate in a variety of sites, including the genitourinary system, liver, skin, or lungs. Angiomyolipomas are commonly associated with the kidneys, whereas CCSTs are rare benign tumors of the lungs. The latter display a slight female predominance and a proclivity for middle age (40–50 years) [3]. Most cases are asymptomatic and are incidentally detected during routine examinations.

Immunohistochemical staining of the processed tissue is crucial for distinguishing PEComas from other soft tissue tumors. PEComas are unique in their high expression levels of HMB-45 and actin markers, which are found in melanocytes and myocytes, respectively [4,5]. Moreover, they lack positivity for cytokeratin or epithelial membrane antigen, as is observed in clear cell renal carcinoma and other metastatic tumors [6].

Although generally benign, malignant PEComas are also increasingly being reported [5,7-9]. Incidental asymptomatic detection is usual in benign tumors, whereas advanced local expansion may occasionally cause chest pain or discomfort [10]. On CT imaging, pulmonary PEComas present as round nodules within parenchymal peripheries, exhibiting intense contrast enhancement of richly vascular stroma that complicates the clinical diagnosis [5,7,11]. A comprehensive preoperative evaluation and staging are warranted to exclude potential malignancies, such as clear cell carcinoma or metastatic sarcoma [10].

Complete surgical excision is currently the recommended treatment of choice. Since chemotherapy and radiotherapy are largely ineffective, complete surgical resection with a pathologically negative resection margin is recommended [9,10]. If invasion of critical structures is suspected, a close or microscopically positive margin may be appropriate for preservation. However, radical resection is only sometimes recommended. Since most PEComas are benign and lymph node invasion has rarely been reported, the need for lymph node dissection is not established. However, according to the National Comprehensive Cancer Network guideline on soft tissue sarcoma, treatment plans depend on nodal staging [12]. Therefore, we recommend performing lymph node dissection if enlarged lymph nodes are seen in preoperative imaging studies. Immunotherapy and targeted therapies are still under investigation, although patients with a tuberous sclerosis complex (TSC) gene mutation, which is known to be associated with PEComas, may respond to mechanistic target of rapamycin inhibitors.

In our patient, the diagnosis of benign PEComa was ultimately achieved through wedge resection, having eluded a precise determination beforehand. Based on preoperative CT findings, Castleman’s disease, paraganglioma, hypervascular metastasis, and primary lung cancer were all diagnostic possibilities. Frozen examination and permanent pathology of the tumor revealed no evidence of malignancy. Consequently, postsurgical follow-up was routine, without any additional therapy.

In conclusion, PEComas are rare mesenchymal tumors originating in various organs, including the lungs. Upon discovery, proper evaluation and management may be challenging. Unlike benign PEComas, which are curable by resection, malignant variants are fraught with therapeutic concerns. Preoperative CT characteristics may simulate those of benign lung tumors, so surgeons must be aware of the potential for malignancy. If malignant behavior is suspected, a thorough evaluation is required, relying on multidisciplinary support until a standard of care is established.

Author contributions

Conceptualization: HRN, JHS, JL. Data curation: HRN. Formal analysis: HRN. Project administration: JL. Visualization: HRN. Writing–original draft: HRN. Writing–review & editing: HRN, JHS, JL.

Conflict of interest

No potential conflict of interest relevant to this article was reported.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

  1. Liebow AA, Castleman B. Benign clear cell ("sugar") tumors of the lung. Yale J Biol Med 1971;43:213-22.
    Pubmed KoreaMed
  2. Folpe AL, Kwiatkowski DJ. Perivascular epithelioid cell neoplasms: pathology and pathogenesis. Hum Pathol 2010;41:1-15. https://doi.org/10.1016/j.humpath.2009.05.011.
    Pubmed CrossRef
  3. Vijayabhaskar R, Mehta SS, Deodhar KK, Pramesh CS, Mistry RC. PEComa of the lung. J Cancer Res Ther 2010;6:109-11. https://doi.org/10.4103/0973-1482.63548.
    Pubmed CrossRef
  4. Liang W, Xu S, Chen F. Malignant perivascular epithelioid cell neoplasm of the mediastinum and the lung: one case report. Medicine (Baltimore) 2015;94:e904. https://doi.org/10.1097/MD.0000000000000904.
    Pubmed KoreaMed CrossRef
  5. Ye T, Chen H, Hu H, Wang J, Shen L. Malignant clear cell sugar tumor of the lung: patient case report. J Clin Oncol 2010;28:e626-8. https://doi.org/10.1200/JCO.2010.29.6939.
    Pubmed CrossRef
  6. Olivencia-Yurvati AH, Rodriguez AE. Clear cell "sugar" tumor of the lung: benign or malignant?. Int Surg 2015;100:924-6. https://doi.org/10.9738/INTSURG-D-14-00021.1.
    Pubmed KoreaMed CrossRef
  7. Lim HJ, Lee HY, Han J, Choi YS, Lee KS. Uncommon of the uncommon: malignant perivascular epithelioid cell tumor of the lung. Korean J Radiol 2013;14:692-6. https://doi.org/10.3348/kjr.2013.14.4.692.
    Pubmed KoreaMed CrossRef
  8. Parfitt JR, Keith JL, Megyesi JF, Ang LC. Metastatic PEComa to the brain. Acta Neuropathol 2006;112:349-51. https://doi.org/10.1007/s00401-006-0105-5.
    Pubmed CrossRef
  9. Zhao J, Teng H, Zhao R, et al. Malignant perivascular epithelioid cell tumor of the lung synchronous with a primary adenocarcinoma: one case report and review of the literature. BMC Cancer 2019;19:235. https://doi.org/10.1186/s12885-019-5383-0.
    Pubmed KoreaMed CrossRef
  10. Sobiborowicz A, Czarnecka AM, Szumera-Cieckiewicz A, Rutkowski P, Switaj T. Diagnosis and treatment of malignant PEComa tumours. Oncol Clin Pract 2020;16:22-33. https://doi.org/10.5603/OCP.2020.0003.
    CrossRef
  11. Song Y, Chen F, Zhang C, Lin X. Spindle cell subtype of pulmonary clear cell tumor with prominent calcification and malignant potential. Thorac Cancer 2017;8:530-4. https://doi.org/10.1111/1759-7714.12457.
    Pubmed KoreaMed CrossRef
  12. von Mehren M, Kane III JM, Agulnik M, et al. NCCN Clinical Practice Guidelines in Oncology: soft tissue sarcoma, version 2. National Comprehensive Cancer Network; 2022 [cited 2022 Dec 29].
    Available from: https://www.nccn.org/professionals/physician_gls/pdf/sarcoma.pdf.

Article

Case Report

J Chest Surg 2023; 56(5): 367-370

Published online September 5, 2023 https://doi.org/10.5090/jcs.22.139

Copyright © Journal of Chest Surgery.

Primary Perivascular Epithelioid Cell Tumor of the Lung: A Case Report

Hye Rim Na , M.D.1, Jong Hui Suh , M.D., Ph.D.2, Jiyun Lee , M.D.2

1Department of Thoracic and Cardiovascular Surgery, Seoul St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Seoul; 2Department of Thoracic and Cardiovascular Surgery, Incheon St. Mary’s Hospital, College of Medicine, The Catholic University of Korea, Incheon, Korea

Correspondence to:Jiyun Lee
Tel 82-32-280-5441
Fax 82-32-280-5556
E-mail jiyunlee.303@catholic.ac.kr
ORCID
https://orcid.org/0000-0002-4554-9676

Received: November 10, 2022; Revised: January 20, 2023; Accepted: January 26, 2023

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Perivascular epithelioid cell tumors (PEComas) are very rare mesenchymal neoplasms, composed of histologically and immunohistochemically distinctive cells that form a sheet-like appearance around vessel lumens. Although most are benign, a malignant subset does exist, complicating clinical diagnostic efforts. Proper evaluation and management are thus essential for long-term patient survival. Herein, we present a rare case of a benign pulmonary PEComa that was diagnosed postoperatively in a 45-year-old woman.

Keywords: Perivascular epithelioid cell neoplasms, Pulmonary neoplasms, Case report

Case report

A 45-year-old woman with a round lung nodule discovered on a simple chest radiograph (Fig. 1A), was referred to Incheon St. Mary’s Hospital. At the time, she was an active smoker (10 pack-years) and reported no symptoms. The physical examination and laboratory results were unremarkable. Non-enhanced computed tomography (CT) of the lung revealed a round and smoothly contoured nodule (16 mm) of low radiodensity (30–60 Hounsfield units [HU]) at the base of the right lower lobe, showing no pre-contrast calcification (Fig. 1B). On contrast imaging, substantial enhancement (110–180 HU) was noted, suggesting intense vascularity (Fig. 1C), but no enlarged lymph nodes were visible. The preoperative differential diagnosis included Castleman’s disease, hypervascular metastasis, and primary lung cancer.

Figure 1. Pulmonary perivascular epithelioid cell tumor imaging studies. (A) Initial plain chest film showing a round, nodular shadow in the right lower lung field (arrow). (B) Pre-contrast computed tomography (CT) view of a round, low-density (30–60 Hounsfield units [HU]) nodule (16 mm) in the right lower lobe (arrow). (C) Post-contrast CT showing a strongly enhanced (110–180 HU), heterogeneous nodule, reflecting intense vascularity (arrow). L-PA, left posterior-anterior.

The patient underwent video-assisted thoracoscopic wedge resection for the initial purpose of frozen-section diagnosis. During exploration, the visceral surface of the right lower lobe demonstrated a round and reddish-tan mass that was removed using an endostapler (Fig. 2A). Once excised, its cut surface exposed a grayish-red lesion well demarcated from the normal parenchyma (Fig. 2B). A frozen section examination favored a benign pathology, pending further immunohistochemical studies. The operation ended without a further procedure and was well tolerated.

Figure 2. Surgical approach to perivascular epithelioid cell tumor of the lung. (A) Ring-shaped forceps were used to ensnare the dark-red, round nodule in the lung, using an endostapler for wedge resection. (B) Incised red-tan tumor, well-demarcated and devoid of necrosis or calcification.

Microscopically, a proliferation of uniform, round-to- cuboidal epithelioid cells was evident in a sheet-like arrangement, with thin-walled vascular networks interposed. The intracellular cytoplasm appeared granular and eosinophilic or variably clear, indicative of high glycogen content. Most importantly, the immunostaining results were positive for melanocytic and myogenic markers, specifically HMB45 and actin (Fig. 3). Other markers, such as CD10, TTF-1, and cytokeratin, proved negative. Hallmarks of malignancy (i.e., infiltration, necrosis, and extreme cellularity) were also lacking. Tumor resection was complete, with no margin involvement.

Figure 3. Histologic attributes of pulmonary perivascular epithelioid cell tumor. (A) Low-power (H&E, ×10) perspective of round and well-circumscribed mass, distinct from normal lung parenchyma. (B) High- power (H&E, ×200) view showing an abundance of round-to-cuboidal cells in a sheet-like arrangement around vessels, with a cytoplasm clear or eosinophilic cytoplasm showing a granular appearance. (C) Positive staining of the tumor marker actin (×40), typically expressed by myocytes, in thin-walled sinusoidal vessels. (D) Focal positivity of HMB-45 (×200), typically expressed by melanocytes, in perivascular epithelioid cells.

The patient recovered uneventfully. On postoperative day 2, the chest tube was removed, and the patient was discharged on postoperative day 5, free of complications. Given the rarity of perivascular epithelioid cell tumors (PEComas), a multidisciplinary conference was held for discussion. Abdominal and pelvic CT studies were also done as a precaution to check for other tumors or metastases. Fortunately, we found no signs of malignant disease, only a diffusely enlarged uterus and multiple myomas, prompting a gynecologic referral.

The Institutional Review Board of Incheon St. Mary’s Hospital approved the study (IRB approval no., OC22ZASI0128). The patient provided written informed consent to publish the clinical details and images.

Discussion

PEComas comprise a family of rare mesenchymal tumors, few of which have been documented worldwide. According to Korea Education and Research Information Service, only 26 cases of PEComa have been reported in Korea, and just 2 have originated in the lung. We thus present this case as another rarity in the medical literature.

The PEComa tumor family is known for its heterogeneity, incorporating a number of entities that share unique pathological and immunohistochemical features. The cellular constituents are described as round and epithelioid, with eosinophilic or clear cytoplasm, forming nests along vessel lumina. This nomenclature was first introduced by Leibow and Castleman [1] in 1971 and currently includes lymphangioleiomyomatosis, angiomyolipoma, pulmonary clear cell “sugar tumor” (CCST), extrapulmonary sugar tumor, clear cell melanocytic tumor of the falciform ligament or ligamentum teres, and others that defy classification [2].

These growths may originate in a variety of sites, including the genitourinary system, liver, skin, or lungs. Angiomyolipomas are commonly associated with the kidneys, whereas CCSTs are rare benign tumors of the lungs. The latter display a slight female predominance and a proclivity for middle age (40–50 years) [3]. Most cases are asymptomatic and are incidentally detected during routine examinations.

Immunohistochemical staining of the processed tissue is crucial for distinguishing PEComas from other soft tissue tumors. PEComas are unique in their high expression levels of HMB-45 and actin markers, which are found in melanocytes and myocytes, respectively [4,5]. Moreover, they lack positivity for cytokeratin or epithelial membrane antigen, as is observed in clear cell renal carcinoma and other metastatic tumors [6].

Although generally benign, malignant PEComas are also increasingly being reported [5,7-9]. Incidental asymptomatic detection is usual in benign tumors, whereas advanced local expansion may occasionally cause chest pain or discomfort [10]. On CT imaging, pulmonary PEComas present as round nodules within parenchymal peripheries, exhibiting intense contrast enhancement of richly vascular stroma that complicates the clinical diagnosis [5,7,11]. A comprehensive preoperative evaluation and staging are warranted to exclude potential malignancies, such as clear cell carcinoma or metastatic sarcoma [10].

Complete surgical excision is currently the recommended treatment of choice. Since chemotherapy and radiotherapy are largely ineffective, complete surgical resection with a pathologically negative resection margin is recommended [9,10]. If invasion of critical structures is suspected, a close or microscopically positive margin may be appropriate for preservation. However, radical resection is only sometimes recommended. Since most PEComas are benign and lymph node invasion has rarely been reported, the need for lymph node dissection is not established. However, according to the National Comprehensive Cancer Network guideline on soft tissue sarcoma, treatment plans depend on nodal staging [12]. Therefore, we recommend performing lymph node dissection if enlarged lymph nodes are seen in preoperative imaging studies. Immunotherapy and targeted therapies are still under investigation, although patients with a tuberous sclerosis complex (TSC) gene mutation, which is known to be associated with PEComas, may respond to mechanistic target of rapamycin inhibitors.

In our patient, the diagnosis of benign PEComa was ultimately achieved through wedge resection, having eluded a precise determination beforehand. Based on preoperative CT findings, Castleman’s disease, paraganglioma, hypervascular metastasis, and primary lung cancer were all diagnostic possibilities. Frozen examination and permanent pathology of the tumor revealed no evidence of malignancy. Consequently, postsurgical follow-up was routine, without any additional therapy.

In conclusion, PEComas are rare mesenchymal tumors originating in various organs, including the lungs. Upon discovery, proper evaluation and management may be challenging. Unlike benign PEComas, which are curable by resection, malignant variants are fraught with therapeutic concerns. Preoperative CT characteristics may simulate those of benign lung tumors, so surgeons must be aware of the potential for malignancy. If malignant behavior is suspected, a thorough evaluation is required, relying on multidisciplinary support until a standard of care is established.

Article information

Author contributions

Conceptualization: HRN, JHS, JL. Data curation: HRN. Formal analysis: HRN. Project administration: JL. Visualization: HRN. Writing–original draft: HRN. Writing–review & editing: HRN, JHS, JL.

Conflict of interest

No potential conflict of interest relevant to this article was reported.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Fig 1.

Figure 1.Pulmonary perivascular epithelioid cell tumor imaging studies. (A) Initial plain chest film showing a round, nodular shadow in the right lower lung field (arrow). (B) Pre-contrast computed tomography (CT) view of a round, low-density (30–60 Hounsfield units [HU]) nodule (16 mm) in the right lower lobe (arrow). (C) Post-contrast CT showing a strongly enhanced (110–180 HU), heterogeneous nodule, reflecting intense vascularity (arrow). L-PA, left posterior-anterior.
Journal of Chest Surgery 2023; 56: 367-370https://doi.org/10.5090/jcs.22.139

Fig 2.

Figure 2.Surgical approach to perivascular epithelioid cell tumor of the lung. (A) Ring-shaped forceps were used to ensnare the dark-red, round nodule in the lung, using an endostapler for wedge resection. (B) Incised red-tan tumor, well-demarcated and devoid of necrosis or calcification.
Journal of Chest Surgery 2023; 56: 367-370https://doi.org/10.5090/jcs.22.139

Fig 3.

Figure 3.Histologic attributes of pulmonary perivascular epithelioid cell tumor. (A) Low-power (H&E, ×10) perspective of round and well-circumscribed mass, distinct from normal lung parenchyma. (B) High- power (H&E, ×200) view showing an abundance of round-to-cuboidal cells in a sheet-like arrangement around vessels, with a cytoplasm clear or eosinophilic cytoplasm showing a granular appearance. (C) Positive staining of the tumor marker actin (×40), typically expressed by myocytes, in thin-walled sinusoidal vessels. (D) Focal positivity of HMB-45 (×200), typically expressed by melanocytes, in perivascular epithelioid cells.
Journal of Chest Surgery 2023; 56: 367-370https://doi.org/10.5090/jcs.22.139

There is no Table.

References

  1. Liebow AA, Castleman B. Benign clear cell ("sugar") tumors of the lung. Yale J Biol Med 1971;43:213-22.
    Pubmed KoreaMed
  2. Folpe AL, Kwiatkowski DJ. Perivascular epithelioid cell neoplasms: pathology and pathogenesis. Hum Pathol 2010;41:1-15. https://doi.org/10.1016/j.humpath.2009.05.011.
    Pubmed CrossRef
  3. Vijayabhaskar R, Mehta SS, Deodhar KK, Pramesh CS, Mistry RC. PEComa of the lung. J Cancer Res Ther 2010;6:109-11. https://doi.org/10.4103/0973-1482.63548.
    Pubmed CrossRef
  4. Liang W, Xu S, Chen F. Malignant perivascular epithelioid cell neoplasm of the mediastinum and the lung: one case report. Medicine (Baltimore) 2015;94:e904. https://doi.org/10.1097/MD.0000000000000904.
    Pubmed KoreaMed CrossRef
  5. Ye T, Chen H, Hu H, Wang J, Shen L. Malignant clear cell sugar tumor of the lung: patient case report. J Clin Oncol 2010;28:e626-8. https://doi.org/10.1200/JCO.2010.29.6939.
    Pubmed CrossRef
  6. Olivencia-Yurvati AH, Rodriguez AE. Clear cell "sugar" tumor of the lung: benign or malignant?. Int Surg 2015;100:924-6. https://doi.org/10.9738/INTSURG-D-14-00021.1.
    Pubmed KoreaMed CrossRef
  7. Lim HJ, Lee HY, Han J, Choi YS, Lee KS. Uncommon of the uncommon: malignant perivascular epithelioid cell tumor of the lung. Korean J Radiol 2013;14:692-6. https://doi.org/10.3348/kjr.2013.14.4.692.
    Pubmed KoreaMed CrossRef
  8. Parfitt JR, Keith JL, Megyesi JF, Ang LC. Metastatic PEComa to the brain. Acta Neuropathol 2006;112:349-51. https://doi.org/10.1007/s00401-006-0105-5.
    Pubmed CrossRef
  9. Zhao J, Teng H, Zhao R, et al. Malignant perivascular epithelioid cell tumor of the lung synchronous with a primary adenocarcinoma: one case report and review of the literature. BMC Cancer 2019;19:235. https://doi.org/10.1186/s12885-019-5383-0.
    Pubmed KoreaMed CrossRef
  10. Sobiborowicz A, Czarnecka AM, Szumera-Cieckiewicz A, Rutkowski P, Switaj T. Diagnosis and treatment of malignant PEComa tumours. Oncol Clin Pract 2020;16:22-33. https://doi.org/10.5603/OCP.2020.0003.
    CrossRef
  11. Song Y, Chen F, Zhang C, Lin X. Spindle cell subtype of pulmonary clear cell tumor with prominent calcification and malignant potential. Thorac Cancer 2017;8:530-4. https://doi.org/10.1111/1759-7714.12457.
    Pubmed KoreaMed CrossRef
  12. von Mehren M, Kane III JM, Agulnik M, et al. NCCN Clinical Practice Guidelines in Oncology: soft tissue sarcoma, version 2. National Comprehensive Cancer Network; 2022 [cited 2022 Dec 29]. Available from: https://www.nccn.org/professionals/physician_gls/pdf/sarcoma.pdf.

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