Skip to main content


Log in

Malignant Pigmented Mass “Sequestrated” in the Lung: A Unique Case Report

  • Published:
Lung Aims and scope Submit manuscript


Primary lung tumors arising in pulmonary sequestration is an exceptional event, usually consisting of common histologic types. On the other hand, malignant perivascular epithelioid cell (PEComatous) tumors with deposition of melanin pigment have never been reported in the lung so far. In this study, we report a challenging case of a 34-year-old man presented with recurrent hemoptysis and CT scan detection of a pulmonary mass at the left lower lobe, vascularized by aberrant communication with the left diaphragmatic artery. After surgical resection, we documented a malignant PEComatous tumor (characterized by TFE3 expression and high mitotic rate) that had arisen in the context of an extralobar sequestration.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2


  1. Savic B, Birtel FJ, Tholen W, Funke HD, Knoche R (1979) Lung sequestration: report of seven cases and review of 540 published cases. Thorax 34:96–101

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. Belchis D, Cowan M, Mortman K, Rezvani B (2014) Adenocarcinoma arising in an extralobar sequestration: a case report and review of the literature. Lung Cancer 84:92–95

    Article  PubMed  Google Scholar 

  3. Thway K, Fisher C (2015) PEComa: morphology and genetics of a complex tumor family. Ann Diagn Pathol 19:359–368

    Article  PubMed  Google Scholar 

  4. Malinowska I, Kwiatkowski DJ, Weiss S et al (2012) Perivascular epithelioid cell tumors (PEComas) harboring TFE3 gene rearrangements lack the TSC2 alterations characteristic of conventional PEComas: further evidence for a biological distinction. Am J Surg Pathol 36:783–784

    Article  PubMed  PubMed Central  Google Scholar 

  5. Marine LM, Valdes FE, Mertens RM, Bergoeing MR, Kramer A (2011) Endovascular treatment of symptomatic pulmonary sequestration. Ann Vasc Surg 25(5):696.e11–696.e15. doi:10.1016/j.avsg.2010.08.012

    Article  Google Scholar 

  6. Leoncini G, Rossi UG, Ferro C, Chessa L (2011) Endovascular treatment of pulmonary sequestration in adults using Amplatzer® vascular plugs. Interact Cardiovasc Thorac Surg 12(1):98–100. doi:10.1510/icvts.2010.246546 (Epub 2010 Oct 12)

    Article  PubMed  Google Scholar 

Download references

Author Contribution

All authors contribute to the diagnosis of the patients and wrote the report. Written consent to publication was obtained.

Author information

Authors and Affiliations


Corresponding author

Correspondence to Filippo Lococo.

Ethics declarations

Conflict of interest


Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Mengoli, M.C., Lococo, F., Depenni, R. et al. Malignant Pigmented Mass “Sequestrated” in the Lung: A Unique Case Report. Lung 194, 699–701 (2016).

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: