Glomangiosarcoma Explained

Glomangiosarcoma
Synonyms:Malignant glomus tumor
Field:Dermatology

Glomangiosarcoma is a low grade[1] tumor of the soft tissue. They rarely metastasize,[2] but metastases are possible.[3] It is also known as malignant glomus tumor.[4] Positive staining for vimentin has been reported.[5]

Signs and symptoms

Glomangiosarcoma usually manifest as painful blue-red dermal nodules that range from 0.5-2cm in size.[4]

Causes

Glomangiosarcomas can arise de novo or from a preexisting benign glomus tumour.[6]

Diagnosis

Typically, a definitive diagnosis cannot be made based solely on radiologic findings and clinical symptoms.[7] To get a conclusive diagnosis of glomangiosarcoma, immunohistochemical staining in addition to hematoxylin and eosin staining is needed.[8]

Histologically, glomangiosarcomas differ from benign glomus tumors in that they include sheets of uniform, round to oval cells with eosinophilic cytoplasm, many vascular spaces, and cellular pleomorphism linked to frequent mitotic figures.[6] [9]

Glomangiosarcomas display many of the same antigens as their benign counterparts on immunohistochemical labeling; these antigens include vimentin, smooth muscle actin, and muscle-specific actin.[10] On the other hand, benign tumors stain more highly for actin and myosin, and malignant tumors stain more intensely for vimentin.[11] Studies conducted in the past have also revealed changes in the expression of Bcl-2 and p53 in comparison to benign glomus tumors.[3] [12]

These lesions in soft tissue and skin need to be distinguished from other cutaneous round cell malignancies such hemangiopericytoma, leiomyosarcoma, melanoma, and Merkel cell carcinoma.[4] [13]

Treatment

Although Mohs micrographic surgery has been previously described, the standard of care for malignant glomus tumors still involves wide local excision with negative surgical margins.[4] It is not currently advised to use adjuvant chemotherapy for primary glomangiosarcoma.[14]

See also

Further reading

Notes and References

  1. Pérez de la Fuente T, Vega C, Gutierrez Palacios A, Sanchez Lorenzo J, Gonzalez Sarasua J . Glomangiosarcoma of the hypothenar eminence: a case report . Chirurgie de la Main . 24 . 3–4 . 199–202 . 2005 . 16121631 . 10.1016/j.main.2005.06.006.
  2. Park JH, Oh SH, Yang MH, Kim NI . Glomangiosarcoma of the hand: a case report and review of the literature . The Journal of Dermatology . 30 . 11 . 827–33 . November 2003 . 14684942 . 10.1111/j.1346-8138.2003.tb00486.x . 21334564 . 2008-10-17 . https://web.archive.org/web/20110722120747/http://www.dermatol.or.jp/Journal/JD/2003/030110827.html . 2011-07-22 . dead .
  3. Watanabe K, Sugino T, Saito A, Kusakabe T, Suzuki T . Glomangiosarcoma of the hip: report of a highly aggressive tumour with widespread distant metastases . . 139 . 6 . 1097–101 . December 1998 . 9990381 . 10.1046/j.1365-2133.1998.02574.x . 34576130 .
  4. Kayal JD, Hampton RW, Sheehan DJ, Washington CV . Malignant glomus tumor: a case report and review of the literature . Dermatologic Surgery . 27 . 9 . 837–40 . September 2001 . 11553174 . 10.1097/00042728-200109000-00011 .
  5. Hiruta N, Kameda N, Tokudome T, etal . Malignant glomus tumor: a case report and review of the literature . The American Journal of Surgical Pathology . 21 . 9 . 1096–103 . September 1997 . 9298887 . 10.1097/00000478-199709000-00015.
  6. EW . Gould . JC . Manivel . J . Albores-Saavedra . H . Monforte . Locally infiltrative glomus tumors and glomangiosarcomas. A clinical, ultrastructural, and immunohistochemical study . Cancer . 1990 . 65 . 2 . 310–318 . 0008-543X . 2153045 . 10.1002/1097-0142(19900115)65:2<310::aid-cncr2820650221>3.0.co;2-q . 2024-04-27 .
  7. Alhroub . Omar A . Mahameed . Shimaa A . Abdelhafez . Mohammad O . Alhroub . Asil . Hour . Hani . Hasasna . Nabil . Kamal . Nazmi . A case of malignant glomus tumor (glomangiosarcoma) of the nasal cavity . Journal of Surgical Case Reports . 2022 . 1 . 2022-01-01 . rjab641 . 2042-8812 . 35096369 . 8791658 . 10.1093/jscr/rjab641 .
  8. Wang . Yingjie . Xiang . Yongbo . Bian . Yanyan . Feng . Bin . Liu . Yong . Zhou . Xi . Zhou . Lizhi . Weng . Xisheng . Glomus tumors associated with the bone and joints: a review of 91 cases . Annals of Translational Medicine . AME Publishing Company . 8 . 21 . 2020 . 2305-5839 . 10.21037/atm-20-6998 . free . 1460. 33313205 . 7723542 .
  9. Folpe . Andrew L. . Fanburg–Smith . Julie C. . Miettinen . Markku . Weiss . Sharon W. . Atypical and Malignant Glomus Tumors . The American Journal of Surgical Pathology . Ovid Technologies (Wolters Kluwer Health) . 25 . 1 . 2001 . 0147-5185 . 10.1097/00000478-200101000-00001 . 1–12. 11145243 .
  10. Maselli . Amy M. . Jambhekar . Amani V. . Hunter . John G. . Glomangiosarcoma Arising from a Prior Biopsy Site . Plastic and Reconstructive Surgery — Global Open . Ovid Technologies (Wolters Kluwer Health) . 5 . 1 . 2017 . 2169-7574 . 10.1097/gox.0000000000001219 . e1219. 28203514 . 5293312 .
  11. M . Aiba . A . Hirayama . S . Kuramochi . Glomangiosarcoma in a glomus tumor. An immunohistochemical and ultrastructural study . Cancer . 1988 . 61 . 7 . 1467–1471 . 0008-543X . 2449949 . 10.1002/1097-0142(19880401)61:7<1467::aid-cncr2820610733>3.0.co;2-3 . 2024-04-27 .
  12. Hegyi . L. . Cormack . G. C. . Grant . J. W. . Histochemical investigation into the molecular mechanisms of malignant transformation in a benign glomus tumour . Journal of Clinical Pathology . BMJ . 51 . 11 . 1998-11-01 . 0021-9746 . 10.1136/jcp.51.11.872 . 872–874. 10193335 . 500988 .
  13. Binesh . F. . Akhavan . A. . Zahir . S. T. . Bovanlu . T. R. . Clinically malignant atypical glomus tumour . Case Reports . BMJ . 2013 . jan03 1 . 2013-01-03 . 1757-790X . 10.1136/bcr-2012-007618 . bcr2012007618. 23291816 . 3603905 .
  14. Oh . Stephen D. . Stephenson . Daniel . Schnall . Stephen . Fassola . Isabella . Dinh . Paul . Malignant Glomus Tumor of the Hand . Applied Immunohistochemistry & Molecular Morphology . Ovid Technologies (Wolters Kluwer Health) . 17 . 3 . 2009 . 1541-2016 . 10.1097/pai.0b013e31818a9d5d . 264–269. 18997618 .

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