Lviv clinical bulletin 2018, 1(21)-2(22): 57-59

https://doi.org/10.25040/lkv2018.01.057

Malignant Neurofibroma (Neurogenic Sarcoma) of the Mammary Gland (Clinical Case Description)

Posted onAuthorAdminЗалишити коментар / Leave a comment

А. Kens1, А.-М. Kens2

1Clinical Hospital of Lviv Railway

2Danylo Halytsky Lviv National Medical University

Background. In the modern literature, much information is devoted to skin and subcutaneous manifestations of neurofibromatosis, much less – to organs’ failure. In the literature available to us, we did not come across the descriptions of plexiform neurofibroma (neurogenic sarcoma) with localization in the mammary gland. The absence in available for authors literature description of malignant neurofibroma of such localization makes this observation quite interesting for professionals.

Aim. To describe the clinical case of malignant neurofibroma (neurogenic sarcoma) of the mammary gland in the patient.

Materials and Methods. The clinical case of malignant neurofibromy (neurogenic sarcoma) of the mammary gland is described with the interpretation of the results of examinations and proposed treatment.

Results. The clinical examination of the disease, the data of the visualization methods of diagnosis (ultrasonography, mammography) and the morphological (cytological and histological) study of biopsy samples gave grounds to suspect the patient’s presence of a wide range of possible diseases: malignant fibroadenoma, myofibroblastoma, nodular fasciitis. The histological examination of the completely removed tumor has made it possible to set the right diagnose and correct the medical tactics. Postoperative period was without any complications, the wound was healed by the primary tension, the patient was discharged under the outpatient observation. Since the patient had the affected organ removed in its anatomical limits, the centers of mitotic activity were located within the “capsule” of the tumor, no therapeutic or adjuvant measures were proposed to the patient.

Conclusions. Malignant neurofibroma of the mammary gland is a disease whose diagnosis is difficult. Only a histological examination of the completely removed tumor made it possible to establish the correct diagnosis, and pronounced macroscopic polymorphism became an explanation for such a discrepancy in the evaluation of the tumor according to the data of the study of biopsy.

References

  1. Abernathy CR, Rasmussen SA, Stalker HJ, Zori R, Driscoll DJ, Williams CA et al. NF1 mutation analysis using a combined heteroduplex/SSCP approach. Hum Mutat. 1997;9:548-554. https://doi.org/10.1002/(SICI)1098-1004(1997)9:6<548::AID-HUMU8>3.0.CO;2-Y
  2. Boyd KP, Korf BR, Theos Neurofibromatosis type 1. J Am Acad Dermatol. 2009;61(1):1-14. https://doi.org/10.1016/j.jaad.2008.12.051
  3. Klesse L, editor. Current Therapies for Neurofibromatosis Type Denver: Mountain States Genetic Foundation, 2010.
  4. Evans, Rosalie E. Ferner, Susan M. Huson, D. Gareth R. Neurofibromatoses in clinical practice. London: Springer, P. 1.
  5. Kluwe L, Tatgiba M, Fünsterer C, Mautner V-F. NF1 mutations and clinical spectrum in patients with spinal neurofibromas. J Med Genet. 2003;40:368-371. https://doi.org/10.1136/jmg.40.5.368
  6. Viskochil D, editor. Neurofibromatosis 1: Current Issues in Diagnosis, Therapy, and Patient Management. Denver: Mountain States Genetic Foundation, 2010.