А. Kens¹, А.-М. Kens²

¹Clinical Hospital of Lviv Railway

²Danylo Halytsky Lviv National Medical University

Introduction.  Tumors of the peripheral nervous system (neurofibromatosis of the first type) described at the end of the XIX century by german pathologist Frederick Daniel von Recklinghauen. The most common manifestations of the disease are spots of “coffee with milk”, freckles, multiple subcutaneous neurofibroma, among the lesions of the organs – the thymus of the optic nerve [2, 3, 6]. The probability of malignant transformation by the neurofibromas, according to researchers [4, 5], varies from 15.0 to 30.0%. In modern literature, much information is devoted to skin and subcutaneous manifestations of the disease, much less – organ failure [2, 4, 5]. In the literature available to us, we did not come across descriptions of Plexiform neurofibroma with localization in the mammary gland. 

The aim of the study.  Describe a clinical case of malignant neurofibroma (neurogenic sarcoma) of the mammary gland. 

Materials and methods of research.  The clinical case of malignant neurofibroma (neurogenic sarcoma) of the mammary gland has been described, with the interpretation of the results of the examinations and treatment. 

Results of the research and their discussion. The clinical course of plexiform neurofibroma with localization in the mammary gland, the results of visualization diagnostic methods (ultrasonography, mamography) and morphologic (cytological and histological) biopsy studies led to suspect the patient’s presence of a whole spectrum of possible diseases (malignant fibroadenoma, myofibroblastoma, nodular fasciitis). The histologic examination of a completely removed tumor has made it possible to put the right diagnose and choose the right therapeutic approach.

On July 26, 2016, during a planned mammalogical examination, a tumor of the left breast, rounded, in a capsule, up to 4.0 cm in diameter (Fig. 1) was detected in a woman for 53 years.  Fine needle aspiration biopsy of the tumor was performed under ultrasound imaging.  A cytologic study of biopsy revealed the presence of a fibroadenoma with a possible transformation to cancer. The patient was offered an excisional biopsy in the amount of a sectoral resection of the left breast for histological examination – the patient refused and for a long time did not consult a doctor. 

Fig. 1. Ultrasound of the tumor, diagnosed during the first survey.

On July 10, 2017, the patient turned for the help of reconsent and agreed to the surgical treatment of the tumor. At the time of the second examination, the tumor of the multicellular structure covered almost the entire breast (Fig. 2-4). 

Fig. 2. Clinical picture.

Fig. 3. Ultrasound.

Fig. 4. Mamorage of the left breast in two projections.

In view of intensive tumor growth, dubious operability and suspicion of malignant transformation, according to the results of a cytologic study, the core biopsy was performed with a histopathologic study of the biopsy speciment. Histologic conclusion: the tumor is represented by spindle-shaped cells with eosinophilic cytoplasm and ovarian nuclei without expressed polymorphism and mitotic activity, forming beam structures in the collagenized stroma. In one of the biopsy speciments the tumor is separated from the breast tissue (without signs of infiltrative growth). The morphological structure most closely matches the breast myofibroblastoma. A similar morphological structure is possible in the presence of nodular fasciitis. As the tumor affected almost the whole of the mammary gland, a simple mastectomy was chosen as an adequate volume of surgical treatment, which was performed under common anesthesia on August 3, 2017. The macroscopic study of the removed breast revealed a number of tumors of varying sizes separated from the glandular tissue with a pronounced capsule, and significant polymorphism of the tumors in the cut (Fig. 5, 6).

Fig. 5. Macroscopic appearance of the tumor.

Fig. 6. Tumor in the cut: pronounced polymorphism.

Histologic examination of the removed tumor: the mammary gland is almost completely replaced by a clearly constrained tuberclelike – ncapsulated benign tumor of nerve fibers – neurofibroma, with multiple concentric structures by type of tactile cells, myxomatosis, gialinosis. Due to some differences in the interpretation of the results of the histopathologic study of core biopsy and the surgical material, the speciments were reviewed at another institution.Diagnosis of neurofibroma has been confirmed, but it has been emphasized on significant mitotic activity in some tumor sites, which gives grounds to consider it as a malignant tumor of the peripheral nerve fibers, presumably on the basis of neurofibroma. Postoperative flow without features, the wound was healed by the primary tension, the patient was discharged under an outpatient observation. Since the affected organ was removed from body 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. Expressed macroscopic tumor polymorphism during histological examination may explain the discrepancy in the tumor evaluation based on the results of the biopsy study. 

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