Primary de novo malignant peripheral nerve sheath tumor of the breast mimicking mastitis. Report of a case and review of the literature

Abstract

Malignant peripheral nerve sheath tumor (MPNST) is a rare tumor representing <0.1% of malignant breast tumors. Here, we report a case of sporadic MPNST of the breast in a 45-year-old woman with a fast-evolving painful mass in the right breast associated with edema, redness, and increased local temperature, simulating mastitis. This presentation has not been reported. A review of the literature since 1992 has revealed 15 MPNST cases, including the present one with sufficient data for analysis. All the cases were women. The ages ranged from 16–60 years (mean 40.5 years). Duration of symptoms varied from four days to 15 years (mean 2.7 years). Tumor size ranged from 2.5–30 cm (mean 10.9 cm). 64.3% of tumors were well-circumscribed. Tumors were graded as grade 1 (7.7%), grade 2 (38.5%), and grade 3 (53.8%). Three (20%) cases showed histopathological peculiarities. Simple mastectomy was performed in four (26.7%) cases, radical mastectomy in six (40%) cases, and simple excision in five (33.3%) cases. There were no metastases in the six cases where axillary dissection was performed. Seven (53.8%) patients received adjuvant therapy, including chemotherapy and/or radiotherapy. Local recurrence was observed in two (16.7%) patients. Distant metastasis was detected in one patient (7.7%) affecting the lung 11 months after radical mastectomy. The follow-up was available for 10 patients (mean 2.2 years, median 1 year). One patient (10%) died of the disease. At the last follow-up, 80% of patients were still alive. The main differential diagnosis includes nonpleomorphic spindle cell tumors.

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“…Cutaneous sarcomatoid carcinomas are diagnostically challenging in that they are a very rare entity that can be easily confused with other diseases. A poorly differentiated carcinomatous element may be missed if ductal or glandular structures that hint at an epithelial origin are poorly formed 5,8 . The sarcomatoid carcinoma can potentially be confused with a wide variety of tumors including squamous cell carcinoma, breast ductal carcinoma, other skin adnexal carcinomas, and a variety of other mixed tumors of the skin which is why staining is vital to a correct diagnosis 5,8,9–13 …”

“…It arises more commonly from primary visceral sites such as the uterus, ovaries, breast, bladder, and lungs, and much less commonly as a primary skin lesion 4 . When it does occur as a primary skin lesion, its epithelial component most often occurs as an epidermal‐derived carcinoma or skin adnexal carcinoma 2,5,6 . Epidermal‐derived carcinomas are more common and include basal or squamous cell carcinomas which tend to occur on sun‐exposed skin of the head and neck, involve older patients, and yield more favorable prognoses with 5‐year survival of 70% 2,5,6 .…”

“…When it does occur as a primary skin lesion, its epithelial component most often occurs as an epidermal‐derived carcinoma or skin adnexal carcinoma 2,5,6 . Epidermal‐derived carcinomas are more common and include basal or squamous cell carcinomas which tend to occur on sun‐exposed skin of the head and neck, involve older patients, and yield more favorable prognoses with 5‐year survival of 70% 2,5,6 . Skin adnexal carcinomas are much rarer and include spiradenocarcinoma, malignant proliferating trichilemmal tumors/cysts, porocarcinomas, and pilomatrical carcinomas 2,5,6 .…”

“…Epidermal‐derived carcinomas are more common and include basal or squamous cell carcinomas which tend to occur on sun‐exposed skin of the head and neck, involve older patients, and yield more favorable prognoses with 5‐year survival of 70% 2,5,6 . Skin adnexal carcinomas are much rarer and include spiradenocarcinoma, malignant proliferating trichilemmal tumors/cysts, porocarcinomas, and pilomatrical carcinomas 2,5,6 . These lesions tend to involve younger patients, grow from long‐standing tumor nodules over many years, and are associated with aggressive behavior and poorer prognosis with 5‐year survival of 25% 2,5,6 …”

“…Skin adnexal carcinomas are much rarer and include spiradenocarcinoma, malignant proliferating trichilemmal tumors/cysts, porocarcinomas, and pilomatrical carcinomas 2,5,6 . These lesions tend to involve younger patients, grow from long‐standing tumor nodules over many years, and are associated with aggressive behavior and poorer prognosis with 5‐year survival of 25% 2,5,6 …”