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

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.

Acquired Elastotic Hemangioma-like Change of the Vulva Associated With Lichen Sclerosus.

Acquired elastotic hemangioma (AEH) is a rare variant of hemangioma that usually presents as an asymptomatic, solitary, slow-growing red plaque on a sun-exposed site of an adult. Ultraviolet radiation can contribute to the pathogenesis of this hemangioma. Lichen sclerosus (LS), a scarring disease, may present a prominent accumulation of elastic fibers in the reticular dermis reflecting a reparative process. Vulvar elastosis, a novel diagnostic entity with fibers similar to solar elastosis, is more common in women 45 yr and older and is related to aging and/or hormonal changes. We herein report for the first time a case of AEH-like change located in the vulva, a sun-protected area, associated with genital LS. An 81-yr-old woman presented with a painful vulvar lesion of 1-yr duration. Clinical examination revealed vulvar LS and 1 cm-flat, erythematous, well-defined plaque with increased consistency located on the left labium minus. Histopathology showed a non-neoplastic proliferation of WT1-positive, small vascular channels surrounded and intertwined by intense elastosis in the reticular dermis. Alpha-smooth muscle actin positive pericytes encircled the vascular channels. The lesion can be understood as a reparative process within an LS with the appearance of epidermal hyperplasia, proliferation of small vascular channels, and hyperplastic elastosis. There is a close link between epidermal hyperplasia and angiogenesis in the formation of this reparative lesion. Recognition of this lesion is crucial to avoid confusion with other significant processes especially Kaposi sarcoma and well-differentiated angiosarcoma.

Myofibroblastic sarcoma of the breast. Report of a case induced by radiotherapys.

Myofibroblastic sarcoma (MFS) is an uncommon tumor rarely located in the breast. Ionizing radiation is a carcinogen capable of inducing sarcomas through DNA damage. A 42-year-old woman was diagnosed with synchronous bilateral breast infiltrating ductal carcinoma with axillary lymph node metastases on the left side. After modified left radical mastectomy and simple right mastectomy, she underwent postoperative radiation with a total volume dose of 50 Gy that included the thoracic wall and the left axillary-supraclavicular region. After a latency period of 6 years and 4 months, the patient developed an MFS in the area of radiation (mammary upper outer quadrant). To our knowledge, only 11 cases of MFS have been previously published in the breast. The study of the 12 cases including the present one revealed that the ages of the patients ranged from 42 to 86 years (mean 60.3 years). There was a clear difference concerning sex (M:F, 1:5). The average duration of the lesion varied from 1 week to 8 months (mean 3.3 months). The size ranged from 2.2 to 22 cm (average 5.1 cm). The tumors showed frequent mitosis and areas of necrosis. The percentage of recurrences, metastasis, and death due to the tumor was 27.3%, 36.4%, and 27.3% respectively. MFS cases differ from those affecting extramammary regions. They are more common in females and show a greater degree of aggressiveness. Correct diagnosis of mammary MFS requires morphological and immunohistochemical study. We present for the first time a case of MFS of the breast induced by radiotherapy.

Incidental, low-fat variant of spindle cell lipoma: a novel tumour of the small intestine.

A woman underwent surgical intervention for a carcinoma of the ovary. In the intervention, a submucosal nodule of the ileum was found. Pathological study revealed a spindle cell lipoma (SCL). This case revealed the presence of CD34-positive spindle and stellate cells with dendritic cytoplasmic prolongations, a feature shared with dendritic fibromyxolipoma. Fluorescence in in situ hybridisation analysis showed 13q14 heterozygous deletion. Spindle cell lipoma of the small intestine has not been previously reported. Spindle cell lipoma, although rare, should be included among the benign mesenchymal lesions of the small intestine. This report extends the range of locations in which this tumour is found to arise.

Oncoplastic breast conserving surgery with tailored needle-guided excision.

BACKGROUND: Breast conserving surgery (BCS) administered with oncoplastic approach (OBCS), when it is required, is currently the gold standard for the treatment of early breast cancer. Wire-guided localization (WL) is the most popular technique used to help surgeon in breast cancer excision. Currently, a universal and undeniable goal is to minimize the rate of positive margins and re-excision operation after BCS improving cosmetic outcome and decreasing health care costs. This study is aimed to report our experience combining OBCS and tailored WL as surgical approach for early breast cancers. METHODS: We performed a retrospective study on 148 breast cancer patients who were treated with OBCS and tailored WL, which consists in individualization of the number and location of wires depending on patient particularities, in our Breast Unit from March 2013 to December 2015. A multivariate analysis was used to determine the association between clinic-pathologic variables, which can be known preoperatively, and margin status. RESULTS: The rate of affected margins was 13.5% and 10.8% patients underwent re-interventions for oncologic reasons. Multifocality was strongly associated with involved margins [odds ratio (OR) 4.67]. CONCLUSIONS: OBCS together with tailored WL obtains an acceptable rate of positive margins and high rate of final BCS.

Vulvar eccrine porocarcinoma: report of a case and literature review.

Adnexal carcinomas of the vulva are rare tumors. A case of an 83-year-old woman with a 3.5 cm vulvar lobulated mass that grew over an 18-month period is reported. Histopathological examination of the initial biopsy revealed a poorly differentiated infiltrating carcinoma. Treatment included radical vulvectomy with bilateral inguinal lymphadenectomy. The histological examination of the vulvectomy specimen resulted in eccrine porocarcinoma (EPC) diagnosis. A review of the literature disclosed eight vulvar cases previously reported. The study of the nine cases including the present revealed that the ages of the patients ranged from 32 to 88 years, with a mean of 66.1 years. The location of the tumor was most common in the labium majus. The size ranged from 2 cm to 5 cm (mean 3.3 cm). A longstanding history suggested that at least two (22.2%) tumors arose from a preexistent benign eccrine poroma. Considering the reduced prevalence of EPC, the diagnosis of this type of tumor is challenging. The follow-up varied from six to 132 months (mean 35.2 months). Two (22.2%) tumors recurred, three (33.3%) patients developed regional lymph node metastases, and two (22.5%) patients showed distant metastases. Only one patient died of the disease, two patients remained alive with tumor, and four (44.4%) patients showed no evidence of disease. Although a rare entity, EPC should be considered in the differential diagnosis of a vulvar mass. In the vulva, it is difficult to establish the clinicopathological predictors of prognosis of EPC. However, the markers of aggressiveness at extragenital sites may also apply to this vulvar tumor.

Dermal plexiform spindle cell lipoma.

Spindle cell lipoma located in the dermis is uncommon. The plexiform variant of this tumor is rare. In fact, only six cases of this variant have been described previously. We report herein a case of dermal plexiform spindle cell lipoma with prominent myxoid matrix. A 47-year-old male patient presented with a solitary, 2.2 cm-cutaneous mass in the right buttock region that had slowly increased in size for over one year. The dermal lesion was characterized by a mixture of mature adipocytes, spindle shaped cells and inconspicuous ropey collagen bundles in a mucinous background. This lesion showed a fascicular and plexiform pattern with adipocytes irregularly arranged, predominant in the depth of the lesion. Immunohistochemically, the spindle cells were positive for CD34, factor XIIIa, and vimentin, and negative for retinoblastoma protein, claudin-1, GLUT-1, epithelial membrane antigen, neurofilament protein, and Sox-10. S100 protein stained a thinned cytoplasmic rim of mature adipocytes and labeled about 25% of spindle cells in the most superficial areas. A review of the seven cases published, including the present report, revealed that there were five females and two males. Most cases located in the thigh-groin-buttock area. The age of the patients ranged from 32 to 58 years with a mean of 45.7 years. Clinical diagnosis suggested a lipomatous or neural tumor in six cases. The main differential diagnosis includes dermal intraneural plexiform neurofibroma and purely intradermal monophasic plexiform spindle cell nevus.

Histopathologic differences between cutaneous vasculitis associated with severe bacterial infection and cutaneous vasculitis secondary to other causes: study of 52 patients.

OBJECTIVES: To determine if cutaneous vasculitis (CV) associated with severe infection has some histopathologic findings that may help us to differentiate patients with this condition from other patients with CV. METHODS: We reviewed the skin biopsy specimens of patients with leukocytoclastic CV associated with a severe bacterial infection. Histopathologic findings of these patients were compared with those observed in leukocytoclastic CV secondary to other causes. Biopsy-proven leukocytoclastic CV were stratified as follows: group a): CV associated with severe underlying bacterial infection; group b): CV without severe bacterial infection but with systemic involvement; group c): CV without systemic involvement. Slides were reviewed by expert pathologists that were blind to the clinical information. The severity of vascular lesions was measured according to a semiquantitative scale (Hodge index). A comparative study between group a) and the other groups was conducted. RESULTS: group a) included 12 patients (2 women/10 men), mean age± SD 56±15 years; group b) 21 patients (10 women/11 men), 52±18 years; and group c) 19 patients (12 women/7 men), 59±24 years. Presence of neutrophilia was significantly increased in biopsies from group a) when compared with the other two groups. Also, a trend to higher frequency of pustular dermatosis was found in patients from group a). Hodge index, degree of inflammatory infiltrate and deep arterioles involvement were similar in all groups. CONCLUSIONS: Neutrophilia is common in skin biopsies of patients with CV associated with severe bacterial infection. No other histopathological findings help us to establish the presence of a severe underlying infection.

Arteriovenous malformation of the uterine cervix.

A uterine arteriovenous malformation (AVM) is an uncommon cause of uterine bleeding. Location of this lesion in the uterine cervix is exceptional. We report a case of a 34-year-old woman who presented with chronic menorrhagias and hypochromic anemia. A sonographic study revealed a 10-cm, fundal, intramural, uterine well-circumscribed mass that distorted the endometrial cavity. The patient underwent hysterectomy for a large uterine leiomyoma. The pathological study revealed an incidental AVM of the posterior half of the cervix measuring 5.5 cm in major diameter. We suggest that in our case cervical AVM might have occurred due to a large corporal leiomyoma distorting the uterine circulation. Differential diagnosis includes capillary hemangioma, venous malformation, or arteriovenous fistula.

Pilonidal sinus associated with cellular blue nevus. A previously unrecognized association.

A neoplasm associated with a pilonidal sinus (PS) is a rare occurrence in the course of a common disease. Early detection is imperative. To our knowledge, pilonidal disease associated with a cellular blue nevus (CBN) has not been reported. There is a 10% diagnostic error rate with this last lesion. Here we report the case of a 19-year-old man with recurrent sacrococcygeal PS infection associated with an indurated dome-shaped blue-black nodule 1.7 cm in diameter. Clinical diagnosis of the nodule was uncertain suggesting a pigmentary or a vascular tumor. A complete resection of the two lesions was achieved. Pathological study showed a CBN showing a predominantly alveolar pattern associated with a chronic pilonidal disease. The tumor cells showed diffuse strong reactivity for melan-A and HMB-45, and focal reactivity for S-100 protein. Staining for Ki-67 (MIB1) was virtually negative. Differential diagnoses included atypical CBN, borderline melanocytic tumor and malignant melanoma. Radical excision provides a good prognosis for the rare association consisting of a common disease such as PS with the uncommon CBN.