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Hindawi Case Reports in Oncological Medicine Volume 2022, Article ID 4042298, 6 pages https://doi.org/10.1155/2022/4042298 Case Report Primary Eccrine Porocarcinoma of the Breast: A Case Report and Review of Literature 1 2 3 Yi Xin Li , Mihir Gudi, and Zhiyan Yan Duke-NUS Medical School, Singapore Department of Pathology and Laboratory Medicine, KK Women's and Children’s Hospital, Singapore Breast Department, KK Women’s and Children’s Hospital, Singapore Correspondence should be addressed to Zhiyan Yan; firstname.lastname@example.org Received 5 January 2022; Revised 4 April 2022; Accepted 6 May 2022; Published 31 May 2022 Academic Editor: Katsuhiro Tanaka Copyright © 2022 Yi Xin Li et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Eccrine porocarcinoma (EPC) is a rare cutaneous neoplasm, with less than 500 reported cases worldwide since it was ﬁrst described in 1963. EPC tends to aﬀect the elderly and most commonly aﬀects the head and neck. The mainstay of EPC treatment is surgery, with lymphadenectomy in the case of nodal involvement or presence of unfavourable characteristics. No evidence exists to guide the use of adjuvant chemotherapy or radiation. EPC is prone to misdiagnosis given its multiple clinical and histopathological mimics, especially in uncommon sites of presentation such as the breast. Herein, we report the case of a 59-year-old woman who presented with a left breast skin lump. The biopsied specimen revealed an inﬁltrative carcinoma involving the dermis and epidermis with positive IHC staining for P63 and CK5/6 and negative staining for ER, PR, and HER2. The tumour was resected and diagnosed as EPC with atypical features as overlapping characteristics of squamous cell carcinoma (SCC) were detected on histopathological analysis. In our case, a simple mastectomy with broad margins and axillary lymph node dissection with adjuvant radiotherapy to a dose of 60 Gy failed to achieve loco-regional control with nodal recurrence occurring 4 months postsurgery—a testament to the aggressive course of this rare malignancy. 1. Introduction the clinical presentation and histopathological ﬁndings and review the pertinent literature. Eccrine porocarcinoma is a rare, invasive, and highly aggres- sive cutaneous tumour originating from the intraepidermal 2. Case Report eccrine sweat duct (acrosyringium), ﬁrst described by Pinkus and Mehregan in 1963 . Its exact prevalence is unknown A 59-year-old woman with a positive family history of breast but estimated to constitute 0.005% to 0.01% of all skin can- cancer presented with a superﬁcial left breast lump of 5 cers . EPC has no predisposition for ethnicity or gender months duration without nipple discharge. Her preexisting and tends to occur in older people in the sixth to seventh medical conditions were hypertension, dyslipidemia, and decade of life although there is a wide distribution of pre- diabetes. Examination revealed a 3×3 cm raised polypoid senting ages ranging from 7 months to 97 years . Surgical lesion at the left periareolar region with palpable left axillary resection remains the mainstay treatment of choice by gen- lymphadenopathy (Figure 1(a)). Figure 1(b) shows the axil- eral consensus with limited evidence on the beneﬁt of adju- lary lymph node dissection specimen. vant radio- or chemotherapy . To our knowledge, only 3 Bilateral mammography revealed a 3.7 cm circumscribed cases of primary EPC in the breast have been reported opacity at the left periareolar region (Figure 2(a)), while [4–6]. We present a rare case of primary breast EPC in a ultrasonography showed a 3:3×3:0×0:9 cm oval hypo- 59-year-old woman with lymph node metastasis and discuss echoic mass (Figure 2(b)) with left axilla lymphadenopathy 2 Case Reports in Oncological Medicine (a) (b) Figure 1: (a) Clinical appearance of the breast lesion. (b) Clinical appearance of the axillary dissection specimen. (Figure 2(c)). Punch biopsy demonstrated an inﬁltrative car- 3. Discussion cinoma involving the dermis and epidermis. Immunohisto- chemical (IHC) staining was P63 and CK5/6 positive and EPC is prone to misdiagnosis given its variable presenta- mammaglobin, estrogen receptor (ER), progesterone recep- tions. Clinically, EPC can present as ulcerating polypoid, tor (PR), and HER-2 negative. Core biopsy conﬁrmed posi- exophytic growths, or verrucous plaques and be misdiag- tive nodal involvement while Computed Tomography (CT) nosed as SCC, Bowen’s disease, seborrheic keratosis, or pyo- imaging did not detect metastasis. These ﬁndings suggested genic granuloma; histopathologically, EPC also has to be a primary skin cancer instead of breast carcinoma. distinguished from basal cell carcinoma, hidradenocarci- Our patient underwent a simple mastectomy with 4 cm noma, and most importantly SCC . gross margins as well as an axillary lymph node dissection In our case, additional clinical mimics such as an inva- up to level II (Figure 1(b)). Macroscopically, the tumour sive mammary ductal carcinoma had to be considered as was continuous with the epidermis with subcutis and breast well. However, triple assessment pointed towards a primary parenchyma invasion; its maximal diameter and thickness skin neoplasm instead of breast carcinoma. Diﬀerentials were 6 cm and 1.3 cm, respectively. 12 out of 17 lymph nodes were narrowed to either a sweat gland tumour or squamous were positive for metastasis with extranodal extension pres- cell carcinoma (SCC), its main histologic mimic. On histo- ent in several involved nodes. The largest nodal metastatic logical analysis, the absence of visible ductal lumina is focus measured about 4.5 cm. The surgical margins were uncharacteristic of EPC while the remarkably uniform “por- tumour-free. oid” appearance of tumour cells is highly unusual for SCC. Histologically, the tumour was poorly diﬀerentiated, IHC staining result was equivocal for either diagnosis consisting of inﬁltrative islands (Figure 3(a)) of solid sheets, although negative CK7 and CEA staining is uncommon in nests, and cords of rounded to polygonal cells surrounded by EPC . This atypical case demonstrates the utility of iden- desmoplastic stroma. Areas of necrosis (Figure 3(b)) and tifying additional IHC stains to diﬀerentiate EPC from SCC keratinisation were observed together with nuclear pleomor- (e.g., S-100, CD117, CK19, and nestin) given their treatment phism and conspicuous mitoses (Figure 3(c)). Lymphovas- diﬀerences which impact prognosis and survival [9, 10]. On cular invasion was identiﬁed (Figure 3(d)). Ductal and the overall, given the tumour’s morphology which was more glandular structures were not identiﬁed. IHC staining was suggestive of EPC instead of SCC, our patient was diagnosed negative for CK7, CEA, ER (Figure 4(a)), PR, and HER2, with EPC and the diagnosis is supported by the tumour’s focally positive for EMA, P16, and GATA3 (Figure 4(b)), highly aggressive nature given its early recurrence despite and diﬀusely positive for CK5/6 (Figure 4(c)) and p63. intensive treatment. The diagnosis of EPC with atypical features of SCC was The etiology behind EPC development is unknown. made, and subsequently, the patient underwent 6 weeks of Suggested risk factors include exposure of skin to burns, adjuvant radiotherapy (60 Gy in 30 fractions) to the left trauma or radiotherapy, immunosuppression, and pro- axilla and chest wall 2 months postsurgery. The dose and longed exposure to ultraviolet light which were all absent fractionation were extrapolated from treatment guidelines in our patient . EPC can arise de novo or via malig- for head and neck SCC given that SCC features were present nant transformation of a benign eccrine poroma usually in this case . However, CT imaging detected nodal recur- over many years . Reportedly, up to 18% to 50% of rence 2 days before the last dose of radiotherapy. The patient EPCs degenerate from initially benign poromas . Unlike rejected reexcision and palliative chemotherapy and is man- poromas which primarily present in regions with high aged supportively at 1-year follow-up. densities of eccrine sweat glands (palmoplantar regions), Case Reports in Oncological Medicine 3 (a) (b) (c) Figure 2: (a) Mediolateral oblique mammogram of the left breast showing a circumscribed periareolar opacity (circle) and dense axillary lymph node (arrow). (b) Superﬁcial hypoechoic nodule on left breast sonography. (c) Lymphadenopathy on left axilla sonography. EPC occurs rarely in these regions and more frequently at [4–6]. An overview of these patient characteristics and the head and neck followed by lower extremities . How- treatment outcomes are summarised in Table 1 which ever, EPC can aﬀect any body part, and to our knowledge, largely correlates with our understanding of EPC presenta- only 3 cases of primary breast EPC have been reported tion and aggressive natural history. The histopathological 4 Case Reports in Oncological Medicine (a) (b) (c) (d) Figure 3: Tumour H&E sections: (a) skin-based carcinoma showing islands of inﬁltrating tumour (×40); (b) focus of central necrosis (×100); (c) cytoplasmic clearing within tumour islands with nuclear pleomorphism (×100); (d) focus of lymphovascular invasion (×200). and immunohistochemical features in these cases were also and metastasis [15, 16]. In cases of recurrent and/or metas- typical unlike in our case where diagnosis of EPC was not tatic EPC, individual reports have demonstrated the possi- as straightforward. bility of partial or complete remission with adjuvant There is no standard guideline for EPC treatment. How- chemotherapy such as docetaxel and 5-ﬂuorouracil [6, 17, ever, the ﬁrst-line approach by consensus is surgical resec- 18]. Unconventional treatments have also been utilised in tion either with Mohs Micrographic Surgery (MMS) or recurrent and/or metastatic EPC such as immunotherapy more commonly a Wide Local Resection (WLE) with broad (interferon alpha, interleukin 2, and pembrolizumab), iso- margins (>2 cm), without which prognosis and survival rates tretinoin, electrochemotherapy, intralesional photodynamic are signiﬁcantly poorer . The curative rate of WLE is therapy, CyberKnife radiosurgery, and hormonal treatment reportedly 80% with a 20% probability of local or nodal (tamoxifen) in the case of positive ER/PR receptors with var- recurrence despite negative excision margins as realised in iable success [19–25]. our case . Recent research may shed light on the high rates of The beneﬁt of adjuvant radio and chemotherapy in EPC recurrence in EPC even with adjuvant treatment—Taghizadeh-Hesary et al. highlighted the impor- remains unproven. Le et al. reported that adjuvant radio or chemotherapy did not signiﬁcantly improve prognosis or tance of mitochondria’s role in cancer metabolism such as overall survival in their meta-analyses of 120 EPC head mediating eﬄux pump expression and scavenging reactive and neck cases . However, there may be a role for adju- oxygen species (ROS) which confers resistance to chemo- vant radiotherapy in EPC with positive resection margins or and radiotherapy . A stronger functional mitochondrial presence of poor prognostic features (as “inﬁltrative” or status may explain why some cancers such as EPC are highly “pushing” subtypes of EPC, >14 mitoses per high-power recurrent and resilient. Further research into antimitochon- ﬁeld, lymphovascular invasion, and tumoural involvement drial therapy may thus have potential in improving treat- >7 mm) as in our case, given the higher risk of recurrence ment outcomes for these cancers. Case Reports in Oncological Medicine 5 GATA3 ER (a) (b) CK5/6 (c) Figure 4: Immunohistochemistry analysis results: (a) ER (-); (b) GATA 3 (+); (c) CK5/6 (+). Table 1: General characteristics, EPC histopathological and immunohistochemical features, treatment modalities, and outcomes in terms of response or survival. Site and Lymph First Age/ largest node/ Histopathological and author Treatment received Treatment outcome sex dimension distant IHC features of EPC and (mm) metastasis year Right Palliative care, adjuvant therapy Bonito 92/F breast, + n.a. Surgery (mastectomy) rejected (2020) 60 mm Inﬁltrative subtype with squamous diﬀerentiation and Right lymphovascular Overall survival of 3.3 years; ﬁrst breast, invasion Surgery (WLE) followed by recurrence 22.5 months after initial Morten 74/F 45 mm — Negative postop reexcision (WLE) and resection and patient died 17.5 months (2018) (ﬁrst margins adjuvant RT (66 Gy) after reexcision and adjuvant RT recurrence) IHC positive for p63, CK5, and EMA and negative for ER and PR Unknown subtype Surgery (WLE) followed by IHC positive for AE1/ Local recurrence and metastasis 1 year 54/ chemotherapy (cisplatin, 5- Aaribi n.a. + AE3 and CEA and postop; complete remission achieved M ﬂuorouracil, and docetaxel) (2013) negative for S-100 and after docetaxel chemotherapy and reexcision (WLE) CK5/6 M: male; F: female; n.a.: not available. 6 Case Reports in Oncological Medicine 4. Conclusion International Journal of Surgery Case Reports, vol. 30, pp. 13–16, 2017. 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Case Reports in Oncological Medicine – Hindawi Publishing Corporation
Published: May 31, 2022
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