Ning Mao,Yin-Hai Dai,Mao Wang,Wen-Xin Ji,Hai-Long Ma

1 The Second Affiliated Hospital of Shaanxi University of Traditional Chinese Medicine,Xianyang,China

*Correspondence:Ning,Mao,The Second Affiliated Hospital of Shaanxi University of Traditional Chinese Medicine,Xianyang,712000,China.E-mail:305942852@qq.com.

Preface

The diagnosis and treatment of breast Paget’s disease have particular difficulties. It is necessary to comprehensively evaluate the individualized treatment plan belonging to the patient and achieve effective regular follow-up.

Clinical information

General information

Female,50 years old,left nipple itching for more than two months,erosion and ulceration for one week.There was no apparent cause of leftnipple itching of the patient before two months.The topical hormonal ointment was not effective for this symptom,and then it was not taken seriously.In the past one week,the left nipple gradually showed exfoliation and rupture(Figure 1),and she came to see a doctor.The patient had no history of hepatitis,hypertension,diabetes,infectious diseases,and surgical trauma denied the history of drug allergy and family history of cancer.Specialist examination:the left nipple erosion with red granulation surface,a little exudate(blood)on the surface,no depression,no redness and swelling of the skin,no tenderness,no abnormal mass in both sides of the breast,the bilateral axilla and the clavicle did not touch the enlarged lymph nodes.Histopathology:(left papillary)Paget’s disease,including breast cancer(Figure 2,Figure 3).Diagnosis:combined with age,history,skin lesions and histopathology,we diagnosed it as a breast Paget’s disease.

Figure 1 General appearance of Paget’s

The left nipple is erosion,ulceration and the granulation changes on the nipple surface.

Treatment method

The breast color ultrasound did not show a palpablemass,followed by mammography,and the results still showed no abnormality.In October 2015,sentinel lymph node biopsy was performed,and five sentinel lymph nodes were taken out.The pathology showed no metastasis of sentinel lymph nodes,followed by the total mastectomy.Postoperative pathological results showed that size of 0.6×0.8×0.3cm was found at 0.5cm below the nipple.The cut surface was grayish white and tough,and the diagnosis was(left)breast invasive ductal carcinoma with mammary glandular Paget’s disease.Immunohistochemistry:ER(estrogen receptor)(-),PR(progesterone receptor)(-),C-erbB2(+++),Ki-67(20%positive rate).The TCH regimen was given four cycles of chemotherapy,and the patient refused to receive targeted therapy.There was no recurrence after 24 months of follow-up.

Figure 2 Pathology of nipp le ulcer tissue(100×)

Figure 3 Pathology of breast cancer(100×)

Discussion

Paget’s disease is a relatively rare disease that occurs in the nipple and areola areas,and a large number of reports report that about 0.5-5%of the total number of breast cancers[1].Its primary clinical manifestations are itching,ulcers,hemorrhage,and erythema of the nipple,often neglected because of no apparent symptoms,or treated with skin diseases[2].When the condition develops again,the skin becomes rough and thick,and there is desquamation,erosion,exudation,and ulceration,after repeated crusting,the granulation is exposed[3].The course of the Paget’s disease is slow and does notheal for a long time.The lesion spreads from the nipple to the areola or nearby skin,and the massive Paget’s disease can make the nipple outline disappear[4].The patient is treated again when the treatment effect is not good,and the cell smear or pathological biopsy is diagnosed as breast Paget’s disease.Most of the patients with Paget’s disease were not able to touch the palpable mass,and the tumor is only about 20%of the manifestations of the patient.However,it has been reported that the prognosis of the Paget’s disease with clinically palpable breast mass is poor.At the same time,the patient with a long-term disease can also have ipsilateral axillary lymph node enlargement,and the prognosis is worse[5].

The patient admitted to our department,50 years old,is similar to the overall age of onset of breast cancer,and the left nipple ulcer is the primary clinical manifestation,which is also consistent with the pathogenesis of Paget’s disease [6].Auxiliary examinations (B-ultrasound,molybdenum target)on both sides did not suggest apparent masses, but postoperative pathological specimens found a mass of about 0.6×0.8×0.3cm.The differential diagnosis was performed by the chief physicians of the two pathology departments.The results indicated that appropriate adjuvant therapy followed the invasive ductal carcinoma of the breast.We believe that the tumor found in the postoperative pathological specimen has no blood flow signal,and its tissue density is similar to that of the surrounding glandular tissue,and there is no calcification point.This situation is rare through an extensive literature review[7].Therefore,for the diagnosis of Paget’s disease,especially in patients without lumps, even in patients with clinically unsuspected breast cancer,if there is no abnormality in breast color ultrasound or molybdenum target,further breast MRI should be evaluated,which can clearly identify the nidus and well display the nipple changes disease,thickening of the nipple areola complex,combined with ductal carcinoma in situ and invasive carcinoma caused by breast Paget’s,and the tumor range is accurately assessed.

There is still a lack of uniform standards for the treatment of Paget’s disease,and surgery is still the first choice[8].If the mammary gland can touch the mass,it is confirmed to be invasive breast cancer during the operation,the treatment plan is the same as the treatment of breast cancer[9],it is feasible to improve the radical mastectomy (mastectomy + axillary lymph node dissection),and postoperative adjuvant therapy was performed according to pathological reports,such as chemotherapy,radiotherapy,endocrine therapy,and targeted therapy.In patients with Paget’s disease whose clinical examination is limited to nipple areola,no lesions other than the nipple areola are found through mammography or ultrasonography.It is feasible to perform breast-conserving surgery,i.e.,resection of the lesion[10],resection of the nipple areola and its deep tissue,together with the surrounding breast tissue at least 2 cm range requires negative margins and postoperative adjuvant radiation therapy[11].If the margin is positive,extended resection or total mastectomy is necessary.Of course,breast reconstruction can be performed according to the needs of patients(stage I)[12].Whether the ipsilateral axillary lymph nodes are cleaned,in principle,if the lesion is limited to the nipple areola,the breast parenchyma is not accompanied by a mass or only breast ductal carcinoma in situ,the axillary lymph nodes cannot be cleaned[13].However,it is difficult to grasp,because even if the clinical examination of the axillary fossa does not touch the enlarged lymph nodes,imaging examination does not show axillary lymph node metastasis,but the pathological report after modified radical surgery can still see patients with axillary lymph node metastasis.Therefore,only by clinical examination,there is a difference between postoperative pathological examinations,so it is recommended that axillary lymph node dissection should still be performed during breast-conserving surgery.

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