br Case reports br Discussion

Case reports
Discussion In 1874, Sir James Paget first described an intraepidermal neoplasm of the nipple, caused by an underlying breast carcinoma. Crocker described the first patient with EMPD involving the penis and scrotum in 1889. Perianal Paget\'s disease was first described in 1893. Other sites of occurrence include the vulva, scrotum, groin, and axilla. The prognosis for primary EMPD without deep dermal invasion is good with appropriate treatment. However, invasive EMPD has a high rate of metastasis. The depth of invasion is an important prognostic factor. The overall survival rate is poor; if the invasion extends into the subcutaneous tissue, and then regional 1 25-dihydroxyvitamin d3 node metastases may occur. In cases of secondary EMPD, prognosis is related to the underlying carcinoma. According to the data of the Veterans General Hospital, Taipei, Taiwan, six of 29 patients (20.7%) died of the scrotal EMPD and three (10.3%) died of associated malignancy. But there are still insufficient data about associated malignancy or the histological characteristics of each lesion. Surgery remains the standard treatment for EMPD. However, high local recurrence rates are seen after a wide surgical excision with an adequate margin. This could be due to irregular margins or the multifocal nature of EMPD. Several papers have shown an overall recurrence rate of about 40% with a wide local excision. Local recurrence rates are higher in cases of invasive disease compared to those limited to intraepithelial involvement. More radical and extensive surgeries are associated with lower rates of local recurrence. Mohs micrographic surgical excision (MMS) both improves cure rates and spares tissue around critical genitourinary structures. The recurrence rate after treatment with MMS is reported in 16% of primary EMPD. In 97% of cases treated with MMS, a safe margin of about 5 cm should be excised from the clinical tumor margin. With a surgical margin of 2 cm, only 59% of tumors are cleared, and the other 41% of EMPD are treated with the standard wide excision. The two cases in this study had noninvasive primary EMPD. Due to the large tumor, both cases accepted a wide excision with a 2 cm safety margin. The defect was so big that a local flap was used for reconstruction. If we choose the 5 cm safety margin, the destruction around the critical genitourinary structures wound is very severe. We need to think about the balance between the benefit and the destruction of a large safe margin. Even if we use the 5 cm safety margin, local recurrence still could happen. It has not been proven whether more extensive surgery would be more effective in preventing local recurrence; the value of excision wider than a 3 cm safe margin is controversial. The recurrence rate has been reported to be about 12.5% in 24 patients who received wide local excision and the excision margin varied from 1 cm to 5 cm. Although surgery remains the traditional treatment for EMPD, radiotherapy may be used when patients are poor surgical candidates or when the level of genitourinary function after extensive surgery is a concern. Radiotherapy has also been used for local recurrence after surgery or as an adjuvant therapy in patients with a high risk of local recurrence. The experience of chemotherapy for EMPD still relies on case reports. Systemic chemotherapy has been used to treat patients with invasive and metastatic disease and may be considered in rare cases when surgery and radiotherapy are contraindicated. Limited reports of systemic chemotherapy to treat EMPD have been described, including a combination of low dose 5-fluorouracil and cisplatin; a combination of 5- fluorouracil, cisplatin, mitomycin C, epirubicin, and vincristine; and docetaxel.
Introduction Tumoral calcinosis (TC) is a rare clinical condition that can be primary (sporadic or familial) or secondary to other diseases, particularly end-stage renal disease (ESRD). Although different types of TC do not differ in their radiologic and histopathologic presentations, the treatment may differ based on etiology. TC (also known as Teutschländer disease, calcifying bursitis, lipocalcino-granulomatosis, calcifying collagenolysis, and Kikuyu bursa) is a deposition of calcium phosphate and calcium hydroxyapatite within periarticular soft tissues. Differential diagnoses of soft-tissue calcification include mixed connective tissue diseases, dermatomyositis, calcinosis circumscripta, calcific tendinitis or calcific bursitis, and heterotrophic calcification. Neoplastic diseases such as parosteal osteosarcoma, chondrosarcoma, and synovial sarcoma should also be considered. Surgical resection of the calcified mass is the main treatment for primary TC, but for hemodialysis-related secondary TC, phosphate lowering measures may be considered first.