Primary cutaneous apocrine carcinoma arising within a congenital nevus: Keratins and filaggrin expression suggesting differentiation into the secretory cells of apocrine glands

Primary cutaneous apocrine carcinoma (PCAC) is a rare neoplasm of skin appendages. To determine the differentiation of apocrine carcinoma, we studied the expression of epithelial keratins and filaggrin immunohistochemically using 10 anti-keratin antibodies againt keratin (K) 1, 7, 8, 10, 14, 15, 16, 17, 18, 19 and the anti-filaggrin antibody. PCAC demonstrated strong positivity for K7, K8, K18 and K19. These keratins are distributed in secretory cells of normal apocrine glands. The tumor cells were negative for K14 and K17. The two keratins exist in myoepithelial cells in normal apocrine glands. Results suggest that PCAC shows differentiation into secretory cells of apocrine glands, although it does not differentiate into myoepithelial cells. K14 is also known as undifferentiated keratin, whereas K17 is considered to be a hyperproliferative keratin. Absence of the expression of K14 and K17 may reflect an indolent clinical course of PCAC.     

Apocrine carcinoma of the breast--a morphologic comparison of the apocrine sweat glands and the apocrine metaplastic epithelia in mastopathy

An operatively removed apocrine carcinoma of the breast from a 62-year-old Japanese lady has been observed by the ABC method, using the monoclonal antibody 115D8. The cancer cells and metaplastic epithelia exhibited similar ultrastructural findings (an apical snout, apocrine granules, etc.) as the apocrine sweat gland cells, although no evidence of apocrine secretion could be detected. The immunohistochemical testing, using monoclonal antibody, 115D8, showed an apical, linear, dot-like, staining in the supranuclear regions on the apocrine sweat gland cells and on the apocrine metaplastic cells of the mammary gland. Similar stainability also was observed in the well-differentiated area of the apocrine carcinoma, while a heterogeneity in staining, such as unstained cells and diffuse cytoplasmic-stained cells were found in the poorly-differentiated areas. These abnormal staining patterns indicate the malignant changes of the apocrine cells.     

Adenoid cystic carcinoma of sweat glands: report of two cases.

Two cases of primary cutaneous adenoid cystic carcinoma (ACC) of sweat gland origin are reported. The patients were a 83-year-old man and a 40-year-old woman. Histologically, the neoplasms showed the classic appearance of ACC. CEA, actin and S-100 protein were immunocytochemically demonstrated to be contained in some neoplastic cells. The literature on sweat gland ACC is reviewed and the clinicopathologic profile of this rare tumor considered.     

Brain metastases from apocrine carcinoma of the scalp: case report

Summary Apocrine carcinoma is an extremely rare malignant neoplasm that occurs most frequently in the axilla. Although it usually shows an indolent clinical course, it often metastasizes to regional lymph nodes and sometimes to lungs or bones. However, a literature search did not reveal any report describing the detailed clinical course of brain metastases from apocrine carcinoma. We report a case of a 54-year-old male who suffered from multiple brain metastases from apocrine carcinoma that had originated in the scalp 6 years before. The brain metastases appeared in spite of several regimens of chemotherapy for lung metastases for two years. The tumor in the right frontal lobe was successfully operated. However, the small tumor in the right occipital lobe was not cured by gamma knife surgery, and eventually required second operation. The operation had contributed to his neurologically independent life for about one year until he died for gradual progression of lung metastases. To our knowledge this is the first reported case of metastatic brain tumor from apocrine carcinoma.     

Treatment strategy for cutaneous apocrine carcinoma

Background

Cutaneous apocrine carcinoma (CAC) is a rare adnexal carcinoma with only scattered reports about long-term follow-up. The aim of this study is to demonstrate clinical findings in the prognosis of CAC and discuss the treatment procedure.

Methods

The subjects were nine patients with a histological diagnosis of CAC who underwent wide excision and regional lymph node dissection as the initial treatment at Shizuoka Cancer Center Hospital. We examined the gender, age, site of involvement, additional treatment to prevent recurrence/metastasis, additional treatment after metastasis, and the follow-up data of the study patients. Then, we calculated the recurrence and 5-year (overall/recurrence-free) survival rates.

Results

The men-to-women ratio was 8:1. The patients ranged in age from 47 to 81 years (median, 67 years). The primary lesion was in the axilla in five patients and in the vulva in the other four patients. The follow-up period ranged from 9 to 204 months (median, 44 months). The recurrence-free 5-year survival rate was 63 %, and the overall 5-year survival rate was 75 %.

Conclusions

We recommend wide local excision for a primary lesion and prophylactic regional lymph node dissection at initial therapy because of the high frequency of regional metastasis of CAC. Although CAC responds poorly to chemotherapy and radiotherapy, adjuvant radiotherapy may be used in advanced local or regional disease.     

Sclerosing sweat duct carcinoma in the peri-anal skin: a case report

We report a case of sclerosing sweat duct carcinoma, a rare tumor, occurring in the peri-anal skin, a rare position. The patient, a 41-year-old Japanese woman, was admitted to our hospital with recurrence of sclerosing sweat duct carcinoma in the peri-anal skin, which had been initially resected at a local hospital. She underwent abdomino-perineal resection. No lymph node metastases or distant metastases were found. Although we allowed a 2-cm resection margin around the tumor, microscopy showed that the tumor had extended to within 2 mm of this margin at several sites, indicating that sclerosing sweat duct carcinoma is locally aggressive. An ample resection margin should therefore be taken at initial surgery for this type of tumor. Moreover, surgical excision that is wider and deeper than primary excision is required when this tumor recurs.      

Primary cutaneous cryptococcosis in a renal transplant recipient: case report

We report a kidney transplant recipient with severe skin‐ and soft‐tissue infection mimicking necrotising fasciitis. Patient failed to respond to empirical antibiotic therapy for presumed bacterial cellulitis. Culture of aspirate from the wound and tissue samples revealed Cryptococcus neoformans. No signs of systemic cryptococcal infection were found. After antifungal treatment and surgical intervention, complete healing was achieved. Clinical and microbiological characteristics of this patient are discussed. Our case indicates that primary cutaneous cryptococcosis must be included in the differential diagnosis of severe cellulitis in solid organ transplant recipients not responding to broad‐spectrum antibiotic regimens. In our case, prompt diagnosis and treatment could dramatically modify the outcome.     

Primary non-Hodgkin lymphoma and invasive ductal carcinoma in the same breast: a rare case report

Primary lymphoma of the breast is an unusual clinical entity. The coexistence in the same breast of an invasive ductal carcinoma is even rarer. We report a 69-year-old woman referred for further evaluation of a palpable mass in her right breast. She was diagnosed and treated for simultaneous primary lymphoma and invasive ductal carcinoma. Primary breast lymphoma should always be considered in the differential diagnosis of breast masses. The presence of both malignancies presents a challenge in treatment decisions.     

Primary squamous cell carcinoma of the colorectum: case report and literature review of a rare entity

A case of primary squamous cell carcinoma of the rectum is presented. Although it is a rare condition, the diagnosis, treatment, and natural history of the disease are similar to that of adenocarcinoma of the colorectum. The patient in this report has done well to date with an abdominal perineal resection and early post-operative radiation. A review of the literature reveals that the precise etiology of this entity remains unknown.     

Ex vivo analysis of DNA repair targeting in extreme rare cutaneous apocrine sweat gland carcinoma

Cutaneous apocrine carcinoma is an extreme rare malignancy derived from a sweat gland. Histologically sweat gland cancers resemble metastatic mammary apocrine carcinomas, but the genetic landscape remains poorly understood. Here, we report a rare metastatic case with a PALB2 aberration identified previously as a familial susceptibility gene for breast cancer in the Finnish population. As PALB2 exhibits functions in the BRCA1/2-RAD51-dependent homologous DNA recombination repair pathway, we sought to use ex vivo functional screening to explore sensitivity of the tumor cells to therapeutic targeting of DNA repair. Drug screening suggested sensitivity of the PALB2 deficient cells to BET-bromodomain inhibition, and modest sensitivity to DNA-PKi, ATRi, WEE1i and PARPi. A phenotypic RNAi screen of 300 DNA repair genes was undertaken to assess DNA repair targeting in more detail. Core members of the HR and MMEJ pathways were identified to be essential for viability of the cells. RNAi inhibition of RAD52-dependent HR on the other hand potentiated the efficacy of a novel BETi ODM-207. Together these results describe the first ever CAC case with a BRCAness genetic background, evaluate combinatorial DNA repair targeting, and provide a data resource for further analyses of DNA repair targeting in PALB2 deficient cancers.     

Primary amyloidosis with high grade transitional cell carcinoma of bladder: A rare case report

Primary amyloidosis of bladder is a rare disease that closely resembles bladder cancer on clinical presentation with painless gross hematuria. Pathologically this is a totally benign non-neoplastic lesion and its association with urothelial carcinoma of the bladder is rare. We herein report a 64-year-old diabetic male who has been treated for primary amyloidosis of bladder for the last 26 years presented recently with high-grade solid urothelial cancer with osseous metaplasia and sarcomatoid change of bladder with area of amyloid deposition.     

Primary neuroendocrine carcinoma of the breast: a case report

Primary neuroendocrine carcinoma of the breast is an extremely rare tumor. We present our experience of primary neuroendocrine carcinoma of the breast in a 76-year-old woman. Surgical biopsies from breast and axillary lymphadenopathy showed a neuroendocrine carcinoma. Immunohistochemical staining of tumor cells with GCDFP15, NSE and chromogranin were positive. Computed tomography scans of the chest and abdomen showed no lesion for metastasis or another primary origin. Adjuvant hormone therapy was given, since the tumor was immunohistochemically receptor positive.     

Primary intraosseous carcinoma of the mandible: a case report

A case of primary intraosseous carcinoma of the mandible in a 75-year-old man is reported. This tumor is an uncommon lesion arising from odontogenic rests. This tumor affects men more than women and is more frequent in the sixth and seventh decades of age. Most tumors occur in the posterior mandible as painful, non-ulcerated lesions, even if several cases have shown complete absence of subjective symptoms in early phases; often they are detected first on routine radiographs. Radiographically the lesion usually shows a fully enclosed, irregular pattern of bone destruction, even if sometimes the margins are well defined. The accepted treatment is radical surgery; but metastases could occur in lymph nodes.     

Primary peritoneal carcinoma in complete remission: a case report

Primary peritoneal carcinoma (PPCa) is a relatively uncommonly diagnosed tumor. It has a similar presentation to ovarian cancer. PPCa has a poor prognosis with survival ranging from 12-18 months. PPCa spreads mainly transperitoneally, but lymphatic and hematological metastases have also been reported. It is a diagnosis of exclusion made after pathological report. Here, a case of a 71-year-old female who presented with early satiety, fatigue, weight loss and left cervical lymphadenopathy and was diagnosed with metastatic PPCa, is reported. The patient was treated with chemotherapy and achieved a complete remission. The management of this rare tumor is discussed herein.     

Primary squamous cell carcinoma of the stomach: a case report

Squamous cell carcinoma (SCC) originating from the stomach is a relatively rare entity. There are theories regarding the development of this rare tumor, but its exact pathogenesis remains obscure. Fewer than 100 cases of primary SCC of the stomach have been presented in the literature. Due to the advanced stage at the time of diagnosis in most of these cases, the prognosis is generally poor. In the case presented here, dissemination of the tumor to the transverse colon, gallbladder and omentum was present at diagnosis. Despite the tumor's advanced stage, complete remission was achieved after six courses of adjuvant chemotherapy with 5-flourouracil and cisplatin. No recurrence has been detected during follow-up. The patient has been healthy with no sign of the disease for three years.     

Primary clear cell carcinoma of the nasopharynx： a case report

Primary dear cell carcinoma of the nasopharynx is a rare and locally invasive minor salivary gland neoplasm, only two cases have been reported yet in the literature. Now a 57-year man, with primary nasopharyngeal clear carcinoma,received radiotherapy and chemotherapy. After treatment, the mass of nasopharynx visibly reduced. Follow-up two months and repeated MRI, CT, abdominal B-ultrasonograpy, electronic nasopharyngoscopy, no tumor recurrence or metastasis.     

阴囊部皮肤原发性腺样囊性癌1例报告及文献复习

目的 探讨原发阴囊部皮肤腺样囊性癌的临床病理特点,免疫组化及鉴别诊断要点.方法 报道1例原发阴囊部皮肤腺样囊性癌的临床、病理组织学形态和免疫组化特点,并复习相关文献对以上特点进行分析.结果 皮肤的腺样囊性癌较少见,可发生于除掌跖以外的任何部位,中老年好发,平均发病年龄为59岁.原发阴囊部皮肤腺样囊性癌未见报道,镜下肿瘤组织形成特殊的筛状结构,囊腔内常含有阿辛兰(pH5.2)阳性的透明质酸和硫酸化的酸性黏蛋白,瘤细胞间和小叶间周围可见透明嗜酸性基底膜物质的沉积,PAS染色呈阳性.免疫组化CK7、S-100、EMA、P63阳性表达.结论 阴囊皮肤原发腺样囊性癌相对少见,其具有与涎腺腺样囊性癌相同的镜下特征和免疫组化特点,临床表现呈惰性过程,预后相对较好.