Endocrine mucin-producing sweat gland carcinoma of the peno-scrotum with systemic metastases: A rare case report.

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare adnexal tumor with a predilection for the skin of the eyelid. It has also been reported in other areas of the face. Extra facial location has rarely been reported. They are twice as common in the females as compared to men and frequently affect the elderly between 50 and 80 years of age. It is a low-grade carcinoma with no reported cases of metastases, although a few cases with recurrences have been reported. Since it was first described by Flieder et al. in 1997, fewer than 60 cases have been reported in the literature. We describe one such case of EMPSGC in an adult male occurring at an unusual location, the peno-scrotal junction with systemic metastases to bilateral inguinal and iliac lymph nodes, multiple bones, and pancreas. Unlike previously reported cases, our patient worsened rapidly and succumbed to the disease six months after initiation of chemotherapy and radiotherapy. To the best of our knowledge, this is the first reported case of its kind in modern published literature.

Do not break a sweat: avoiding pitfalls in the diagnosis of sweat gland tumors.

The group of malignant sweat gland neoplasms is characterized by a wide biologic spectrum, including tumors with indolent behavior, low-grade malignant potential with locally destructive tumor growth and high local recurrence rates and high-grade malignant potential characterized by risk for disseminated disease and disease-related mortality. Reliable diagnosis to predict behavior may be challenging for a number of reasons. The clinical presentation is often nonspecific. Many of the tumors are rare, and they are only infrequently encountered in routine diagnostic practice. A significant subset of tumors shows bland and innocuous histologic features. They are easily mistaken for benign tumors despite their potential for destructive growth and aggressive disease course. At the other end of the spectrum the tumors may resemble poorly differentiated carcinoma or adenocarcinoma and recognition relies entirely on sampling and carful histological examination. The tumors may be inseparable from cutaneous metastases from visceral primaries by morphology and immunohistochemistry, requiring careful clinical correlation and work-up. Conversely, cutaneous metastases are readily mistaken for cutaneous primary tumors. While the presence of a myoepithelial layer is a helpful feature in excluding metastatic deposits, it does not imply benign behavior of sweat gland tumors in general. The above issues and challenges are exemplified in the discussion of selected sweat gland carcinoma in this manuscript, with a focus on recently described entities and those with novel findings.

Primary cutaneous adenoid cystic carcinoma: an unusual case.

Primary cutaneous adenoid cystic carcinoma (PCACC) is an uncommon adnexal skin tumor. It is usually located on the head or neck of people over 50 years of age, affecting men and women equally. The etiology is unclear; the source used to be considered the eccrine glands, but more recent hypotheses defend this carcinoma as originating in the apocrine glands or modified apocrine glands. The tumor may clinically appear as a normal skin-colored nodule with ill-defined edges and slow growth. Histologically, a proliferation of neoplastic cells appears, which could present in a tubular, cribriform, or solid pattern. Perineural invasion is characteristic for this tumor. For diagnosis of PCACC it is essential to exclude metastasis, especially in the salivary glands. Treatment involves surgical excision. We present a case of primary adenoid cystic carcinoma in a young woman located on the skin of the upper limb, which is an unusual place.

Metastatic apocrine sweat gland adenocarcinoma in a terrier dog.

This report describes the clinical and pathological aspects of an apocrine sweat gland carcinoma with distant metastasis in an aged dog. A 7-year-old male terrier dog was referred to small animal hospital of Shahid Bahonar University of Kerman with a 5.5×3.5 centimeter pedunculated mass on its head near left auricular region which had been progressively growing since three months ago. The radiography showed no local and distant metastasis. Surgical excision and histological evaluation was done. Histologically, the mass was composed of epithelial cells arranged in glandular and solid patterns. The morphologic findings suggested either a primary or metastatic apocrine-gland carcinoma. Immunohistochemically, the tumor cells were intensely positive for cytokeratin 7 and 20 and negative for S100 protein. On the basis of histopathological and clinical findings, the tumor was diagnosed as a malignant apocrine gland tumor, arising from apocrine sweat glands of the skin. Local tumor recurrence with anorexia and weight loss was reported by the owner nine month later. Severe submandibular and prescapular lymphadenomegaly was noted in clinical examination. Several large pulmonary nodules were noted in chest radiographs resembling mediastinal lymph node metastasis. Second surgery and chemotherapy was rejected by the owner due to grave prognosis of the patient. The animal was died 45 days later due to respiratory complications. Tumors of apocrine sweat glands are relatively uncommon in dogs whereas apocrine gland adenocarcinoma with distant metastasis is extremely rare.

An immunohistochemical panel to differentiate metastatic breast carcinoma to skin from primary sweat gland carcinomas with a review of the literature.

CONTEXT: Approximately 25% of patients with breast cancer develop cutaneous metastases. Sweat gland carcinomas (SGCs) account for about 0.05% of all cutaneous neoplasms. Cutaneous metastases of breast carcinoma (CMBCs) (especially the ductal type) can be difficult to distinguish from SGCs. Treatment and prognoses for these 2 types of tumors differ radically, making accurate histologic diagnosis crucial. Although a few studies attempt to differentiate these entities employing immunohistochemical (IHC) studies (some of which we review here), to date, no panel of IHC stains exists, to our knowledge, to distinguish these entities. OBJECTIVE: To devise a panel of IHC stains to distinguish CMBC from SGC. DESIGN: Twelve cases of ductal CMBCs (11 not otherwise specified type, and 1 basal phenotype), 11 cases of SGCs (5 eccrine carcinomas, 3 porocarcinomas, and 3 microcystic adnexal carcinomas), 2 benign sweat gland neoplasm cases, and 2 primary breast cancer cases were retrieved and analyzed with the following IHC panel: mammaglobin, gross cystic disease fluid protein (GCDFP) 15, p63, basal cytokeratins (CK5, CK14, and CK17), androgen receptor, and PAX5. RESULTS: The p63 was only weakly expressed in 1 of 12 CMBC cases (8.3%), whereas it was strongly expressed in 10 of 11 SGC cases (90.9%) (P < .001). Basal cytokeratins demonstrated a similar immunoprofile in the SGC group, with 10 of 11 cases (90.9%) expressing all 3 markers, and a variable immunoprofile in the CMBC group with 0% (CK14) (P < .001) to 16.7% (2 of 12 cases; CK5 and CK17) (P < .001) expression. Mammaglobin was expressed in 8 of 12 cases (66.7%) of CMBC. CONCLUSIONS: Together, these 5 IHC stains were combined to make a panel that was 100% sensitive and 91% specific in distinguishing between CMBC and SGC.

Mammaglobin immunostaining in the differential diagnosis between cutaneous apocrine carcinoma and cutaneous metastasis from breast carcinoma.

The differential diagnosis between cutaneous apocrine carcinoma (CAC) and cutaneous metastases from breast carcinoma is commonly difficult. Many times, clinical information is crucial in the final diagnosis, because help that can be obtained from immunohistochemistry is usually limited concerning this subject. We used the antibody mammaglobin in order to study 10 cases of cutaneous metastasis of ductal breast carcinoma, and 2 cases of CAC. One of the CAC cases showed only scattered positive cells, while the other did not show any positivity. Four cases of metastatic breast carcinoma also showed scattered positive cells. In other five metastatic cases, positive cells were abundant, representing up to 60% of the tumoral cells. One case of metastatic breast carcinoma did not show any expression of mammaglobin at all. Although, more cases of CAC should probably be studied in the future before any categorical conclusion can be obtained, our results seem to indicate that a pattern of immunostaining with expression of mammaglobin in many cells would favor a metastatic origin of the tumor.

[Primary mucinous carcinoma of the skin]. / Primaert mucinøst karcinom i huden.

Primary mucinous carcinoma of the skin is a rare malignant tumour originating from the sweat glands. It is often misdiagnosed clinically since it has an uncharacteristic and variable presentation, and microscopically because it resembles a cutaneous metastasis from the more frequent mucinous adenocarcinomas of the colon, mammae, lungs and ovaries. The tumour often recurs, but mortality is low. We present a case with regional lymph node metastases five years after excision of the primary tumour.

[Eccrine adenocarcinoma in a patient with familial breast carcinoma]. / Ekkrines Adenokarzinom bei einer Patientin mit familiärem Mammakarzinom.

A 67-year-old patient developed a subcutaneous, non-tender nodule in her left groin over a period of about seven years. The patient underwent surgery and 4 procedures were required to obtain complete excision. The histopathologic findings showed eccrine adenocarcinoma, a member of the heterogeneous group of sweat gland tumors which occur primarily in adults with a peak of 50-60 years of age. Sweat gland carcinomas are extremely rare neoplasms of the skin and exhibit a slow growth rate with a rather high local recurrence rate. The tumor has a disposition to metastasize and shows a poor response rate to adjuvant therapy regimens. Therefore wide, deep surgical excision with an excision margin of 2-3 cm is the treatment of choice. Nevertheless there are some case reports on successful therapy of a metastasized sweat gland carcinoma with 5-fluorouracil and tamoxifen. Here further studies are needed to achieve a better survival rate for patients with metastatic disease.

A rare and unusual case of bilateral, axillary, metachronous apocrine carcinoma.

Apocrine carcinoma is a rare sweat gland neoplasm with very few cases reported in the published literature. We report a case of primary axillary apocrine carcinoma with later recurrences in both axillae. A 55-year-old man was clinically diagnosed with hydradenitis suppurativa in the right axilla, and after excision of lesion, histology showed metastatic adenocarcinoma of probable breast origin. However, no primary focus was found after extensive work-up except for metastatic lymph nodes in the ipsilateral axilla treated with axillary clearance. After 4 years, the patient developed metastatic lymph nodes in the contralateral axilla and had surgery. He had a further recurrence in the right axilla and was treated with surgery and radiotherapy. He continues to do well at this time, with no evidence of local or distant metastases.

[Surgical treatment of groin soft tissue defects]. / Chirurgische Therapieoptionen bei Weichteildefekten der Leiste.

BACKGROUND: Treatment of groin defects remains challenging due to their location and origin. Such defects commonly result from resection of tumours but can also occur after surgical or medical therapy. MATERIAL AND METHODS: From 2003 to 2005, 11 patients were treated with groin defects following radiation therapy and resection of lymph node metastasis, primary carcinoma, and sarcoma. Seven patients received wound closure with rectus femoris muscle flaps, and three had vertical rectus abdominis muscle flaps. One patient was amputated at the hip, and the defect was covered with dorsally pedicled muscle and skin flaps. RESULTS: All flaps healed primarily. There were no secondary infections or lymphorrhea. CONCLUSION: The flaps described here are well vascularised standard flaps which are easily harvested with no major donor site morbidity. They provide sufficient coverage for the groin, reduce long-term conservative treatment, hospitalisation, and problems such as scar contracture.

[A case of apocrine adenocarcinoma suspected as an axillary lymph node metastasis from cecal cancer].

The patient was a 69-year-old male. He had a 2-year history of subcutaneous tumor in the left axilla. Biopsy of the tumor showed the features of metastatic adenocarcinoma. FDG PET to check the primary lesion revealed the collection of an ileocecal junction and left axilla. After CT scan and colonoscopy, we diagnosed cecal cancer and metastasis of the axillary lymph nodes from it. He underwent ileocecal resection and a wide local resection of the axillary tumor. Histopathological examination showed the axillary tumor was different from the cecal cancer. Axillary tumor was diagnosed not as metastasis of axillary lymph nodes from cecal cancer but apocrine adenocarcinoma.

Successful adjuvant tamoxifen therapy for estrogen receptor-positive metastasizing sweat gland adenocarcinoma: need for a clinical trial?

We report on successful adjuvant tamoxifen therapy for a metastasizing sweat gland adenocarcinoma of the scalp in a 64-year-old woman. Before the antihormonal therapy, the patient had undergone repeated surgery for ipsilateral intraparotid, soft tissue, and lymph node metastases and had had disease-free intervals of less than 5 months. As the immunohistochemical analysis of the tumor tissue revealed a 100% nuclear reactivity to estrogen and progesterone receptors, we started empirical tamoxifen citrate therapy, which dramatically changed the course of the disease. The patient has been in complete remission for 3 years. This is the third report in the literature of substantial therapeutic benefit of antiestrogen therapy in metastasizing eccrine gland adenocarcinoma with positive hormone receptor immunohistochemistry. We suggest examining the hormone receptor expression in these neoplasms regularly. A prospective study should be commenced to assess the benefit of adjuvant antihormonal therapy in eccrine gland adenocarcinomas.

Her-2 expression in cutaneous eccrine and apocrine neoplasms.

Cutaneous eccrine and apocrine glands have many histologic and immunologic similarities to ducts and acini of the breast. Thus, differentiating a primary cutaneous process from a metastatic breast carcinoma can be nearly impossible. In all, 10-34% of breast carcinomas overexpress HER-2 protein, a membrane-associated protein that functions in cell differentiation, adhesion and motility. As expression of this gene in cutaneous neoplasms has not been well characterized, we sought to determine HER-2 expression in a sample of benign and malignant cutaneous eccrine and apocrine neoplasms and to determine if there is value in using this protein expression in differentiating primary cutaneous from metastatic breast lesions. Totally, 85 primary cutaneous neoplasms and 11 cutaneous metastases from HER-2-positive breast carcinomas were retrieved from archived material at our institute. All cases were evaluated for HER-2 protein expression using the Dako Hercept Test kit. Membranous HER-2 staining was noted in three of the 85 cutaneous adnexal neoplasms: one hidrocystoma and two nodular hidradenomas. Seven of the 11 cutaneous metastases from HER-2-positive breast carcinomas maintained moderate-to-strong HER-2 expression. In conclusion, while 10-34% of breast carcinomas overexpress the HER-2 protein, only 3.5% of cutaneous apocrine and eccrine neoplasms in this study stained with the HER-2 antibody. These HER-2-positive cutaneous neoplasms typically do not pose a diagnostic dilemma in the setting of differentiation from breast metastasis. Additionally, although histologically these breast and cutaneous lesions may have morphologic similarities, the relative lack of HER-2 overexpression suggests that they are different nosologically. Finally, this study suggests that HER-2 protein expression can be a useful tool in differentiating a primary cutaneous appendageal neoplasm from HER-2 expressing metastatic breast carcinoma.

Metastatic malignant acrospiroma of the hand.

We present the case of a 37-year-old man with multiple pulmonary metastases of a primarily unknown primary tumour. Thorough revision of the medical history yielded that he had already passed three resections of a right palmar mass, which had been described as a benign tumour. Clinical examination showed a thickened scar with a suspicious palpable mass in the right hand. Excision of this scar and the tumour mass with histopathological examination now revealed a malignant acrospiroma. Resection of the pulmonary metastases histologically also confirmed a malignant acrospiroma. The following radical resection of the metacarpals II and III with the index and middle finger under the assumption of a wide compartment resection achieved tumour free margins and proved to be efficient with the patient being relapse free for 4 years from this operation. Although the prognosis of this tumour is generally unfavourable this particular case demonstrates the value of a radical surgical resection as the mainstay of treating such highly malignant sweat gland tumours of the hand.

[Cancer of the sweat glands (its clinico-morphological characteristics)]. / Rak potovykh zhelez (kliniko-morfologicheskaia kharakteristika).

Based on a review of the literature and the authors' own experience comprising 20 cases, clinical and histological characteristics of sweat gland cancers (SGC) are detailed. Two clinical variants of SGC are distinguished: exophytic (or exophytic-endophytic) with ulceration and endophytic (intradermal). The lapse of time between detection of a SGC and its removal generally ranges from 12 months to 2 or more years. It is shown that diagnostic and prognostic considerations warrant classifying these cancers into two groups: those arising from benign tumors and retaining their histologic and ultrastructural features (one example of such a tumor is malignant poroma) and primary cancers (e. g., mucoid adenocarcinoma). Cancers of the first group, which comprise a majority of all SGC, run a more favorable course.