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Malignant adnexal neoplasms of the skin are a heterogeneous group of rare malignancies with eccrine, apocrine, sebaceous and follicular differentiation. Essential clinical practice recommendations for the overall management of these cancers are presented. Moreover, specific evidence-based findings according to diagnosis, therapy and follow-up of porocarcinoma, sebaceous carcinoma and microcystic adnexcarcinoma will be explained.

We report on an atypical clinical course of a patient with the very rare diagnosis of an apocrine sweat gland carcinoma with lymphatic metastasis, a single metachronous distant metastasis and a now reached survival time of more than 4 years and give a review about the current literature. Only a very small number of cases have been described. The recommendations for diagnostics and treatment of this tumor, therefore, are not based on prospective randomized studies but upon case reports and on new immunohistochemical and genetic markers.

Poromas were originally classified as eccrine tumors which predominantly consist of poroid ductal cells and differentiate in the direction of sweat gland ducts. However, there have now been many reports on poromas with additional differential characteristics differentiating in the direction of sebaceous and/or apocrine glands and/or hair follicles. These tumors have been termed apocrine poromas. Multilineage differentiation within a poroma can be explained by the embryological association of the sweat duct with the so-called folliculo-sebaceous-apocrine unit. The clinical and histopathological features of apocrine poromas are reviewed in comparison to classical eccrine poromas by taking into account seven own cases of apocrine poroma and a review of the literature. It is important for histopathologists not to confuse apocrine poroma with other tumors with multilineage differentiation. Apocrine poroma needs to be distinguished from sebaceoma and from basal cell carcinoma with sebaceous differentiation, in particular, because these tumors have therapeutic consequences for the patient. The main histopathological differences between apocrine poroma, sebaceoma and basal cell carcinoma with sebaceous differentiation are explained.

Hidradenocarcinomas are rare, yet highly malignant tumors of eccrine sweat gland origin. Due to its locally aggressive growth and likelihood for metastasis it should be considered as a differential diagnosis especially in case of suspicious intraoperative findings. We report the case of a 73-year-old female patient presenting with a hidradenocarcinoma of the wrist. Treatment of hidradenocarcinomas is similar to the treatment of sarcomas: The first step is an incisional biopsy and obtaining an expert second opinion on the histopathological diagnosis as well as staging. The second step is a clear margin resection of the tumor and the plastic-surgical reconstruction. A long-term follow-up is mandatory to detect potential recurrence or metastasis., (© Georg Thieme Verlag KG Stuttgart · New York.)

Background: Eccrine porocarcinoma is a rare malignant tumor, arising from the intraepithelial ductal parts of the sweat glands. Although the clinical appearance is variable, some tumors present with a diameter of 10 cm or more. In most cases the tumor presents as a slowly growing painless mass., Patients and Methods: A case of a 61 year old male patient is presented, who was admitted because of a peripheral facial nerve palsy. The patient reported about a sting in this area some ten years ago with a persistent swelling. Four years ago this lesion began to grow slowly., Results: Clinically a huge mass in the left cheek extended to the parotid gland was obvious. The patient underwent radical tumor excision with histologically clear margins. The defect was reconstructed by a microvascular forearmflap, the facial nerve was reconstructed using either a graft from the suralis nerve (for the upper part of the facial nerve) along with a hypoglossal facial nerve anastomosis for the lower part of the facial nerve. Postoperative radiation therapy was added. Histologically an eccrine porocarcinoma was confirmed with an infiltration of the facial nerve and the parotid gland. Lymph nodes in the neck were negative. DNA image cytometry achieved a DNA malignancy grade of 0.73, the immunohistochemical assessment using a monoclonal antibody against the proliferation antigen MIB-1 achieved a proliferation rate of < 10 %., Conclusions: Although very rare eccrine porocarcinoma should be considered in the differential diagnosis of slowly growing skin masses in the face. Therapeutically a radical resection with histological clear margins is recommended, in case of lymph node metastases with additional postoperative radiotherapy.

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.

Sweat gland carcinomas are rare malignant tumors of the skin. The well-defined entities porocarcinoma, microcystic adnexal carcinoma, aggressive digital papillary adenocarcinoma, mucinous eccrine carcinoma, adenoid cystic carcinoma, spiradenocarcinoma, cylindrocarcinoma, hidradenocarcinoma are described. The article summarizes essential clinical, prognostic and histopathological findings of these tumors and takes in focus special recommendations for dermatologists and surgeons to plan biopsies and operations.

Introduction: Malignant eccrine poroma is a very rare tumour of the sweat glands with high malignancy and presenting with a polymorph clinical and histological picture., Case Report: We describe the case of a 99-year-old patient with a malignant poroma on the buttock. Despite the large size of the tumour, no metastasis was found with standard examination techniques. Radical excision and defect closure with a Limberg flap was performed., Result: The healing course was uneventful and without complications., Conclusion: The malignant poroma is a tumour of high malignancy which can easily be misdiagnosed because of its different forms of presentation. Radical surgical therapy is the only known effective treatment.

A 37-year-old female presented for surgery with central perforation of the eardrum with granulation. Mastoidectomy had been performed 18 years ago following chronic mastoiditis. As the clinical picture now suggested a suspected cholesteatoma, radiological imaging was performed. The CT scan revealed specification of the mastoid and the tympanic cavity. In addition, MRI scan showed signal enhancement in the same areas. However, the suspected cholesteatoma could not be confirmed intraoperatively. Pathohistology revealed a ceruminal gland adenoma. They are a rare phenomenon and should be distinguished from middle ear adenomas, pleomorph ceruminal gland adenomas, ceruminal gland adenocarcinomas and cylindromas of the ceruminal glands. Owing to a high recurrence rate, complete surgical removal is necessary. Despite its rare occurrence, a ceruminal gland adenoma must be taken into consideration in the differential diagnosis of individual cholesteatoma cases.

The eccrine carcinoma is a rare tumour originating from the sweat glands. They are mainly located in the palm of the hand and the sole of the foot, the digits, the head and the trunk. We report on a now 68-year-old male, who underwent partial amputation of the thumb at the level of the proximal phalanx due to an eccrine carcinoma in 1990. In the following years, three local recurrences were excised. After the exclusion of metastases an amputation at the MP I level was performed at our institution. To restore grip function, we performed a distraction of the first metacarpal bone followed by deepening of the first web space. So far, there are no defined treatment recommendations due to the rarity of the tumor. In some cases, metastatic disease has been reported, and in most cases, a high rate of local recurrence. After diagnostic biopsy, we recommend staging to exclude metastatic spread of the tumor followed by aggressive local surgical treatment.

A case of Malignant Syringoma (syn. = Microcystic Adnexal Carcinoma, Sclerosing Swat Duct Carcinoma) of the nose in a 44 year old female patient is reported. The tumor had been misdiagnosed as a Rhinophyma some 20 years before and had thus been treated under cosmetic aspects. Besides the appearance, the patient did not suffer any complaints and was referred to the ENT-department of the Klinikum Hannover for cosmetic reasons. Histopathological examination after removal revealed a malignant syringoma, which, due to its extensive size and subepithelial growth pattern, made a complete ablation of the entire nose and the adjacent soft tissue of the face necessary. Pathohistologically cellular atypia, invasive growth pattern, perineural and perivascular infiltration was characteristic. Quantitative DNA anaylsis revealed a tumor with a diploid stem line and only few aneuploid cells. Malignant syringoma is a rare differential diagnosis of face skin tumors. The present case is discussed based on a review of the literature.

Patient: A 60-year-old woman was evaluated for a nodular tumor in her right upper eyelid, which has developed over the last four years. The tumor measured 10 x 8 mm and was ulcerated. The patient's ophthalmologic und medical history was normal. Clinically, a basal cell carcinoma was suspected, and an excision was carried out under local anaesthesia. The histopathology was suggestive for an eccrine spiradenoma., Conclusion: Eccrine spiradenomas rarely involve the eyelids. This tumor represents a benign, usually painful, sweat gland tumor. The possibility of sweat gland tumor should be kept in mind in the diagnosis of eyelid tumors. The complete excision is the only therapeutic option.

Introduction: A great number of consmetically disturbing, cutaneous lesions are localized in the periocular region. While various approaches for treatment such as excision, electrocauterization or cryosurgery often show unsatisfactory results, the use of laser technology is of increasing interest., Material and Methods: A total of 104 patients with different cutaneous periorbital lesions (wrinkles, xanthelasma, syringoma) were treated with a new erbium: YAG laser system (wavelength 2940 nm, pulse duration 0.350 ms and pulse energy 0.1-1.7 Joules), which works on the principle of vaporization., Results: The erbium: YAG laser allows athermic ablation of very thin skin layers without scarring and with minimal lateral thermal injury due an extremely short pulse duration. Periorbital lesions can be treated effectively by erbium: YAG laser, and good to excellent cosmetic results can be obtained., Conclusions: The use of pulsed erbium: YAG laser is an effective and promising new method for treatment of different superficial periorbital cutaneous lesions.

Two cases of generalized syringomata showing an uncommon dissemination and density of tumors particularly on thighs, back and abdomen, are demonstrated. Clinically there is similarity with urticaria pigmentosa adultorum. Histological investigation is essential for clinical diagnosis.

Periocular syringoma are a frequent symptom of the trisomy 21 of adolescent mongoloids. This is seldom mentioned in the literature. Disseminated eruptions at the trunc seem to be a rare phenomena in mongolism.

In a 37-year-old female with the clinical appearance of urticaria pigmentosa histologic examination revealed disseminated syringomata. By toluidine blue staining, a marked increase of tissue mast cells was demonstrated in the interstitial connective tissue, thus explaining the clinical appearance.