Pregnancy-Related Eccrine Angiomatous Hamartoma: Case Report.

Eccrine angiomatous hamartoma is rare, slow-growing, and benign neoplasm that is diagnosed based on clinical characteristics and histological findings. It usually presents as a solitary nodule on the extremities and may arise at birth or in childhood. Although it is usually asymptomatic, in some cases it can cause pain and hyperhidrosis. From a histological perspective, it is characterized by an increase in the number of eccrine glands and a proliferation of vascular channels. We present the case of a 26-year-old woman who developed an eccrine angiomatous hamartoma in her right leg. The rapid growth of the lesion during pregnancy coupled with the challenges posed by a superficial biopsy, complicated the differential diagnosis.

Metastatic hidradenocarcinoma: a report of two rare cases.

BACKGROUND: Hidradenocarcinoma is a rare malignant sweat gland tumour, characterized by a slow but aggressive course, with high rates of local recurrence and metastasis. Due to its rarity, histological criteria and therapeutic guidelines are poorly defined, posing a major challenge for clinicians and pathologists. OBJECTIVES: To present two new cases of metastatic hidradenocarcinoma as well as a review of the literature. MATERIALS & METHODS: We describe two case studies and a review of the literature based on a search using the MEDLINE (PubMed) electronic database. RESULTS: The first patient was a 61-year-old woman with a perimamillary hidradenocarcinoma that arose from the malignant transformation of a benign childhood lesion and developed regional lymph node metastases after wide excision and adjuvant radiotherapy. The second patient was a 63-year-old man who developed cutaneous and renal metastases several years after the complete excision of a lumbar hidradenocarcinoma. As far as we can ascertain, kidney metastasis from hidradenocarcinoma has not previously been described. CONCLUSION: Most authors recommend wide excision as the treatment of choice for hidradenocarcinoma, however, optimal adjuvant therapy remains to be determined. Our cases add to the limited knowledge available, but high-quality studies to find new effective treatments are needed.

Complex sweat gland hamartoma: A case report.

Syringocystadenoma papilliferum (SCAP), tubular apocrine adenoma (TAA), and eccrine nevus are rare benign sweat gland tumors with varied clinical presentations but generally distinctive histomorphologic profiles. TAA and SCAP have been associated with other cutaneous hamartomas, most commonly with nevus sebaceus. Additionally, TAA and SCAP have uncommonly co-occurred in the same lesion. In contrast to nevus sebaceus, eccrine nevus is considerably less common and is rarely associated with other benign adnexal lesions. Here we present an unusual case of a complex sweat gland hamartoma containing features of syringocystadenoma papilliferum, tubular apocrine adenoma, and eccrine nevus in a 7-year-old female.

Clear Cell Hidradenoma in a Pregnant Female.

Solid cystic hidradenoma, or clear cell hidradenoma, is a distinct and histologically rare tumor formed at the sweat glands, found mainly in adults and majorly among women. In this case, a 26-year-old female presented with asymptomatic swelling in her left inguinal area. Similar cases have been discussed in the literature considering the same kind of tumor. The present case is reported owing to the rarity of the type of tumor in terms of size and region of occurrence with the associated condition of pregnancy.

Case Report: Dermoscopic, High-Frequency Ultrasound, Contrast-Enhanced Ultrasound Appearances and Special Treatment of a Patient With Syringoid Eccrine Carcinoma on the Chest.

This article aims to explain the use of a variety of noninvasive of minimally invasive examinations to obtain reliable diagnostic clues. The choice of treatment methods and repair techniques for wound defects are also critical in terms of the prognosis. Here, we describe the case of a 53-year-old male patient who visited our dermatology clinic due to a red plaque on the inner side of his left nipple without any symptoms for more than 30 years. He was given dermoscopy, high-frequency ultrasound (HFUS), Color Doppler flow imaging (CDFI), and contrast-enhanced ultrasound (CEUS) examinations. Currently, there are no literatures on these auxiliary examinations for this disease. Dermoscopy revealed that there were abundant blood vessels on the periphery of the skin lesion with obvious dilation. HFUS revealed an inhomegeneous hypoechoic solid mass in the dermis with clear borders and irregular shape. CDFI indicated that there are abundant blood flow signals in the periphery and central of the tumor. CEUS showed a mixed inhomogeneous, grid-like high-enhancement pattern. Based on the above auxiliary findings, the possibility of malignant lesion was suspected. Therefore, the patient was given a pathological examination, which showed that many luminal structures of the dermis layer were embedded in the hyperplastic fibrous tissue. The atypical cells were not obvious but showed an infiltrating growth pattern. Immunohistochemistry showed positive reaction for cytokeratin 7 (CK7), epithelial membrane antigen (EMA), and carcinoembryonic antigen (CEA) and a weak positive results was obtained for S-100. There was also a negative result for CK20, gross cystic disease fluid protein 15 (GCDFP-15), and P63. As a result, the patient was diagnosed with "syringoid eccrine carcinoma." The treatment was surgical excision. Mohs microsurgery was combined with the looped, broad, and deep-buried suturing technique (LBD tension-reduced suturing technique). This technique directly sutures the wound instead of carrying out traditional skin grafting or flap transfer. The postoperative follow-up results were satisfactory as no obvious keloid formed on the wound during the follow-ups. In conclusion, ultrasound is greatly advantageous in tumor morphology and hemodynamics. It orients the therapeutic management and assesses the therapeutic efficacy and the tumoral prognosis. In surgical treatments, a less-traumatic operation should be selected to reduce the patient's pain.

Multicentric endocrine mucin-producing sweat gland carcinoma and mucinous carcinoma of the skin: A case report.

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare low-grade sweat gland carcinoma. EMPSGC is thought to be a precursor to mucinous carcinoma of the skin (MCS). Since the first description of EMPSGC in 1997, only a few cases have been reported, and its etiology and mechanisms remain unknown. In this report, we describe a 71-year-old Japanese woman with two isolated EMPSGC and one MCS lesion on her face. She was simultaneously diagnosed with invasive ductal carcinoma of the breast. She had a history of uterine cancer of unknown histopathological diagnosis 24 years previously. The presence of in situ lesions confirmed by myoepithelial cells suggested that the cutaneous lesions were primary tumors. To the best of our knowledge, this is the first case of multiple primary EMPSGC/MCS tumors. Additionally, this might be the first case with multiple primary carcinomas including adnexal cutaneous tumors, breast cancer, and uterine cancer, which may share the common feature of expressing female hormonal receptors. This case indicates that EMPSGC/MCS may be triggered by a hormonal receptor abnormality, perhaps because of genetic defects. A larger number of reports examining this issue may be necessary to further assess our initial observations.

Brooke-Spiegler Syndrome With Cervical Spine Lesion.

Brooke-Spiegler syndrome (BSS) is a rare hereditary autosomal dominant disorder with variable phenotypic expressivity that results in a variety of benign cutaneous face, scalp, and neck tumors with a histology profile of cylindroma, spiradenoma and trichoepithelioma. Reports of lymph node and distant metastasis are scarce. We present the first case of Brooke-Spiegler syndrome with metastasis to the cervical spine. An 86-year-old female with Brooke-Spiegler syndrome presented to the clinic with a finding of cervical spine lesion involving vertebral body, prevertebral, paraspinal, foraminal, and epidural spaces. The histopathology of the lesion showed benign cylindroma. Considering the location of the lesion and local invasion of neural structures, the malignant transformation of existing tumors could not be excluded. Brooke-Spiegler syndrome rarely presents with malignant transformation and distant metastatic spread. It is important to be aware of these rare cases while monitoring the disease and addressing clinical symptoms. This is to our knowledge the first case of metastatic spread of the cylindroma to the cervical spine resulting in local bone destruction and neural elements compromise. Physicians should be aware of this rare possibility.

Clear Cell Hidradenoma of the Breast Diagnosed on a Core Needle Biopsy: A case report and review of the literature.

Clear cell hidradenoma (CCH) is a tumour originating from the eccrine sweat glands. It usually presents in the limbs, axilla or trunk. CCH of the breast is rare and can present as a cystic lesion in the breast that can be easily misdiagnosed as malignancy. We report a 36-year-old female patient who presented at the Sultan Qaboos University Hospital Breast Clinic, Muscat, Oman, in 2018 with a lump in her left breast. Ultrasound examination reported a complex cystic lesion with a solid, vascular component. An ultrasound-guided core needle biopsy was suggestive of clear cell hidradenoma. Surgical excision was performed and histopathology confirmed the diagnosis of CCH of the breast. This is the first ever case of a diagnosis of CCH made using core needle biopsy. CCH can be challenging to diagnose; therefore, awareness of its histopathological and ultrasonographic features are essential to avoid misdiagnosis and over treatment.

Dermatoscopy: an auxiliary resource for the diagnosis of poroid neoplasms

Abstract: Poroid neoplasms are benign epithelial proliferations with eccrine sweat gland differentiation. They are a challenging diagnosis because of the clinical heterogeneity, being able to mimic several malignant neoplasms. They are classified into classic poroma, hidroacanthoma simplex, dermal duct tumor and poroid hidradenoma. Association of histological subtypes occurs in more than 25% of cases. We report a case of a combined poroid neoplasia of classical poroma and poroid hidradenoma, reviewing its dermatoscopic features.

Clear Cell Hidradenoma: Characteristic Imaging Features on Ultrasonography, Computed Tomography, and Magnetic Resonance Imaging.

OBJECTIVES: The purpose of this study was to evaluate the imaging features of clear cell hidradenoma on ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI). METHODS: The radiologic and pathologic databases at 2 medical institutions were searched retrospectively from 2004 to 2016 to identify patients with a diagnosis of clear cell hidradenoma. Ultrasonographic, CT, and MRI features were described, and pathologic specimens were reviewed. RESULTS: There were 5 female and 4 male patients. The mean patient age was 48.9 years (range, 28-70 years). Five patients underwent only US; 2 patients underwent only CT; 1 patient underwent both US and CT; and 1 patient underwent US and MRI. Most of the tumors were located primarily in the subcutaneous fat layer. The mean tumor size was 18.4 mm. On US, 6 masses had a heterogeneous echo texture, including an anechoic portion with protruding echogenic portions. Two masses had multiple septa in the anechoic portion. On color Doppler US, blood flow was both central and peripheral in 5 patients. All 3 cases seen on CT presented as a low-attenuation mass with an enhanced solid internal nodule. On MRI, the mass showed heterogeneous signal intensity on T2-weighted images and enhancement of the peripheral wall and internal solid component on contrast-enhanced T1-weighted images. CONCLUSIONS: Clear cell hidradenoma is usually located in the subcutaneous fat layer, has a well-defined margin, appears as a cystic mass with an internal solid nodule, and occasionally has multiple septa on US, CT, and MRI.

Sonographic appearance of an eccrine spiradenoma: A case report.

Eccrine spiradenoma is a rare, benign, adnexal skin tumor of the sweat gland, which may manifest in any part of the body. It is typically located in the dermal or the subcutaneous fat layer. Eccrine spiradenomas rarely progress to malignant transformation and only a few cases of malignant transformation have been reported. Due to its rarity, there have been few reports about the sonographic appearances of eccrine spiradenoma. We report the sonographic findings in one case of eccrine spiradenoma, located in the subcutaneous fat and the deep dermal layers of the upper arm in a middle-aged woman.

Ultrasonographic Features of Eccrine Spiradenoma.

The purpose of this series was to evaluate the features of eccrine spiradenoma on ultrasonography (US). We reviewed the clinical data of 8 patients with eccrine spiradenoma who underwent preoperative US at 4 different medical institutions from 2004 to 2016 and analyzed the US features in terms of the tumor location, size, shape, margin, echo texture, echogenicity, posterior acoustic enhancement, calcification, septum, and color Doppler flow. There were 7 female patients and 1 male patient. The mean patient age was 45.6 years (range, 28-60 years). Most of the tumors were located primarily in the subcutaneous fat layer. The mean size of the tumors was 14.3 mm. The masses had a lobular appearance in 7 patients and had a tractlike structure in 3 patients. In 6 patients, the masses had a heterogeneous echo texture. Six cases showed hypoechogenicity with more hypoechoic foci in the masses, and 2 cases showed hypoechogenicity only. Color Doppler flow was evaluated in 7 patients; the blood flow was central and peripheral in 4 patients and only peripheral in 3 patients. All cases showed posterior acoustic enhancement and had well-defined margins. Calcification and septa were not seen in any cases. Eccrine spiradenoma is usually located in the subcutaneous fat layer, has a well-defined margin, a lobulated appearance, occasionally with a tractlike structure, a heterogeneous echo texture, a hypoechoic appearance with internal hypoechoic foci and posterior acoustic enhancement, and shows blood flow in the peripheral portion, with or without blood flow in the central portion.

Syringoid Eccrine Carcinoma of the Thigh.

Syringoid eccrine carcinoma (SEC) is a rare cutaneous malignant tumor thought to be derived from eccrine sweat apparatus. It is usually present in the head, neck and trunk region, and often occurs in the fourth to seventh decades of life. A 94-year-old male patient visited our department with an 80-year history of a lesion showing a 2×2 cm sized well-demarcated round shaped erythematous to pinkish colored nodule with ulcer on his left thigh. Histological findings revealed a tumor consisted mainly of numerous small cords and nests forming luminal or tubular structures and tumor cells showing variable atypia. Some ductal structures showed tadpole appearance. On immunohistochemical staining, epithelial membrane antigen, S-100, cytokeratin 7 and carcinoembryonic antigen were reactive and Ki-67 showed less than 10% positivity. Based on these findings, the final diagnosis was made as SEC. The patient was treated with local wide excision and didn't show any recurrence during the follow-up period of 12 months. Herein, we report a very rare case of SEC which occurred on the left thigh and discuss 10 cases of SEC presented on the extremities, including our case.

Concomitant poroma and porocarcinoma

Abstract: Eccrine poromas and porocarcinomas are adnexal tumors derived from the sweat duct epithelium. However, eccrine poroma is benign in nature, whilst eccrine porocarcinoma is regarded as its malignant counterpart. We report the case of a man who presented simultaneously with an eccrine poroma and eccrine porocarcinoma. Both lesions had no clear distinctive features, enhancing the need of high-level clinical suspicion together with surgical excision and histopathology for prompt diagnosis confirmation.

Case for diagnosis. A bluish nodule on the scalp

Abstract: Acrospiroma, also known as hidradenoma, is a rare cutaneous tumor that has several histological characteristics. As a consequence, a high index of suspicion is necessary for its diagnosis. Here we report a case that illustrates the importance of a good clinical-pathologic correlation in order to recognize this disease.

Reversed cellular polarity in primary cutaneous mucinous carcinoma: A study on tight junction protein expression in sweat gland tumors.

Primary cutaneous mucinous carcinoma (PCMC) is a rare sweat gland tumor characterized by the presence of abundant mucin around the tumor islands, but the molecular mechanisms for this structure are not well elucidated. Because mucin is epithelial in nature, it is likely to be produced by epithelial tumor cells, not by surrounding stromal cells. We hypothesized that the abundant mucin is a result of reversed cellular polarity of the tumor. To test this hypothesis, we conducted an immunohistological study to investigate expression of tight junction (TJ) proteins occludin and ZO-1 in PCMC, as well as in normal sweat glands and other sweat gland tumors. Dot-like or linear expression of TJ proteins was observed at ductal structures of sweat glands, and ductal or cystic structures of related tumors. In PCMC, however, TJ protein expression was clearly visible at the edges of tumor cell islands. This study provides evidence to show that the characteristic histological structure of PCMC is caused by inverse polarization of the tumor cells, and that TJ proteins are useful markers of ductal differentiation in sweat gland tumors.

Espiroadenocarcinoma localizado en pabellón auricular y cuero cabelludo: reporte de un caso de localización inusual / Spiroadenocarcinoma located on ear auricle and scalp: a case report of unusual location

Introducción: El espiradenocarcinoma es una neoplasia maligna inusual que suele surgir de un espiradenoma benigno solitario preexistente. La mayoría de las lesiones aparecen en tronco y extremidades, pero casos extremadamente raros se han reportado en la región del cuero cabelludo y pabellón auricular. Objetivo: Describir el caso de un paciente en quien se diagnosticó espiradenocarcinoma. Diseño: Reporte de caso. Materiales y métodos: Se presenta el caso de un paciente adulto mayor con masa en región auricular y cuero cabelludo, de crecimiento progresivo. Resultados: Los estudios imagenológicos e histopatológicos mostraron una lesión tumoral maligna derivada de los anexos cutáneos complicada con infección y miasis. Se le informa la importancia de resección quirúrgica, pero los familiares se negaron a dicho procedimiento. Por lo cual se le ofrecen medidas paliativas. Conclusión: Describimos un caso extremadamente raro de un espiroadenocarcinoma en cuero cabelludo cerca del pabellón auricular; siendo el primer caso descrito en Colombia.

Introduction: Spiradenocarcinomas an extremely rare malignant neoplasm. Most often arises from a preexisting solitary benign spiroadenoma. Most of the lesions often appear on the trunk, limbs and unusually, on the region of the scalp near the pinna. Objective: To describe a case of a patient who was diagnosed with spiroadenoma. Design: Case report. Methods: We present the case of an elderly patient with a progressive growth mass on the scalp near the pinna. Results: Imaging studies in conjunction with histopathology allowed to evidence a malignant tumor lesion derived from skin annexes and complicated with an infection process and secondary myiasis. We told him the importance of performing surgery but the family refused this procedure. Conclusion: We report an extremely rare case of a spiroadenocarcinoma of the scalp near the pinna; this is the first case reported in Colombia

[Spiradenocarcinoma of the forehead : A rare skin tumor in the head and neck region]. / Spiradenokarzinom der Stirn : Ein seltener Hauttumor im Kopf-Hals-Bereich.

Spiradenocarcinomas are rare malignant tumors that originate from the sweat glands of the skin and demonstrate aggressive growth. We report the case of an 86-year-old female patient presenting with a growth on the forehead which had been apparent for 2 years. After surgical removal of the tumor, histological workup culminated in the diagnosis of a spiradenocarcinoma. Surgical margins were free of tumor on pathological examination. Metastasis was excluded by positron-emission tomography-computed tomography (PET-CT). Due to the advanced age of the patient and the absence of metastatic disease, no adjuvant therapy was performed. Six months postoperatively there is no evidence of relapse.

Zosteriform configuration of multiple eccrine spiradenomas of the scalp.

BACKGROUND: Eccrine spiradenoma (ES) is a benign adnexal tumor predominantly located in the head and neck regions. Multiple neoplasms located on the scalp have been reported but never with a zosteriform configuration on the first trigeminal area. CASE REPORT: We describe an original case report of a 75-year-old Caucasian man presenting multiple subcutaneous blue and purple nodules disseminated on the first left trigeminal dermatome. All the nodules appeared gradually on a one-year period. Biopsy revealed a nodular adnexal tumor in the dermis without malignant eccrine spiradenoma (MES) transformation. The surgical procedure was performed in a manner to protect the galea aponeurotica in the upper half on the first left trigeminal area. The frontalis muscle was raised with the surgical specimen in the lower half of the first trigeminal area. A split-thickness skin graft was applied on the surgical defect. Histological examination revealed multilobular well-defined tumors located in the dermis. CONCLUSION: The presence of multiple subcutaneous nodules in a trigeminal pattern should suggest a multiple localized zosteriform ES. The diagnosis is focused on clinical findings and the treatment is based on a large surgical excision. The histological examination is essential for not to fail a MES transformation.