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Malignant tumors with apocrine or eccrine differentiation I. (A1) (20×, HE) and (A2) (20×, CK7): adnexal adenocarcinoma, not otherwise specified, deeply invasive lesion, involves deep dermis and extends to subcutaneous tissues. (B1) (40×, HE) and (B2): Porocarcinoma characterized by infiltrative borders, and atypical cytological features, increased mitotic activity, and focal necrosis. (C1) (400×, HE) and (C2) (200×, Ki67): Hidradenocarcinoma, predominantly composed of bland, clear cells, with increased mitotic activity.
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Adnexal tumors of the skin are a rare group of benign and malignant neoplasms that exhibit morphological differentiation toward one or more of the adnexal epithelium types present in normal skin. Tumors deriving from apocrine or eccrine glands are highly heterogeneous and represent various histological entities. Macroscopic and dermatoscopic featur...
Citations
... Hidradenocarcinoma is a rare and fearsome malignant tumour that originates from the ducts of the eccrine sweat glands. It accounts for less than 6% of malignant eccrine and 0.001% of all tumours [1,2]. Usually it arises de novo, rarely from a pre-existing benign hidradenoma, with a slight predominance in the female sex [1,3]. ...
... It accounts for less than 6% of malignant eccrine and 0.001% of all tumours [1,2]. Usually it arises de novo, rarely from a pre-existing benign hidradenoma, with a slight predominance in the female sex [1,3]. Hidradenocarcinoma rarely progresses from hidradenitis suppurativa [4]. ...
... Hidradenocarcinoma commonly arises de novo, although it may derive from pre-existing eccrine acrospiroma [2]. Histopathological examination of biopsied specimens from the skin is essential for making a correct diagnosis [3]. The optimal therapeutic choice is total surgical excision [4][5][6]. ...
... CT, in this case, is less informative than MR imaging and duplex ultrasonography. Histological analysis remains the gold standard for diagnosing eccrine acrospiroma [3]. Rarely, benign neoplasms can undergo malignant transformation. ...
Background: Cutaneous adnexal tumors are a rare cluster of neoplasms deriving from the adnexal epithelial parts of the skin, including the eccrine sweat gland epithelium. They are uncommon and heterogeneous histological entities. Generally, they present as solid or cystic lesions that seldom undergo malignant transformation. Due to their rarity in routine clinical practice, they often pose a diagnostic dilemma. Management usually involves appropriate wide local resection margins of the primary tumor. Recurrence after incomplete excision is well-known, as observed in our case. Case presentation: A patient presented here with a recurrent cutaneous adnexal tumor on the dorsum of the forearm, histopathologically proven to be a syringoepithelioma (eccrine acrospiroma), which was planned to be managed by repeat surgical excision with adequate volume. Conclusion: Eccrine acrospiroma is a solitary benign cutaneous tumor arising from the eccrine sweat gland epithelium. Only pathological analysis will lead to the diagnosis. The surgical management approach of adequate excision is the only effective treatment to prevent recurrence and malignant transformation.
... The etiology remains unknown, but certain risk factors that may favor their carcinogenesis are ionizing radiation, exposure to ultraviolet rays, immunosuppression and antecedents of trauma [2,3]. ...
... Apocrine adnexal carcinoma occurs at an average age of 66 years [4]. There is no difference in frequency between the sexes [3,5,6]. Clinically, they appear as asymptomatic nodules or plaques with a slowly progressive evolution; they may reach large diameters and ulcerate [1,7]. ...
... Therefore, diagnosis is based primarily on morphological assessment. However, the diversity and rarity of eccrine carcinomas makes correct morphological classification challenging [1,5]. In DEA, the appearance of marked ductal proliferation mimics metastatic adenocarcinoma from non-cutaneous sites such as the breast, further complicating the differential diagnosis [3]. ...
... The mainstay of management for localised eccrine carcinomas is surgical resection. This involves either wide local excision or Mohs micrographic surgery to achieve negative resection margins [5]. Regional lymph node dissection is recommended only if there is clinical suspicion of nodal disease [2]. ...
... Regional lymph node dissection is recommended only if there is clinical suspicion of nodal disease [2]. Radiotherapy is often utilised as an adjunct to resection in highrisk cases such as large tumours, close resection margins, or lymphovascular or perineural invasion [5]. ...
Eccrine carcinoma, a subtype of which is ductal eccrine adenocarcinoma (DEA), is a rare cutaneous malignancy. For metastatic eccrine carcinoma, there are very limited data to guide treatment. Conventional chemotherapy is of limited benefit and there is only a small body of evidence for the use of immunotherapy in non-DEA eccrine carcinomas. We report the first case of metastatic DEA treated with a multimodality approach including surgery, radiotherapy, and immunotherapy, with an excellent prolonged response to pembrolizumab, and provide a review of the literature on pathological and management aspects for this rare tumour subtype. A 60-year-old male with a history of pT1N0M0 left scalp DEA, managed 2 years prior with excision and adjuvant radiotherapy, represented with a symptomatic right pontine metastasis. Imaging demonstrated intracranial, pulmonary, and hilar disease; biopsy of the cranial and lung lesions showed metastatic adenocarcinoma, morphologically similar to the previously resected scalp DEA. The patient was treated with stereotactic resections of his pontine metastases and adjuvant cranial radiotherapy, then commenced on immunotherapy with pembrolizumab. The patient has completed 21 months of pembrolizumab with a significant radiological response of the pulmonary and hilar disease and nil evidence of intracranial recurrence or further metastases. In this case report, we provide the first evidence of efficacy of immunotherapy in metastatic DEA, demonstrating an excellent and prolonged response of metastatic DEA to pembrolizumab. Further research is required to better establish the role of immunotherapy within the management protocol for this uncommon but aggressive tumour subtype.
... Presence of nodal metastasis was found to be a poor prognostic factor. Plachta I et al 10 have discussed the management approach of skin adnexal tumours, after summarizing the key points of diagnosis. ...
Adnexal or appendageal tumours of skin comprise an important subcategory of cutaneous neoplasms, although thorough understanding of this entity is still in its early stages. The role of immunohistochemistry in diagnosis is particularly limited, and, it is morphology that actually guides pathologists for clinching diagnosis. Here, we present three uncommon cases of skin adnexal tumours- a case of retiform trichoblastoma, trichilemmal carcinoma and sebaceous carcinoma; especially highlighting the morphological approach of diagnosis and the associated clinical implications based on literature review.
... Hidradenocarcinomas are malignant adnexal tumors that develop from sweat glands, more specifically of eccrine or apocrine origin [1]. These neoplasms present a very low incidence, accounting for only 0.001% of all tumors [2]. Often affecting the head and neck, hidradenocarcinomas may rarely arise in the extremities, the trunk, or the abdomen [3]. ...
... Considering there is no consensus guiding the proper management of hidradenocarcinomas owing to their rarity, wide local excision with at least a 3-cm surgical margin remains the cornerstone of treatment [2]. In some instances, an initial excisional biopsy or wide local excision can be performed, followed by a subsequent re-intervention to achieve a free margin resection and local control of the disease [8,9]. ...
... A surgical approach aiming for a wide local excision with at least a 3 cm surgical margin is the most crucial key aspect regarding hidradenocarcinoma treatment [2]. To properly address these neoplasms, surgeons must be proficient in oncoplastic techniques to achieve a free-margin resection without compromising the local control of the disease or the aesthetic outcome [20]. ...
Clear-cell hidradenocarcinomas are extremely uncommon sweat gland tumors with a predilection for the head and neck. In the limited number of articles reporting breast involvement, the primary focus concerns this entity's histological and immunohistochemical characteristics.
Since hidradenocarcinomas of the breast have the potential to resemble a primary breast carcinoma closely, diagnosis may be challenging. Therefore, the authors report the first case of hidradenocarcinoma of the breast, which features its macroscopic morphology. In addition, to increase physicians’ awareness of this rare neoplasm, the article also aims to detail its surgical approach.
... CMANs are a group of rare and aggressive tumors that originate from the adnexal structures of the skin, including hair follicles, sweat glands, and sebaceous glands [68]. These tumors exhibit malignant behavior and can pose diagnostic challenges due to their diverse histopathological features [69,70]. ...
... Additional rare CMANs include malignant pilomatricoma, malignant cylindroma, and malignant mixed tumors. These tumors exhibit malignant features such as cellular atypia, mitotic activity, and infiltrative growth [69]. ...
Skin cancers require a multidisciplinary approach. The updated guidelines introduce new insights into the management of these diseases. Melanoma (MM), the third most common skin cancer, a malignant melanocytic tumor, which is classified into four major histological subtypes, continues to have the potential to be a lethal disease. The mortality–incidence ratio is higher in Eastern European countries compared to Western European countries, which shows the need for better prevention and early detection in Eastern European countries. Basal cell carcinoma (BCC) and squamous cell carcinoma (cSCC) remain the top two skin cancers, and their incidence continues to grow. The gold standard in establishing the diagnosis and establishing the histopathological subtype in BCC and SCC is a skin biopsy. Sebaceous carcinoma (SeC) is an uncommon and potentially aggressive cutaneous malignancy showing sebaceous differentiation. It accounts for 0.7% of skin cancers and 3–6.7% of cancer-related deaths. Due to the rapid extension to the regional lymph nodes, SeC requires early treatment. The main treatment for sebaceous carcinoma is surgical treatment, including Mohs micrographic surgery, which has the advantage of complete margin evaluation and low recurrence rates. Primary cutaneous lymphomas (PCLs) are a heterogeneous group of lymphoproliferative diseases, with no evidence of extracutaneous determination at the moment of the diagnosis. PCLs have usually a very different evolution, prognosis, and treatment compared to the lymphomas that may secondarily involve the skin. The aim of our review is to summarize the important changes in the approach to treating melanoma, non-melanoma skin, cutaneous T and B cell lymphomas, and other types of skin cancers. For all skin cancers, optimal patient management requires a multidisciplinary approach including dermatology, medical oncology, and radiation oncology.
... Cutaneous adnexal neoplasms are notorious for masquerading as skin cancers due to their close proximity to the skin; thus, they should be considered on the list of differential diagnoses for suspicious skin lesions [1]. The majority of adnexal neoplasms arise from apocrine and eccrine sweat glands due to an unknown etiology, but hypothetical causes include UV radiation, immunosuppression, trauma to the affected area, and genetic predisposition [1]. ...
... Cutaneous adnexal neoplasms are notorious for masquerading as skin cancers due to their close proximity to the skin; thus, they should be considered on the list of differential diagnoses for suspicious skin lesions [1]. The majority of adnexal neoplasms arise from apocrine and eccrine sweat glands due to an unknown etiology, but hypothetical causes include UV radiation, immunosuppression, trauma to the affected area, and genetic predisposition [1]. For instance, mutations in the epidermal growth factor receptor (EGFR), Wnt family member 10A (WNT10A), HRas proto-oncogene (HRAS), and retinoblastoma (RB1) genes have been associated with the tumorigenesis of benign and malignant adnexal neoplasms [1,2]. ...
... The majority of adnexal neoplasms arise from apocrine and eccrine sweat glands due to an unknown etiology, but hypothetical causes include UV radiation, immunosuppression, trauma to the affected area, and genetic predisposition [1]. For instance, mutations in the epidermal growth factor receptor (EGFR), Wnt family member 10A (WNT10A), HRas proto-oncogene (HRAS), and retinoblastoma (RB1) genes have been associated with the tumorigenesis of benign and malignant adnexal neoplasms [1,2]. Syringocystadenocarcinoma papilliferum (SCACP) is a rare cutaneous adnexal neoplasm that typically arises from its benign precursor lesion, syringocystadenoma papilliferum (SCAP), or nevus sebaceous (NS) [3][4][5]. ...
Syringocystadenocarcinoma papilliferum (SCACP) is an exceptionally rare cutaneous adnexal tumor that is infrequently encountered by clinicians worldwide. The tumor typically appears in the older population, affecting patients in their fifth and sixth decades of life without male or female predominance. Patients frequently present with a variable-sized hyperpigmented ulcerative lesion containing an exudate that has a long-standing course of progression from its benign counterpart, Syringocystadenoma papilliferum (SCAP). Additionally, the clinical presentation and morphology of the neoplasm can be easily confused with a variety of other skin cancers, such as squamous cell carcinoma (SCC), basal cell carcinoma (BCC), cutaneous lymphoma, and cutaneous metastasis. Therefore, histopathology and tissue analysis play an essential role in establishing an accurate diagnosis. However, the lesion is so rare that no definitive diagnostic markers have been established yet. We present a case of SCACP localized to the scalp of the patient. Our case study highlights the presence of specific tumor markers that could potentially serve as objective criteria for diagnosis.
... Considering all the data we diagnosed adnexal adenocarcinoma of not otherwise specified type. 6 MCATs are rare neoplasms that do not have a well-studied treatment algorithm. 7 According to the risk of recurrence and metastasis, we have discussed the case at multidisciplinary commission. ...
Malignant cutaneous adnexal tumors are rare dermatosis that can have extradermal twins. Diagnosis of adnexal skin tumors is sometimes difficult. Immunohistochemistry has limited value in the diagnosis. Most adnexal skin tumors are localized on the head and neck. Difficulties in their diagnosis associated with absence of strict clinical criteria.
... For this reason, this technology seems ideal for solid and superficial tumors, such as melanomas, one of the leading causes of death from skin cancer [11]. This field of study attracts the scientific community's interest, considering that the therapies against malignant melanoma still lack efficacy due to the heterogeneity [12] and metastatic dissemination [13] of this cancer kind [14,15]. Different types of fillers have been successfully loaded in electrospun membranes [16,17], but understanding how to tune the drug release is still unclear. ...
Electrospun systems are becoming promising devices usable for topical treatments. They are eligible to deliver different therapies, from anti-inflammatory to antitumoral. In the current research, polycaprolactone electrospun membranes loaded with synthetic and commercial antitumoral active substances were produced, underlining how the matrix-filler affinity is a crucial parameter for designing drug delivery devices. Nanofibrous membranes loaded with different percentages of Dacarbazine (the drug of choice for melanoma) and a synthetic derivative of Dacarbazine were produced and compared to membranes loaded with AuM1, a highly active Au-complex with low affinity to the matrix. AFM morphologies showed that the surface profile of nanofibers loaded with affine substances is similar to one of the unloaded systems, thanks to the nature of the matrix-filler interaction. FTIR analyses proved the efficacy of the interaction between the amidic group of the Dacarbazine and the polycaprolactone. In AuM1-loaded membranes, because of the weak matrix-filler interaction, the complex is mainly aggregated in nanometric domains on the nanofiber surface, which manifests a nanometric roughness. Consequently, the release profiles follow a Fickian behavior for the Dacarbazine-based systems, whereas a two-step with a highly prominent burst effect was observed for AuM1 systems. The performed antitumoral tests evidence the high-cytotoxic activity of the electrospun systems against melanoma cell lines, proving that the synthetic substances are more active than the commercial dacarbazine.
