Benign Adnexal Tumors Research Articles

Dermoscopy of nodular hydradenoma

Hidradenoma Nodularis (HN) is a benign adnexal tumor that was generally considered to be of eccrine origin, although apocrine forms also exist. It develops from sweat glands located in the superficial or deep dermis or sometimes even in the subcutaneous tissue. Clinically, it appears as a small, solitary, firm papule, which may be dark or transparent in color, although usually a single papule, it is less common to find multiple papules [1]. the confirmation of the diagnosis is based on the histology spectra which will find a dermal nodule well bounded by a pseudocapsule of collagen which lobules filled with epithelial cells and tubular structures. Tumor proliferation in HN is very typical, dense and often hyalinized, making it an important element in the diagnosis. It is characterized by two cell types: polyhedral cells with elongated nuclei and slightly basophilic cytoplasm, and cells with small rounded nuclei and clear cytoplasm [2]. few publications in the literature have described the dermoscopic aspect which has structureless white-pink areas; red lacunalike areas; and hairpin, dotted, and linear irregular vessels [3,4], we describe a new case of dermoscopy of nodular hydradenoma.

Genetic and Immunohistochemical Profiling of Mammary Hidradenoma and Comparison to Mucoepidermoid Carcinoma

Mucoepidermoid carcinoma (MEC) is exceedingly rare in the breast, with <45 cases reported in the literature. Although estrogen receptor/progesterone receptor/human epidermal growth factor 2 triple-negative, MEC is characterized as a special subtype of breast carcinoma with significantly better prognosis than conventional basal-type tumors. Cutaneous hidradenoma (HA) is considered a benign adnexal neoplasm showing histomorphologic overlap with MEC. Rare cases of HA have also been reported in the breast, but these are relatively uncharacterized. In this study, we examined the clinicopathologic, immunohistochemical (IHC), and genetic features of 8 breast HAs, in comparison to 3 mammary MECs. All cases were positive for MAML2 break-apart fluorescence in situ hybridization. Eight cases demonstrated a CRTC1::MAML2 fusion, and one MEC harbored a CRTC3::MAML2 fusion; the latter is a novel finding in the breast. Mutational burden was very low, with only one HA exhibiting a MAP3K1 pathogenic alteration. By IHC, both MEC and HA demonstrated cell type–dependent expression of high- and low-molecular-weight keratins and p63, as well as negative to low-positive estrogen receptor and androgen receptor. Smooth muscle myosin and calponin highlighted an in situ component in the 3 cases of MEC; expression of these myoepithelial markers was negative in HAs. Additional distinguishing characteristics included the growth pattern and tumor architecture, the presence of glandular/luminal cells in HA, and overall higher IHC expression of SOX10, S100 protein, MUC4, and mammaglobin in MEC. Morphologic findings were also compared to a series of 27 cutaneous nonmammary HAs. Mucinous and glandular/luminal cells were identified in significantly more mammary HAs than nonmammary lesions. The findings provide insight into the pathogenesis of MAML2-rearranged neoplasms of the breast, underscore the overlapping genetic features of MEC and HA, and highlight similarities to their extramammary counterparts.

Linear syringocystadenoma papilliferum of neck: a rare variant adnexal tumour

Syringocystadenoma papilliferum is a rare benign adnexal tumour presenting as exuberant proliferating lesion showing differentiation in apocrine pattern. The three clinical variants are linear, plaque and solitary of which linear variant is the rarest. We report a case of linear variant of syringocystadenoma papilliferum, which had typical histopathological features. As there is risk of malignancy in long standing cases, early diagnosis and treatment is of utmost importance.

Trichoepithelioma of Upper Eyelid: A Case Report

Solitary trichoepithelioma is a rare benign adnexal tumour of hair follicles, especially over the upper eyelid. Reconstruction in these cases has not been discussed in the literature. We are reporting a case of a 60-year-old male patient with trichoepithelioma of the upper eyelid involving 70% of the upper eyelid which was managed by primary closure; thus avoiding unnecessary extensive and time-consuming reconstructive procedures.

Eruptive syringomas – An uncommon presentation in a male

Syringomas are benign adnexal tumors derived from the intraepidermal portion of eccrine glands. They are mostly found in young to middle aged women as asymptomatic (sometimes pruritic), soft, and yellowish-brown or skin-colored papules with bilaterally symmetrical distribution. The lesions have a predilection for periorbital area (primarily lower eyelids) and cheeks. A variant-eruptive syringoma presents as successive crops of disseminated lesions in atypical areas. There are very few reports of such cases. Therefore, we report this rare variant of syringoma in a male on an unusual site.

A rare tumor of atypical scrotal location

Apocrine hidrocystoma is a rare benign adnexal tumor considered a cystic proliferation of the apocrine glands, rather than simple retentive cysts of the apocrine sweat glands [3]. It occurs most often in adults on the neck and head and especially on the external canthus of the eye [1,2]. They vary in size from 3 to 15 millimeters and are most often pigmented, blue, blue-black, grayish, translucent or violet in color. Apocrine hidrocystomas located in other sites such as the shoulders, fingers, abdomen and genital region have been rarely described.Differential diagnoses of apocrine hidrocystomas of scrotal location include eccrine hidrocystomas, blue nevus, melanoma, median raphe cyst, milium grain, epidermoid or pilar cyst, and hemolymphangioma

Recurrent PAK2 rearrangements in poroma with folliculo-sebaceous differentiation.

Poroma is a benign adnexal neoplasm with differentiation towards the upper portion of the sweat gland apparatus. In 2019, Sekine etal. demonstrated recurrent YAP1::MAML2 and YAP1::NUTM1 fusion in poroma and porocarcinoma. Follicular, sebaceous and/or apocrine differentiation has been reported in rare cases of poroma and whether these tumours constitute a variant of poroma or represent a distinctive tumour is a matter to debate. Herein we describe the clinical, immunophenotypic, and molecular features of 13 cases of poroma with folliculo-sebaceous differentiation. Most of the tumours were located on the head and neck region (n = 7), and on the thigh (n = 3). All presented were adults with a slight male predilection. The median tumour size was 10 mm (range: 4-25). Microscopically, lesions displayed features of poroma with nodules of monotonous basophilic cells associated with a second population of larger eosinophilic cells. In all cases, ducts and scattered sebocytes were identified. Infundibular cysts were present in 10 cases. In two cases high mitotic activity was noted, and in three cases cytologic atypia and areas of necrosis were identified. Whole transcriptome RNA sequencing demonstrated in-frame fusion transcripts involving RNF13::PAK2 (n = 4), EPHB3::PAK2 (n = 2), DLG1::PAK2 (n = 2), LRIG1::PAK2 (n = 1), ATP1B3::PAK2 (n = 1), TM9SF4::PAK2 (n = 1), and CTNNA1::PAK2 (n = 1). Moreover, fluorescence in situ hybridisation (FISH) analysis revealed PAK2 rearrangement in an additional case. No YAP1::MAML2 or YAP1::NUTM1 fusion was detected. Recurrent fusions involving the PAK2 gene in all analysed poroma with folliculo-sebaceous differentiation in this study confirms that this neoplasm represents a separate tumour entity distinct from YAP1::MAML2 or YAP1::NUTM1 rearranged poromas.

Apocrine Papillary Hidrocystoma With Mucinous Metaplasia (Goblet Cell Type): A Case Report and Review of the Literature.

Mucinous metaplasia (goblet cell type) is exceptionally rare in the skin. This is the second case of apocrine papillary hidrocystoma with mucinous metaplasia (goblet cell type) and a review of the literature exploring the significance and frequency of mucinous metaplasia with goblet cells in nongenital skin. The patient is an elderly man who presented with a blue-pigmented nodule on the scalp that was clinically suggestive of an atypical nevus. Histologically, the lesion was composed of a simple cyst of cuboidal cells with decapitation secretion and mucinous metaplasia with goblet cells. Papillary formation was identified in the cysts. Most cases of cutaneous mucinous metaplasia have been reported on genital skin, usually after chronic inflammation of the area. This type of mucinous metaplasia is categorized as benign mucinous metaplasia of the genitalia (BMM) and is believed to be unrelated to apocrine glands owing to the different histologic features and absence of apocrine differentiation by immunohistochemistry. Mucinous metaplasia (goblet cell type) has been previously reported in benign adnexal tumors (eccrine acrospiroma/hidroadenoma, mixed tumor, and syringocystadenoma papilliferum) and in malignant tumors (apocrine hidradenocarcinoma and squamous cell carcinoma). To date, mucinous metaplasia has not been identified in the histologically normal apocrine glands.

CHONDROID SYRINGOMA :A RARE UPPER EYELID TUMOR

Chondroid syringoma is a benign adnexal mixed tumor of skin characterized by sweat gland elements in a cartilaginous stroma1. It is a tumor found to be arising from the sweat glands which can be apocrine (found throughout the surface of lid) or eccrine (glands of moll) with a mean age of 50 years2. Chondroid syringoma is a rare benign tumour that accounts for 0.01% of all primary skin tumours3 and occurs rarely in the upper eyelid region. Head and neck region involvement is common (nose is the most common site).It is morphologically similar to pleomorphic adenoma or benign mixed tumour of the salivary gland4. A 18-year-old male patient presented to us with a slow growing, painless mass on right upper eyelid since 6 months. Complete surgical excision of the mass was done under local anaesthesia. This report aims to discuss about this rare benign tumor of the eyelid and to study the histopathological aspects of it.

TRPS1 Is Differentially Expressed in a Variety of Malignant and Benign Cutaneous Sweat Gland Neoplasms

Neoplasms of sweat glands and the breast may be morphologically and immunophenotypically similar. A recent study showed that TRPS1 staining is a highly sensitive and specific marker for breast carcinoma. In this study, we analyzed TRPS1 expression in a spectrum of cutaneous sweat gland tumors. We stained five microcystic adnexal carcinomas (MACs), three eccrine adenocarcinomas, two syringoid eccrine carcinomas, four hidradenocarcinomas, six porocarcinomas, one eccrine carcinoma-NOS, 11 hidradenomas, nine poromas, seven cylindromas, three spiradenomas, and 10 syringomas with TRPS1 antibodies. All of the MACs and syringomas were negative. Every cylindroma and two of the three spiradenomas demonstrated intense staining in cells lining the ductular spaces, with negative to relatively weak expression in surrounding cells. Of the 16 remaining malignant entities, 13 were intermediate to high positive, one was low positive, and two were negative. From the 20 hidradenomas and poromas, intermediate to high positivity was revealed in 14 cases, low positivity in three cases, and negative staining in three cases. Our study demonstrates a very high (86%) expression of TRPS1 in malignant and benign adnexal tumors that are mainly composed of islands or nodules with polygonal cells, e.g., hidradenomas. On the other hand, tumors with small ducts or strands of cells, such as MACs, appear to be completely negative. This differential staining among types of sweat gland tumors may represent either differential cells of origin or divergent differentiation and has the potential to be used as a diagnostic tool in the future.

Regional recurrence of digital papillary carcinoma in ipsilateral axillary lymph nodes 12 years after digital amputation – a case report

Digital papillary adenocarcinoma (DPA) is a rare malignant adnexal tumour that affects 0.08 per 1,000,000 people/year.1 It often presents as a solitary painless mass in the digital extremities where eccrine sweat glands are abundant. It is reported to have a recurrence rate of 50% and 14% rate of metastasis.2 Due to its rarity and vague clinical presentation, it has frequently been misdiagnosed as a benign adnexal tumour, pyogenic granuloma and ganglion cyst. We report a rare case of regional recurrence of DPA in an ipsilateral axillary node of a 66- year-old male, 12 years after a right ring finger distal phalanx amputation.

Eccrine poroma on the forearm in a child: A rare presentation

Eccrine poroma (EP) is a benign adnexal tumor arising from the terminal duct of the sweat gland. It represents 10% of all sweat gland tumors and is most commonly found on the sole or the side of the foot [1]. Herein, we report a case of EP on the right forearm in a child. A thirteen-year-old female with no past medical history presented with an asymptomatic nodule on the right forearm (Fig. 1). The nodule had gradually increased in size and was not associated with pain, pruritus, or bleeding from the lesion. A physical examination revealed a firm, mobile flesh-colored nodule on the right forearm 7 × 6 mm in size. A dermoscopic examination revealed chalice-like vessels, whitish-pink areas, and yellow structureless areas (Fig. 2). The nodule was completely excised and histopathological findings showed a tumor proliferation connecting to the epidermis, organized on thick, cellular cords and composed of small, cohesive, round cells forming homogeneous layers. These cellular cords were separated by fibrous interstitial tissue, not especially inflammatory, and traversed by regularly distributed capillaries (Figs. 3 and 4). Based on these histological findings, a diagnosis of EP was reached.

Dermoscopy of Cylindroma in Fitzpatrick Type IV Skin: A Case Report and Review of Literature.

Dermoscopy of Cylindroma in Fitzpatrick Type IV Skin: A Case Report and Review of Literature.

Clinicopathological Spectrum of Syringoma: A Report of 50 Cases From a Tertiary Care Hospital in Eastern India.

Background Syringoma is a benign adnexal neoplasm and is considered safe with very low malignant potential. However, multiple tiny lesions typically affect the face and exposed area, which may cause a cosmetic concern for the patient.After a clinical diagnosis, there are two methods to diagnose syringoma: fine needle aspiration cytology (FNAC) and histopathology. FNAC is generally used for the initial evaluation of syringoma, while histopathology is used as a confirmatory test to diagnose syringoma. In developing and resource-limited settings, the combination of FNAC and histopathology would cause a financial and logistics burden. Objective This study aimed to observe the cytological and histopathological features of cases clinically diagnosed as syringomas in a tertiary care hospital to suggest the use of either FNAC or histopathology for diagnosing syringoma. Materials and Methods This cross-sectional observational study was conducted in the Department of Dermatology and Department of Pathology of a tertiary care hospital in eastern India from November 2021 to April 2022. Any clinically provisionally diagnosed case of syringoma was recruited for the study after obtaining informed consent for voluntary participation. With aseptic precautions, the tissue aspirates and punch biopsy were obtained in the Department of Dermatology and the samples were sent to the Department of Pathology. Cytological and histological examination was conducted by a single expert pathologist. Result A total of 50 cases (36 female, 14 male) with a median age of 23 years (range 10-40 years) were included in the study. A total of 43 cases were presented with papular lesions and seven with nodules. In the majority of the cases (40%), the lesion was in the eyelid followed by 26% in the arm. In FNAC, 22 cases were found to be benign adnexal lesions, 16 were suggestive of syringoma, eight were diagnosed as xanthoma, two were diagnosed as warts, and two cases were inadequate for opinion. Histologically, 42 cases were confirmed as syringoma, six were diagnosed as xanthoma, and two cases were diagnosed as warts. There was a significant difference between diagnosis by FNAC and histopathology (McNemar χ2 = 24.038, p-value = 0.0001). Conclusion We found that FNAC and histopathological diagnosis of syringoma may not be corroborative. Benign adnexal lesions are difficult to categorize by FNAC. Histopathological examination of clinically diagnosed cases of syringoma is of help for definitive diagnosis. Hence, FNAC may be avoided for saving time and discomfort for the patients and clinically diagnosed cases may be diagnosed by histopathological examination.

“ CHONDROID SYRINGOMA OF NOSE-AN UPDATE OF MIXED ADNEXAL SKIN TUMOR.”

Chondroid Syringoma is a rare benign adnexal skin tumor arising from sweat glands(apocrine or eccrine) situated in the dermis. They are often mistaken for other benign adnexal tumors of the skin and are most commonly seen in the head and neck region and, in individuals above the age of 40 years and predominantly in males. Rarely they can turn malignant and diagnosis is only by histopathology. We had an occasion to treat a 50 years old female patient with this tumor occupying the entire right alanasi. We could treat her well by complete excision with out any deformity of the alanasi. We are reporting this case, since this is the rst case reported arising from the alanasi in the world literature and also review the available literature.

CeruminousSyringocystadenoma papilliferum: An Extremely Rare Neoplasm of the External Ear

Abstract Introduction/Objective Syringocystadenoma papilliferum (SCAP) is a rare benign adnexal tumor derived from the apocrine or the eccrine sweat glands on the face or the scalp. Ceruminous glands are modified apocrine glands in the external auditory canal and produce cerumen that helps the ear canal against physical damage and microbial invasion. Neoplastic transformation of these glands is even rarer among these benign tumors. Methods/Case Report Here we report a case of a 29-year-old female with a three-month history of progressively worsening right-sided ear pain, muffled hearing, aural fullness, and right-sided shooting aches. She reported that her ear felt sensitive to touch and was painful if she lay on it. Previous treatments included steroids and oral antibiotics without any improvement in symptoms. A physical exam revealed a right narrow ear canal with a cystic mass to the superior lateral canal wall. A CT Temporal Bone showed a 1 cm oval soft tissue lesion in the lateral osseous portion of the right external auditory canal with no underlying osseous erosions identified. This is nonspecific, and neoplasm cannot be excluded. The decision was made for excision. The mass ruptured during excision with dishwater appearing fluid. Microscopic examination showed papillae lined by bilayered glandular epithelium projecting into a cystic lumen. Well defined myoepithelial cell layer is appreciated. Decapitation secretions and plasma cells are noted to be present in the stroma. A final diagnosis of Syringocystadenoma papilliferum is made. Results (if a Case Study enter NA) NA. Conclusion Ceruminous Syringocystadenoma papilliferum is an extremely rare benign tumor of the ceruminous glands of the external ear. Complete excision is the treatment of choice

Three cases of eruptive syringoma with dermoscopic features

Syringoma is a benign adnexal neoplasm originating from the intraepidermal eccrine duct. Eruptive Syringoma (ES) is a rare variant that is characterized by skin-colored to reddish-brown and shiny papules. Here we report three cases of ES, one of them initially misdiagnosed as Darier disease. Dermoscopy showed light brown pigment networks and dotted or linear vessels. All these cases were confirmed as ES by histopathological examination, highlighting the importance of histopathological and dermoscopic evaluation.

Contrast-enhanced ultrasonography for differential diagnosis of adnexal masses.

Quantitative contrast-enhanced ultrasonography parameters are affected by various factors. We evaluated corrected quantitative contrast enhanced ultrasonography in differentiating benign adnexal tumors from malignant tumors. Patients with adnexal masses who underwent conventional and contrast-enhanced ultrasonography were included. Contrast-enhanced ultrasonography parameters such as base intensity, arrival time, peak intensity, time to peak intensity, ascending slope, and descending slope were measured. Corrected (time to peak intensity - arrival time) mass/(time to peak intensity - arrival time) uterus and (peak intensity - base intensity) mass/(peak intensity - base intensity) uterus were calculated. Lesions were confirmed by pathologic examination of surgical specimens. This study included 31 patients with 35 adnexal lesions including 20 (57.10%) benign and 15 (42.90%) malignant lesions. The corrected contrast-enhanced ultrasonography quantitative parameters in lesions were statistically different between malignant and benign groups (P<0.05). The optimal cut-off value for (time to peak intensity - arrival time) mass/(time to peak intensity - arrival time) uterus, ascending slope, and (peak intensity - base intensity) mass/(peak intensity - base intensity) uterus, and descending slope for differentiating malignant adnexal masses from benign tumors were 1.05 (area under curve: 0.93,P<0.05), 1.11 (area under curve: 0.83,P<0.05), 0.82 (area under curve: 0.73,P<0.05), and -0.27 (area under curve: 0.66,P=0.16), with sensitivity and specificity of 93.33% and 85.00%, 86.67% and 75.00%, 86.67% and 60.00%, and 54.55% and 66.67%, respectively. Corrected contrast-enhanced ultrasonography parameters provide practical differential diagnosis value of adnexal lesions with high reliability for sonologists.

Eccrine poroma: When dermoscopy is useful for diagnosis

Eccrine Poroma is a rare benign adnexal tumor. It presents the problem of differential diagnosis with several entities. Dermoscopic features are suggestive and may guide the diagnosis. We report a new observation. A 44-year-old woman, presented with an asymptomatic lesion on the dorsal side of the left foot for 2 years. Clinically the nodule was brownish, well delimited with superficial erosions, and firm on palpation. Dermoscopy showed linear and irregular vessels with perivascular white halos. Based on the clinical and dermoscopic features a diagnosis of eccrine poroma was made and confirmed by histological findings.

Hidradenoma with an atypical localization mimicking lipoma

Sir, Nodular hidradenoma is a rare, benign adnexal tumor usually located in the head, neck, and extremities [1]. The difficulty of the diagnosis is due to the non-specificity of the clinical and histological presentation, often requiring a clinicopathological correlation [2]. Herein, we report a case of atypical abdominal hidradenoma mimicking lipoma. Our patient was a 51-year-old Moroccan female operated for bowel obstruction and fibroma presenting with a slowly growing, painless, subcutaneous abdominal nodule persistent for the last two years. The nodule had gradually increased in size in a context of apyrexia and general state preservation. A clinical examination was remarkable for a subcutaneous nodule (2.5 cm long axis) at the left iliac fossa, painless, indurated, and not fixed to the deep tissues, with slightly purplish skin (Fig. 1). The rest of the examination was featureless. In addition, dermoscopy was non-specific.