Hidradenocarcinoma: A Case Series From the Scripps Clinic With a Systematic Review of the Literature.

BACKGROUND: Hidradenocarcinoma (HAC) is a rare adnexal carcinoma. To the best of the authors' knowledge, there are no published systematic reviews on HAC. OBJECTIVE: To incorporate a case series from the authors' institution and systematically integrate reported information to provide a reference tool for optimization of diagnosis and management. METHODS: A comprehensive MEDLINE search was conducted from database inception to 2021 using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. This yielded 225 studies with 165 cases of HAC. References of included articles were also searched. In addition, 9 patients with HAC were identified from the authors' institution over the past 10 years. RESULTS: The mean age of HAC presentation is 60 years with a slight male predilection (60%). The head and neck is the most commonly affected region. Over 36% of cases either presented with metastatic disease or went on to metastasize. The most common treatment type was wide local excision, followed by Mohs micrographic surgery. CONCLUSION: Early detection with accurate histologic interpretation is prudent in all cases of HAC. Wide local excision is the current first-line treatment. However, Mohs micrographic surgery offers complete marginal analysis with evidence of reduced risk of metastasis and better outcomes compared with wide local excision. Currently, there are no National Comprehensive Cancer Network guidelines for the treatment of HAC, and consensus guidelines are limited to tumor and nodal metastasis staging provided by the American Joint Committee on Cancer, eighth edition. Thus, this case series and systematic review integrates important aspects of diagnosis, workup, and management of HAC.

Direct intracranial invasion of eccrine spiradenocarcinoma of the scalp: a case report and literature review.

BACKGROUND: Eccrine spiradenocarcinoma (SC), also known as malignant eccrine spiradenoma, is a rare malignant cutaneous adnexal neoplasm arising from long-standing benign eccrine spiradenoma. Malignant skin tumors rarely show direct intracranial invasion. However, once the intracranial structure is infiltrated, curative excision with sufficient margins can become extremely difficult, particularly when the venous sinuses are involved. No effective adjuvant therapies have yet been established. Here, we report an extremely rare case of scalp eccrine SC with direct intracranial invasion, which does not appear to have been reported previously. CASE PRESENTATION: An 81-year-old woman presented with a large swelling on the parietal scalp 12 years after resection of spiradenoma from the same site. The tumor showed intracranial invasion with involvement of the superior sagittal sinus and repeated recurrences after four surgeries with preservation of the sinus. The histopathological diagnosis was eccrine SC. Adjuvant high-precision external beam radiotherapy (EBRT) proved effective after the third surgery, achieving remission of the residual tumor. The patient died 7 years after the first surgery for SC. CONCLUSIONS: Scalp SC with direct intracranial invasion is extremely rare. Radical resection with tumor-free margins is the mainstay of treatment, but the involvement of venous sinuses makes this unfeasible. High-precision EBRT in combination with maximal resection preserving the venous sinuses could be a treatment option for local tumor control.

Value of Immunohistochemistry to Differentiate Digital Papillary Adenocarcinoma From Acral Hidradenoma With Papillary Structures.

ABSTRACT: Digital papillary adenocarcinoma is a malignant adnexal tumor with a predilection for acral sites. Hidradenoma is a benign solid and cystic sweat gland neoplasm with focal ductal and glandular differentiation and good outcomes. Hidradenomas can occur at acral sites and show papillary structures; for this reason, they are included in the differential diagnosis of digital papillary adenocarcinoma, and immunohistochemistry is a valuable tool in this scenario. We described a case of a 43-year-old man with an epithelial tumor showing papillary structures in the intermediate phalanx of the fourth finger. There was diffuse positivity for p63 and negativity for S100 protein, suggesting that this tumor was an acral hidradenoma with papillary structures.

Clear Cell Hidradenoma-Basal Cell Carcinoma Collision.

ABSTRACT: A 91-year-old man presented with a tumor on the left temporal area, clinically suspicious of basal cell carcinoma. The histopathologic study showed a central solid-cystic tumor composed by 3 different types of cells (clear or finely granular cells, polygonal cells, and squamoid cells). It had a sclerotic stroma. At the periphery, another tumor composed by smaller interconnected nests was evident. Some nests were separated from the stroma by clefts. The stroma of this second tumor was highly cellular. There was a sharp delimitation between both tumors, with no transitional area. Immunochemistry demonstrated they are different tumor. A diagnosis of clear cell hidradenoma-basal cell carcinoma collision was performed. To the best of our knowledge, this is the first description of this challenging association.

A rare case of Hidradenoma of the lower lip.

Hidradenoma are rare benign tumors with eccrine or apocrine differentiation that most often presents as a small, firm, solitary dermal nodule. At times, hidradenoma shows striking cytologic and histologic similarity to other neoplasms, including its malignant counterpart, hidradenocarcinoma. We present the first reported case of benign hidradenoma of the lower lip. The case illustrates important diagnostic features of hidradenoma and discusses the current understanding of its malignant potential and the appropriate management of such lesions.

Rapidly Enlarging Hidradenoma of the Eyelid.

Hidradenoma is a rare benign neoplasm, with few cases reported to involve the eyelid. When affecting the eyelid, hidradenomas may mimic other benign or malignant lesions. Rarely, a benign hidradenoma may transform into a malignant hidradenoma and metastasize. The authors present a case of a benign hidradenoma arising from the eyelid, presenting with rapid growth, ulceration, and bleeding, suggestive of a malignant lesion.

PIGMENTED NODULAR CYSTIC HIDRADENOMA OF THE ANKLE.

Solid-cystic hidradenoma is a benign cutaneous tumor that is most commonly found in the head, neck, trunk, and upper extremity regions of patients in the middle to older aged groups. Presentation on lower extremities and in particular on the foot is uncommon. Nodular hidradenomas represent a dermatological pitfall, being difficult to differentiate from basal cell carcinoma and melanoma. We report on a 53-year-old male patient with a pigmented nodular hidradenoma on his ankle that was surgically removed. We discuss histopathology and differential diagnosis of this eccrine tumor of skin. This is the second reported case in the English literature.

Malignant acrospiroma: a case report in the era of next generation sequencing.

BACKGROUND: Malignant acrospiroma is a rare tumor of the eccrine sweat glands accounting for around 6% of all malignant eccrine tumors. Typically, it presents as large ulcerated nodules, and diagnosis can be challenging as it has great overlap with its benign counterpart. CASE PRESENTATION: We herein report a case of acral malignant acrospiroma, initially treated with surgical excision and adjuvant radiotherapy. After metastatic disease was confirmed, subject received multiple lines of chemo- as well as targeted therapy. Genomic testing was also done using next generation sequencing. CONCLUSION: To the best of our knowledge, this is the first case of acral malignant acrospiroma with reported next generation sequencing results.

Nodular hidradenoma arising on the site of a BCG scar.

Hidradenomas are tumours that arise from the adnexal structures, both eccrine and apocrine and are histologically benign. The tumours that arise from eccrine differentiation are known as poroid hidradenomas and when they arise from the apocrine glands they are called nodular hidradenomas. In our centre a 13-year-old boy presented with a slow-growing, painless erythematous fungating nodule on the left upper arm over a period of 18 months at the site of the BCG vaccination. The nodule was surgically excised and sent for histopathological examination, leading to a diagnosis of nodular hidradenoma. This case is presented to highlight its rarity, together with its clinical features that were suggestive of malignancy but proved ultimately to be benign.

Hidradenoma of the Chin.

Clear cell hidradenoma is a rare skin tumor in the head and neck. Clear cell hidradenoma of skin is an uncommon soft tissue tumor originating from the sweat glands. The authors report a case of clear cell hidradenoma developing in the chin in a 61-year-old woman, which occurred during the course of 8 months. The clinical and histologic findings of the tumor are documented. The lesion was totally removed by excision and revealed no evidence of recurrence.

[Malignant transformation of an eccrine spiradenoma]. / Transformation maligne d'un spiradénome eccrine.

INTRODUCTION: Malignant eccrine spiradenoma is a rare and aggressive tumor, developed on the epithelium of eccrine sweat glands. Typically, it occurs after malignant transformation of benign eccrine spiradenoma, but sometimes it happens de novo. OBSERVATION: We report a case of malignant eccrine spiradenoma in a 62-year-old woman. The patient presented a rapid increase in size of a long-standing tumoral lesion of her forearm. There was no secondary lesion on the chest, abdomen or pelvis at the CT-scanner. Cutaneous biopsy of the lesion was performed and showed a carcinoma with no contact with epidermis. On this biopsy, we could not affirm if the tumor was a metastatic process or a primary tumor of the skin. Histologic examination of the surgical removal of the tumor showed an undifferentiated carcinoma with adjacent nodules of eccrine spiradenoma. Immunohistochemical assessment of Ki67 expression showed a weak expression (5%) in the benign spiradenoma nodules and a high rate expression (80%) in the malignant neoplasm. The final diagnosis was an undifferentiated carcinoma arising from preexisting benign spiradenoma. DISCUSSION: Malignant eccrine spiradenoma is not frequent and is rarely described in the international literature that may lead to diagnostic difficulties.

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.

Mohs micrographic surgery for the treatment of hidradenocarcinoma: the Mayo Clinic experience from 1993 to 2013.

BACKGROUND: Hidradenocarcinoma (HAC) is a rare malignant adnexal neoplasm with reported metastatic potential and undefined optimal treatment. OBJECTIVE: To review clinical characteristics and outcomes of patients with HAC treated with Mohs micrographic surgery (MMS). MATERIALS AND METHODS: The authors performed a retrospective chart review of patients with HAC treated by MMS at Mayo Clinic from 1993 to 2013, recording patient demographics, tumor characteristics, MMS stages to clearance, follow-up, recurrence, metastasis, and mortality. RESULTS: Ten patients underwent MMS for HAC more than 20 years. The average age was 62.8 years, with 6 females and 4 males. Occipital scalp was the most common location (40%), followed by extremities (30%) and face (20%). In 5 of 7 cases (71%), "cyst" was the working clinical diagnosis. The average preoperative lesion area was 3.18 cm, with an average of 1.5 MMS stages required for clearance. Mean postoperative follow-up was 7 years (range, 5-205 months). No tumors treated with MMS recurred, metastasized, or led to disease-related mortality. CONCLUSION: Mohs micrographic surgery seems to be a useful treatment modality for HAC. This is the largest reported series of HAC treated with MMS with long-term follow-up.

Pseudoxanthoma elasticum-like change adjacent to a benign adnexal neoplasm: a histopathologic reaction pattern.

The progressive calcification and fragmentation of elastic fibers, primarily affecting the skin, are histopathologic features typically associated with pseudoxanthoma elasticum (PXE), pseudo-pseudo PXE, or even acquired PXE. We present a case of a 65-year-old woman, with a known history of breast carcinoma, status after wide excision (T1N0Mx) in 2010, who presented with a 0.6 × 0.5-cm, nontender pink nodule in the right inferior axilla of 1 week duration on the same side as her mastectomy. Histopathologically, the lesion revealed a circumscribed, nonencapsulated bland dermal tumor with poroid and clear cells and foci of ductal differentiation-features diagnostic for hidradenoma. Of note, immediately adjacent to the hidradenoma, the specimen also exhibited short, frayed elastic tissue fibers (positive for EVG and Von Kossa stains)-features reminiscent of PXE. With this case, we expand the spectrum of reaction patterns observed in the skin to include a PXE-like change, a reaction pattern that, to the best of our knowledge, has not been previously reported in association with a benign cutaneous tumor.

A periclitoral mass as a cause of persistent genital arousal disorder.

INTRODUCTION: Persistent genital arousal disorder (PGAD) is an intrusive and unremitting disorder for which several possible etiologies and treatments have been suggested. AIM: To describe a woman who developed PGAD in association with a periclitoral mass, a potential physical cause of the disorder that has not been previously described in the medical literature. METHODS: A postmenopausal woman presented with 6 months of persistent, unrelenting genital arousal and clitoral pain that was unrelated to sexual stimuli. Careful examination revealed a tender, firm, mobile, left-sided mass that appeared to compress the dorsal nerve of the clitoris. RESULTS: Complete excision of the mass resulted in full resolution of her symptoms over several weeks. CONCLUSION: Localized causes of persistent genital arousal, though rare, should be included in the differential diagnosis PGAD as detection and treatment can lead to a complete recovery.

Nodular hidradenoma of the caruncle.

An 84-year-old man presented with a tumor of the right caruncle causing epiphora due to insufficient eyelid closure. The tumor was cystic and mucinous in appearance. MRI showed a solitary tumor of the caruncle with no adhesions to neighboring tissue. The mass was removed by carunculectomy. Histopathologic examination revealed a tumor composed of epithelial cells with intervening strands of hyalinized stroma. Solid areas were predominant but a cribriform area could also be distinguished. Periodic acid-Schiff positive, diastase-labile material (glycogen) was detected in many tumor cells. Immunohistochemical staining of the tumor cells demonstrated positive staining with antibodies against CK-CAM 5.2, BER-EP4, p63, and smooth muscle actin-positive cells in some areas. The tumor was diagnosed as a nodular hidradenoma of the caruncle, which has never been reported before and should be included in the diagnoses of caruncular tumors.

Hidradenocarcinoma of the finger.

Hidradenocarcinoma is a rare adnexal neoplasm representing the malignant counterpart of hidradenoma derived from eccrine sweat glands. Misdiagnosis of this disease is common due to the wide variety of histological patterns and rarity of this malignancy. We report an 87-year-old man presenting with a rare case of biopsy-proven hidradenocarcinoma of the finger. There is no standard care of treatment of hidradenocarcinoma, especially of those tumors in rare locations such as the fingers, given its rarity, variable tumor behavior and histology. Although limited treatment strategies exist, detailed data including TNM, location, histologic type and grade, and patient age should be gathered for optimal treatment strategy. The literature supports a 3-fold approach to these malignancies involving margin-free resection, sentinel lymph node biopsy to evaluate possible metastasis, and long-term follow-up given high risk of recurrence. Our treatment strategy involved a 4-fold, multidisciplinary approach involving reconstruction to optimize tumor-free remission and hand function.

Hidradenoma papilliferum of the orbit.

PURPOSE: Hidradenoma papilliferum (HP) is a cystic lesion of apocrine gland origin that occurs most commonly in the perineal region. Although there are scattered reports of HP involving the eyelid, to our knowledge, we present the first case of HP of the orbit. METHODS: A 63 year-old female presented with progressive left upper eyelid fullness over an 18 month period. RESULTS: The lesion was excised via transconjunctival anterior orbitotomy without incident. CONCLUSIONS: The pathologic features of HP are presented.

Clear cell hidradenocarcinoma of the eyelid: a case report with a review of the literature.

Clear cell hidradenocarcinomas are extremely rare neoplasms, with very few well-documented cases reported in the literature. The most common sites are the head and neck regions. These tumors are histologically malignant but are not always aggressive. They are known for recurrence and may metastasize widely. Treatment is wide local resection. We report on a case of clear cell hidradenocarcinoma occurring over the eyelid together with a review of the literature.