Malignant Eccrine Spiradenoma Research Articles

Demographic and socioeconomic risk factors in treatment of malignant eccrine spiradenoma.

e21595 Background: Malignant Eccrine Spiradenoma (MES) is a rare malignancy that affects the eccrine sweat glands. Although a rare malignancy, MES commonly metastasizes to various regions of the body. Tumor resection with tumor-free margins has a recurrence-free survival of only 23%. There is a lack of analysis on the demographic and socioeconomic factors of MES patients in the literature. Therefore, the National Cancer Database (NCDB) database was utilized in order to address the insufficient epidemiological and demographic information in MES patients. Methods: The National Cancer Database (NCDB) retrospective cohort analysis from 2004 - 2020 included patients with histologically-confirmed diagnosis of MES (N = 188). Regression analysis was used to interpret demographic factors (race, Hispanic origin, facility type, urbanization, income, and Charleson-Deyo score). Results: A total of 188 confirmed cases of MES were identified in the database between 2004 – 2020 with a gradual increase of diagnosed patients per year (R2 = 0.2). The average age of diagnosis was 64.7 years (SD = 15.1, range = 19-90 years). The most common primary site of MES was the skin of the trunk (30.9%), however, primary sites on the skin of upper limb and shoulder (19.1%) and lower limbs and hip (16.5%) were also prevalent. A majority of patients (78.7%) had a Charlson-Deyo comorbidity score of 0. Out of the 188 cases, 83.5% were classified as White. Patients skewed more towards the top quartile in income (30.3%) followed by the second highest quartile (24.5%). The primary source of insurance for patients was Medicare (45.7%) followed by private insurance (41.5%). Most of the patients resided near a metropolitan area (93.6%). The majority of patients were treated in a non-academic facility (59%), rather than an academic facility (41.0%). On average, patients lived 42.3 miles (SD = 184.4, range = 0.6 – 2,319.5) away from their treatment facility. Conclusions: This is the first NCDB analysis on MES, addressing a discrepancy in knowledge on demographic factors contributing to MES. Most MES patients are non-Hispanic and White with their primary site diagnosis being found at the trunk, as seen in case reports. MES patients are more likely to live near a metropolitan area, be treated in a non-academic facility, and have Medicare insurance. More research is imperative to establish an effective treatment protocol for patients with metastatic involvement and to better comprehend the influence of socioeconomic factors on diagnosis and overall survival of patients.

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

BackgroundEccrine 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 presentationAn 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.ConclusionsScalp 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.

Low grade malignant eccrine spiradenoma of the vulva: case report, review of the literature and discussion about the role of p53 and HPV

BackgroundMalignant eccrine spiradenoma is one of the rarest sweat-gland tumors. Here, we describe a rare case of low grade malignant eccrine spiradenoma located at the vulva.Case presentationThe vulvar lesion was described as a mass measured 3.5 cm and located in the dermis and subcutis with no attachment to the epidermis. The neoplasm was arranged in ragged sheets or solid nodules sometimes with focal necrosis. The tumor cells had hyperchromatism, pleomorphism, and prominent nucleoli with high mitotic index and KI-67 estimated at 70–80%.ConclusionsIt’s only the fifth case of malignant eccrine spiradenoma localized at the vulva. This is the first time that an HPV genotyping was made in this type of lesion with no HPV found while the p16 expression was diffuse. Moreover, it’s the first time that a p53 mutation is detected by sequencing in this location.

A rare case report of an aggressive malignant spiradenoma chest wall masquerading as carcinoma of unknown origin - Course of events and review of literature

Malignant eccrine spiradenomas are exceedingly rare and aggressive tumours normally arising in long-standing benign eccrine spiradenomas. We present a case of malignant eccrine spiradenoma (MES) that was initially misdiagnosed as carcinoma of unknown origin in spite of extensive workup to look for primary and presented with a large exophytic tumour on the right side of his chest wall, which had fungated. Radiological imaging of the chest region revealed extensive involvement of the local tissues. Patient underwent wide local excision with a 1 cm margin along with right-sided axillary lymph node dissection. Histopathological findings were consistent with MES with tumour deposits in multiple lymph nodes in the axilla. Our case report shows MES is a rare and aggressive cancer, which is difficult to diagnose, highly aggressive with propensity to involve the lymph nodes. May present with local recurrence and also develop distant metastases significantly compromise the quality of life of the patient. It requires the surgeons to have a high index of suspicion for its initial diagnosis, and a multidisciplinary management with surgery, radiotherapy, and chemotherapy is required for complete treatment and needs close follow up.

Transformation maligne d’un spiradénome eccrine

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. 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. Malignant eccrine spiradenoma is not frequent and is rarely described in the international literature that may lead to diagnostic difficulties.

Pigmented Low Grade Malignant Eccrine Spiradenoma of the Eyelid. Unusual Presentation of a Rare Tumor. A Case Report and Review of Literature

Pigmented Low Grade Malignant Eccrine Spiradenoma of the Eyelid. Unusual Presentation of a Rare Tumor. A Case Report and Review of Literature

Malignant Eccrine Spiradenoma with Squamoid and Chondroid Differentiation

Malignant eccrine spiradenoma (MES) is an extremely rare sweat gland tumor that arises in a pre-existing benign eccrine spiradenoma. We describe a case of malignant eccrine spiradenoma of a 41-year-old male with a history of swelling over nape of neck for 6 yrs and recent onset of increase in size since 6 months. Microscopically, the tumour showed benign component, which was composed of two nodules with interwining bands of 2 types of epithelium, inner differentiated cuboidal cells and peripheral undifferentiated cells. Malignant component was composed of large clear cells with areas of squamoid and chondroid differentiation, lymph nodes showed metastasis. With all these features, a diagnosis of malignant eccrine spiradenoma with Squamoid and Chondroid differentiation was made.

Zosteriform configuration of multiple eccrine spiradenomas of the scalp

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. 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. 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.

Malignant eccrine spiradenoma の 1 例

Malignant eccrine spiradenoma の 1 例

De novo malignant eccrine spiradenoma and metachronous lung cancer

ENWEndNote BIBJabRef, Mendeley RISPapers, Reference Manager, RefWorks, Zotero AMA Unal D, Tasdemir A, Oguz A, Koc A, Benderli Y. Case reportDe novo malignant eccrine spiradenoma and metachronous lung cancer. Contemporary Oncology/Współczesna Onkologia. 2014. doi:10.5114/wo.2014.40598. APA Unal, D., Tasdemir, A., Oguz, A., Koc, A., & Benderli, Y. (2014). Case reportDe novo malignant eccrine spiradenoma and metachronous lung cancer. Contemporary Oncology/Współczesna Onkologia. https://doi.org/10.5114/wo.2014.40598 Chicago Unal, Dilek, Arzu Tasdemir, Arzu Oguz, Ali Koc, and Yasemin Cihan Benderli. 2014. "Case reportDe novo malignant eccrine spiradenoma and metachronous lung cancer". Contemporary Oncology/Współczesna Onkologia. doi:10.5114/wo.2014.40598. Harvard Unal, D., Tasdemir, A., Oguz, A., Koc, A., and Benderli, Y. (2014). Case reportDe novo malignant eccrine spiradenoma and metachronous lung cancer. Contemporary Oncology/Współczesna Onkologia. https://doi.org/10.5114/wo.2014.40598 MLA Unal, Dilek et al. "Case reportDe novo malignant eccrine spiradenoma and metachronous lung cancer." Contemporary Oncology/Współczesna Onkologia, 2014. doi:10.5114/wo.2014.40598. Vancouver Unal D, Tasdemir A, Oguz A, Koc A, Benderli Y. Case reportDe novo malignant eccrine spiradenoma and metachronous lung cancer. Contemporary Oncology/Współczesna Onkologia. 2014. doi:10.5114/wo.2014.40598.

English

Rare group of tumors with potential for local destruction and metastasis, Taxonomically segregated into 2 main groups, as follows: those that are histologically similar to certain benign appendage tumors (e.g., sclerosing sweat duct carcinoma, porocarcinoma, malignant chondroid syringoma, malignant nodular hidradenoma, and malignant eccrine spiradenoma) and those that show a diverse array of histologic features, not recapitulating to any degree aspects of a benign counterpart derived de novo from any portion of the normal eccrine apparatus or result from the transformation of an existing benign eccrine tumor. An etiologic role for UVR has been suggested; Abbate et al reported that 5 out of 10 patients with MAC gave a prior history of radiation therapy. Chiller et al reported a potential etiologic role for UVR as they describe MAC predominantly affecting the left side of the face, corresponding to higher UVR exposures while driving. Immunosuppression has been known to increase the risk of nonmelanoma skin cancers, particularly squamous cell carcinomas, Harwood et al suggested that patients who are immunosuppressed have a propensity to also develop cutaneous appendageal tumors over their immunocompetent counterparts, with increased rates of both benign eccrine tumors and malignant eccrine tumors. REPORT OF A CASE: It is exceedingly rare, accounting for roughly 1 of 13,000 specimens submitted to a dermatopathology laboratory. The more common subtypes include microcystic adnexal carcinoma, eccrine porocarcinoma, and hidradenocarcinoma. The less common subtypes include eccrine mucinous carcinoma, malignant eccrine spiradenoma, malignant mixed tumor, malignant cylindroma, and papillary eccrine adenoma. Only several hundred cases of eccrine carcinoma have been reported in the literature worldwide. Many of these tumors metastasize (up to 60%), with a fatal outcome. MAC was previously only described in white patients; however, Peterson et al and Gardner et al reported the first and second cases of MAC affecting African Americans, respectively. Sex incidence would appear to be equal for eccrine carcinoma, although this has not been definitively stated. Exceptions to this are the malignant chondroid syringoma and primary cutaneous adenoid cystic eccrine carcinoma, both of which occur more commonly in females than in males. Eccrine carcinomas most commonly are diagnosed in patients in their fifth through eighth decades of life. Here we describe a unique and a rare case of malignant eccrine spiradenoma which mimicked like a basal cell carcinoma, squamous cell carcinoma or a malignant melanoma.

Malignant eccrine spiradenoma of the nasolabial fold. A case report

Malignant eccrine spiradenoma (MES) is a rare malignancy of the eccrine sweat glands. It usually presents as a small, firm, reddish painful and small solitary nodule. Head and neck are rare locations. Etiology is unknown although previous trauma is believed to be an implicated factor. MES arises over a prior benign spiradenoma. Clinical behavior is aggressive with a high rate of recurrences and distant metastases. Prognosis is poor. Diagnosis is based on histological findings and treatment must be aggressive from the beginning to achieve the best results.Since Kersting and Helwig first described the case in 1956, and Beekley et al., reported its malignant transformation in 1971, only a few cases can be found in the literature.Based on these particular features we report a case of a 75-year-old man diagnosed on a MES that arises in a very unusual location, with a peculiar histopathology and behavior.

Low grade malignant eccrinespiradenoma with florid squamous differentiation: A Potential Diagnostic Pitfall

Malignant eccrinespiradenomas (MES) are rare, fatal neoplasmsand typically manifest as rapid enlargement of long-standing benign eccrinespiradenomas. Low grade malignancyarising in benign eccrinespiradenomas are difficult to diagnose as the morphological changes are subtle and needs careful examination by the pathologist. MES frequently shows focal squamous differentiation, which may be florid in rare instances. We herein report a case of low grade malignancy in an eccrinespiradenoma with florid squamous differentiation. The present tumor was removed twomonths after rapid increase in size of a long standing nodule over the left forearm was noted by the patient. Histopathological examination showed the earliest stage of malignant transformation within eccrinespiradenoma. The early changes of malignant transformation was characterized by nuclear pleomorphism, loss of two cell types, increased mitotic activity with atypical mitoses, partial loss of PAS positive basement membrane material and florid squamous differentiation. Immunostaining for p53 revealed absent staining in benign areas and increased expression in malignant areas. Ki 67 score was less than 1% in benign areas and was increased (4-5%) in the malignant area. Since patient had tumor free margin, no palpable lymph node in the axilla or evidence ofdistant metastasis, he was not put on any adjuvant therapy. Such cases must be followed up carefully. In conclusion, we need more information about low grade malignant eccrinespiradenoma, its biological behavior, and treatment modalities.

Rare Tumors Through the Looking Glass

To identify prognostic factors related to malignant cutaneous adnexal tumors. Population-based study using the Surveillance, Epidemiology, and End Results database from January 1, 1988, through December 31, 2006. Seventeen population-based cancer registries. A total of 4032 patients with malignant cutaneous adnexal tumors. Overall survival (OS) and disease-specific survival (DSS). Ten-year OS and DSS rates were 54% and 97%, respectively. Unfavorable factors for OS were increasing age (hazard ratio [HR] 1.08; 95% confidence interval [CI], 1.07-1.09; P < .001), distant metastases (3.26; 2.34-4.53; P < .001), no surgical procedure (1.27; 1.01-1.59; P = .04), grade IV tumors (1.97; 1.18-3.28; P = .009), nodal metastases (2.19; 1.40-3.43; P = .001), and T3 tumors (1.37; 1.00-1.87; P = .04). Favorable factors for OS were a wide surgical excision (HR, 0.78; 95% CI, 0.68-0.89; P < .001), female sex (0.73; 0.65-0.82; P < .001), malignant eccrine spiradenoma (0.72; 0.53-0.99; P = .04), and histologic findings of sweat duct carcinoma (0.63; 0.44-0.90; P = .01). Unfavorable factors for DSS included N1 status (HR, 6.77; 95% CI, 2.11-21.68; P < .001), distant metastases (12.24; 6.03-24.85; P < .001), histologic findings of malignant eccrine spiradenoma (5.62; 1.25-25.34; P = .02), and no surgical procedure (2.81; 1.09-7.23; P = .03). Favorable factors for DSS included female sex (HR, 0.52; 95% CI, 0.30-0.91; P = .02). Five-year survival among patients with malignant cutaneous adnexal tumors is good in the absence of distant metastases. Wide resection may be preferable to less aggressive excision. The prognostic importance of lymph node metastases warrants consideration of lymph node basin staging.

Rare Tumors Through The Looking Glass: An Examination of Malignant Cutaneous Adnexal Tumors

Results: Ten-year OS and DSS rates were 54% and 97%, respectively. Unfavorable factors for OS were increasing age (hazard ratio [HR] 1.08; 95% confidence interval [CI], 1.07-1.09; P .001), distant metastases (3.26; 2.344.53; P .001), no surgical procedure (1.27; 1.01-1.59; P=.04), grade IV tumors (1.97; 1.18-3.28; P=.009), nodal metastases (2.19; 1.40-3.43; P=.001), and T3 tumors (1.37; 1.00-1.87; P=.04). Favorable factors for OS were a wide surgical excision (HR, 0.78; 95% CI, 0.68-0.89; P .001), female sex (0.73; 0.65-0.82; P .001), malignant eccrine spiradenoma (0.72; 0.53-0.99; P=.04), and histologic findings of sweat duct carcinoma (0.63; 0.440.90; P=.01). Unfavorable factors for DSS included N1 status (HR, 6.77; 95% CI, 2.11-21.68; P .001), distant metastases (12.24; 6.03-24.85; P .001), histologic findings of malignant eccrine spiradenoma (5.62; 1.2525.34; P=.02), and no surgical procedure (2.81; 1.097.23; P=.03). Favorable factors for DSS included female sex (HR, 0.52; 95% CI, 0.30-0.91; P=.02).

Malignant eccrine spiradenoma: a new case report

Malignant eccrine spiradenoma is an extremely rare skin tumor of sweat gland origin. In most cases, it arises in pre-existing benign eccrine spiradenoma. We report an additional case of malignant eccrine spiradenoma. The present case is of a 75-year-old man with malignant eccrine spiradenoma developed in the right shoulder. He had a 6-year history of a recurrent mass that was removed 2 years ago. The patient then underwent a complete excision. The gross pathologic specimen showed a large cutaneous and subcutaneous multinodular tumor, measuring 6 cm in maximal dimension. Microscopically, there were two distinct morphological components: a benign eccrine spiradenoma and a malignant eccrine spiradenoma of low grade with extensive necrosis. Different histologic patterns were observed such as cylindromatous features. Morphological differentiation in malignant eccrine spiradenoma is variable, sometimes with almost complete loss of eccrine differentiation. Extensive sampling to look for a probable previously benign component is necessary. In the other hand, malignant changes can be easily missed without adequate sampling.

Not all stubbed toes are innocuous—A case report of rare malignant eccrine spiradenoma (spiradenocarcinoma) of the toe

Malignant eccrine spiradenoma (MES) is a rare adnexal tumour derived from cutaneous sweat glands. We describe a case of MES arising on the tip of the right middle toe. The lesion had been managed as a non-specific chronic ulcer for 4 years. The lesion was treated by elective amputation. We wish to emphasise through this case report the importance of keeping this rare but potentially lethal tumour in mind when managing chronic non-healing ulcers in extremities.

Malignant eccrine spiradenoma: case report and review of the literature, including 15 Japanese cases

Malignant eccrine spiradenoma (MES) is an extremely rare cutaneous malignant tumour. An 86-year-old man presented at our hospital with an enlarging tumour on the dorsum of the left hand. An excisional biopsy was taken and histological examination showed a solid island of cells of two distinct types: cells with abundant cytoplasm and oval vesicular nuclei, and small round cells with less cytoplasm and hyperchromatic nuclei with a high frequency of mitosis. We diagnosed this tumour as MES. Although we did not perform further treatment because of the patient's age, there was no sign of recurrence or metastasis in the 2 years of follow-up after excisional biopsy. We reviewed cases of malignant eccrine spiradenoma in the English and Japanese literature and found that 'sarcomatous' or 'squamous' change in histopathology was significantly correlated with a poorer prognosis. It is therefore important for treatment planning to evaluate the entire specimen histologically.

Malignant eccrine spiradenoma on the lateral margin of nose as an infrequent localization

Malignant eccrine spiradenomas are exceedingly rare tumors. They can arise from a preexisting eccrine spiradenoma or occur as a primary malignant tumor. Clinical features of these tumors may include a history of enlargement in a previously stable lesion. Tumor can be of low or high grade. Low-grade malignant eccrine spiradenoma has a better prognosis. We present a malignant eccrine spiradenoma arising from a preexisting eccrine spiradenoma, which has an infrequent localization between lateral edge of nose and medial canthus.

Direct Intracranial Extension of Malignant Eccrine Spiradenoma of the Face

Malignant eccrine spiradenomas are exceedingly rare tumours. They are aggressive tumours normally arising in long-standing benign eccrine spiradenomas. We present a case of malignant eccrine spiradenoma of the right side of the face with direct intracranial extension without distant metastasis. A 48 years old woman presented with a large exophytic tumour on the right side of her face. Radiological imaging of the head and neck region revealed extensive invasion of the facial tissues around right orbit, maxilla and extension into the middle cranial fossa involving the right temporal lobe. She underwent craniotomy and debulking of the right temporal lobe and biopsy of the facial tumour. Histopathological findings were consistent with malignant eccrine spiradenoma. This is a rare case of facial malignant eccrine spiradenoma with direct intracranial extension with no distant metastasis. A literature search revealed that it is first case to be reported of this kind.KeywordsEccrine carcinoma; Spiradenoma; Adnexal carcinoma; Malignant eccrine spiradenoma