Syringoid Eccrine Carcinoma Research Articles

MALIGNANT SWEAT GLAND TUMOURS: A CASE SERIES

Objectives: Malignant sweat gland tumor is a very rare skin cancer with variable presentations. The objectives of this study are to examine the clinicopathological and immunohistochemical features of malignant sweat gland tumors and to discuss the treatment and follow-up for postsurgical recurrence rates. Methods: Six patients diagnosed with sweat gland carcinoma at the National Hospital of Dermatology and Venereology between 2018 and 2023 were carefully reviewed. Results: The cases occurred in four men and two women, ranging in age from 31 to 78 years (mean 59.3). Four of the lesions were from the scalp, one from the nose, and one from the right arm. There were three cases of Eccrine Porocarcinoma, two cases of Hidradenocarcinoma, and one case of Syringoid Eccrine Carcinoma. All of them were treated with surgery, five cases with wide local excision, and one case with Mohs micrographic surgery. Only one case had a recurrence. Conclusion: Malignant sweat gland tumors demonstrate significant variability in clinicopathology and immunohistopathology. Surgery is the mainstay of treatment. Received 28 June 2023Revised 26 September 2023Accepted 29 November 2023

Rare syringoid eccrine carcinoma of the upper lip and nasal base treated with resection and subsequent innovative reconstruction using an Abbé flap, turbinate flaps and three-stage forehead flap: a case report

BackgroundAlthough syringoma is a common benign tumour of the sudoriferous gland, there is also an extremely rare malignant form known as syringoid eccrine carcinoma (SEC). SEC usually exhibits slow growth with deep invasion and a frequent tendency to relapse. The treatment of choice is radical wide resection, which poses a difficult reconstructive problem, especially when the tumour is located in the centre of the face.Case presentationIn this case, a 70-year-old man was diagnosed with an SEC at the same location as a benign syringoma of the upper lip and nasal base that had undergone primary excision 7 years prior. Primary radical resection was performed with immediate Abbé flap reconstruction. Nevertheless, histology revealed positive margins, and 3 additional re-excisions were needed to achieve clear margins. Four months after the initial resection, the patient had undergone an innovative reconstruction technique including not only the Abbé flap but also a turbinate flap harvested with functional endonasal surgery and a three-stage forehead flap.ConclusionTo the best of our knowledge, this is the first case report of a suspect malignant transformation of a benign syringoma after 7 years. In addition, from oncoplastic and reconstructive points of view, the bilateral use of the turbinate flap for reconstructing the intranasal lining of the alar base is unusual, and the use of functional endonasal surgery in nasal reconstruction for reducing the risk of damaging the vascular supply of the flap is innovative.

Syringoid Eccrine Carcinoma in the Abdominal Wall: A Rare Case Report and Literature Review

Patient: Male, 58-year-oldFinal Diagnosis: Syriongoid eccrine carcinomaSymptoms: Burning sensationMedication: —Clinical Procedure: Surgical excisionSpecialty: SurgeryObjective:Rare diseaseBackground:Syringoid eccrine carcinoma (SEC) is an extremely rare malignant adnexal neoplasm derived from eccrine sweat glands, of unknown pathogenesis. We report a case of this rare entity presenting in the abdomen, which is the only one reported in this area and the only case of SEC in a patient with so many comorbidities.Case Report:A 58-year-old black male from Brazil reported a nodular lesion in the abdomen with a progressive increase in size and pain and local burning sensation. The histopathological examination showed a syringoid eccrine carcinoma.Conclusions:We present a rare case of SEC and did an extensive literature review in order to describe the clinical characteristics, histopathological findings, immunohistochemical profile, treatments, and difficulties found in the diagnosis of this tumor. To avoid misdiagnosis, we gave special attention to biopsy quality.

Syringoid eccrine carcinoma of the vulva: differential diagnosis, treatment, and follow-up

Syringoid eccrine carcinoma of the vulva: differential diagnosis, treatment, and follow-up

Syringoid eccrine carcinoma: A case report and review of the literature

Syringoid eccrine carcinoma: A case report and review of the literature

Syringoid eccrine carcinoma: A case report and literature review of therapeutic options

Syringoid eccrine carcinoma: A case report and literature review of therapeutic options

Syringoid eccrine carcinoma of the eyelid presenting as cicatricial entropion.

Syringoid eccrine carcinoma of the eyelid presenting as cicatricial entropion.

Epidermodysplasia verruciformis: An unusual malignant transformation

Epidermodysplasia verruciformis (EV) is a rare, life-long heritable disease caused due to a unique susceptibility to human papilloma virus. The disseminated verrucous lesions and pityriasis versicolor-like lesions persist from early childhood and can transform into a cutaneous malignancy in a fourth of patients. Malignant transformation into syringoid eccrine carcinoma (SEC) has been reported only once so far. SEC is an extremely invasive, rare, locally destructive, slowly growing adnexal tumor. We hereby report the association of EV with SEC in a 29-year-old male.

Photodynamic Therapy as Palliative Therapy for Invasive Syringoid Eccrine Carcinoma of the Auricle: A Case Report

Recurrent eccrine syringomatous carcinoma is an extremely rare adnexal tumor predominantly found in the head and neck region. We present a case of a 75-year-old woman with a 7-year history of recurrent syringoid eccrine carcinoma in the scalp and auricle. She had undergone five previous oncologic resections and declined further oncologic ablation of the auricle, including temporal bone and external ear canal resection. We performed five sessions of palliative photodynamic therapy using topical aminolevulinic acid HCL 20% (ALA) and between 25 and 33 pulses of Intense Pulsed Light (Quantum) with an intensity of 30 Joules. The ALA incubation time was 3 hours, while the time between each session was 4 weeks. This treatment helped to control ulceration, pain, and swelling. We saw the patient 1 month after the last treatment, by that time she moved and we lost contact.

Metastatic syringoid eccrine carcinoma of the nipple

Syringoid eccrine carcinoma is a very rare skin tumor. Herein we describe a 72-year-old male patient presenting with a syringoid eccrine carcinoma of the nipple with associated axillary lymph node metastases. Surgery associated with adjuvant radiotherapy was performed. To the best of our knowledge, this is the first case of syringoid eccrine carcinoma of the nipple ever reported.

An Iranian male with syringoid eccrine carcinoma misdiagnosed as basal cell carcinoma: a case report

Syringoid carcinoma (syringoid eccrine carcinoma, or eccrine epithelioma) is a rare cutaneous tumor with some controversy regarding its correct definition. This tumor shows a slow growth and has often been for many years, some decades before diagnosis. It may also be difficult to differentiate from its benign counterpart (syringoma) or other adnexal carcinoma and cutaneous metastasis. There have been limited case reports of syringoid carcinoma in foreign literatures but none from Iran. Here we report a case of syringoid carcinoma in a 52 year-old Iranian man. Syringoid eccrine carcinoma is a very rare and uncommon diagnosed tumor thought to be derived from eccrine sweat apparatus. It locally invasive, destructive and often shows recurrence. It may also be difficult to differentiate from metastatic adenocarcinoma.

Syringoid eccrine carcinoma: Case report of a rare tumor occasionally detected in the vulva

► Syringoid eccrine carcinoma (SEC) is a rare tumor. ► There are no published reports describing diagnosis of this tumor on the vulva. ► We report a case of a 58-year-old female with a SEC of left labia majora.

Syringoid eccrine carcinoma: a clinicopathological and immunohistochemical study of four cases

BackgroundSyringoid eccrine carcinoma (SEC) is a rare malignant adnexal tumour with variable presentations.AimTo examine the clinicopathological and immunohistochemical features of SEC.MethodsFour cases were reviewed by three dermatopathologists and the immunohistochemical...

Syringoid eccrine carcinoma of external auditory canal. A case report

First described in 1969, syringoid eccrine carcinoma (SEC) is a rare cutaneous tumor with some controversy regarding its correct definition. It consists of solid nests and small cords in a dense fibrocollagenous stroma. As it is rare, its clinical appearance is not well characterized and its biological behaviour is not defined. It usually affects skin of the scalp, extremities and more rarely, other sites. It behaves as locally aggressive tumor but metastases are rare. Although there have been some previous reports describing clinical presentation and management of SEC in the skin, there has been no previous reports describing clinical findings and management of this tumor in the external auditory canal. We report a case of a 57-year-old female with small solitary mass in left external auditory canal associated with discharge, severe itching and bleeding on manipulation. Complete local excision is the recommended method for diagnosis and treatment of this tumor in the external auditory canal. This extremely rare case serves as a springboard for the diagnosis as well as the management of SEC in external auditory canal.

A case of syringoid eccrine carcinoma with circumscribed abundant stroma

A case of syringoid eccrine carcinoma with circumscribed abundant stroma

Adnexal carcinomas of the head and neck

ABSTRACTAdnexal carcinomas of the skin are rare and they derive from structures such as sweat glands, sebaceous glands, and hair follicles. Adnexal tumors represent 1-2% of skin cancers. Between 1998 and 2004, eight patients with malignant adnexal tumors of the head and neck were treated in the Plastic Surgery Service in Argerich Hospital in Buenos Aires, Argentina. Four (50%) of them had malignant cylindromas, two (25%) had sebaceous carcinoma, and the other two (25%) syringoid eccrine carcinoma. Tumor resection and local flaps were made in all cases. In one case, a radical neck dissection with superficial parotidectomy was performed to treat the metastatic cervical nodes. Local recurrence observed in two cases (25%) was associated with distant metastasis and death of the patients. In other six cases, the survival rate was 75% after five years.

Adnexal carcinomas of the head and neck

Adnexal carcinomas of the skin are rare and they derive from structures such as sweat glands, sebaceous glands, and hair follicles. Adnexal tumors represent 1–2% of skin cancers. Between 1998 and 2004, eight patients with malignant adnexal tumors of the head and neck were treated in the Plastic Surgery Service in Argerich Hospital in Buenos Aires, Argentina. Four (50%) of them had malignant cylindromas, two (25%) had sebaceous carcinoma, and the other two (25%) syringoid eccrine carcinoma. Tumor resection and local flaps were made in all cases. In one case, a radical neck dissection with superficial parotidectomy was performed to treat the metastatic cervical nodes. Local recurrence observed in two cases (25%) was associated with distant metastasis and death of the patients. In other six cases, the survival rate was 75% after five years.

Syringoid eccrine carcinoma with apparently aggressive transformation: case report and review of the literature

A 65-year-old man presented with a 20-year history of a lesion on his lower abdomen. The lesion had started as an indolent papule that slowly developed to form an extensive plaque. Physical examination revealed a flat-topped, elevated, well-demarcated, erythematous plaque measuring 7 cm × 5 cm in diameter (Fig. 1). The lesion had an elastic, hard induration beneath the plaque and was fixed to the underlying tissue. Inguinal lymphadenopathy was present. Hematologic and biochemical investigations were all within the normal ranges. The patient underwent incision biopsy. Figure 1. Clinical appearance of the lesion. An indurated plaque can be seen on the lower abdomen Download figure to PowerPoint A biopsy specimen, measuring 2.7 cm, revealed that the tumor was located in the dermis, and consisted of solid nests and small cords in a dense fibrocollagenous stroma. The tumor located in the upper dermis consisted mainly of solid nests with calcified central necrosis (Figs 2 and 3). The tumor located in the deep dermis consisted of small cords forming luminal, tubular, and tadpole-shaped structures, resembling those of syringoma (Figs 2 and 3). Solid tumor nests consisted of tumor cells which were basaloid and polymorphic, with hyperchromatic nuclei. A few mitotic figures were observed. A palisading arrangement of tumor cells was not observed at the periphery of the solid tumor nests. Keratinous cysts and follicular differentiation were not observed. No apocrine differentiation, such as decapitation secretion, was observed. There were no connections between the tumor nests and the epidermis. The extent of tumor invasion could not be determined from the biopsy specimen because the tumor had spread beyond the edges of the excision. Immunohistochemical analysis showed diffuse nuclear and cytoplasmic positivity for S-100 protein (1 : 1000; Dakopatts, Glostrup, Denmark) and focal positivity for carcinoembryonic antigen (CEA, 1 : 500; Mochida, Tokyo, Japan). Figure 2. The tumor consisted of solid undifferentiated nests and small cords forming tubular structures in a dense fibrocollagenous stroma (original magnification, ×40) Download figure to PowerPoint Figure 3. The tumor located in the upper dermis consisted mainly of solid undifferentiated nests with calcified central necrosis. Small cords forming tubular structures resembling those of syringoma can also be observed (original magnification, ×200) Download figure to PowerPoint The extent of tumor spread was evaluated by computed tomography (CT) and magnetic resonance imaging. The primary tumor had infiltrated the rectus abdominis muscle, and multiple metastases, up to 1 cm in diameter, were observed in the lungs, right bronchus, para-aortic lymph nodes and Th11, right S1, and bilateral ilium. CT scan revealed no continuity between the tumor and the prostate. After consulting a urologist, the possibility of prostate cancer was ruled out. After diagnosis and evaluation of tumor spread, the patient was treated with seven cycles of combination chemotherapy as follows: 5-fluorouracil (350 mg/m2 intravenously) on days 1–5, epirubicin (40 mg/m2 intravenously) on day 1, mitomycin C (3.5 mg/m2 intravenously) on day 1, vincristine sulfate (0.7 mg/m2 intravenously) and carboplatin (300 mg/m2 intravenously) on day 2, every 21–28 days. The treatment was relatively well tolerated without major complications. After seven courses of chemotherapy, evaluation by CT showed no change in the size of the primary lesion, or in the size and number of distant metastases. It is now 6 months since the last course of treatment and the patient appears to have stable disease.

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A case of sweat gland carcinoma with von Recklinghausen's disease were reported. We considered this case syringoid eccrine carcinoma that had reported by Mehregan A.H., in 1983.A 61-year-old man had a complaint of asymptomatic eruption on the vertex of the scalp on July 1991, that had increased in size slowly since resection of the traumatic scar on January 1990. Physical examination revealed a 15×15mm erosive lesion with crust on the vertex of the scalp and a lot of neurofibromas on the whole body. A 15×15mm red nodule was close to the scalp lesion.Examination of the hematoxylin-and-eosinstained specimens showed many variously sized cysts extended in upper dermis and a lot of branching tubular structures embedded in a fibrous stroma of lower dermis. Tumor cells consisted by cystic and tubular structures were moderately atypical basaloid cells and did not form palisading. There was evidence of perineural and periarterial invasion. Evident connection between the tumors and neurofibromas was not found.Immunohistochemical stain with Avidin-Biotinylated-Peroxidase-Complex method showed that luminal cells of cysts stained positive with carcinoembryonic antigen (CEA) and S-100 protein and tubular structural cells staind positive with CEA negative with S-100 protein. CAM5.2, antihuman cytokeratin, was reactive to the both structures. Vimentin showed negative activities.Enzyme histochemical examination revealed tumor structures gave positive reactions for succinic dehydrogenase and negative for β-gluculonidase.Electron microscopic findings were numerous intracellular ducts with prominent microvilli were present and tumor cells connected with desmosomes. Immature tonof filaments were found in some cytoplasmes.In July 1991, the tumor was totally excised and skin-grafted. But the remnants of tumor cells were found at the margin with histological examination. In spite of a 40Gy electron radiation therapy, it recurred on previous skin graft in September 1991. The patient had wide surgical excision. It has not recurred in 11 months.In 1983, Mehregan, studied 35 patients diagnosed in the past 20 years and classified eccrine adenocarcinomas the following 4 distinct variants histologjcally: eccrine porocarcinoma, syringold eccrine carcinoma, mucimous eccrine carcinoma and clear cell eccrine carcimoma.Syringoid eccrine carcinoma (SEC) occurred on the scalp in four instances, on the extremities in four, on the back in two, on the ear and the cheek in each one. The lesions were single in all cases and measured from 1.5 to 4cm. Local lymphnode metastasis was rare.Histologically, SECs were characterized by cystic and tubular structures resembled syringoma were composed by basaloid cells and invasively prolifetared in a fibrous stroma. Tubular structures were lined by a single layer of luminal cells and one or more rows of cells. Mitosis were often found. In some cases, tumor nests showed connections with the overlying epidermis. Perineural or perivascular invasion may be present. Enzyme histochemical showed positive reactions for succinic dehydrogenase and leucine aminopeptidase and negative for alkaline phosphatase.Basal cell carcinoma has no activity to CEA that SECs react. Syringoma does not exhibit the aggressive infiltrative growth seen in the SECs. Cylindromas have more classic basaloid nests arranged in a jigsaw puzzle pattern. Primary cutaneus adenoid cystic carcinomas have its characteristic cribriform patterns.We concluded our case differentiated into dermal duct through secretary portion by our immunohistochemical exmanination.In Japan, 63 cases of non-neurogenic malignant neoplasms were reported among 2, 513 cases of von Recklinghausen's disease. It's rate was 2.5%. These complications may be accidental but a mutation theory is proposed that von Recklinghausen's disease patient may be given a tendency of occuring malignant neoplasms by gene mutation.

Syringoid eccrine carcinoma

Eccrine carcinomas are locally aggressive and have an infiltrative growth pattern. They have a variable clinical appearance and a diversity of histologic findings that often make the diagnosis difficult. The histologic findings, together with immunoperoxidase and enzyme his-tochemical studies, usually allow differentiation between eccrine carcinomas, other cutaneous neoplasms, and visceral adenocarcinomas with skin metastases. Provided there is no evidence of distant metastases, surgical excision is the treatment of choice. We report a case of syringoid eccrine carcinoma that was treated with Mohs micrographie surgery.