Solitary Vulvar Syringoma With Deep Extension; Potential for Misdiagnosis as the Microcystic Adnexal Carcinoma (MAC).

ABSTRACT: A 43-year-old woman presented with a palpable, pruritic, minimally painful right vulvar lesion. Physical examination revealed approximately 2.0-cm tender nodule at 70' clock in the right labia majora. Histological sections of the excision specimen showed an unremarkable epidermis with large, well-circumscribed dermal proliferation with extension to the reticular dermis. Within this proliferation are small solid and ductal structures relatively evenly distributed in the sclerotic stroma. The epithelial elements consisted of monomorphous cuboidal cells and assumed round, oval, curvilinear, or have other peculiar geometric shapes, including "comma-like" or "tadpole"-like configurations. The tumor cells were positive for CEA, EMA, and estrogen receptor and negative for progesterone receptor. The clinical presentation and the deep extension of the tumor were similar to the microcystic adnexal carcinoma. Although a syringoma generally presents with multiple lesions and usually involves the superficial dermis, a syringoma with deep extension was favored based on the lack of follicular differentiation, atypia, mitoses, and perineural invasion. Microcystic adnexal carcinoma and syringoma have a morphologic overlap and are misdiagnosed in 30% of the cases. Thus, it is exceptionally important for pathologists to be aware of and be able to distinguish these entities. To the best of our knowledge, this is the first case of a solitary, painful vulvar syringoma with deep extension.

Syringomatous dermatitis: a myth or an existing entity?

Syringoma is rare disease that in classical variant occurs mainly on lower eyelids. In previously published researches, there is increasing evidence that eruptive syringomas must be discussed as an inflammatory dermal reaction pattern. And there was a proposal to change a name from eruptive syringoma to reactive syringomatous proliferation in appropriate cases. We conduct retrospective study on histopathological archived material to study the histopathological findings in cases of eruptive syringomas and correlate it with hypothesis that eruptive syringomas is not true adnexal neoplasms "de novo" but a hyperplastic response of the acrosyringium to an inflammatory process.According to obtained data and literature correlation, we can conclude that there is apparent diversity in eruptive syringomas. Part of cases can be calculated as neoplastic lesions arising "de novo," and another part as reactive syringomatous proliferation due to preceding cutaneous inflammatory process. Thus, term "eruptive syringoma" may be changed in appropriate cases to a "reactive syringomatous proliferation."Clinical variants of eruptive syringoma with evidence of underlying inflammatory process may be more responsive on types of treatments used for inflammatory disorders. Thus, more global clinicopathological correlative researches should be conducted and classification with terminology should be appropriately changed.

Clear Cell Syringoma of the Eyelids, a Distinctive Histopathologic Variant Associated with Diabetes Mellitus.

The authors describe the clinical and histologic findings of the clear cell variant of syringoma. Three adult female patients (age range 39-76 years old) were found to have multiple, flesh-colored lower eyelid papules, clinically consistent with syringomas, but histologically displaying abundant clear cell change. Two patients had known diagnoses of uncontrolled diabetes.

Eruptive syringomas: Summary of ninety cases and a brief literature review.

BACKGROUND: Eruptive syringomas is a rare variant of syringoma, which is a benign adenoma differentiated from the terminal ducts of the eccrine glands. Nowadays, it's widely valued because of obvious skin lesions, large scope of influence, and high misdiagnosis rate. OBJECTIVES: We aim to explore the clinical features of eruptive syringomas and the current research progress. MATERIALS AND METHODS: We firstly summarized the clinical features of 90 cases of eruptive syringomas. Then, the chi-square test was used to analyze the relationship between the onset site of eruptive syringomas and age, as well as gender. Finally, we briefly reviewed the previous literature. RESULTS: During 12 years, 90 cases of eruptive syringomas were diagnosed in our hospital, including 28 males (31.1%) and 62 females (68.9%). The average diagnosed age was 28.8. Patients from 20 to 40 years old is 63 (70%), which is the most. 60 (66.7%) patients had the course for more than 1 year. Among onset sites, the neck, chest, and abdomen were in the top three. The chi-square test showed that there were no significant differences in the onset sites of patients aged ≤ 20 and >20 years old (p-value = 0.181), as well as male and female (p-value = 0.363). CONCLUSION: We found that more female than male was affected, and the most common onset sites were the neck, chest, and abdomen. Neither age nor gender was significantly associated with onset site distribution. Our study provides some data support for the research of eruptive syringomas.

Syringomatous Adenoma of the Nipple with Associated Invasive Carcinoma: A Case Report.

Syringomatous adenoma of the nipple is a rare benign infiltrative neoplasm that was first described in 1983. At the time of this writing, a literature search revealed no cases of syringomatous adenoma of the nipple in association with invasive carcinoma of the breast. We report a case of syringomatous adenoma of the nipple in a 40-year old female who also had bilateral invasive ductal carcinoma and ductal carcinoma in situ of the breasts. Syringomatous adenomas of the nipple have been postulated to originate from eccrine structures of the nipple due to their microscopic similarity to other tumors of eccrine origin, such as syringomatous carcinoma. However, their exact origin is uncertain. Despite their benign behavior, they usually demonstrate an infiltrative and expansile proliferation into adjacent nipple and breast tissue. They have been confused with tubular carcinoma and low-grade adenosquamous carcinoma of the breast, both clinically and histologically. Complete excision is the therapy of choice, and only incompletely excised lesions have shown recurrence. We present this case to raise awareness that syringomatous adenoma of the nipple may present in patients with a simultaneous invasive carcinoma of the breast.

Generalized Eruptive Syringoma in a Nigerian Woman: A Case Report and a Brief Literature Review.

Generalized eruptive syringomas are a very rare variant of syringoma which appear on the chest, neck, abdomen, upper and lower arms, thighs, legs and back. They often affect adolescent or post-adolescent females, although cases have been described in children and older adults. Generalized eruptive syringomas are mostly sporadic but familial cases, and those associated with eczematous skin conditions, solid organ transplants, renal cell carcinoma and some genodermatoses have also been reported. Although eruptive syringomas may resemble cutaneous mastocytosis, disseminated granuloma annulare, eruptive xanthomata, steatocystoma multiplex, eruptive velus hair syndrome, verruca plana and other skin conditions clinically, the histological appearance characterized by upper dermal epithelial cords with a tendency to ductule formation and tadpole extension of outer epithelial layer is diagnostic. We report the case of a 20-year-old Nigerian woman with generalized eruptive syringoma.

[Skin-coloured vulvar papules]. / Huidkleurige vulvaire papels.

We report a 43-year-old woman with asymptomatic vulvar papules. Histopathology showed ducts with luminal differentiation and eosinophil debris with a tadpole shape. Based on these findings, the diagnosis vulvar syringoma was made. Syringoma are benign eccrine sweat gland tumour mostly located on lower eyelids and cheeks. Vulvar syringoma are rare.

Recurrent syringomatous adenoma of the nipple following a misdiagnosis: A case report.

Infiltrating syringomatous adenoma of the nipple (SAN) is a rare benign neoplasm of the breast that is often misdiagnosed. SAN may present with a subareolar lesion and clinical, mammographic, and ultrasonographic findings associated with malignancy. We present the case of a 60-year-old woman with a painful, firm, solid tumor in her left breast and deformation of the left nipple-areolar complex (NAC). Histopathological test results were conflicting. The tumor, including the NAC were locally excised. Postsurgical immuno-histochemical tests revealed squamous histology, whereas myoepithelial cells were present in the resected specimen, a feature consistent with SAN. The pathologist noted microscopically positive surgical margins. Three months after surgery, tumor recurrence occurred. The patient underwent revision surgery with wide excision of the skin and gland around the lesion, followed by immediate breast reconstruction, using a pedicled myocutaneous latissimus dorsi (pLD) flap. Extreme care should be taken when diagnosing SAN to ensure proper treatment and prevent recurrence.

Múltiples pápulas parduzcas asintomáticas de reciente aparición en el tronco / Recent Onset of Multiple Asymptomatic Brownish Papules

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Ln-γ 2 chain of laminin-332 is a useful marker in differentiating between benign and malignant sclerosing adnexal neoplasms.

AIMS: Laminin (Ln)-γ 2, one of the chains of Ln-332, is a marker of invasive tumours and is frequently expressed as a monomer in malignant tumours. Desmoplastic trichoepithelioma (DTE), some types of basal cell carcinoma (BCC) (infiltrating and morphoeic BCC) and microcystic adnexal carcinoma (MAC) belong to a group of tumours known as sclerosing adnexal neoplasms (SAN) that are frequently difficult to differentiate and often require immunohistochemistry for diagnosis. The aim of this study was to assess the usefulness of Ln-γ 2 expression in the differential diagnosis of DTE, infiltrating/morphoeic BCC, MAC and syringoma. METHODS AND RESULTS: In this study, we compared the expression of Ln-γ 2 in infiltrating/morphoeic BCC (n = 28), DTE (n = 26), MAC (n = 10) and syringoma (n = 20). Immunohistochemically, Ln-γ 2 positivity was noted in 96% (27 cases) of infiltrating/morphoeic BCC and 90% (nine cases) of MAC, while all DTE and syringoma cases were negative. Furthermore, Ln-γ 2 expression pattern in infiltrating/morphoeic BCC was different from that in MAC. Ln-γ 2 expression was found in the cytoplasm of tumour cells in infiltrating/morphoeic BCC tumour cells, while in MAC linear expression was noted both along tumour nests and in the cytoplasm. CONCLUSION: Ln-γ 2 is a helpful adjunct in the differential diagnosis of SAN.

Multiple eruptive and vulvar syringomas and the incipient role of dermoscopy in diagnosis / Siringomas eruptivos múltiples y vulvares y el papel incipiente de la dermatoscopia en el diagnóstico

INTRODUCCIÓN: Los siringomas son tumores benignos derivados de la porción intraepidérmica de los conductos sudoríparos ecrinos. Ocasionalmente pueden iniciar de forma súbita como siringomas eruptivos o localizarse en sitios atípicos que retrasan el diagnóstico por años. La dermatoscopía tiene un rol incipiente en diferenciar siringomas de su extenso diagnóstico diferencial. MÉTODOS: Estudio retrospectivo descriptivo de serie de casos de siringomas de localización atípica. Los datos fueron extraídos de fichas clínicas electrónicas. Todos incluyen dermatoscopía y correlación histopatológica. RESULTADOS: Cinco pacientes. Cuatro hombres y una mujer entre 40 y 79 años de edad con siringomas atípicos: cuatro casos eruptivos y un caso de siringomas vulvares. DISCUSIÓN: Proponemos la dermatoscopía basada en nuestros hallazgos como una herramienta útil con estructuras ovales amarillas y una pseudo-red café clara difusa en su superficie. Estas estructuras amarillas se pueden correlacionar con la proliferación ductal ecrina y el denso estroma en la histopatología. CONCLUSIÓN: Enfatizamos que se debe considerar esta entidad en el diagnóstico diferencial de dermatosis papulares y conocer sus manifestaciones clínicas para optimizar la sospecha diagnóstica.

INTRODUCCTION: Syringomas are common benign tumors, probably of origin derived from the intraepidermal portion of the eccrine sweat ducts. Occasionally they may develop suddenly and extensively as eruptive syringomas or be located in atypical sites delaying the diagnosis for years. Dermoscopy has an incipient role in differentiating syringomas from their extensive differential diagnosis. METHODS: Retrospective descriptive case-series study of atypical location syringomas. Data extraction from clinical history from electronic files. They all include dermoscopy and histopathological correlation. RESULTS: Five patients: Four men and one woman between 40 and 79 years old, with atypical syringomas diagnosis: four eruptive and one vulvar syringomas. DISCUSSION: We propose dermoscopy, based on our findings, as a useful tool to this entity, with its oval yellow structures and a diffuse light-brown network-like structure on its surface. These yellow enlargements may be correlated with the ductal eccrine proliferation and the dense stroma seen in the histopathology. CONCLUSION: We emphasize that they should be considered in the differential diagnosis of papular dermatosis, as they tend to be underdiagnosed, and to know their clinical manifestations to optimize the diagnostic suspicion.