Association of HPV42 with digital papillary adenocarcinoma and the use of in situ hybridization for its distinction from acral hidradenoma and diagnosis at non-acral sites.

Digital papillary adenocarcinoma (DPAC) is a rare tumor of sweat gland origin that preferentially affects the digits and has the potential to metastasize. Its tumor diagnosis can be difficult. Well-differentiated variants of DPAC can be confused with a benign sweat gland tumor, in particular nodular hidradenoma. With the recent detection of HPV42 DNA in DPAC by next-generation sequence analysis, we reasoned that this association could be used for diagnostic purposes. To this end, we performed in situ hybridization for HPV42 on 10 tumors diagnosed as DPAC as well as 30 sweat gland tumors of various histology types, including 8 acral hidradenomas. All DPAC were positive for HPV42. Positive hybridization signals for HPV42 were seen in both primary and metastatic DPACs. All other tumors and normal tissues were negative. This study confirms the association of HPV42 with the tumor cells of DPAC through in situ hybridization. The positive test result in all lesions of DPAC and lack of detection of HPV42 in any of the acral hidradenomas or other sweat gland tumors examined in this series is encouraging for the potential diagnostic utility of the assay. As documented by two scrotal tumors of DPAC, the in situ hybridization test for HPV42 can also help support the rare occurrence of this tumor at a non-acral site.

Tubulopapillary hidradenoma-like tumor of the mandible: Two case reports of an underrecognized entity.

Tubulopapillary hidradenoma-like tumor of the mandible is extremely rare, with only 3 cases published in the English-language literature. The clinicopathological characteristics and histogenesis of this tumor are unclear. Few pathologists and perhaps fewer clinicians are familiar with this entity, and it is likely underrecognized and under-reported. Herein, we present two additional cases, both misdiagnosed as malignancies preoperatively and postoperatively by different unwary pathologists. Awareness and knowledge of this enigmatic entity and its clinical and radiographic features, together with careful morphological assessment should enable the correct diagnosis and prevent unnecessary treatment.

Histological findings and pathologic diagnosis of spiradenocarcinoma: A case series and review of the literature.

BACKGROUND: Atypical spiradenoma and spiradenocarcinoma present a diagnostic challenge. We aim to assess the significance of certain histologic features, which may facilitate diagnosis of these tumors. METHODS: A natural language search for cases of "atypical spiradenoma" and "spiradenocarcinoma" diagnosed between 2009 and 2018 was performed. Original slides were retrieved and a subset of cases (n = 5) were stained for Ki-67, p53, carcinoembryonic antigen (CEA), and S100. All cases (n = 7) were assessed for overall architecture, atypical mitotic figures, abnormal cytology, necrosis, ductal proliferation, dilated vessels, and loss of dual cell population. RESULTS: All our cases showed an abrupt transition from benign to malignant morphology, nuclear atypia, atypical mitotic figures, and a monomorphic loss of the dual cell population (7/7; 100%). The majority also had dilated vessels (6/7; 85.7%), and ductal dilation or proliferation (5/7; 71.4%). Fewer cases showed tumor encapsulation (3/7; 43%), massive necrosis (3/7; 43%), and focal cellular necrosis (1/7; 14%). All cases showed a relatively increased Ki-67 proliferation index at the transitional interface (5/5; 100%). Almost all cases stained positively for p53 (4/5; 80%). Malignant areas of tumor or at the transitional interface showed more intense S100 staining (3/5; 60%). All cases were negative for CEA. CONCLUSION: Histologic features that strongly favor atypical spiradenoma or spiradenocarcinoma include abrupt transition to malignant foci, atypical mitotic figures, and monomorphic loss of the dual cell population. Ki-67, p53, and S100 may help delineate areas of atypical or malignant transformation in spiradenomas.

Eccrine spiradenoma mimicking a painful traumatic neuroma: case report.

Diagnosing and treating patients with persistent neuropathic pain associated with peripheral nerve lesions can be challenging. The authors report the rare case of a painful eccrine spiradenoma treated as a traumatic neuroma for many years because of a history of acute trauma, the presence of a tender palpable mass, and symptoms of allodynia. Surgical excision of the neoplasm completely relieved the pain and hypersensitivity that 2 prior surgeries and other nonsurgical treatments failed to resolve. The diagnosis of eccrine spiradenoma was not established until resection and histopathological analysis of the tissue. This case highlights the need to develop and consider an extensive list of differential diagnoses, including eccrine spiradenoma, for peripheral nerve lesions that fail to respond to treatment.

Multiple eccrine spiradenomas in a zosteriform pattern.

Eccrine spiradenoma (ES) typically presents as a solitary tender lesion. Multiple ES is a rare variant of ES and can present in a segmental, linear, blaschkoid, or zosteriform pattern. The etiology of multiple ES is unknown, but several theories have been suggested including a multipotent stem cell origin. We report the case of a 30-year-old woman with multiple painful ES in a zosteriform pattern on the mid-back and abdomen. Skin biopsy of a representative lesion demonstrated a circumscribed tumor nodule encapsulated by a fibrous capsule with diffuse dense basophilic proliferation located in the dermis. The lesions were then excised on two separate sessions without recurrence.

[Tubulo-papillary apocrine adenoma in association with syringocystadenoma papilliferum]. / Adénome tubulo-papillaire apocrine associé à un syringocystadénome papillifère.

Tubulo-papillary apocrine adenoma (TAA) is a very rare sweat gland tumor. TAA in association with syringocystadenoma papilliferum (SCP) is exceptional. A 2-year-old Tunisian child developed a mixed tumor on the scalp: TAA in association with SCP. Histologically, the tumor consisted of dilated duct-like areas with some apocrine gland-like areas. The superficial part of the tumor was connected to the epidermis and showed the characteristics of SCP. The characteristics and differences in histopathologic and immunohistochemical findings in this mixed tumor are described.

Eccrine Syringofibroadenomatosis of the Soles.

Eccrine syringofibroadenoma is a rare adnexal tumor with acrosyringeal differentiation. It was first described by Mascaro in 1963. Its clinical presentation varies from a solitary nodule, keratotic papule, verrucous plaque, or palmoplantar keratoderma to multiple lesions with linear or diffuse distributions. Age at onset ranges from 16 to 80 years. Lesion distribution is wide and includes the face, back, abdomen, buttocks, extremities, and, rarely, nails. There are five subtypes of eccrine syringofibroadenoma. Histologically, eccrine syringofibroadenoma, independent of the subtype, is characterized by anastomosing cords of acrosyringeal cells forming ductal structures in a fibrovascular stroma and attached to the undersurface of the epidermis in multiple foci. In this article, we describe a 51-year-old woman diagnosed as having eccrine syringofibroadenomatosis.

A unexpected growth arising within nevus sebaceous of Jadassohn.

The predisposition to epithelial neoplasms in nevus sebaceous is well established; most tumors occur in adults and are benign. Hidradenoma is a relatively rare benign tumor of sweat gland origin that can rarely arise within a nevus sebaceous. We present an interesting case of a hidradenoma and sebaceoma arising within a nevus sebaceous and present a literature review of the 2 conditions. Even though hidradenoma is a benign tumor, we would advocate complete excision given the potential for malignant transformation.

Linear syringocystadenoma papilliferum on the retroauricular area associated with nevus sebaceus.

Syringocystadenoma papilliferum is a rare cutaneous adnexal tumor that usually arises in the head and neck region. It may develop de novo or within a nevus sebaceus. Linear syringocystadenoma papilliferum is an uncommon variant of this benign tumor. We report a child with linear retroauricular distribution of syringocystadenoma papilliferum. A background nevus sebaceus was shown histologically. Total excision was curative with no recurrence. An association between the linear variant of syringocystadenoma papilliferum and nevus sebaceus has not been reported previously.

Lymphangiectatic Variant of Eccrine Spiradenoma - A Diagnostic Challenge.

Conventional eccrine spiradenoma is a benign, slow growing and painful tumor of the skin. While the tumor does not usually present a diagnostic dilemma, a rare variant with marked stromal lymphedema can be a challenge to interpret. We present a case of lymphangiectatic variant of eccrine spiradenoma in an 82-year-old white male who presented with a persistent left flank lesion for several months. The patient was initially asymptomatic and subsequently developed a suspected abscess that was excised to reveal a 6.5 cm subcutaneous mass. Microscopic examination reveals strands and cords of dark, epithelial, round to oval cells with inconspicuous nucleoli streaming between prominently dilated and congested vascular spaces. Within the cystic component there are small ductular structures. Additionally, prominent stromal lymphedema is present. To the best of our knowledge, there is only one reported case of this entity in the English literature. This case represents a diagnostic challenge and the purpose of reporting it is to alert surgical pathologists, dermatopathologists and dermatologists of the existence of this unusual variant of eccrine spiradenoma.

Malignant eccrine spiradenoma of the external ear.

Eccrine spiradenoma is a benign tumor of sweat gland origin. Malignant eccrine spiradenoma is a rare neoplasm which almost always arises from a pre-existing spiradenoma lesion. In this article, we present a patient with malignant eccrine spiradenoma of the auricula with a prior diagnosis as benign eccrine spiradenoma. Although malignant transformation is usually associated with aggressive behavior, we did not detect recurrence or metastasis in four years of follow-up after performing wide excision.

Malignant Eccrine Spiradenoma of the Face.

Malignant eccrine spiradenoma, or spiradenocarcinoma, is an exceedingly rare sweat-gland tumor, with only 102 reported cases. Low-grade carcinomas are especially rare with only a few cases reported. Because of the limited number of case reports, the biologic behavior of low-grade malignant eccrine spiradenoma is poorly understood and no evidence-based therapeutic approach is established. Here, the authors report a 29-year-old woman who presented with a history of left-sided facial lesions present since the age of 2 months. Histopathologic examination revealed multiple benign spiradenomas, several of which showed foci of low-grade malignant transformation evidenced by loss of the characteristic 2-cell population seen in the benign tumor component. Included are the clinical presentation, histopathologic description, and surgical decision making in an effort to guide recognition of this rare entity.

[Cutaneous adnexal and salivary gland tumours. Similarities and differences]. / Hautadnex- vs. Speicheldrüsentumoren. Analoges und Divergentes.

Despite major discrepancies in basic microscopic anatomy, remarkable similarities are manifest within the wide spectrum of cutaneous adnexal and salivary gland tumors. In this study salivary gland and adnexal tumors were identified and investigated with respect to similarities in histology, terminology and pathogenesis. Histological similarities of certain types of salivary gland tumors relate to eccrine, apocrine and rarely sebaceous (but not trichofollicular) types of adnexal tumors. The most striking similarity was found with salivary gland pleomorphic adenoma and cutaneous mixed tumor. Multistep carcinogenesis starting with intraductal carcinoma, identified in carcinoma ex pleomorphic adenoma is identical to that found in cutaneous carcinoma ex spiradenoma. Further histological and terminological similarities are shown for mucinous and mucoepidermoid carcinoma, for lymphadenoma and lymphoepithelial carcinoma, for sebaceous adenoma and carcinoma, for adenoid-cystic carcinoma, as well as for salivary gland basal cell adenoma versus cutaneous spiradenoma. Manifest diagnostic problems related to histologically similar salivary gland and adnexal tumors are rare and are topographically limited to the parotid and oral areas.

A malignant eccrine spiradenoma of the scalp.

Malignant eccrine spiradenoma (MES) is an extremely rare but aggressive tumour, resulting in high mortality. We report the first case in the UK of metastatic MES on the scalp of a 37-year-old man who initially presented with a raised nodular mass which had grown rapidly over a few months. Excision biopsy of the lesion was performed and histology confirmed MES with evidence of cytological atypia and necrosis. A further wide local excision of the lesion with a 1 cm surgical margin was performed and full clearance was achieved. At 10-month follow-up another similar lesion had developed close to the previously excised area. A CT scan demonstrated metastatic lesions in the lungs, liver and pelvis, and subsequently chemotherapy was initiated. Although MES is uncommon, it has a high propensity to metastasise, therefore prompt identification, treatment and close follow-up of these tumours is essential.

Siringomas múltiples eruptivos / Multiple eruptive syringoma

Los siringomas son nevos benignos derivados del epitelio ductal intraepidermico de las glándulas sudoríparas ecrinas (acrosiringio). Predomina en mujeres y la variedad más frecuente es la de localización palpebral. La forma eruptiva es rara. Presentamos un varón de 29 años de edad, con siringomas múltiples eruptivos y compromiso en cara, antebrazos,abdomen y muslos con poca respuesta a tretinoína...