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Brooke–Spiegler Syndrome
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Spiradenoma forms single or several variably sized, well-demarcated nodules of blue/black appearance, commonly in the extremities, trunk, and less frequently in the scalp. Due to its common co-occurrence with cylindroma, giving rise to spiradenocylindroma, spiradenoma appears to have a similar apocrine origin to cylindroma. Histologically, spiradenoma shows small epithelial basaloid cells with hyperchromatic nuclei at the periphery in a trabecular, reticular, solid or diffuse growth pattern, along with large cells containing pale nuclei and sparse lymphocytic infiltrate in the center, in addition to droplets of PAS-positive hyaline basal membrane material (Figure 36.2) [23].
Spiradenoma
Published in Longo Caterina, Diagnosing the Less Common Skin Tumors, 2019
Spiradenoma is a benign adnexal tumor originating from apocrine glands. It usually presents as a solitary gray-pinkish nodule, less than 1 cm in diameter, arising on the head and neck or on the truncal region. Moreover, spiradenoma may arise in a sebaceous nevus and is commonly seen in patients affected by the Brooke–Spiegler syndrome. Malignant transformation is rare and has been reported in spiradenomas associated with Brooke–Spiegler syndrome.1 At histopathologic examination, one or more large dermal nodules are commonly seen, sometimes extending into the subcutis. Within these nodules, two cell populations may be found, consisting of small basaloid cells and more frequent larger cuboidal cells with a pale nucleus. Periodic acid–Schiff (PAS)-positive hyaline material is often present at the periphery of the aggregates, together with a few duct-like structures. In the stroma between lobules, dilatated vessels or even hemorrhage may be seen.2 Cylindroma-like features are frequently seen in spiradenoma, in particular in patients with Brooke–Spiegler syndrome.
Malignant tumors
Published in Archana Singal, Shekhar Neema, Piyush Kumar, Nail Disorders, 2019
Histopathology demonstrates well-circumscribed nodules of typical benign eccrine spiradenoma with its characteristic two cell types of peripheral cuboid basal cells and luminal cells. The carcinomatous change may be seen as a gradual transition to a malignant area in the tumor where the two-layered spiradenoma architecture changes to monomorphous carcinoma with ill-defined nests and cords of tumor cells. Duct-like structures may be completely missing and glandular components as well as hyaline globules are decreased. The other type usually presents as a spiradenocarcinoma adjacent to the benign tumor without transition. There may be Bowenoid, squamous, ductal carcinoma-like, tubular, histiocytoid, and carcinosarcomatous changes with rhabdomyoblastic and osteosarcomatous differentiation.85 Necrosis and hemorrhage are found in advanced spiradenocarcinoma. The most important differential diagnosis is benign spiradenoma as the diagnosis of malignant transformation may be missed in case of an insufficient biopsy.
Extraskeletal Ewing's Sarcoma with CD7 Positivity and T-cell Receptor/Immunoglobulin Rearrangement Masquerading as T-lymphoblastic Lymphoma
Published in Fetal and Pediatric Pathology, 2022
Yin Cheng, Li Yang, Na Zhang, Guang-Sheng Chen, Juan Li, Yan-Fei Liu, Chun-Ju Zhou
A nine-year-old boy presented with recurrent and fast-growing mass in his right thigh. One year earlier, he had undergone a surgical excision at a local hospital due to subcutaneous, painless, and mobile mass (the size of a broad bean) at the same site, which was diagnosed as a “spiradenoma”. Physical examination showed an egg-like bulk, solid, non-mobile mass and a surgical scar on the surface of the mass. There was no liver, spleen, lymph node enlargement and any other obvious signs. Magnetic resonance imaging showed a huge, slightly lobulated mass measuring 8.7 cm × 5.6 cm × 3.9 cm under the skin of the right thigh (Figure 1A). The border was clear, and there was no femoral destruction. Peripheral blood routine, liver and kidney function tests, and bone marrow biopsy were normal. The tru-cut biopsy was performed.
Clinical, genetic and experimental studies of the Brooke–Spiegler (CYLD) skin tumor syndrome
Published in Journal of Plastic Surgery and Hand Surgery, 2019
Mattias K. Andersson, Lars Kölby, Jonas A. Nilsson, Göran Stenman
Brooke–Spiegler syndrome (BSS) is an autosomal dominant inherited skin disorder characterized by the appearance of multiple skin adnexal tumors such as cylindroma, spiradenoma and trichoepithelioma [1]. The tumors typically occur in the head and neck region, particularly on the scalp, and increase in size and number during the lifetime of the patient. They are usually benign but may occasionally undergo malignant transformation. In addition to skin tumors, BSS patients may on rare occasions develop salivary gland tumors morphologically resembling their cutaneous counterparts [1,2]. Cylindromas may rarely also occur in the breast [1,3]. Despite the fact that BSS tumors are benign they can become quite large and adjacent lesions can coalesce (so called turban tumors). The treatment of BSS skin tumors is limited to repeated surgical excisions with or without concomitant skin transplantation. Thus, there is a need for novel, non-surgical treatment options for these patients.