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Less Common Lung and Bronchial Tumours; Bronchiolo-Alveolar Ca., Carcinoids, Hamartomas, Reticuloses, Protein Disorders, Lung Deposits and Leukaemia.
Published in Fred W Wright, Radiology of the Chest and Related Conditions, 2022
These arise in the trachea and larger bronchi, especially the main bronchi, from mucous cells and mucous glands. Radiologically they cause narrowing of the airway within these structures, often with a smooth outline of the swelling within them. Bronchoscopy shows a pale tumour with an intact mucosa. For the latter reason bleeding and haemoptysis are uncommon. The cut surface of an excised specimen looks waxy or gelatinous. Occasionally these tumours are multiple. Surgical removal may be difficult because of their position, but a sleeve resection may be possible. Local recurrence is not uncommon. Regional and distal metastases may appear late. Death is usually due to bronchial obstruction. Examples are shown in Illus. CYLINDROMA.
Brooke–Spiegler Syndrome
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Cylindroma is a benign adnexal tumor with no attachment to the epidermis and shows a folliculosebaceous distribution (specifically the areas that are rich in hair follicles and sebaceous glands). It appears as solitary or multiple skin-colored or erythematous, smooth/pedunculated or ulcerated/disfiguring lesions of a few mm to several cm in size on the scalp, neck, face, and sometimes trunk and extremities (<10%), usually in adults. Solitary cylindroma often occurs sporadically in middle-aged and elderly persons who have no family history of similar lesions; whereas multiple cylindromas begin in early adulthood and grow in size and number throughout the life, coalescing in the scalp to form the so-called turban tumor that is associated with alopecia. In FC, multiple, erythematous nodules typically arise on the scalp but also on the forehead or pubic area. Histologically, cylindroma is a circumscribed, nonencapsulated dermal nodule composed of islands and cords of monomorphic basaloid cells surrounded by a thick, eosinophilic, PAS-positive hyaline sheath closely resembling a basement membrane and arranged in an interlocking “jigsaw puzzle”-like architecture. The cells at the periphery are small and dark with a tendency for palisading, while those in the center are large and pale, with occasional presence of lumina or pseudolumina (Figure 36.1) [20–22].
The Head and Neck
Published in E. George Elias, CRC Handbook of Surgical Oncology, 2020
Polyps can be snared or surgically excised. On the other hand, malignant tumors are rare and are more common in women than in men. Such tumors can be accompanied by benign polyps. The most common malignant tumor is squamous cell carcinoma. Other tumors of the nasal fossa may include adenocarcinoma (including cylindroma), chondrosarcoma, neurogenic tumors (including olfactory neuroblastoma), and melanoma. Surgical extirpation of the disease followed by irradiation is indicated in squamous cell carcinoma and adenocarcinoma, including cylindroma. Melanoma and sarcomas should be treated surgically with wide margins of resection. This is followed by reconstruction or prosthetic coverage. Lymphomas and plasmacytoma are sensitive to radiation therapy. However, the patient may have to be managed systemically by chemotherapy for these potentially systemic diseases. Similarly, metastatic tumors to the nasal area are managed by controlling the primary disease.
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
Histopathological and immunohistochemical analyses of the PDX tumors revealed that they recapitulate the histological and molecular features of the respective primary tumors. Thus, they showed the typical jigsaw pattern with islands and nests of basaloid tumor cells surrounded by a rim of hyalinized basement membrane material. The xenografted tumors also stained positive for MYB, a driver of growth of both sporadic and CYLD-mutated inherited cylindromas [7,8]. In addition, the PDXs expressed NTRK3 (TRKC), a tyrosine kinase receptor regulating cell survival and differentiation. NTRK3 signaling has previously been shown to promote colony formation and proliferation of cylindromas and the receptor is overexpressed in primary, CYLD-defective tumors [10]. Both MYB and NTRK3 are potential therapeutic targets in CYLD-defective cylindromas [7,10] that can be evaluated using the present preclinical PDX-model. Our studies, although preliminary, demonstrate that the cylindroma xenografts survive and retain their morphological and molecular characteristics for up to at least 6 months in vivo, which is ample time to allow for preclinical treatment studies. The reason why the PDX-tumors did not grow progressively may be due to the lack of specific factors in the mouse subcutaneous tissue microenvironment necessary for continous growth of cylindromas. This may, however, potentially be resolved by co-transplantation of human cylindroma tissues containing both epidermis, tumor, and dermal connective tissue.