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PubMed1991

[Cytological diagnosis and differentiation of tracheobronchial cylindromas].

From study of histocytological parallels in 12 cases of cylindromas the authors determined the criteria of cytologic diagnosis which allowed these tumors to be differentiated from other neoplasms of the lungs. Cylindromas are characterized by the presence in the cytogram of 2 types of cells (small cells and larger ones with light nuclei), homogeneous spherical structures with cells occurring centrally or on the periphery, ball-shaped clusters of cells, and an oxyphilic structureless mass forming the background of the preparation. The absence of oxyphilic masses in solid cylindroma, atypism and polyphormism of the epithelial cells makes it very difficult to differentiate it from moderately and poorly differentiated glandular carcinoma. However, careful study of the preparations and the discovery of an intermediate substance makes it possible to differentiate cylindroma from glandular carcinoma.

Adenocarcinoma↗
PubMed1987

[Multiple trichoepitheliomas, cylindromas and milia. An entity].

Four patients from two different families presented with multiple papular trichoepitheliomas of the face associated with cylindromas of the scalp and, in one of them, milium. This association, first described by Adamson, has now become classical. It is transmitted as an autosomal dominant trait with variable penetrance. Histochemical studies gave the following results: ATPase negative in the two types of tumour, phosphorylase weakly positive, NADH diaphorase positive in the basal cells of the trichoepitheliomas and diffusely in cylindromas. These results suggest that the cylindromas are of apocrine origin. Using monoclonal antibodies, it has been possible to demonstrate the presence of Langerhans cells in both trichoepitheliomas and cylindromas. The BL9 and KL3 antikeratinocyte monoclonal antibodies were negative, whereas the KL3 antibody, which recognizes the 55-57 Kd polypeptides of keratin, marked the suprabasal part of the tumours. These results are in favour of incomplete cell differentiation. Treatment with retinoids was ineffective, as in all other cases reported. Electrocoagulation or surgical excision om request for cosmetic reasons seem to be only possible treatments.

Antibodies, Monoclonal↗
PubMed1982

Cylindroma and eccrine spiradenoma coexistent in the same lesion.

Two patients complained of having a tender nodule. One patient had the nodule on the scalp and the other on the face. Each nodule contained a cylindroma and an adjacent eccrine spiradenoma. While eccrine spiradenoma is of eccrine derivation, the origin of cylindroma is controversial because of variable histochemical and enzyme histochemical findings. The presence of cylindroma and eccrine spiradenoma in one clinical nodule contributes circumstantial evidence for the similar derivation of both tumors.

Adenoma, Sweat Gland↗
PubMed1995

[Multiple eccrine spiradenoma. Histological association with dermal cylindroma].

A case of multiple eccrine spiradenomata is reported. One of the tumours was histopathologically associated with dermal cylindroma, and immunohistochemical studies showed that the cylindroma cells differentiate towards the secreting portion of the eccrine sweat gland. The relationship among multiple eccrine spiradenomata, dermal cylindroma and multiple trichoepitheliomata is discussed.

Adenoma↗
PubMed1995

Familial cutaneous cylindromas: investigations in five generations of a family.

BACKGROUND: Multiple cutaneous cylindromas are probably inherited in an autosomal dominant way. OBJECTIVE: Our purpose was to describe a large family with cutaneous cylindromas, trichoepitheliomas, and milia occurring in five generations and to elucidate further the mode of inheritance. METHODS: We examined 39 family members and obtained information on 31 other members from reports of relatives. RESULTS: The pedigree included 237 members, 118 male and 119 female, with 30 affected patients (11 male, 19 female). Between 33% and 100% of the children of affected family members had one or more of these skin lesions. Female-to-female, female-to-male, male-to-female, and male-to-male inheritance occurred. CONCLUSION: Multiple cutaneous cylindromas are inherited in an autosomal dominant way with variable clinical expression. Penetrance reaches 100% in adult life. This condition is associated with trichoepitheliomas and milia.

Adenoma↗
PubMedDate not supplied

Benign dermal cylindroma of the external auditory canal: a clinicopathological report.

Considerable confusion exists regarding the nature of benign dermal cylindroma of the external auditory canal. In the past, authors have used the term cylindroma to refer to malignant lesions such as adenoid cystic carcinoma. Benign dermal cylindroma is a specific clinical entity consisting of benign tumors arising from the epidermal appendages; however, whether they are apocrine- or eccrine-derived is not conclusively known. Six per cent are reported to be related to the ear. Histologically they are characterized by cords of basophilic cells, hyaline material, and cystic duct-like structures. Rarely, cases of malignant degeneration are reported. However, for the most part, these lesions are benign and should be treated with complete local excision. Radiotherapy may be useful in treating massive lesions not amenable to surgery.

Adult↗
PubMed2000

Brooke-Spiegler syndrome: treatment of cylindromas with CO2 laser.

Brooke-Spiegler syndrome is an autosomal dominant inherited disease characterized by the development of multiple trichoepitheliomas and cylindromas. We report two patients with Brooke-Spiegler syndrome whose scalp cylindromas were treated with a high-energy continous wave CO2 laser after debulking with bipolar scissors in one of the patients. The special feature in our report is the coexistence of three adnexal skin neoplasms in one case, and the close association of cylindroma and eccrine spiradenoma in the same lesion. We also support that debulking with bipolar scissors prior to laser therapy can be a technique of great benefit in large tumors.

Aged↗
PubMed2002

[Nasolacrimal duct obstruction caused by a cylindroma. Report of an atypical tumor manifestation and review of the literature].

BACKGROUND: The purpose of this report is to describe the clinical characteristics, management and histological findings in a patient with obstruction of the nasolacrimal duct caused by a cylindroma. About 618 lacrimal sac tumours have been reported previously in reviews and case reports, which were reviewed after searching in medline database. CASE REPORT: A 79-year old man suffered from multiple cylindromas (Spiegler tumour) on his head for about 40 years. He presented with a 6 months' history of left-sided epiphora and a painless medial canthal mass of various size. A tumour which caused nasolacrimal duct obstruction was found during dacryocystorhinostomy. After complete excision histological examination revealed a cylindroma, which had shown expansive growth from dermis to the lacrimal sac area. The patient felt well; there was no longer any epiphora or dacryocystitis after the operation. CONCLUSION: Although very rare, lacrimal sac tumours must be considered in the differential diagnosis of nasolacrimal duct obstruction. To our knowledge this case represents the first report on an atypical manifestation on a Spiegler tumour in the lacrimal sac region.

Aged↗
PubMed1996

Cutaneous adnexal carcinoma arising within a solitary cylindroma-spiradenoma.

Malignant transformation within an adnexal neoplasm with features of spiradenoma and cylindroma is reported in a 60-year-old woman. The malignant component of the tumor consisted of areas of solid carcinoma, with focal squamous metaplasia. In the benign component, apart from the features of spiradenoma and cylindroma, sebaceous differentiation was present adjacent to a cystic space lined by apocrine epithelium. The morphologic composition of this unusual neoplasm supports the concept of a histogenetic relationship between cylindromas and spiradenomas, including their differentiation toward the pilosebaceous-apocrine unit.

Adenoma, Sweat Gland↗
PubMed2002

Molecular analysis of chromosome 16q regions in dermal analogue tumors of salivary glands: a genetic link to dermal cylindroma?

Dermal analogue tumor, an uncommon subtype of basal cell monomorphic adenoma of the parotid gland, has a remarkable clinical and histologic resemblance to dermal cylindroma. Molecular studies of familial and sporadic cylindromas have shown frequent alterations at chromosome 16q12-13 that have recently been found to house the cylindromatosis gene (CYLD). To determine the involvement of the chromosome 16q12-13 region in dermal analogue tumors, we performed loss of heterozygosity analysis using microsatellite markers flanking the cylindromatosis gene locus in 21 sporadic dermal analogue salivary tumors and 12 salivary and dermal lesions from two sisters. Loss of heterozygosity was identified in 17 (80.9%) of the 21 sporadic tumors and in nine of the 12 dermal and salivary gland dermal analogue tumors from the two sisters; a parathyroid adenoma from one sister and two lymphoepithelial lesions from the second sister showed no microsatellite alterations. Microsatellite instability was only identified in three sporadic tumors at marker D16S308. Markers D16S409 (centromeric), D16S541, and D16S308 (telomeric) to the CYLD gene showed the highest incidence of loss of heterozygosity (>65%). The minimally deleted region was flanked proximally by marker D16S389 and distally by marker D16S419 and spanned the 771.5-megabase fragment that included the CYLD locus. We conclude that dermal analogue tumor and cylindroma share similar incidence of alterations at the 16q12-13 region, supporting a common molecular origin.

Adult↗
PubMed1991

Cylindroma overexpresses collagen VII, the major anchoring fibril protein.

The cell clusters of the human adnexal tumor, cylindroma, are surrounded by an unusual, thick, unorganized basement membrane-like zone in vivo. Ultrastructural analysis of this electron-dense zone revealed recesses that contained numerous anchoring fibrils. Indirect immunofluorescence staining with antibodies to collagen VII, the major structural component of the anchoring fibrils, showed strong fluorescence of this zone. Collagen VII could be isolated from tumor tissue by limited pepsin digestion or by extraction with chaotropic agents. Cylindroma cells were cultured from the tumor and passaged several times. The cells exhibited a strong expression of collagen VII in vitro, when assayed with immunofluorescence staining or immunoblotting of culture extracts. These results suggest that cylindroma can be used as an abundant source to produce collagen VII in vitro to study the biosynthesis and regulation of collagen VII and the formation of the anchoring fibrils, as other culture systems have failed to produce ample amounts of this anchoring fibril protein.

Aged↗
PubMed1997

Multiple malignant cylindromas of skin in association with basal cell adenocarcinoma with adenoid cystic features of minor salivary gland.

This unusual case is that of a middle-aged man exhibiting a tumor diathesis including a basal cell adenocarcinoma with features of adenoid cystic carcinoma arising in minor salivary gland of lip in association with multiple primary malignant cylindromas of skin. The labial lesion showed invasive tubules, solid epithelial sheets and cribriform structures. It did not exhibit PAS positive juxta-tubular basement membrane material. The skin lesions all showed features of a highly infiltrative cylindromatous carcinoma with two cell types, peripheral palisading and prominent PAS positive juxta-tubular basement membrane material. Immunocytochemical studies of the lip lesion and one of the skin lesions showed similarities, including positive staining for high and low molecular weight keratins and S-100 with negative staining for CEA. The precious descriptions of tumor diatheses involving dermal cylindromas and dermal analogue tumors of salivary glands and the distinctions with the present study are noted. If benign and even malignant cylindromas were described in the literature to be associated with basal cell adenocarcinoma of the major salivary glands, our case is unique by its association with this rare malignant tumor in a minor salivary gland.

Adenocarcinoma↗
PubMed1992

Dermal cylindroma. Expression of intermediate filaments, epithelial and neuroectodermal antigens.

We report on immunohistochemical staining patterns in so-called apocrine tumors of skin with special emphasis on the dermal cylindroma. The results were compared with apocrine tubular adenoma, syringocystadenoma papilliferum and the normal eccrine sweat gland. A relationship of dermal cylindroma to the apocrine gland is suggested by expression of lysozyme and alpha 1-antichymotrypsin. The tumor shares keratin, epithelial membrane antigen (EMA) and EGF-receptor expression with eccrine and apocrine glands. The presence of intermingled cells with a coexpression of keratin and vimentin argues for a partial myoepithelia-like differentiation. Neuroectodermal antigens are missing. Therefore, dermal cylindroma is classified as an adnexal tumor of skin with a variable rate of cells of apocrine secretory, myoepithelial and undifferentiated phenotypes.

Adenoma↗
PubMed1977

[Basal cell adenomas of the salivary glands. Analysis of 7 cases and review of the literature. Comparative study with cylindromas].

Basal cell adenomas, of which 7 cases are reported here together with 63 others collected in the literature, are benign tumours of the salivary glands, preferentially localised in the parotid and in the upper lip. Mobile, non-ulcerated and painless, these nodules with an average diameter of 2 cm are well circumscribed, fleshy or cystic. Their morphology is characterised by the proliferation of uniform, small cells, with scarcely visible cytoplasm, arranged in layers, cords and canals, which may be ectasic or cystic. Peripheral elements, individualising these different structures, are arranged in palissades and are regularly surrounded by a basal membrane. The histological analogies between basal cell adenomas and cylindromas merely reflect their ultrastructural similarities, cellular as well as architectural. These two neoplasms may be distinguished on the basis of two essential morphological criteria:--basal cell adenomas are well circumscribed, whilst cylindromas are invasive;--the cells in adenomas are closely juxtaposed, endowing the various structures of these tumours with a dense appearance, whilst cylindroma cells are generally separated by clear spaces corresponding to extreme development of the intercellular spaces.

Adenoma↗
PubMed1987

Malignant transformation in a solitary dermal cylindroma.

We describe a case of a solitary dermal cylindroma with focal malignant transformation. The scalp tumor measured 3 cm in greatest dimension and was successfully surgically excised. The patient has been followed up for the last two years with no recurrences. Changes of malignant transformation included loss of peripheral palisading, destruction of the hyaline sheath, areas of necrosis, and pleomorphism of nuclei with frequent mitosis. We reviewed data on 12 other reported cases of dermal cylindromas with malignant transformation. Morphologic features of both benign cylindromas and their malignant counterparts are described. The literature was reviewed regarding the controversy of the cell of origin.

Aged↗
PubMed1995

Unusual abdominal location of a dermal cylindroma.

Cylindromas are rare benign adenexal tumors that classically arise on the head and neck, thus earning the name of turban tumor. They are usually benign, but may be multiple and recurrent. A 78-year-old woman noted an abdominal cylindroma, an unusual presentation of this rare cutaneous tumor. The clinical presentation, histologic characteristics, and variations of the dermal cylindroma are reviewed.

Abdominal Muscles↗
PubMed1996

The cylindromatosis gene (cyld1) on chromosome 16q may be the only tumour suppressor gene involved in the development of cylindromas.

Hereditary cylindromatosis is a rare autosomal dominant disease characterised by the development of multiple benign neoplasms of the skin. We recently localised the gene responsible for this disease (cyld1) to chromosome 16q12-q13 and provided evidence that it is a tumour suppressor gene (Biggs et al., 1995). We have now examined polymorphic markers on every chromosome, some of which are close to known tumour suppressor genes, in 25 tumours from 4 individuals with familial cylindromatosis. No loss of heterozygosity (LOH) was detected other than at loci on chromosome 16q. This observation suggests that the cyld1 gene may be the only tumour suppressor gene implicated in the development of cylindromas. We have also demonstrated LOH using markers on chromosome 16q in 8/14 (57%) sporadic cylindromas, indicating that the cyld1 gene is likely to be involved in the genesis of both familial and sporadic cylindromas.

Carcinoma, Adenoid Cystic↗
PubMedDate not supplied

Florid dermal cylindroma (turban tumor).

A florid case of dermal cylindroma is presented as representative of the natural history and usual clinical findings in a patient afflicted by this tumorous growth. Although histologically and clinically benign, dermal cylindroma can result in considerable disfigurement of the patient. Treatment varies from isolated primary resection to serial resections with subsequent electrocautery or cryosurgery for small recurrences. Total scalping with skin grafting is reserved for only the most severe cases where normal integument has been completely involved by the disease process.

Carcinoma, Adenoid Cystic↗