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Ten dermal cylindromas have been studied using histological, ultrastructural and immunocytochemical techniques. The clinical data from the ten patients have also been reviewed. The results indicate histogenesis from the intradermal coiled duct region of the eccrine sweat gland.
We have studied four patients from two families and obtained the clinical data from four other affected family members who demonstrated the coexistence of multiple cylindromas and trichoepitheliomas. An autosomal dominant pattern of inheritance with variable penetrance was exhibited in both families, with a female to male ratio of 3:1. The clinical and pathological features of this rare association are discussed.
Multiple dermal cylindromas on the scalp and back and eccrine spiradenoma on the face of a 59 year old woman are described. A case for a common origin is discussed.
The management of multiple cylindromas presents a therapeutic challenge to the clinician. The condition has significant cosmetic implications, and malignant degeneration can occur. Reported treatments include excision and repair of individual lesions, high-dose radiation, and complete scalp removal with grafting. We review the proposed histogenesis of this tumor, discuss its associations, and report the first use of the carbon dioxide laser as a successful treatment.
This report describes the ultrastructural characteristics of a Poncet-Spiegler tumor (cylindroma, turban tumor). This tumor was made up of two cell types and showed a ductal differentiation. Cells bordering duct-like structures were joined together by innumerable desmosomes, especially in places where the cell membranes were markedly invaginated. The arguments in favor of an eccrine or an apocrine for the origin of this sweat gland tumor are discussed.
A patient with coexisting dermal cylindromas (Turban tumour), eccrine spiradenomas and a variant of basal cell adenoma of the parotid gland characterized by prominent focal deposition of hyaline (membranous basal cell adenoma of parotid gland) is reported. The microscopic appearance of the cutaneous and parotid gland tumours was very similar. This rare association of analogous tumours of sweat and parotid glands has been described only in 5 previous cases. It is hypothesized that the occurrence of the 3 different tumours found in this individual may be explained by the multipotential reserve cell concept and these tumours perhaps could be considered as different expressions of a single genetic disorder.
Four cases of adenoid cystic carcinoma (cylindroma) of accessory salivary tissue are described, and the diagnostic difficulties experienced are emphasized. The tumour infiltrates cranial and other nerves and may invade the central nervous system by this route. It is highly malignant and may metastasize widely both by haematogenous and lymphatic pathways. The tumour is radiosensitive and it is suggested that the treatment of choice is radiotherapy combined, when possible, with surgical excision of the primary lesion. Metastases should be treated with radiotherapy since useful remission may occur.
Three cases of trichoepithelioma associated with cylindroma (Brooke-Spiegler syndrome) in the same family are reported. Three generations were affected. The syndrome usually beginning at puberty is inherited as an irregular autosomal dominant trait.
Adenoidcystic carcinoma or cylindroma of the parotid gland is a slow-growing, relentless, and often lethal disease which kills usually by local occurrence and direct extension of the disease. The reported incidence of adenoidcystic carcinoma with reference to other parotid malignancies varies from 5% to 37%. The incidence in this series is 36%. The presence of facial nerve involvement adversely affects the long-term survival. Some authors regard facial nerve involvement as an indication of noncurability. The incidence of local pain is reported to be as high as 50% and is thought to be caused by perineural invasion by the tumor. This is well-documented histopathologically. There is no uniform or standard treatment for this disorder. Extensive resection of the parotid gland (including the facial nerve if there is any indication of involvement), followed by external irradiation to the parotid area and upper neck is the most rational approach to the problem. This is supported both in the review of the literature and from the present series. A radical neck dissection is not included as part of initial therapy unless there are clinically suspicious cervical nodes. Postoperative radiotherapy appears indicated in all cases of adenoidcystic carcinoma of the parotid gland. Four case histories are presented which demonstrate the hallmark characteristics of the disease, i.e. multiple cranial nerve deficits, facial pain and subsequent death by direct intracranial extension of disease. The protracted, relentless course of the disease is well-documented in one case in which 25 local recurrences developed over an 18-year period.
Certain dermal appendage tumors have a striking histologic and clinical homology with some tumors arising from the salivary duct system. The dermal eccrine cylindroma is an exemplar. Arising from the eccrine ducts, this neoplasm is only occasionally found outside the head and neck, has a recurrence rate of over 40%, and in its malignant form is a high-grade carcinoma. Its counterpart in salivary glands, the dermal analogue tumor, manifests a histologic similarity, if not identity, also has a relatively high recurrence rate, and can also undergo malignant change.
The onset of sweat gland and salivary gland tumors in the same patient is a rare association known only for a few years. The case is reported of a man who died at 63 with lung metastases and bearing a turban and salivary tumor (both of the eccrine cylindroma dermal type). This "cutaneous and salivary neoplastic syndrome" may be explained by a common stem cell in different organs.
Cylindromas are rarely encountered in the neurosurgical field. Four cases of this rare tumor are presented. Conventional and computerized tomography scanning were most useful in establishing the diagnosis; angiography did not provide any further information. Because these tumors are well demarcated from surrounding structures, even in cases of recurrence, surgery is the treatment of choice. Radiation therapy is useful in the postoperative management. Long survival times with multiple recurrences are the rule.
The authors show a case of parapharyngeal space cylindroma paying attention to large diagnostic difficulties. This tumor has been characterized by a slow local growth but after five years disease involved many organs with metastases.
A case of malignant eccrine cylindroma of the skin studied immunohistochemically is reported.
We report a rare case of adenoid carcinoma of the endocervix (cylindroma). Laparoscopic lymphadenectomy and extended vaginal hysterectomy (Schauta-Dargent operation) was successfully performed. We present illustrative pathology findings and review the literature on this severe disease.
We report a new case of multiple trichoepitheliomas, milia and cylindromas syndrome. This new case differed from the others in that it was complicated by the occurrence of a basal cell carcinoma presenting as an ulcus rodens affecting the nasal pyramid. In such cases, the main differential diagnosis to be excluded is basal cell naevomatosis.
The authors present the results of medical treatment in patients with head and neck cylindromas hospitalized in the Department of Otolaryngology. The Zeromski Hospital in Kraków, within the period 1961 to 1988.
A superomedial orbital dermal cylindroma in an adult female is described.