No full-text available
To read the full-text of this research,
you can request a copy directly from the authors.
Malignant Dermal Cylindromas. Do they Exist? A Morphological and Immunohistochemical Study and Review of the Literature
Abstract
Malignant dermal cylindromas are very rare. We present a case of multiple cylindromas of the scalp with metastasis to a cervical lymph node. The morphology of the tumour was unusual in that it contained eccrine spiradenoma-like areas and foci of squamous differentiation with keratin formation. The immunohistochemical phenotype of the eccrine spiradenoma-like areas and the metastatic tumour was similar, but different from the areas of typical cylindroma.
Although alleged “malignant” cylindromas have been reported, none have been described to have metastasized, whereas metastatic eccrine spiradenoma is well-documented. We reiterate that overlaps between dermal cylindroma and eccrine spiradenoma are more common than has been documented. In the rare event of metastases, it is the eccrine spiradenomatous component that is metastatic. We contend that there is no evidence that pure dermal cylindromas have metastasized.
... Although the literature contains approximately 100 neoplasms reported as spiradenocarcinoma, cylindrocarcinoma, and spiradenocylindrocarcinoma, based on the illustrations provided, not all of those cases may be accepted as such. 56 Spiradenocarcinoma is the most commonly reported neoplasm, and spiradenocylindrocarcinoma is the rarest form, but this is likely due to underrecognition of benign spiradenocylindroma. 9,31,51 As for their benign counterparts, most malignant cases have occurred sporadically, but some have arisen in the setting of BSS. [1][2][3]8,12,15,19,21,24,31,38,39,41,[45][46][47][52][53][54]57,66,67,71 Most have been published as case reports, with the only series being that of Granter et al, 22 comprising 12 spiradenocarcinomas. ...
... 56 Spiradenocarcinoma is the most commonly reported neoplasm, and spiradenocylindrocarcinoma is the rarest form, but this is likely due to underrecognition of benign spiradenocylindroma. 9,31,51 As for their benign counterparts, most malignant cases have occurred sporadically, but some have arisen in the setting of BSS. [1][2][3]8,12,15,19,21,24,31,38,39,41,[45][46][47][52][53][54]57,66,67,71 Most have been published as case reports, with the only series being that of Granter et al, 22 comprising 12 spiradenocarcinomas. Most malignant neoplasms have been found to arise in a preexisting benign neoplasm, a residuum of which is included as a diagnostic criterion, among others listed in the World Health Organization classification of cutaneous neoplasms. ...
... Analysis of the follow-up of our BSS patients combined with previously reported cases shows an approximately 80% disease-related death rate, which may be even higher, as the follow-up in some of the previously reported cases was short or not available. [1][2][3]8,12,15,19,21,24,31,38,39,41,45,46,47,[52][53][54]57,66,67,71 As far as we can judge from the illustrations accompanying these papers, the BCAC morphology, either high-grade or low-grade prevailed. Of note, some of those patients, in addition to benign and malignant cutaneous neoplasms, also developed basal cell adenoma or BCAC of salivary glands. ...
The authors present a series of 24 malignant neoplasms arising in preexisting benign spiradenoma (20), cylindroma (2), and spiradenocylindroma (2). Nineteen patients (12 females, 7 males; age range, 41 to 92 y) had a solitary neoplasm (size range, 2.2 to 17.5 cm; median 4 cm), whereas the remaining 5 (4 females, 1 male; age range, 66 to 72 y) manifested clinical features of Brooke-Spiegler syndrome (BSS), an autosomal dominantly inherited disease characterized by widespread, small, benign neoplasms on which background larger malignant lesions appeared. Microscopically, all cases showed the residuum of a preexisting benign neoplasm. The malignant components of the lesions were variable and could be classified into 4 main patterns, occurring alone or in combination: 1) salivary gland type basal cell adenocarcinoma-like pattern, low-grade (BCAC-LG); 2) salivary gland type basal cell adenocarcinoma-like pattern, high-grade (BCAC-HG); 3) invasive adenocarcinoma, not otherwise specified (IAC-NOS); and 4) sarcomatoid (metaplastic) carcinoma. In 1 case of IAC-NOS, an in situ adenocarcinoma was also found, presumed to have evolved from an adjacent adenomatous and atypical adenomatous component. Cases harboring a sarcomatoid carcinoma featured a malignant epithelial component composed of varying combinations of BCAC-HG, BCAC-LG, IAC-NOS, or squamous cell carcinoma, whereas the sarcomatoid component appeared as either a pleomorphic or spindle-cell sarcoma. Additionally, in 2 cases there were foci of heterologous chondrosarcomatous differentiation and in 1 case there was rhabomyosarcomatous differentiation. Of the 21 patients with available follow-up (range, 3 mo-15 y; average 4.8 y; median 3.5 y), 10 were without evidence of disease, 1 was alive with metastatic disease, 1 was alive with BSS, 3 developed local recurrences, 4 had died of disease, and 2 were dead of other causes. The histologic pattern of the malignant neoplasm correlated to some extent with the clinical course. BCAC-LG neoplasms showed a less aggressive course, with local recurrences but no distant metastases, whereas the BCAC-HG neoplasms typically followed a highly aggressive course resulting in the death 3 of 6 patients with BCAC-HG. Patients with sarcomatoid carcinoma had a relatively good survival. Molecular genetic investigations revealed no mutations in the CYLD gene in the 4 sporadic cases investigated. One patient with BSS revealed a novel missense germline mutation in exon 14 (c. 1961T>A, p. V654E), whereas a living descendant of another deceased patient demonstrated a recurrent nonsense germline mutation in exon 20 (c. 2806C>T, p. R936X). Given the morphologic diversity and complexity of the neoplasms in question, we propose using a more specific terminology with the precise description of the neoplasm components, rather than generic and less informative terms such as "spiradenocarcinoma" or "carcinoma ex cylindroma."
... Further microscopic analyses of the tumors confirmed the presence of "residuum of a pre-existing benign neoplasm" indicative of in situ development of malignancies from the apparently benign lesions. A handful of malignant cases developed in patients with BSS have also been reported by others [30][31][32][33][34][35][36][37][38][39][40][41][42][43][44][45][46][47][48][49]. These malignancies included salivary gland type basal cell adenocarcinoma-like pattern, low-grade (BCAC-LG), and high grade (BCAC-HG), invasive adenocarcinomas (IACs), squamous cell carcinomas (SCCs), anaplastic neoplasms and sarcomatoid (metaplastic) carcinomas [34,[50][51][52][53][54][55][56][57][58][59]. ...
Germline CYLD mutation is associated with the development of a rare inheritable syndrome, called the CYLD cutaneous syndrome. Patients with this syndrome are distinctly presented with multiple tumors in the head and neck region, which can grow in size and number over time. Some of these benign head and neck tumors can turn into malignancies in some individuals. CYLD has been identified to be the only tumor suppressor gene reported to be associated with this syndrome thus far. Here, we summarize all reported CYLD germline mutations associated with this syndrome, as well as the reported paired somatic CYLD mutations of the developed tumors. Interestingly, whole-exome sequencing (WES) studies of multiple cancer types also revealed CYLD mutations in many human malignancies, including head and neck cancers and several epithelial cancers. Currently, the role of CYLD mutations in head and neck carcinogenesis and other cancers is poorly defined. We hope that this timely review of recent findings on CYLD genetics and animal models for oncogenesis can provide important insights into the mechanism of head and neck tumorigenesis.
Electronic supplementary material
The online version of this article (doi:10.1186/s41199-016-0012-y) contains supplementary material, which is available to authorized users.
The clinical presentation of skin appendage tumours for the most part is not as specific and diverse as the histological features of this extremely heterogeneous tumour group. Differential diagnosis of certain appendageal tumours can be extremely subtle, yet greatly influence final treatment outcome. Occasional association of certain entities with inherited tumour syndrome s can be extremely important in terms of hereditary cancer screening. This chapter aims to provide the most important clinical, histological and genetic features for selected, most important cutaneous appendage tumours.
This chapter on cutaneous appendageal tumours covers the spectrum of lesions displaying eccrine, apocrine, sebaceous and follicular differentiation. It includes cysts, hamartomas and benign and malignant tumours. Traditionally, appendageal tumours have been classified according to rigid criteria separating lesions with eccrine, apocrine, sebaceous and follicular differentiation. This separation is clearly artificial and it is now acknowledged that lesions regarded as strictly eccrine may well have apocrine differentiation and lesions classified as purely follicular may exhibit apocrine differentiation reflecting the close embryological relationship between the pilosebaceous unit and the apocrine gland.
Cylindromas are adnexal tumours of either eccrine or apocrine origin. Two common variants- Solitary and Multiple are seen, of which the malignant transformation of a solitary lesion is rare. Solitary tumours occur sporadically in the head and neck and are without a familial history whereas the multiple tumours are inherited in an autosomal dominant pattern occurring in scalp and rarely on trunk. Malignant transformation is observed commonly in multiple tumours, although transformation of solitary lesions has also been reported. We report a rare case of malignant transformation of a solitary cylindroma involving the fronto-nasal region in a 72-year-old diabetic and hypertensive male reported to the Department of Oral and Maxillofacial Surgery, with a chief complaint of rapidly growing, painful growth over fronto-nasal region for 3-years and difficulty in breathing since 3-months. on examination; a single, large, well circumscribed, tender, firm and fixed multinodular growth with focal areas of ulcerations and reddish brown crustations of approx. 9.0×3.5 cm measuring in its greatest dimension was seen in naso-frontal region. A CECT Head showed a well defined, heterogeneously enhancing lobulated soft tissue mass, occupying the left nasal cavity, ethmoid sinus and extending into medial extraconal space of left orbit. The incisional biopsy report suggested low grade skin adnexal tumor of malignant cylindroma. Based on the finding in the biopsy, the CECT surgical excision of the lesion was planned. The patient was kept on follow-up of 3 years and no local recurrence or distant metastases was noted.
Multiple cylindromas on the scalp seems to be the most common predisposing factor for the development of a cylindrocarcinoma. The diagnosis of cylindrocarcinoma or spiradenocarcinoma should be suspected when sudden rapid growth occurs in a pre-existing long-standing lesion.
Background:
Multiple skin cylindromas are of autosomal dominant transmission and may be associated with spiradenoma and trichoepithelioma. This condition is known as Brooke-Spiegler syndrome. The aim of this study is to compare surgical and laser treatment for these lesions.
Case reports:
Five patients from a single family (two male and three female) consulted at the dermatology department for the treatment of multiple cylindromas of the scalp. The female patients presented a more severe form that had developed into "turban tumour". All patients were initially treated by surgery. Two had undergone multiple surgical excisions alone while the others had been treated with CO2 laser. One of the female patients treated with surgery had undergone complete excision of the scalp covered by total skin graft. The three patients treated with CO2 laser were completely satisfied with their therapy, which enabled a larger number of lesions to be treated at each session. They underwent one session every three months under local anaesthesia.
Conclusion:
CO2 laser constitutes an alternative to surgery in the treatment of multiple skin cylindroma. It gives excellent cosmetic result with excellent safety and a high degree of satisfaction among treated patients. It should be proposed for patients refusing surgery or where the number of lesions enables first-line use of this approach to be envisaged.
Introduction:
Cylindromas are usually benign tumors as small, solitary, slow-growing nodules of the scalp, face and trunk. Multiple cylindromas may form a "turban tumors" in the autosomal dominant Brooke-Spiegler syndrome. Malignant dermal cylindromas are very rare. There are few cases of malignant transformation of dermal cylindromas in the literature. We present an unusual case of malign cylindroma of the scalp with multiple metastasis to cervical lymph nodules and also this case is the only case that has the most lymph node metastasis.
Presentation of case:
A 52-year-old man initially presented to our clinic with hyperemic plaque located on his scalp. Measures of the plaque was 20cm×10cm with bilateral, multiple palpable submandibulary lymph nodes. Entire scalp was removed reaching in depth the periostal level and bilateral modified radical neck dissection was performed. The histopathological examination of the scalp specimen was malignant skin ecrine tumor, cylindroma and on the neck dissection specimen, 55 lymph nodes were reported as metastatic out of 79. The patient was treated with chemotherapy and radiotherapy. No recurrence or metastasis was observed during a 5 year follow-up period.
Discussion:
Malign cylindroma of the scalp rarely presents with multiple cervical lymph node metastasis, however it is important to be aware of this possibility.
Conclusion:
The case reported below is outstanding in literature for being the only case that has the most lymph node metastasis. Although malign transformation of dermal cylindromas is rare, aggressive surgery should be considered with locoregional metastasis of the tumor.
Hair-follicle tumoursExternal root-sheath tumoursHamartomas and hair germ tumours and cystsHair matrix tumoursLesions of the hair-follicle mesenchymeSebaceous gland tumoursApocrine gland tumoursEccrine gland tumoursEccrine or apocrine/follicular tumoursEccrine gland carcinomasEccrine or apocrine/follicular carcinomas
Hair-follicle tumours External root-sheath tumours Hamartomas and hair germ tumours and cysts Hair matrix tumours Lesions of hair-follicle mesenchyme Sebaceous gland tumours [1] Apocrine gland tumours [1] Eccrine gland tumours [1] Eccrine or apocrine/follicular tumours Sweat gland carcinomas, including ductal apocrine/follicular carcinomas Eccrine gland carcinomas Eccrine or apocrine/follicular carcinomas Miscellaneous tumours References
Cylindroma is a rare tumor which originates from skin appendages and occurs predominantly on the scalp and face. It is considered to be benign, but malignant transformation has been reported. It has two distinct clinical presentations, solitary and multiple. A review of the literature revealed that the majority of malignant cylindromas occur among multiple type cylindromas. Only 36 malignant cylindromas have been reported, and only 9 were of the solitary type. A case with a solitary type of cylindroma exhibiting malignant transformation of the scalp is presented.
Brooke-Spiegler syndrome is a rare condition with a predisposition to develop cutaneous adnexal neoplasms, especially cylindromas, trichoepitheliomas and spiradenomas. Malignant transformation of cylindromas is rare. In such cases usually cylindrocarcinomas develop within these lesions. We present an unusual case of basal cell carcinoma developing within a preexisting cylindroma.
58-year-old woman with a 30-year history of multiple dermal cylindromas extensively involving her scalp was referred for dermatological treatment. The patient reported that one of the long-lasting lesions, 5.5 cm in size, ulcerated within the foregoing few weeks. Histopathology confirmed cylindromas and basal cell carcinoma within the ulcerating tumor. Surgical excision of largest cylindroma tumors led to cosmetic and functional improvement. Magnetic resonance and computed tomography showed tumor infiltration into the skull lamina externa. Metastases were excluded by chest radiography and abdominal ultrasound examination.
Patients with Brooke-Spiegler syndrome should be followed-up for malignant transformation of skin tumors to prevent deep penetration and possible metastases.
Malignant cutaneous cylindroma is a rare tumor. It has been described in 26 cases, both in the solitary form and in the autosomal dominant inherited multiple tumor form. The authors present two new cases that occurred in one family with a history of multiple cylindromas.
Clinical and histopathologic data of both tumors were compared with those of 26 other cases in the literature. Immunohistochemical examinations were performed.
The malignant tumors were distinguished from the benign lesions by rapid growth, long-standing ulceration, or bleeding. Histopathologic examination showed a well-differentiated carcinoma in one patient and a poorly differentiated tumor in the other. In the latter, lymph node metastasis developed, and the patient died 2.5 years later. Histopathologic criteria of malignancy included cell pleomorphism, frequent mitoses and loss of jigsaw pattern, peripheral palisading, hyaline sheaths, and dual cell population.
These observations are in accord with those in the literature. Malignant cutaneous cylindroma developed more often in the multiple tumor form than in the single tumor form. Malignant cylindroma is an aggressive carcinoma with a tendency to local destructive growth and metastases.
Cylindromas are benign tumours arising as small, solitary, slow-growing nodules on the head and neck. Multiple cylindromas may form a 'turban tumour' in the autosomal dominant Brooke-Spiegler syndrome. We report two unusual cases of multiple cylindromas with transformation into cylindrocarcinomas. The first patient, a 63-year-old white woman, developed a cylindrocarcinoma on pre-existing multiple cylindromas on her right shoulder. Eight months after resection she developed a lymph node metastasis in the right axilla. The second patient, a 68-year-old white woman, presented with multiple cylindromas of the scalp. One of these transformed into a cylindrocarcinoma, infiltrating the dura mater, with local recurrence 2 years after incomplete resection and postoperative radiation.
Familial cylindromatosis (Brooke-Spiegler syndrome) is a rare autosomal dominant inherited disease characterized by the development of adnexal tumors, mostly cylindromas, but also trichoepitheliomas and spiradenomas. Malignant tumors may occur, usually with the features of a cylindrocarcinoma. The authors describe the case of a 75-year-old woman with the Brooke-Spiegler syndrome who presented with multiple nodules of the scalp, face, and trunk. In 1997 she underwent surgical excision of the entire forehead and scalp with skin grafting. Histologic examination revealed multiple cylindromas, some with areas of spiradenoma and one with an extensive adenomatous component; some trichoepitheliomas were also evident. In 2002, a nodule of the trunk suddenly increased in size and became painful. The lesion was excised and histologic and immunohistochemical evaluation revealed a malignant cutaneous biphasic tumor extending into the subcutis, consisting of a major portion with the features of an adnexal carcinoma and of a minor one of atypical spindle cells. Biphasic malignant skin tumors are rare and only a limited number have been described, none in association with the Brooke-Spiegler syndrome. The authors discuss the morphogenesis of the folliculosebaceous-apocrine unit from which the tumors in this syndrome derive, and the pivotal role of mesenchymal cells in determining the process. Since the Brooke-Spiegler syndrome is characterized by a germline mutation in the CYLD oncosuppressor gene, a biphasic tumor in this setting may represent a true carcinosarcoma.
Multiple malignant Spiegler tumors were observed on the scalp of a 67-year-old female patient with brachydactyly and racket-nails affecting all fingers of both hands. The occurrence of similar clinical features in the family of the patient is suggestive of a hereditary disorder. The light and electron microscopical findings are described.
• 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.
(Arch Dermatol 1982;118:273-274)
• Two patients are described in whom malignant transformation occurred within an eccrine spiradenoma. Their findings were compared with those of six similar patients from the literature, and a characteristic clinicopathologic picture was evident. The typical history was one of rapid enlargement of a cutaneous nodule of long standing. The neoplasms were composed, at least in part, of large, markedly atypical basaloid cells with numerous mitotic figures. Adjacent benign spiradenoma was observed microscopically in each case, and some of the carcinomas retained architectural features reminiscent of spiradenoma. One patient died of disseminated tumor, but follow-up was short or lacking in several cases. Malignant transformation of eccrine spiradenoma shares many clinical and pathologic features with malignant transformation of dermal cylindroma.
(Arch Dermatol 1985;121:1445-1448)
A case of locally invasive cutaneous cylindroma is reported This neglected scalp tumor 6 5 cm in greatest dimension, initially invaded the calvana at the time of the first operation. A recurrence infiltrated the dura and presented as an intracranial space occupying lesion. No brain invasion was present and distant metastases were not documented. The patient remains well 2 1/2 years after tumor resection. Light and electron microscopic appearance of the recurrent tumor revealed distinct morphologic features, which may be of use in predicting the biologic behavior of cutaneous cylindromas. This case documents the low biologic aggressiveness of cylindroma and stresses the necessity of complete surgical excision of neoplasms with atypical morphologic features
Sweat glands from normal skin obtained at autopsy or as routine biopsies were examined using a panel of immunoperoxidase-linked antibodies. The results indicate that such a panel of antibodies defines all known functional regions of the eccrine sweat gland and provides a reliable distinction from all other skin elements.
The morphological and immunohistochemical characteristics of 37 atypical fibroxanthomas of the skin were examined. Twenty-four tumours were nodular ulcerative lesions on the head and face of patients with a median age of 75 years, whereas 13 tumours occurred on the trunk and limbs of patients with a median age of 48 years. Both pleomorphic polygonal and giant cells as well as the spindle cell component of the tumours stained for the histiocytic markers alpha 1-antichymotrypsin, alpha 1-antitrypsin, lysozyme and, less frequently, for ferritin. Leu M1 antigen and peanut agglutinin receptors were not demonstrable in tumour cells. This antigenic profile was contrasted with the findings in six cases of dermatofibroma which were largely not reactive with the antisera used. The immunohistochemical findings in atypical fibroxanthomas suggest that they represent a homogeneous group of tumours which are related to tissue histiocytes. These results are discussed in the context of the published findings in other so-called fibrohistiocytic tumours including dermatofibrosarcoma protuberans and malignant fibrous histiocytoma. The diagnoses in three cases coded as atypical fibroxanthomas were revised on the basis of their showing a different immunohistochemical profile.
Malignant degeneration of dermal eccrine cylindromas is extremely rare. Only six cases are on record. Here an additional case is described in a 96-yr-old woman.
Three consecutive generations of a family have had members affected with trichoepitheliomas and milia. One member, the proband, also had cylindromas. The pattern of inheritance was a dominant one, with only the male members affected. Histopathologically, the trichoepitheliomas and milia contained keratinizing cysts with laminated centers, peripheral basaloid cells, and a thin granular layer. Fibroblastic stroma and fronding of basaloid cells were also seen in both tumors. The trichoepitheliomas and milia differed mainly in the greater proportion of keratinized cells found in the milia. Clinically, the milia were found only in areas of vellus hairs.
A basal cell adenoma of parotid, eccrine dermal cylindromas and trichoepitheliomas occurring in the same patient were examined by light and electron microscopy and histochemistry. The eccrine and parotid adenomas were similar both structurally and histochemically except for the presence of Langerhans cells in the cutaneous adenoma and well differentiated mucinous cells in the parotid tumor. The three different hamartomas found in this individual may represent the effect of a single pleiotropic gene acting on ontogenetically related stem cells.
Two patients had carcinoma arising in eccrine spiradenoma. In both patients, recent changes in a long-standing cutaneous nodule prompted excision, and microscopic examination disclosed carcinoma intimately associated with and apparently arising from typical benign eccrine spiradenoma. One patient had lymph node metastasis.
Skin disorders are readily accessible to inspection, palpation, and biopsy. In many instances, the physician's discovery of a neoplasm leads to excision of part or all of the lesion. By combining the clinical presentation and histopathologic appearance, most of these disorders can be diagnosed with accuracy. In some instances, however, it may be difficult to differentiate one neoplastic process from another with routine stains. Traditionally, the histologic diagnosis of cutaneous neoplasms has been facilitated by application of special histochemical stains and electron microscopy. Despite the use of these techniques, some neoplasms in the skin defy definitive diagnosis. With the availability of antibodies against certain characteristic cellular antigens, the immunoperoxidase technique has proven to be a useful tool in the diagnosis of a number of infectious, autoimmune, and neoplastic skin diseases. In this review, I will examine the application of the immunoperoxidase technique to the histogenetic assessment of a number of dermatologic neoplasms, its practical benefits, and its limitations in the differential diagnosis of some commonly encountered neoplasms of the skin.
In an attempt to characterize the immunocytochemical attributes of eccrine sweat gland carcinoma, we studied 32 examples of this tumor with antibodies to epithelial membrane antigen (EMA), cytokeratin (CK), carcinoembryonic antigen, S100 protein, alpha-lactalbumin, salivary amylase, blood group isoantigens, beta-2-microglobulin, and Leu M1. All cases expressed EMA and CK, and 28 of 32 cases also displayed at least 2 of the 6 remaining antigens. No significant variations were noted in the immunophenotypes of histologic subtypes of eccrine carcinoma. These results provide an objective means of diagnostic separation between sweat gland carcinoma and other primary malignant cutaneous tumors. However, they do not appear to correlate with the degree of tumoral differentiation, and are of no assistance in the separation of benign and malignant sudoriferous neoplasms. The ability of immunocytochemical techniques to distinguish between primary malignant adnexal cutaneous tumors and metastases to the skin appears unlikely, but remains to be studied further. Also, the use of immunostaining panels is advised in the study of adnexal carcinomas, since no single determinant in isolation is specific for these neoplasms.
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.
We herein report two cases of sweat gland carcinoma that arose in association with eccrine spiradenoma. These lesions presented as enlarging masses that previously had been stable for many years. One produced widespread metastasis and death 5 months after diagnosis. Immunohistochemical studies demonstrated an antigenic relationship between the benign and malignant components of sweat gland carcinoma ex eccrine spiradenoma, but ultrastructural analyses showed a paucity of specialized differentiation. This neoplasm appears to display a range of microscopic appearances and has proven fatal in 20% of reported cases.
A case of locally invasive cutaneous cylindroma is reported. This neglected scalp tumor, 6.5 cm in greatest dimension, initially invaded the calvaria at the time of the first operation. A recurrence infiltrated the dura and presented as an intracranial space-occupying lesion. No brain invasion was present and distant metastases were not documented. The patient remains well 2 1/2 years after tumor resection. Light and electron microscopic appearance of the recurrent tumor revealed distinct morphologic features, which may be of use in predicting the biologic behavior of cutaneous cylindromas. This case documents the low biologic aggressiveness of cylindroma and stresses the necessity of complete surgical excision of neoplasms with atypical morphologic features.
Two patients are described in whom malignant transformation occurred within an eccrine spiradenoma. Their findings were compared with those of six similar patients from the literature, and a characteristic clinicopathologic picture was evident. The typical history was one of rapid enlargement of a cutaneous nodule of long standing. The neoplasms were composed, at least in part, of large, markedly atypical basaloid cells with numerous mitotic figures. Adjacent benign spiradenoma was observed microscopically in each case, and some of the carcinomas retained architectural features reminiscent of spiradenoma. One patient died of disseminated tumor, but follow-up was short or lacking in several cases. Malignant transformation of eccrine spiradenoma shares many clinical and pathologic features with malignant transformation of dermal cylindroma.
Dermal-type cylindroma (turban tumour, Spiegler tumour) is a tumour characterized by the occurrence of firm, pink-to-red skin nodules. The nodules vary from 2 to 60 mm. in diameter and show the histological features of a dermal eccrine cylindroma (Montgomery, 1967). The disease generally affects the scalp which may become completely covered with tumour masses, forming the so-called turban. Tumours also frequently occur in the face, neck and shoulder regions, but almost any skin surface may be affected.
Malignant transformation occurred in pre-existing sweat gland tumors in 7 patients. Three lesions showed an histologic pattern of eccrine spiradenoma, 2 eccrine poroma, one cylindroma and one papillary eccrine adenoma. Malignant transformation was histologically characterized by the presence of solid tumor areas populated with large cells having irregularly shaped nuclei and mitotic figures. There were multiple foci of squamous metaplasia, areas of loss of basement membrane and invasion of the surrounding connective tissue.
Sweat glands from normal skin obtained at autopsy or as routine biopsies were examined using a panel of immunoperoxidase-linked antibodies. The results indicate that such a panel of antibodies defines all known functional regions of the eccrine sweat gland and provides a reliable distinction from all other skin elements.
We applied a peroxidase-antiperoxidase technique for S100 protein to 73 tumors of skin and skin adnexa. These included 15 eccrine tumors, 11 apocrine tumors, 18 tumors with differentiation toward hair, two sebaceous adenomas, one mixed tumor of the scalp, ten dermatofibromas, ten basal cell carcinomas, five squamous cell carcinomas, and one clear cell acanthoma. Consistent results were obtained. Occasional cells in eccrine tumors showed strong positive staining, as did the Langerhans' cells in the squamous cell carcinomas and the clear cell acanthoma. The cells of the apocrine tumors showed moderate to weak staining, and the tumors with differentiation toward hair, the sebaceous adenomas, and the mixed tumor of the scalp showed uniform negative staining, as did basal cell carcinomas and dermatofibromas.
Using immunoperoxidase methods, normal sweat glands, 44 benign and 4 malignant sweat gland tumors were tested for the presence of carcinoembryonic antigen (CEA), pregnancy-specific-B1-glycoprotein (SP1) and actin (ACT). CEA and SP1 stained the secretory and duct-lining cells of normal eccrine glands. Among benign tumors, 74% were positive for CEA and 44% for SP1. The staining reaction was found mainly in luminal secretions and surrounding cells. Staining by SP1 was reduced, but not suppressed, after absorption with the purified antigen. ACT was found in myoepithelial cells of the secretory tract of normal glands and in basal cells of all cases of hidradenoma papilliferum. Only 3 sweat gland carcinomas reacted for CEA. In a malignant chondroid syringoma, no ACT-positive cells were seen in the myxochondroid stroma. The potential value of CEA, SP1 and ACT in the diagnosis of sweat gland tumors is discussed.
A series of 10 sweat-gland carcinomas is reported. The ages of the patients at presentation ranged from 12 to 79 years. Three of the tumours had shown long latent periods with a mass present for up to 20 years. Prognosis proved to be related to the histological subtype. Thus, two papillary syringocystadeno-carcinomas, one recurring cylindroma, one hybrid cylindroma/adenoid cystic carcinoma and two mucoid carcinomas were apparently all cured by surgery. Two mucoepidermoid carcinomas and one histologically malignant chondroid syringoma were not only histologically, but also clinically, malignant. The last case, a chondroid syringoma of benign histology, proved nevertheless to be malignant clinically.
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.
A clinical and pathologic study of 49 eccrine spiradenomas occurring in 46 patients is presented. The study showed that pain and/or tenderness are not such characteristic clinical features of this tumor as has previously been suggested; either or both were present in only 23% of the 35 patients with well-documented clinical histories. Some pathologic features, not previously documented in this tumor, were seen and included: cylindromatous foci, aggregates of stromal clear cells, clear cells lining ducts and ulceration of the overlying epidermis. Two tumors had undergone malignant transformation, but there were no recurrences in the 35 patients with adequate follow-up.
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.
Fifty benign sweat gland tumours were studied for the expression of carcinoembryonic antigen (CEA) and apocrine epithelial antigen (AEA), using immunohistochemical methods. CEA was found in thirty-two and AEA in thirty-three neoplasms. Both antigens were located in the epithelium of the luminal structures and in the intraluminar material and CEA was occasionally found also in proliferating cells. Co-expression of CEA and AEA occurred frequently in cases of syringoma, syringocystadenoma papilliferum, hidradenoma papilliferum, eccrine spiradenoma and clear cell hidradenoma. AEA was seen also in tumours showing eccrine differentiation, even though it is not present in normal eccrine sweat ducts.
There is still some debate about which tumors should be included under the terms eccrine and apocrine. Using histochemistry and electron microscopy, it is possible to identify varying degrees of differentiation towards normal apocrine or eccrine structures. Some would argue that this does not establish certain origin from these structures, as reserve cells or pluripotential cells could give rise to tumors with intermediate features, or characteristics different from those of the gland in which they were resident. These problems are not yet completely resolved, although the development of monoclonal antibodies directed against eccrine or apocrine antigens may help elucidate this matter. The following classification of eccrine tumors is proposed. The emphasis will be placed on recent papers, and those aspects of morphology discussed in standard textbooks will be dealt with only briefly.
The Journal of Investigative Dermatology publishes basic and clinical research in cutaneous biology and skin disease.
Dermal eccrine cylindroma, epithelioma adenoides cysticum of Brooke and eccrine spiradenoma
- Gottschalk