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PubMed1977

[Familial epithelioma of Ferguson-Smith about two familial cases].

Two cases of multiple self-healing epithelioma (Ferguson-Smith) are reported in a French family. This dermatosis has been present since the age of 30 in both father and son. The father aged 66 years, farmer, had suffered since 1939 from recurrent spontaneously healing tumors of the face and neck, with deeply pitted scars on the face. He was treated during fifteen years in a cancer institute for multiple well-differentiated " squamous cell carcinoma " on the neck, face, and tongue; local recurrence did not occur after treatment of the active tumors and there was no clinical evidence of secondary lymphnode involvement. His son, aged 30, teacher, came in July with a papular active tumour on the cheek, which was surgically removed; his face and ears soon became affected. In the two cases, numerous biopsies showed in the early stage a great down growth of the epidermis with a central horny plug, like in keratotic papules or small kerato-acan-thomas and in more active lesions, typical appearances of squamous cell epithelioma grade I. The familial incidence is proved in the majority of the 30 cases reported since the first description by Ferguson-Smith in 1934. Only caucasians are concerned, most of them being Scottish. There is no sex predominance. First changes generally occur in young adults on light exposed areas but mucous membranes can be affected. The individual lesions last for several months, but fresh ones appear continuously, so that the disease remains extensive. The cause of these tumors is still unknown; histological and ultrastructural findings cannot explain this condition. Our ultrastructural study is parallel to previous works and fails to find viral particles in keratinocyte nuclei or cytoplasma.

Carcinoma, Squamous Cell↗
PubMed1996

Frequency of skin cancer and other ultraviolet radiation provoked skin lesions of eyelid and periocular region in the 1981-1992 period.

It is well known that the region of eyelids and periocular skin is exposed to ultraviolet radiation (UV rays), so it is the location of skin changes associated with the effects of this radiation. The analysis of basal cell carcinomas, keratoses, keratoacanthomas and cornu cutaneum of this regions covered a 12-year period. In the total of 1,398 skin biopsies from the Department of Ophthalmology, Sestre Milosrdnice University Hospital from Zagreb, the above mentioned lesions were found in 498 cases. Most frequent were basal cell carcinomas (BCC), found in 377 cases, followed by squamous cell carcinoma (SCC) in 64, keratoses in 37, cornu cutaneum in 8 and keratoacanthoma in 12 cases. As BCC was the most frequent finding in the bioptic material from this clinic, special emphasis was put on this tumor, its age and sex distribution, and localization. In the discussion and material analysis, particular reference is given to the causative relation with the effects of UV radiation. As a special contribution to the discussion on the connection of these changes with UV radiation, 3 cases with the occurrence of BCC and seborrheic keratoses in the same lesion and a patient with senile elastosis and BCC in the same segment of the skin of an eyelid are described.

Adolescent↗
PubMed1996

The surgical management of melanoma: from diagnosis to local treatment.

It is critical for physicians who may play a role in the diagnosis of melanoma to be knowledgeable of how to approach a suspicious nevus. A full thickness of tissue is necessary to microstage the melanoma and thereby guide subsequent treatment decisions. The past two decades have witnessed a dramatic change in the surgical management of this disease in that the margin of excision seems to be constantly shrinking. Well-conducted randomized studies have shown conclusively that narrower margins are sufficient for thin and intermediate thickness melanomas. Thus, the survival is comparable with that achieved for more radical excisions with less functional and cosmetic deficit.

Biopsy↗
PubMedDate not supplied

[A system for the clinico-functional staging of the quality of life of patients with malignant neoplasms of the oro-facial area].

The anatomical region identified with the "head and neck region" has got the highest functional concentration of the human body and the highest incidence of malignant tumours per unit of surface. A pathology, that forced to serious and destroying surgical interventions in this area, will carry necessarily out a series of mutilations and functional restrictions on the whole organism. Leawing out the central nervous system, the head is site of important functions like breathing and feeding, in addition to essential functions for the relationship life like vocal expression and sight. For a correct interpretation of the quality of life in patients with neoplastic lesions in this anatomical area, it is very important to assess what kind of function has been more or less compromised and therefore what kind of limitation interfere between patient and the surrounding environment and between patient and himself. In order to provide a useful instrument for the measurement of the therapeutical acts consequences and in order to quantify the qualitative aspects of oncological patient's residual life, has been elaborated a test of "functional evaluation of the oro-facial district". This system, according to the modern oncology requests that value the dificoltous but important concept of "life quality", allows the interpretation of elements otherwise dispersive because of the various and different physiological functions localized in the considered anatomical district. The examined physiological functions have been subdivided in vital, primary or secondary, and through the use of an objective evaluation score card, they have allowed to classify the patients with orofacial oncological pathology in definite and listed functional stages.

Adult↗
PubMed1997

Fractional cryosurgery. A new technique for basal cell carcinoma of the eyelids and periorbital area.

BACKGROUND: Cryosurgery is an established method to treat malignant tumors of the eyelids and periorbital area. Nevertheless, it has been abandoned for tumors greater than 10 mm, because it gives irregular esthetic results and, in some cases, lagophthalmos. OBJECTIVE: To devise a new method for the treatment of such tumors. METHOD: Fractional cryosurgery is performed in stages: the center of the lesion is frozen, resulting in a reduction of the tumor; this procedure is repeated, as necessary, until the lesion's diameter is smaller than 10 mm; the standard cryosurgical procedure is then carried out. RESULTS: The treatment of the first 20 basal cell carcinomas with diameters between 10 and 24 mm is described, with excellent clinical and cosmetic results. CONCLUSION: With fractional cryosurgery, the final scar bears no relation to the size of the original tumor but, instead, corresponds to the size of the lesion preceding the final cryosurgical procedure.

Aged↗
PubMed1997

[Neurocutaneous syndromes with vascular alterations].

There are several syndromes in which neurological and cutaneous alterations of vascular origin, among other symptoms, occur. The key point of this fact is that these cutaneous signs permit early diagnosis, thus helping in further recognition of more complex syndromes and preventing unnecessary, harmful and costly diagnostic procedures or having to wait until the appearance of neurological signs. Therefore, these diseases should be classified attending to the most notorious vascular lesions they show, though they may show other less frequent cutaneous vascular lesions. In this way, these syndromes can be classified as associated with nevus flammeus (Sturge-Weber, Shapiro-Shulman, Bonnet-Dechaume-Blanc, Cobb, Klippel-Trenaunay, Fegeler, Robert), cavernous hemangiomas (Maffucci, blue-rubber-bleb-nevus, Proteus, Bannayan-Zonana, Riley-Smith, familial cavernous angiomatosis, POEMS syndrome), capillary hemangiomas (Rubinstein-Tayabi, Coffin-Siris, PHACE syndrome), telangiectasia (congenital telangiectatic cutis marmorata, Rendu-Osler-Weber, ataxia telangiectasia, Cockayne, De Sanctis-Cacchione), livedo reticularis (Sneddon, Divry-van-Bogaert), angioqueratoma (Fabry disease, Fucosidosis) and hemangioblastoma (Von Hippel-Lindau). Though we have tried that these vascular lesions should be named as angiomas if they are malformations and hemangiomas if they are benign neoplasias, they are called following morphological aspects rather than other criteria, due to their unknown origin.

Brain Neoplasms↗
PubMed1997

[Facial pain].

Whether acute, chronic or recurrent, facial pain remains an therapeutic challenge. Neurological tests, otorhinolaryngologic, dental or psychiatric examinations do not always provide a precise diagnosis. We propose a review of painful diseases most often found in the head and neck region. A differential diagnosis between psychiatric and functional diseases is proposed although the subjective component is always present. Neuralgias, migraine, cluster headache, tension-type headache, atypical facial pain and cancer pain are reviewed.

Acute Disease↗
PubMed1977

[Cryotherapy in basalioma].

The biological basis of cryotherapy of tumours is explained. The enveloped cryosurgical instruments in Dresden were presented. Oculists and dermatologist have treated 200 basal cell epitheliomas by cryotherapy. Good functional and cosmetical results were seen. The time of control completes up to 5 years.

Basal Cell Carcinoma↗
PubMed1997

Therapeutic efficacy of interferon alfa-2b in infants with life-threatening giant hemangiomas.

BACKGROUND: Because of their size and the possibility of complications, giant hemangiomas represent a therapeutic challenge. Various forms of treatment have been used, with variable results, including surgery, embolization, lasers, pentoxifylline, and corticosteroids. Interferon alfa has been used successfully to treat life-threatening hemangiomas, possibly by means of its antiangiogenic activity. OBSERVATIONS: We treated 7 infants with organ-interfering and/or life-threatening giant hemangiomas with subcutaneous injections of 3 million U/m2 per day of interferon alfa-2b during the first month and subsequently every 48 to 72 hours, depending on the evolution in each case. The treatment lasted from 3 to 12 months. In 2 patients, interferon alfa-2b was administered while prednisone therapy was being tapered. In all 7 patients, there was considerable reduction of the volume of the hemangiomas and remission of their complications. All patients presented with fever, neutropenia, and an increase in serum aminotransferase levels. The patients who received interferon alfa-2b and prednisone seemed to improve at a faster rate. CONCLUSIONS: Interferon alfa-2b is a good option for the treatment of patients with steroid-resistant, organ-interfering and/or life-threatening giant hemangiomas. In our experience, the adverse effects were transient and minor and did not require the interruption of the treatment.

Antineoplastic Agents↗
PubMed1998

[Bilateral aggressive basal cell carcinoma of the eyelids and face--case report].

In this paper we present a case of sclerosing basal cell carcinomas affecting both sides of periocular regions in a 68-years old woman. Neoplastic tissues were surgically removed and the extend of resection was controlled microscopically during the operation. After fixing the remaining palpebral structures to ocular muscles and bones the split-thickness skin grafts were placed at the defects. We planned to perform the second step reconstructive procedures of the eyelids, but we resigned because of unexpectedly good functional nad esthetic results. We present this case because the cancers were long-term, multiple and of a great advancement, the treatment was difficult and the results were very good.

Aged↗
PubMed1998

[Sinusoidal hemangioma].

INTRODUCTION: Sinusoidal hemangioma (SH) was described by two pathologists, Calonje and Fletcher, in 1991. This uncommon cutaneous benign vascular lesions with distinctive histologic features was considered a subset of lesions known as "cavernous hemangiomas". It is made of dilated interconnecting ("sinusoidal"), thin-walled channels with a single-layered lining endothelium and a lobular architecture. PATIENTS AND METHODS: Clinical, radiological (CT, MRI and arteriography), evolutive, and histologic data of 4 patients, whose lesions appeared very similar clinically and histologically, were reviewed and compared to the patterns of the so-called SH. RESULTS: There were 2 males and 2 females. Lesions had an infantile onset in 3 (congenital in 2). They exhibited a protracted course over years in the 4, and they all invaded the right forehead, eyelids and orbit areas. Clinically, they appeared as multilobulated, building, firm tumors under a normal shiny skin. Radiologically, lesions were strikingly nodular, with criteria of slow-flow vascular lesions on MRI and arteriograms. Histologically, all lesions were comprised of well-circumscribed nodules with a lobulated architecture, made of greatly dilated, blood-filled, thin-walled vascular channels, with scanty fibrous stroma between them. Interconnecting channels had the distinctive sinusoidal pattern. There were some elongated pseudopapillary structures. Islands of spindle cells were noted in 2 lesions. DISCUSSION: On the one hand histologic features of the lesions in our 4 patients are identical to those described by Calonje and Fletcher as SH, a lesion not to be confused with classic, involuting-type, hemangioma of infancy and childhood, and with venous or lymphatic malformations of the same location. SH fits into the group of lesions defined by pathologists as "cavenous hemangiomas". On the other hand the lesions in our 4 patients differed from the clinical description of SH as a solitary acquired small (less than 3 cm) nodule in adults. Otherwise the four lesions described herein constitute a distinctive clinicopathologic entity with a striking aggressive local growth although there is a histologic malformative pattern. The presence in 2 cases of islands of spindle cells led us to discuss the differences with spindle cell hemangioendothelioma.

Adolescent↗
PubMed1976

[Sebaceous hyperplasias, kerato-acanthomas, epitheliomas of the face and cancer of the colon. A new entity?].

Numerous skin lesions: sebaceous hyperplasias, benign acanthoma, keratoacanthomas and squamous cell epitheliomas appeared on the face, especially on the nose, on the cheeks and around the lips of a 45-year-old obese man with mild diabetes. In 1969, an adenocarcinoma had been found in the rectosigmoid and another one in the colon. In 1974, an excision of a well-differenciated epidermoid carcinoma was performed in the right external auditory meatus. A rectoscopy showed a micropolyp at the rectosigmoid junction. In 1975, an abdominal exploration was performed following a sudden relapse of about 50 skin lesions in the face, partly sebaceous adenomas, partly sebaceous gland epitheliomas, and about 10 precancerous lesions on the dorsum of the hands. A villous adenoma of the colon was removed. Inheritance appears to the autosomal dominant with variability in the expressiveness of the cutaneo-intestinal symptoms.

Adenocarcinoma↗
PubMed1987

Primary neoplasms of the facial nerve.

A series of 30 primary facial nerve tumors is reviewed. Most of them were benign (n = 26); there were four malignant tumors. Neoplasms originating within the temporal bone were found to have preoperative facial paralysis in 84 percent of cases; the extracranial tumors had a 35 percent incidence of preoperative facial paralysis. All tumors in this series were treated surgically--by means of a middle fossa or transmastoid approach for the intratemporal group of tumors; the extracranial tumors were removed by the technique of parotid tumor surgery with complete facial nerve dissection. All the patients with preoperative facial weakness required facial nerve transection. Facial paralysis was rehabilitated with nerve grafts, hypoglossal crossover, or muscle transfers. Because "normal" facial expression is still not attainable following repair of complete facial nerve transection, an early diagnosis, hopefully prior to total neurotmesis, is essential. All patients with unexplained facial weakness, especially that which is progressive and persistent, should have the entire course of the facial nerve investigated for the possibility of treatable etiology.

Cranial Nerve Neoplasms↗