LIFE HISTORY OF ORGANOID NEVI. SPECIAL REFERENCE TO NEVUS SEBACEUS OF JADASSOHN.
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Malignant fibrous xanthoma is an uncommon tumor of histiocytic origin. It is a difficult tumor to diagnose, and may have an unpredictable and confusing clinical course. The malignant fibrous xanthoma described herein probably represents the first reported case of metastasis to the cerebellopontine angle. When properly diagnosed, the preferred treatment is wide excision. Metastases are usually to the lungs and regional lymph nodes, and portend a poor prognosis. Radiotherapy appears to be of little value.
While the majority of patients with basal cell carcinoma are cured by the initial therapeutic approach, high risk patients who have had recurrences of basal cell carcinoma have been identified. From April 1976 to November 1979, 811 patients with 1,271 cutaneous carcinomas were seen at the Mohs' Histographic Surgery Unit of the Cleveland Clinic Foundation. Of these, 496 represented recurrent basal cell carcinomas. Histologic subtype, location of the tumor, and age of the patient were studied in an attempt to identify factors which place patients at risk for persistent or recurrent carcinoma. The physician who first sees a patient with basal cell carcinoma should look for these risk factors and, after identifying such patients, consider an aggressive management with frequent follow-up.
Although sun exposure is believed to be associated causally with cutaneous melanoma, the high incidence on less sun-exposed areas such as the back, as well as on chronically exposed sites such as the face, suggests that the association with sunlight is less straightforward than for other skin cancers. To explain this enigmatic site distribution, a theory of site-dependent susceptibility of melanocytes to malignant transformation is proposed. As possible evidence, all melanomas diagnosed in the state of Queensland, Australia, over a one-year period were surveyed for histologic evidence of benign melanocytic nevus cells adjacent to the melanoma, and analyzed according to anatomic distribution. Results showed a regional variation in the proportion of melanomas with adjacent nevi not explicable by regional variation in nevus density, which suggests that there is a varying susceptibility of nevi to malignant change. Given that nevus cells are equivalent to melanocytes, this finding would support the hypothesis that melanocytes at-large have a differential response to the mitogenic stimulus of sunlight according to anatomic site.
We report on the case of a 70-year-old caucasian woman with a large linear sebaceous naevus with multifocal intralesional basaliomas and a lipodermoid of the left eye. This rare finding has been preserved because of the special history of the patient. The naevus spread on the left side from parietal to retroauricular, nuchal and sternal. Because of the patient's cardio-pulmonary insufficiency we excised the basaliomas under local anaesthesia. We also made a partial stripped excision of the left-sided parietal parts of the naevus.
PURPOSE: To describe three Hispanic patients with oculodermal melanocytosis and uveal melanoma. METHOD: Case series. RESULTS: Three Hispanic patients with oculodermal melanocytosis and uveal melanoma underwent enucleation. The diagnosis of choroidal melanoma was confirmed by histopathologic examination. CONCLUSION: In the Hispanic population, uveal melanoma can occur in the presence of oculodermal melanocytosis.
PURPOSE: The authors document the association of peripapillary staphyloma and an atypical variant of hemimegalencephaly with the linear nevus sebaceous syndrome. BACKGROUND: Linear nevus sebaceous syndrome is an uncommon neurocutaneous disorder that has a propensity to involve the eyes. METHODS: Clinical, histopathologic, and neuroimaging findings are examined in a child with linear nevus sebaceous syndrome. RESULTS: In addition to bilateral peripapillary staphylomas, ophthalmologic abnormalities included a corneal dermoid, a complex conjunctival choristoma, macular hypoplasia, and optic nerve hypoplasia with contralateral optic atrophy. Magnetic resonance imaging disclosed a rare form of hemimegalencephaly characterized by hypoplasia of an optic radiation within the enlarged, dysgenetic cerebral hemisphere. CONCLUSION: The spectrum of oculocerebral dysgenesis in the linear nevus sebaceous syndrome can be expanded to include peripapillary staphyloma and atypical hemimegalencephaly with hypoplasia of an optic radiation.
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Inactivation of wild-type p53 tumor suppressor function is the primary mechanism of tumor initiation in Li-Fraumeni syndrome (LFS) individuals with germline p53 mutations. Tumors derived from LFS patients frequently retain the normal p53 allele, suggesting that alternative mechanisms in addition to gene deletion must be involved in inactivating wild-type p53 protein. DNA tumor viruses, such as SV40, target p53 for inactivation through the action of viral oncoproteins. We studied the probands from two unrelated LFS families, each of whom presented with multiple malignant neoplasms. Patient 1 developed an embryonal rhabdomyosarcoma (RMS) and a choroid plexus carcinoma (CPC), while patient 2 developed a CPC and subsequently presented with both an osteosarcoma (OS) and renal cell carcinoma (RCC). We utilized DNA sequence analysis and immunohistochemistry to determine p53 gene status in the germline and tumors, as well as evidence for SV40 T-antigen oncoprotein expression. Each patient harbored a heterozygous germline p53 mutation at codons 175 and 273, respectively. In patient 1, the normal p53 gene was lost while the mutant p53 allele was reduced to homozygosity in the RMS. Both normal and mutant genes were maintained in the CPC. In patient 2, normal and mutant p53 alleles were retained in both the CPC and RCC. Both specific PCR and immunostaining detected SV40 T-antigen in both CPCs and the RCC. In addition to chromosomal alterations, epigenetic mechanisms may disrupt p53 function during tumorigenesis. In two LFS patients, we found SV40 DNA sequences and viral T-antigen expression that could account for inactivation of the normal p53 protein. Inactivation of p53 or other tumor suppressors by viral proteins may contribute to tumor formation in specific tissues of genetically susceptible individuals.
BACKGROUND: Cutaneous cylindromas are benign adnexal tumors that may occur as a solitary lesion or in a multiple familiar pattern. Malignant change is rare. The association of multiple trichoepitheliomas and cylindromas, the so called Brooke-Spiegler Syndrome, is supposed to be inherited in an autosomal dominant manner. PATIENTS: A 55 year old woman presented with multiple skin tumors on the scalp, as well as the nasolabial and periauricular area since the age of 20 years. Her daughter and her nephew were also affected. Tumors were surgically removed for cosmetic reasons and showed histological signs of benign cylindromas and trichoepitheliomas. 35 years after diagnosis patient presented with a large exulceration on the right occipital area with intracranial invasion. Staging revealed multiple metastases in both lungs. Patient died 1 month later. RESULTS: Histological examination showed beside zones of massive necrosis multiple mitosis. The jigsaw puzzle pattern and the thick PAS positive basal layer, the striking characteristics of benign cylindromas, were lost. These histological findings and the clinical presentation led to the diagnosis of cylindrocarcinoma. CONCLUSIONS: Cylindrocarcinoma is an aggressive tumor with tendency to a local destructive growth and metastases. As malignant transformation of a benign cylindroma occurs more often in the multiple form a close follow-up of patients with multiple cylindromas is necessary. A family study is indicated.
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A 56-year-old man had approximately forty apocrine cystadenomas of the face and ears. Clinical examination and light microscopic findings were typical for apocrine cystadenomas. Electron microscopic findings showed an absence of annulate lamellae, thus differing from the only previously reported study. Review of the literature indicates that a large number of apocrine cystadenomas, such as on our patient, has not been reported previously.
At the University of Michigan the dermatologic surgeon works closely with the head and neck surgeon in resecting extensive cutaneous malignancies that could benefit from the combined skills of both surgical specialists. Mohs surgery offers complete microscopic controlled resection of the cutaneous portion of skin cancers. Tumors extending deeply from the skin into underlying bone and soft tissue are resected with the assistance of the head and neck surgeon familiar with the anatomy and trained in the protection of the vital structures of the head and neck. It is evident that patients with large or aggressive basal cell carcinomas will best be served when this interdisciplinary approach has become commonplace.
BACKGROUND: The accepted method for securing full thickness skin grafts (FTSG) is with a tie-over bolster dressing, with or without basting sutures. We question the need for tie-over bolster dressings for small FTSGs. OBJECTIVE: We describe our method of FTSG reconstruction and the outcome in a consecutive series. METHODS: Thirty patients with surgical defects following tumour excision from the face24, scalp1, ear2, and finger3 ranging in diameter from 8 to 45 mm (mean 20 mm) were reconstructed with a FTSG. Interrupted monofilament nylon perimeter sutures only were used, with antibiotic ointment at the wound edge with either a light dressing or no dressing. RESULTS: A series of 30 FTSG secured without a tie-over bolster dressing or basting sutures is described, all with good to excellent long term results. In two cases there was early superficial necrosis of the graft, but ultimately 100% graft take. CONCLUSION: We have found tie-over bolster dressings and basting sutures to be unnecessary in our series of small FTSG. This technique saves time and material and minimizes handling of the graft.