Epidermoid carcinoma of the skin involving the parotid gland.
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Basal cell carcinoma is the most common human malignancy. This locally invasive tumor rarely metastasizes, but can cause considerable morbidity when on the eyelids and periocular skin. The successful management of BCC requires an awareness of its broad clinical spectrum and knowledge of the risk factors associated with therapeutic failure. The optimal therapy for basal cell carcinoma is controversial. Treatment strategy is formulated on an individual basis, taking into account tumor size, anatomic location, and growth pattern. The immune status and life expectancy of the patient also must be considered. While complete eradication of the tumor is desirable, this can be challenging since basal cell carcinomas often extend beyond their apparent clinical margins. Since prevention is preferable to treatment, the public needs to be informed that the incidence of periocular skin cancers can be reduced with the longterm use of sunscreens, sunglasses, and hats with brims.
In treating periocular basal cell carcinoma the goal is to achieve the most complete eradication of affected tissue while preserving as much healthy tissue as possible. In the first of these "Viewpoints" articles, Drs. Leshin and Yeatts discuss the success of Mohs' microsurgical technique (MMS) in both regards. Moreover, they note that MMS offers the additional advantage of placing all surgical and histological phases of tumor excision in the hands of one specialist, thus circumventing problems that may arise with procedures requiring the separate involvement of several specialists. In the second article, Drs. Anscher and Montana point out that modern radiotherapy techniques may offer adequate tumor control with less damage to surrounding tissues than is caused by surgical excision and reconstruction. With small lesions, radiotherapy has a success rate similar to that of surgery. With large lesions, the success rate is lower; however, if surgical excision would cause extensive disfigurement or functional damage, an initial trial of radiotherapy may be warranted, then followed by surgery, if necessary. In an editorial, Dr. Dutton summarizes the indications for and advantages and disadvantages of both techniques.
A case of angiosarcoma of the right corpus cavernosum with direct extension to the bladder, prostate, and seminal vesicles and metastasis to brain, liver, and cheek is presented, and the literature is reviewed.
Radiation damages endothelial cells (EC) of malignant and normal tissues. Shortly after radiation injury platelet thrombi cause small vessel obstruction. Radiation doses of 20 Gy or higher release von Willebrand factor (VWF) from cultured human umbilical vein EC. Angiosarcoma (AS) is a vascular tumor with abnormal appearing EC which have detectable cytoplasmic VWF. We examined the effect of radiation therapy on VWF in three patients with AS. Each patient's malignant tissue was demonstrated to have cytoplasmic VWF present. Venous blood samples were drawn immediately before and at weekly intervals during treatment. Patient 1 received 80 Gy over 6 weeks and had no alterations in the VWF. Patient 2 received 64 Gy over 6 weeks and was noted to have loss of the high molecular weight multimers of VWF without loss of FVIII:C, VW: RIST, or von Willebrand antigen activity (VW:Ag). Patient 3 was treated with 64.4 Gy over 9 weeks and had a disproportionate increase of the VW: Ag and VW: RIST to FVIII: C. This returned to normal after completion of therapy. These changes were minimal and might be explained by either limited local release or no increased release of VWF from the irradiated tissue. The minimally abnormal multimeric pattern in patient 2 may be due to the release of an abnormal VWF in normal amounts or small amounts of proteolysis. The alteration in the VW: RIST and VW: Ag to FVIII: C ratio can be explained by activated coagulation secondary to radiation injury.
A 43-year-old man with xeroderma pigmentosum, XP97TO, was allocated to complementation group D. He had had moderate photosensitivity at age 1 year and freckles by age 6 but no neurologic abnormalities. Nevertheless, his fibroblasts in culture had the XP-D phenotype. They showed a sevenfold hypersensitivity to killing by 254 nm ultraviolet radiation and a diminished level (29%) of unscheduled DNA synthesis. Phototesting revealed delayed maximum erythema at 72 hours after UVB exposure and a lowered minimal erythema dose. Lentigo maligna developed on the patient's face, and a rapidly growing malignant schwannoma was found on the left trigeminal nerve. This may be the first case of a peripheral nervous tissue neoplasm in xeroderma pigmentosum.
Infantile hemangiomas are frequent benign vascular tumors that are often easily recognized. However, the diagnosis between infantile hemangiomas and other vascular tumors, whether benign or malignant, may be difficult. This chapter describes the different clinical presentations of hemangiomas and details the investigations that are needed to confirm the nature of the lesion and to diagnose the potentially associated anomalies. Knowledge on differential diagnosis enables clinicians to detect hemangiomas that can lead to complications and that necessitate a multidisciplinary approach.
BACKGROUND: To present the clinical characteristics and treatment outcomes in a relatively large series of patients with periocular metatypical cell carcinoma. METHODS: This is a retrospective study of 35 patients with periocular metatypical cell carcinoma, who were seen between January 2000 and December 2004 in Izmir Ataturk Research and Training Hospital, Turkey. The patients were histologically confirmed metatypical cell carcinoma with radiologic evidence of periocular region. The main outcome measures were patients' demographics, duration of tumour, clinical presentation, tumour site, treatment modalities, recurrence rate and tumour-related exenteration. Statistical comparisons between the nonrecurring group and recurring group were completed using the Cox regression analysis and the log rank test. Significance was determined for all statistical tests as pé 0.05. RESULTS: The median age of patients at time of diagnosis was 61 years. The most common site for metatypical cell carcinoma was medial canthus and lower eyelid region. The median follow-up for all patients was 18 months. The average length of time until recurrence was 13 months. Five of the 35 patients received adjuvant therapy to the primary tumour site after excision. Signs suggestive of orbital involvement included bone fixation of the mass, limitation of ocular motility and globe displacement. The early lack of diagnostic criteria for metatypical cell carcinoma created considerable debate and confusion regarding its diagnosis. We believe that once this diagnosis is made, the treatment modality should be individualized and chosen with consideration of the extent of orbital involvement, visual function, and the patient's general health.
OBJECTIVE: To characterize the causes and treatment of facial nerve paresis (FNP) in pediatric patients. METHOD: Retrospective study in a tertiary care pediatric hospital. Thirty-four patients identified with partial or complete FNP evaluated between 1997 and 2003. A review of the medical records including sex, age, laterality, etiology, therapy, severity of paralysis according to House-Brackman (HB) six-point grading scale, duration, and degree of recovery. RESULTS: Thirty-five cases of FNP. Causes of FNP were infectious (13), traumatic (7), iatrogenic (5), congenital (4), Bell's/Idiopathic (3), relapsing (2) and neoplastic (1). Peak age distributions for both infectious and traumatic etiologies were bimodal: 1-3 and 8-12 years. Of the 13 infectious cases, 11 were associated with acute otitis media with effusion (AOME). Four (4/11) were bacterial-culture negative. Seven (7/11) were bacterial-culture positive, four (4/7) of which required prolonged, broth-medium culture. Bacteria cultured predominantly included Staphylococcus non-aureus species (5/7) and Propionobacterium acnes (3/7). One (1/13) was viral culture positive (Herpes Simplex Virus). All six patients who received intravenous steroids for OME-associated FNP received the doses within the first week of presentation and had complete recovery (HB I/VI); three of five patients who did not receive steroids had complete recovery. There were five iatrogenic cases; two (2/5) were planned surgical sacrifices and three (3/5) were complications of middle ear/mastoid surgery. Facial nerve function associated with infection returned in 0.5-2 months while, when associated with trauma, returned in 0.25-30 months. CONCLUSIONS: In infectious or traumatic FNP, children aged 1-3 and 8-12 years are the primary groups involved. In AOME FNP, culture-identified organisms may not be representative of traditional pathogens. Infectious FNP averaged 1 month for recovery while traumatic FNP averaged 9 months. Intravenous steroid therapy may improve the outcome. Recovery was complete (HB I/VI) in 8/10 infectious and 4/6 traumatic cases.
An epithelioid hemangioendothelioma and Paget's disease of the breast were treated with topical imiquimod 5% cream with very impressive results. In both of the tumors complete disappearance of the lesions occurred. In this article, in addition to describing the treatment approach, the findings in biopsy specimens of the epithelioid hemangioendothelioma and the Paget's disease before, during, and after therapy will be described. The histologic findings show ample evidence that there is both a lymphocytic T helper 1-like and T helper 2-like response from imiquimod. Direct evidence of lymphocytotoxicity was found. Furthermore, mast cells appear to be involved in the development of regression in the vascular tumor. Although the study is very limited and reports only two cases, the results are striking. We considered it important to describe these findings because of their possible use in developing strategies for the application of imiquimod in the treatment of other tumors in human beings.
Although lipomas are relatively common and found in most anatomical sites, we are not aware of any previous reports of lipomas arising between the two layers of the deep temporal fascia above the zygomatic arch. This is surprising because a fat pad is seen and used during surgery in this area to protect the frontal branch of the facial nerve. This case highlights the importance of understanding the anatomy of this region, because lipomas can potentially arise in any of three fat pads laying near the facial nerve in this area.
Five patients had eyelid and periocular Merkel cell carcinoma. The tumor was located on the left lower eyelid in two patients, the left upper eyelid in one patient, the right upper eyelid in one patient, and was metastatic to the right outer canthus in one patient. The mean duration of symptoms was approximately four months. The diagnosis of Merkel cell carcinoma was not suspected clinically in any of the four primary eyelid cases, but was only established on histopathologic examination of biopsy specimens. Light microscopy disclosed carcinoma with small primitive cells in all five tumor biopsy specimens. Immunohistochemical studies showed neuron-specific enolase and keratin and transmission electron microscopy demonstrated neurosecretory granules typical for Merkel cell carcinoma. All five patients in this study were treated with wide surgical excision of the eyelid tumors with intraoperative frozen-section monitoring of the margins of resection. The left lower eyelid Merkel cell carcinoma spread to the preauricular lymph node in one patient. This patient subsequently died of metastatic Merkel cell carcinoma. One patient with metastatic right outer canthus Merkel cell carcinoma received radiotherapy (6,550 cGy). Eyelid Merkel cell carcinoma has the potential for recurrence and metastatic spread. We recommend lifetime follow-up for patients treated for eyelid Merkel cell carcinoma.
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A series of 11 sebaceous carcinomas is reported. None of the tumours recurred or metastasized. Two growth patterns were observed: basal-cell carcinomas with sebaceous differentiation, and pure sebaceous carcinomas. Since both these types may occur together they are considered to be merely variants of a single tumour entity. The clinical and histological characteristics of sebaceous carcinomas of our series are compared with those recorded in the literature. It is concluded that sebaceous carcinomas originate in the epidermis, in the skin appendages, or in both.
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A patient who developed a partial relapse of Bell's palsy following superficial radiotherapy to a basal cell carcinoma in the temple is reported. Nerves injured by Bell's palsy may be more susceptible to radiation induced damage.