Photodynamic laser therapy of basal-cell carcinoma of the lid

Photodynamic therapy (PDT) remains an experimental approach for the treatment of small, mainly superficial malignant tumors. When given intravenously, hematoporphyrin derivative (HpD) selectively photosensitizes tumor tissue. Activated by light of 630 nm wave-length, HpD leads to tumor necrosis. This paper presents the results of PDT to eyelid basal-cell carcinomas in 21 patients. All lesions primarily responded to the treatment and became necrotic. A generalized photosensitization lasting for more than 4 weeks was seen in all patients. In five patients, lid malformations due to scar formation were noted, being marked in three cases. Ten patients showed a recurrence of tumor after 3-12 months. At present, PDT has no advantage over well-established therapies for basal-cell carcinomas of the eyelid.     

The Stomatology Information Retrieval (Reference) System]

BACKGROUND: Langerhans cell histiocytosis (LCH) is characterized by monoclonal proliferation of activated Langerhans cells. Neither etiology nor pathomechanism of this disorder is presently known. However, despite monoclonality LCH might represent a reactive clonal disorder induced by immune dysfunction rather than a malignant process. To investigate a putative cytokine dysregulation in the pathogenesis of this disorder and searching for parameters of both disease activity and prognosis, serum concentrations of proinflammatory and T-cell derived cytokines were evaluated in LCH patients. MATERIALS AND METHODS: Serum levels of IL-1 beta, IL-2, sIL-2R and TNF-alpha were determined by ELISA in seven children with different types of LCH: Three children (aged 6, 10 and 14 years, respectively) with single system/single bone disease; one child (11 years) with recurrent single system/multiple bone disease and three children (1, 2 and 2 years, respectively) with multisystem disease. RESULTS: sIL-2R was elevated at diagnosis in seven children as compared to healthy adults (mean +/- SEM: 5,256 +/- 3,751 U/ml vs. 73 +/- 5.5 U/ml; P < 0.005) or healthy children (mean +/- SEM: 10,195 +/- 2,798 pg/ml vs. 2,638 +/- 156 pg/ml; P < 0.01). A positive correlation between serum levels of sIL-2R and extent of the disease could be observed. During remission, sIL-2R levels declined. IL-1 beta, IL-2, and TNF-alpha remained within the normal range during the study period. CONCLUSIONS: Elevated sIL-2R levels seem to correlate positively with both extent and activity of LCH, thus indicating a pathological T-cell activation as a pathogenetic factor. sIL-2R level is a promising parameter to monitor disease activity in LCH and may also be of prognostic relevance.     

Controversies and new approaches to treatment of Langerhans cell histiocytosis

There continues to be genuine ambivalence as to whether Langerhans cell histiocytosis (LCH) is a primary neoplastic or immuno-dysregulatory disorder. Treatment strategies have moved from one camp to the other depending upon the most current alleged successes or failures. This has been particularly true for patients who fall outside of the sphere where treatment is minimal or where known treatment approaches are clearly beneficial. However, there is growing evidence that LCH is both the result of clonal proliferation of Langerhans cells and the immunologic consequence of increased cellular activation. This new knowledge should be the basis for the development of new therapeutic approaches for patients with LCH and its complications.     

Production of endothelin-1 by cultured human synoviocytes

Langerhans-cell histiocytosis (LCH) is a rare condition with a wide clinical spectrum and variable prognosis. Patients with multisystem LCH have been treated with a variety of agents but may develop resistant and progressive disease. Based on a preliminary encouraging report on the activity of 2 chlorodoxyadenosine in this disease, we administered this agent to a patient with LCH which was resistant to corticosteroids and etoposide. After 4 courses of treatment the patient achieved a complete remission which is currently ongoing for 12 months. 2 CdA appears to be effective in patients with resistant LCH and warrants investigation in previously untreated patients with poor risk disease.     

Anterior capsulotomy created by radiofrequency endodiathermy and continuous curvilinear posterior capsulorhexis in a patient with intumescent cataract and primary capsular fibrosis

PURPOSE: To report a clinicopathologic correlation of angiosarcoma affecting the eyelid skin. METHODS: An 82-year-old man developed multiple bruise-like maculopapular lesions, subcutaneous nodules, and diffuse edema over his scalp, face, and eyelids. Biopsy disclosed angiosarcoma, and the patient was treated with wide-field external beam radiotherapy (5,000 cGy). RESULTS: He responded to radiotherapy with partial regression of the tumor. Twelve months later, he developed extensive lymphedema secondary to the residual tumor. He was also found to have parotid gland and bone metastases, treated with radiotherapy. The patient died of widespread bone metastases 3 months later. CONCLUSIONS: Angiosarcoma is a rare skin tumor that has a poor prognosis despite treatment. It should be considered in the differential diagnosis of eyelid tumors and edema because it may affect only the central portion of the face.     

Leiomyosarcoma of the maxillary gingiva: a case report

An unusual case of leiomyosarcoma (LMS) of the maxillary gingiva is discussed here; this case presents a unique pattern of tumor growth and a long period between initial discovery and correct pathological diagnosis. The tumor was incompletely resected twice by a private dentist over a period of 3 years, with a clinical diagnosis of epulis, no pathological examination was conducted during this period. When it was finally removed, the tumor was very large (50 x 35 x 12 mm in size and 18 g in weight), consisting of an easily hemorrhagic mass originating in the gingival mucosa with the growth pattern of a polyp. Following an extensive surgical excision and a unilateral radical neck dissection, the patient has been free of LMS for 8 years. In light of this case, we strongly emphasize the importance of conducting a pathological examination, even though clinical examination seems to indicate a diagnosis of epulis or granulation. In this way, the presence of LMS can be ascertained in a timely manner with better prognosis for treatment and recovery.     

Individual DNA bands obtained by RAPD analysis of canine genomic DNA often contain multiple DNA sequences

We present a case of Langerhans' cell histiocytosis (LCH) of the liver and spleen in an adult. The imaging features are different from those in the few previously reported cases of individual organ involvement by LCH.     

Effect of contrast concentration on abdominal enhancement in the rabbit: spiral computed tomography evaluation

BACKGROUND: Langerhans' cell histiocytosis (LCH) is an uncommon, poorly understood granulomatous disease, characterized by the idiopathic proliferation of Langerhan's cells or their marrow precursors. In 1985, the Philadelphia Work-shop adopted the term "Langerhans' cell histiocytosis" (LCH) to differentiate it from reactive and neoplastic causes of histiocytosis. METHODS: This study includes 73 pediatric patients diagnosed with this condition in Dublin, Ireland, and Nottingham, England, during a 34-year period (1959 to 1993). These patients are reviewed with respect to clinical presentation, difficulty with making a histological diagnosis, their management, and outcome. RESULTS: A total of 49 patients (67%) had head and neck involvement. Bony involvement was the most frequent sign, most frequently located in the skull. There were 11 deaths (15%) in this series, all associated with multisystem disease, and nine of these deaths were in children younger than 2 years of age. CONCLUSIONS: The role of otolaryngologists is important in the early and accurate evaluation, staging, and diagnosis of LCH. It may mimic more common diseases, such as otitis externa, acute mastoiditis, skin rash, gingivitis, or cervical lymphadenopathy. Patients with multisystem disease may be so ill at presentation that the head and neck lesions may be overlooked. The current management of LCH has become increasingly conservative, and in the 1990s, fewer cases are given chemotherapy or radiotherapy. The prognosis is very good for single-system disease and poor for multisystem disseminated disease with early onset.     

Detection of GM-CSF in the sera of children with Langerhans' cell histiocytosis

GM-CSF induces proliferation and activation of Langerhans' cells in vitro. The density of Langerhans' cells in human tumours is correlated to the in situ density of GM-CSF, and intradermal injection of GM-CSF induces local accumulation of Langerhans' cells. Therefore, we investigated the presence of GM-CSF in the sera of children with Langerhans' cell histiocytosis (LCH). We detected GM-CSF in the sera of all children with disseminated and active LCH, but not in the sera of patients with localized (i.e. bone) LCH. These results suggest that GM-CSF level is related to extent and the activity of LCH.     

Primary ductal adenocarcinoma of the lacrimal gland

The morphological similarity of salivary and lacrimal gland tumors is known. Many clinicopathological studies and characteristics of salivary duct carcinoma, which bears histological similarities to mammary duct carcinoma, have been recently reported; however, only one case of lacrimal duct carcinoma is reported. A second case of lacrimal duct carcinoma is presented. A 67-year-old male with a painless mass in the right upper eyelid underwent total removal of the tumor mass. Microscopic examination of the tumor mass revealed ductal adenocarcinoma of the lacrimal gland, which was the equivalent of salivary duct carcinoma. The immunohistological studies of the lacrimal duct carcinoma showed similar results to those reported for salivary duct carcinoma. The recurrent tumor in the subdural spaces was removed 2 years after the initial surgery and the patient is followed as an outpatient.     

Witches, multiple personalities, and other psychiatric artifacts

Apolipoprotein E (apoE) has been found in association with several different types of systemic and cerebral amyloid deposits and the presence of the epsilon 4 allele constitutes a risk factor for Alzheimer's disease. It has been shown that apoE binds and promotes the fibrillogenesis in vitro of Alzheimer's amyloid beta-peptide, suggesting an important role for apoE in the modulation of amyloidogenesis. Due to the co-localization of apoE with several biochemically distinct amyloid deposits, it has been proposed that apoE plays a general role modulating and/or participating in amyloidosis. In the present study, we show for the first time that apoE, isolated from human plasma, increases fibril formation of synthetic peptides comprising the amyloidogenic sequences of gelsolin amyloid related to familial amyloidosis Finnish type, and amyloid A found in secondary amyloidosis and familial Mediterranean fever. Our results suggest that apoE acts as a general pathological chaperone in various amyloidoses by enhancing the transition from soluble peptides into amyloid-forming, pathological molecules.     

Endocrinological aspects of Langerhans cell histiocytosis complicated with diabetes insipidus

Langerhans cell histiocytosis (LCH) is a rare disorder and may be complicated with hypopituitarism and diabetes insipidus (DI) due to invasion of the hypothalamic-pituitary area. In this study, 10 patients with complete (4) and partial (6) type central DI were found among 125 LCH patients in our hospital records. The water deprivation test, followed by the pitressin test, was performed to confirm DI. Hypothalamic-pituitary endocrine function tests were carried out on these 10 patients at the initial diagnosis and during follow-up. All patients revealed growth hormone insufficiency in the insulin hypoglycemic tolerance test. Four patients had impairment of cortisol secretion, demonstrated by insulin hypoglycemic stimulating test results. Two patients had poor response in the thyrotropin releasing hormone stimulating test. Two patients had only partial responses in the luteinizing hormone releasing hormone test. Four patients had hyperprolactinemia. All patients underwent surgical treatment followed by chemotherapy and/or radiotherapy. One patient completely recovered from the endocrine disorder, 3 patients required smaller doses of desmopressin, and one patient had normal adrenal, thyroid, and gonadal function. Hypothalamic-pituitary disorders in LCH should not be neglected. Treatment of LCH can partially or completely reverse associated endocrine disorders. Therefore, endocrine studies and hormone replacement should be mandatory for patients with LCH.     

What''s new in colorectal cancer screening?

BACKGROUND/AIM: Patients with thyroid eye disease with upper eyelid retraction often develop overaction of the accessory muscles of eyelid closure, the glabellar muscles corrugator supercilii and procerus. The resultant glabellar furrowing (frown lines) contributes to the typical thyroid facies. The aim of this study was to evaluate the use of botulinum toxin A reversible chemodenervation of the glabellar muscles as adjunctive treatment in the rehabilitation of patients with thyroid eye disease. METHODS: 14 patients (13 females) ages 39-76 years (mean 52) with inactive thyroid eye disease and associated medial eyebrow ptosis and prominent glabellar frown lines were recruited. All patients had a history of upper eyelid retraction. Each patient was treated with a single botulinum toxin injection (Dysport 0.2 ml, 40 units) into each corrugator supercilii and sometimes procerus muscles as an outpatient procedure. The effectiveness and acceptability of the treatment was assessed clinically and from a patient questionnaire. RESULTS: The injections were tolerated by 13/14 (93%) patients. There was resultant flattening of the glabellar region and improvement of medial eyebrow contour in all patients, with onset of paralysis within 1 week. All patients reported a subjective improvement in appearance. Side effects included one patient (7%) with reversible partial ptosis. The beneficial effect lasted 4-6 months, with a gradual return of function. Repeat treatment was indicated where there was persistent upper eyelid retraction and protractor overaction. CONCLUSION: Botulinum toxin A chemodenervation of the glabellar muscles in these patients was effective and acceptable. Chemodenervation should be considered in the rehabilitation of patients with thyroid eye disease where there is upper eyelid retraction and overacting protractors resulting in a thyroid frown. Once the eyelid retraction has been successfully treated by surgery, the need for further glabella muscle chemodenervation is considerably reduced.     

Lower eyelid reconstruction using pedicled skin flap and palatal mucoperiosteum

BACKGROUND: Full-thickness lower eyelid reconstruction requires functional as well as aesthetic considerations to be successful in the long term. The three elements necessary for a stable result are skin, mucosa, and a semirigid "skeleton" to provide the support that prevents the development of ectropion. A number of techniques combining these elements are described in the literature, with reconstruction in one or more stages. OBJECTIVE: We report a simple method of reconstruction of a full-thickness lower eyelid defect, in which palatal mucoperiosteum provided both the mucosal and the "skeletal" components following tumor excision. METHODS: Literature review and report of illustrated case. RESULTS: A cosmetically acceptable result with no functional deficit or donor site morbidity. CONCLUSION: Palatal mucoperiosteum provides an abundant supply of tissue that may be used successfully in the reconstruction of the lower eyelid. It functions both as a mucosal lining in addition to a semirigid supporting framework.     

The zygorbicular dissection in composite rhytidectomy: an ideal midface plane

Traditional face lift and blepharoplasty techniques are based on two consistent principles. Conventional face lift techniques have always incorporated unopposed lateral vector tissue advancement, which is typical of subcutaneous lifts or rhytidectomies that include the platysma muscle (SMAS) or cheek fat (malar fat). When they are not adequately repositioned, the tissues of the lower eyelid and upper cheek continue to age, which may create a "lateral sweep" of the lower face as those malar soft tissues descend at a more rapid rate than the repositioned SMAS. Removal of the lower eyelid fat in conventional blepharoplasties may also lead to a more hollow-appearing lower eyelid. Hollow eyes and the lateral sweep could be prevented with a rhytidectomy technique that includes orbicularis repositioning and preservation of the lower eyelid fat with an arcus marginalis release. All patients who have been operated on who exhibit these unfavorable signs can have an impressive correction by utilizing the principles of superomedial vector orbicularis repositioning to counter the "lateral sweep" and arcus marginalis release to recreate a youthful shallow and narrow lower eyelid contour. This technique is invaluable to patients seeking secondary surgery to regain harmony of the rejuvenated face.     

The sonographic appearance of lesions of the spleen and pancreas in an infant with Langerhans' cell histiocytosis

Langerhans' cell histiocytosis (LCH), the term now used to describe the group of diseases known as histiocytosis X, is a rare disorder of the bone marrow-derived histiocytes that may involve the skin, bone, bone marrow, liver, spleen, lungs, lymph nodes, and rarely the pancreas. Sonographically demonstrable lesions of the spleen and pancreas have not been reported. We present a case of disseminated LCH in a 4-week-old infant to demonstrate the sonographic appearance of splenic and pancreatic lesions that occur with this disease.     

Ocular toxicity following topical application of anesthetic cream to the eyelid skin

BACKGROUND AND OBJECTIVE: The use of topical anesthetic cream in the periorbital region may be of clinical value. The potential for toxic effects from such use has not been studied in a controlled manner. This study was performed to evaluate the potential ocular toxicity of anesthetic cream topically applied to the eyelid in an animal model. MATERIALS AND METHODS: Ten rabbits underwent periorbital eutectic mixture of local anesthetics (EMLA) (2.5 percent lidocaine and 2.5 percent prilocaine) application and were observed for evidence of gross or microscopic ocular toxicity. Baseline external and anterior segment examinations were performed, including biomicroscopy and fluorescein staining, after which a standard quantity of EMLA cream (0.75 g) was applied along the upper eyelid and covered with an occlusive dressing. After 1 hour of treatment, the eyelid and anterior segment were examined for evidence of adverse reaction. The eyelids were excised and examined histopathologically. RESULTS: No significant adverse effects were noted on external lid and anterior segment examination. The histopathologic findings were within normal limits. CONCLUSIONS: This study suggests that external application of EMLA cream to the eyelid does not induce local toxicity in the rabbit model. The external application of EMLA cream may be safe in the periorbital region.     

Immune surveillance: paraneoplastic or environmental triggers of autoimmunity

Autoimmunity associated with tumor cell development seems an important mechanism by which to prevent progression to clinical cancer. In this brief review, tumor autoantigens associated with paraneoplastic syndrome, non-HLA-associated organ-specific autoimmune diseases, and the highly cell-specific autoimmune eradication of the islet beta cells in type 1 diabetes are compared and discussed. It is suggested that autoreactivity is important in preventing tumor formation; however, it may be at the expense of the development of autoimmune disease. Although the cytotoxic T lymphocytes (CTL) induction by HLA class I has been studied and used in clinical trials, little is understood about the initiation and HLA class II mediated induction of an immune response to neoplastic cells. This induction apparently takes place because paraneoplastic disorders are often due to an immune response to the tumor cell resulting in a cross-reactivity with a normally expressed autoantigen on a remote nontumor-associated target cell. The problem of immune surveillance to eradicate neoplasm or downregulate pathological autoimmunity are therefore closely related phenomena. An improved understanding of immune mediated tumor suppression should therefore greatly benefit immunotherapy of type 1 diabetes, and the two areas of research would benefit from an interdisciplinary endeavor.     

Ophthalmic tumor formation in Waldenstr?m's macroglobulinemia

Waldenstr?m's macroglobulinemia is a malignant disease of the reticuloendothelial system; ocular complications reported previously have been concerned chiefly with hyperviscosity manifestations. Ophthalmic tumor formation is rare, although not unheard of, in this particular condition. This report describes two rare instances of Waldenstr?m's macroglobulinemia which involved conjunctival and orbital tumors. One patient developed the tumors after a lengthy clinical course; in the other patient, eyelid and orbital tumors were the first symptoms. Careful examination of the external eye, especially the fornix of the conjunctiva, in patients with Waldenstr?m's macroglobulinemia can identify an initial lesion or invasions to other organs.     

Eye movement in amyotrophic lateral sclerosis: a longitudinal study

Conservative therapy in cases of severe dysthyroid ophthalmopathy (DO) has not given satisfactory results. Since 1986, 51 patients (99 orbits) with marked DO have been treated by surgical decompression of the orbits. The procedure begins within the upper eyelid. The excision of the excessive skin and subcutaneous tissue is performed and eyelid fat is removed. In order to obtain the correction of the upper eyelid retraction, the levator aponeurosis is divided by 2-3 transverse incisions in its central part and Muller's muscle is sectioned at the level of its tarsal insertion. Through an incision in the lower eyelid, the posterior part of the orbital floor, the lateral orbit wall, as well as the periorbital and intraorbital fat are removed. Through an incision made over the medial margin of the orbit, the ethmoidal part of the medial orbital wall and the retrobulbar fat are removed. The periorbital periosteum should be incised at several sites. After operation all patients showed a significant reduction of exophthalmos (5-11 mm, 7.16 mm on average), significant reduction of intraocular pressure, marked improvement in ocular muscle function, as well as considerable reduction or disappearance of subjective symptoms. There was an improvement in vision in 68% patients who had impaired vision before the operation. There were no cases of subsequent impairment of vision or ocular motility. Mild relapse was recorded in three cases only and only one patient required unilateral reoperation. Strabismus surgery had to be performed in five patients due to unsatisfactory correction of double vision. It can be concluded that this method of orbital decompression gives very good functional and aesthetical long-term results.