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Biomedical subjects

W L White

Publications and source records attributed to W L White.

At least 19 recordsLinked to original sources

Rudimentary meningocele: remnant of a neural tube defect?

BACKGROUND: Rudimentary meningocele, a malformation in which meningothelial elements are present in the skin and subcutaneous tissue, has been described in the past under a variety of different terms and has also been referred to as cutaneous meningioma. There has been debate as to whether rudimentary meningocele is an atretic form of meningocele or results from growth of meningeal cells displaced along cutaneous nerves OBJECTIVE: We reviewed the clinical, histological, and immunohistochemical characteristics of rudimentary meningocele in an attempt to assess the most likely pathologic mechanism for it. DESIGN: Retrospective study. SETTING: University hospitals. PATIENTS: Thirteen children with rudimentary meningocele. MAIN OUTCOME MEASURES: Medical records were reviewed and histopathologic examination as well as immunohistochemistry studies were performed for each case. A panel of immunoperoxidase reagents (EMA, CD31, CD34, CD57, S-100, and CAM 5.2) was used to assess lineage and to confirm the meningothelial nature of these lesions. RESULTS: Recent evidence indicating a multisite closure of the neural tube in humans suggests that classic meningocele and rudimentary meningocele are on a continuous spectrum. CONCLUSION: Rudimentary meningocele seems to be a remnant of a neural tube defect in which abnormal attachment of the developing neural tube to skin (comparable to that in classic meningocele) could explain the presence of ectopic meningeal tissue. In the majority of cases, no underlying bony defect or communication to the meninges could be detected. However, in light of the probable pathogenesis, imaging studies to exclude any communication to the central nervous system should precede any invasive evaluation or intervention.

Child↗

Delayed orbital hemorrhage after cataract surgery in a patient with an acquired factor VIII inhibitor.

PURPOSE: To report the occurrence of delayed orbital hemorrhage after cataract surgery with retrobulbar anesthesia in a patient with previously unknown acquired factor VIII antibodies. METHODS: Interventional case report. A 79-year-old woman underwent uneventful cataract surgery with retrobulbar anesthesia and subsequently incurred a severe orbital hemorrhage 12 hours postoperatively. RESULTS: Despite aggressive surgical and medical intervention, the patient lost all vision in the involved eye. CONCLUSION: Delayed postoperative retrobulbar hemorrhage may cause total loss of vision in patients with acquired factor VIII antibodies.

Aged↗

Expression of microtubule-associated protein 2 in benign and malignant melanocytes: implications for differentiation and progression of cutaneous melanoma.

Cutaneous melanocytic neoplasms are known to acquire variable characteristics of neural crest differentiation. Melanocytic nevus cells in the dermis and desmoplastic melanomas often display characteristics of nerve sheath differentiation. The extent and nature of neuronal differentiation characteristics displayed by primary and metastatic melanoma cells are not well understood. Here, we describe induction of a juvenile isoform of microtubule-associated protein 2 (MAP-2c) in cultured metastatic melanoma cells by the differentiation inducer hexamethylene bisacetamide. Up-regulation of this MAP-2 isoform, a marker for immature neurons, is accompanied by extended dendritic morphology and down-regulation of tyrosinase-related protein 1 (TYRP1/gp75), a melanocyte differentiation marker. In a panel of cell lines that represent melanoma tumor progression, MAP-2c mRNA and the corresponding approximately 70-kd protein could be detected predominantly in primary melanomas. Immunohistochemical analysis of 61 benign and malignant melanocytic lesions showed abundant expression of MAP-2 protein in melanocytic nevi and in the in situ and invasive components of primary melanoma, but only focal heterogeneous expression in a few metastatic melanomas. In contrast, MAP-2-positive dermal nevus cells and the invasive cells of primary melanomas were TYRP1-negative. This reciprocal staining pattern in vivo is similar to the in vitro observation that induction of the neuronal marker MAP-2 in metastatic melanoma cells is accompanied by selective extinction of the melanocytic marker TYRP1. Our data show that neoplastic melanocytes, particularly at early stages, retain the plasticity to express the neuron-specific marker MAP-2. These observations are consistent with the premise that both benign and malignant melanocytes in the dermis can express markers of neuronal differentiation.

Acetamides↗

Iatrogenic complications related to the use of Herrick Lacrimal Plugs.

PURPOSE: To report a series of lacrimal complications associated with a specific type of lacrimal plug (Herrick Lacrimal Plug; Lacrimedics Incorporated, Rialto, CA.) DESIGN: Retrospective, noncomparative case series and survey. METHODS: Members of the American Society of Ophthalmic Plastic and Reconstructive Surgery were asked to submit personally treated cases of patients referred for treatment of complications after placement of a Herrick Lacrimal Plug. MAIN OUTCOME MEASURES: Failure of the device to be removed by simple lacrimal irrigation. RESULTS: The clinical courses of 41 patients were analyzed. Patients ranged in age from 19 to 81 years, and all had symptomatic epiphora related to the presence of the lacrimal plug. Several interventions were used to treat lacrimal obstruction. Nasolacrimal duct probing with irrigation was used in 15 lacrimal systems, whereas six systems were probed and subsequently stented with silicone tubing. Eyelid margin cutdown was used in eight cases. Balloon dacryoplasty was performed in three systems, dacryocystorhinostomy in 18 instances, and conjunctivodacryocystorhinostomy in two patients. CONCLUSIONS: The Herrick lacrimal occlusion device sometimes cannot be removed by simple irrigation and is capable of inducing permanent, irreversible, symptomatic lacrimal drainage system obstruction.

Adult↗

The autogenous dermis-fat orbital implant in children.

PURPOSE: The autogenous dermis-fat graft orbital implant is one alternative for volume augmentation in the adult anophthalmic socket. We have reviewed our experience with dermis-fat grafts after primary enucleation in the pediatric population. METHODS: We reviewed the clinic charts of all patients under 8 years of age who underwent primary enucleation combined with dermis-fat graft implantation. We assessed subsequent orbital growth by measuring lid and fissure symmetry, superficial soft tissue socket volume, prosthetic fit, and periorbital symmetry. All measurements were obtained by 2 of the authors. RESULTS: Eight children had primary enucleations combined with a dermis-fat implant (Table 1). Age at the time of surgery ranged from 1 week to 8 years. Indications for enucleation were retinoblastoma (4), malignant teratoid medulloepithelioma (1), blind, painful eye secondary to trauma (2), or phthisis secondary to end-stage retinopathy of prematurity (1). Two patients were lost to follow up at 3 months and 6 months post enucleation. The remaining 6 patients had between 1 year and 6 years of follow up. All implants maintained appropriate volume allowing proper prosthetic fit and facial symmetry. Two children experienced excessive growth of their implants, managed by surgical debulking. Two children developed a central graft ulcer, managed by superficial revision and reclosure. CONCLUSIONS: We have observed that dermis-fat grafts in pediatric primary enucleations demonstrate good maintenance of orbital soft tissue volume and periorbital symmetry. The complications of excessive dermis-fat growth or central ulceration were easily corrected. We feel this is a promising implant for the pediatric anophthalmic socket.

Adipose Tissue↗

Benign cutaneous Degos' disease: a case report with emphasis on histopathology as papules chronologically evolve.

The following case report details a 53-year-old man with a 6-year history of the benign cutaneous or skin-limited form of Degos' disease. Clinically, the patient demonstrated a diffuse eruption of papules on the upper trunk and arms. Many papules demonstrated the classic porcelain-white centers characteristic of Degos' disease, but others exhibited different clinical morphologies that corresponded to the evolutionary stages of papules originally described by Degos. Over the course of several clinic visits, the patient underwent a total of 5 punch biopsies, the histologies of which were correlated with their clinical morphologies. Early papules were skin-colored and demonstrated a superficial and deep perivascular, periadnexal, and perineural chronic inflammatory cell infiltrate associated with interstitial mucin deposition. The overlying epidermis showed a mild vacuolar interface reaction and the histologic appearances at this early stage resembled tumid lupus erythematosus. Fully developed papules were raised with umbilicated porcelain-white centers and a surrounding erythematous rim. Histologically these exhibited a prominent interface reaction with squamatization of the dermo-epidermal junction, melanin incontinence, epidermal atrophy, and a developing zone of papillary dermal sclerosis that resembled the early stages of lichen sclerosus et atrophicus in miniature. These interface reactions were invariably confined to the central portion of the punch biopsy specimen, corresponding to the central porcelain-white area seen clinically. Additional features of fully developed papules included a prominent lymphocytic vasculitis affecting venules, a mild periadnexal infiltrate of neutrophils and/or eosinophils, and interstitial mucin deposition. In late-stage papules, the porcelain-white areas were better developed and the lesion flattened. Histologically, the degree of inflammation was generally sparse and the overall picture mirrored the classic histologic description of Degos' disease with a central roughly wedge-shaped zone of sclerosis surmounted by an atrophic epidermis and hyperkeratotic compact stratum corneum. These late-stage papules closely resembled a miniaturized version of fully developed lichen sclerosus et atrophicus confined to the center of the punch biopsy specimen.

Adult↗

Primary signet ring cell carcinoma of the eyelid: report of a case demonstrating further analogy to lobular carcinoma of the breast with a literature review.

Primary signet ring cell carcinoma of the eyelid is a rare neoplasm with less than ten cases described. This report details another case, which shows further parallels to lobular carcinoma of the breast, and reviews the literature on this subject. A 73-year-old white female presented with diffuse induration of her left eyelids. Histopathology revealed a delicate infiltrate of epithelial cells scattered throughout the lid stroma in a non-destructive pattern. The cells were relatively monomorphous and showed only mild atypia and rare mitotic figures. Many had slightly granular amphophilic cytoplasms, others showed distinct signet ring cell morphology, and all were strongly positive for epithelial mucin. Immunomicroscopy showed strong reactivity for estrogen receptor (ER), progesterone receptor (PR) and gross cystic disease fluid protein-15 (GCDFP-15), and was negative for Her-2/neu (erb-2) and cytokeratin 20. An extensive workup for other primary sites was negative. Orbital exenteration showed extensive involvement of both lids and soft tissue, including diffuse muscle and lacrimal gland infiltration. In the breast, signet ring cell carcinoma is considered a variant of lobular carcinoma. The delicate infiltrating pattern in our case and the ER+, PR+, GCDFP-15+, Her-2/neu-phenotype further strengthen this analogy. Together, these data also support apocrine differentiation of primary eyelid signet ring cell carcinoma.

Aged↗

Use of heat-malleable thermoplastic splints in eyelid skin grafting.

PURPOSE: To report on the usage of a thermoplastic material in eyelid reconstruction. METHODS: Retrospective single-surgeon case review. RESULTS: Thirty-eight full-thickness skin grafts in 37 patients are reported. No major complications were encountered. All outcomes with the use of this technique yielded satisfactory results. CONCLUSIONS: The use of thermoplastic materials in eyelid reconstruction appears to achieve results equal to those seen with traditional materials. This technique in eyelid surgery may save operative time and be less expensive than traditional methods.

Aged↗

Testicular lymphoma is associated with a high incidence of extranodal recurrence.

BACKGROUND: Testicular lymphoma is a rare extranodal presentation of non-Hodgkin lymphoma. The authors report long term follow-up information regarding a group of patients with testicular lymphoma evaluated at the Mayo Clinic and describe the outcome with special attention to patterns of recurrence. METHODS: The medical records of patients with testicular lymphoma seen at the Mayo Clinic between January 1970 and March 1993 were reviewed. Patients were included if they had evidence of testicular involvement at the time of diagnosis of lymphoma. Pathology specimens were reviewed for confirmation of diagnosis. RESULTS: Sixty-two patients with a diagnosis of testicular lymphoma were identified. Their median age was 68 years, and 60 patients underwent orchiectomy as the initial therapeutic and diagnostic procedure. Most of patients (79%) had localized or regional disease at the time of presentation. Other treatment modalities after diagnosis included radiotherapy (37%), combination chemotherapy (37%), and combination chemotherapy and radiotherapy (16%). Although 88% of patients had no residual disease after primary treatment, 80% subsequently experienced disease recurrence. There was no significant difference in the rate of recurrence, including Ann Arbor Stage I disease. Treatment did not appear to affect the recurrence rate. At a median follow-up of 2.7 years, 60% of patients had died of disease. Late recurrences were observed, and there appeared to be no plateau in the disease free survival curve. In half (51%) of the patients with disease recurrence, only extranodal locations were involved. Thirteen patients experienced recurrence in the central nervous system, 11 of whom had parenchymal lesions. In 8 of these 13 patients, the central nervous system was an isolated site of disease recurrence. CONCLUSIONS: Testicular lymphoma is a unique and aggressive extranodal non-Hodgkin lymphoma. Better treatment strategies are needed to prevent recurrences. The risk of extranodal recurrence is high, especially in the central nervous system.

Adult↗

Combined intraepithelial squamous neoplasia and atypical fibroxanthoma of the cornea and limbus.

PURPOSE: To investigate a case of an unusual neoplasm of the cornea and limbus. METHODS: A 59-year-old man presented with a highly vascularized, nodular mass involving the left cornea and limbus. An excisional biopsy and, subsequently, a superficial lamellar keratectomy and multiple conjunctival biopsies were performed. At the 6-month follow-up examination, repeat conjunctival biopsies were performed. RESULTS: Histopathologic examination of the corneal specimen showed a high-grade intraepithelial squamous neoplasia (in situ carcinoma) overlying an atypical fibroxanthoma. CONCLUSION: We report the clinical and histologic appearance of a corneal/limbal neoplasm consisting of an intraepithelial squamous neoplasia and an atypical fibroxanthoma.

Carcinoma in Situ↗

Fusiform incisional biopsy for pigmented skin lesions.

Current recommendations for biopsy of a pigmented skin lesion suspected of being melanoma include complete excision when possible. Because benign pigmented lesions do not warrant excisional biopsy, especially when they are large and/or on cosmetically sensitive sites, incisional biopsy is desirable. For such lesions we recommend a fusiform incisional biopsy. This tissue sampling technique not only yields an excellent cosmetic outcome, but also provides the pathologist with an optimal biopsy specimen in which he/she can assess the symmetry, circumscription, breadth, and depth of the lesion.

Biopsy↗

Utility of rush paraffin-embedded tangential sections in the management of cutaneous neoplasms.

BACKGROUND: Mohs micrographic surgery (MMS) modified by the use of tangential, formalin-fixed, paraffin-embedded histologic specimens is advantageous in treating selected skin neoplasms. OBJECTIVE: To review the use of our experience with a modification of MMS to treat lentigo maligna melanoma (LMM), lentigo maligna (LM) and other melanoma in situ (MIS) lesions, dermatofibrosarcoma protuberans (DFSP), atypical fibroxanthoma (AFX), and angiosarcoma. METHODS: Our experience utilizing a modification of MMS in the treatment of 77 patients with LM or other MIS, 23 patients with LMM, 11 patients with DFSP, 1 patient with AFX, and 1 patient with angiosarcoma was reviewed. Length of follow-up and rate of recurrence were examined. A literature review of this pertinent modification of the Mohs technique was performed. RESULTS: One hundred fourteen patients underwent MMS for melanocytic (LM, MIS, LMM), spindle cell (DFSP, AFX), and vascular malignant neoplasms. One patient developed locally recurrent LM and one patient with LMM developed satellite metastasis. Regional lymph node metastasis occurred in one patient with LMM and in a patient with angiosarcoma. CONCLUSION: The use of Mohs micrographic surgery in conjunction with rush formalin-fixed, paraffin-embedded tangential histologic sections provides the accuracy and tissue conservation of the Mohs procedure while ensuring more confident interpretation of histology in cases of lentigo maligna, lentigo maligna melanoma, dermatofibrosarcoma protuberans, atypical fibroxanthoma, and angiosarcoma.

Aged↗

Epithelioid blue nevus: neoplasm Sui generis or variation on a theme?

The epithelioid blue nevus has recently been associated with the Carney complex, which is characterized by myxomas, spotty skin pigmentation, endocrine overactivity, and schwannomas. Using the general criteria proposed by Carney and Ferreiro, similar lesions were identified in 33 patients with no evidence of the Carney complex. Those lesions presented on the face, trunk and extremities of 15 males and 18 females. The mean age was 35 years, much older than those in the Carney complex (mean 16.3 years). Clinical diagnoses included malignant blue nevus, atypical nevus, melanoma, congenital nevus, and dermatofibroma. The lesions were symmetric, predominantly dermal melanocytic proliferations arranged as short fascicles, small nests, and single cells. Large polygonal and epithelioid melanocytes with moderate pleomorphism, and occasional nuclear pseudoinclusions were admixed with heavily pigmented dendritic and spindled melanocytes and melanophages. Rare mitotic figures were seen in some cases. The neoplasms showed a morphologic spectrum that encompassed a group of combined blue nevi with epithelioid melanocytes and other Spitz's nevus characteristics. These epithelioid combined nevi (ECN) fell into three phenotypes with morphologies that most closely paralleled those pictured by Carney and Ferreiro in the Carney complex: the classic or Carney complex pattern (ECN-CC), those that showed overlap with deep penetrating nevus (ECN-DPN), and those that have many dermal Spitz's nevus features, [BLue + SpITZ's nevus; (ECN-BLITZ)I. In six cases, there was such an admixture of features that it was difficult to ascribe them to one of the groups. Nine lesions had associated banal congenital nevus. Follow-up that averaged over 2.5 years (31 months) (range 6-162 months) showed no evidence of malignancy or recurrent disease after excision. Epithelioid combined nevus is a type of combined nevus with blue nevus and Spitz's nevus features, which may or may not be associated with the Carney complex. It shows morphologic overlap with the epithelioid blue nevus described by Carney (ECN-CC), deep penetrating nevus (ECN-DPN), and blue nevus with intradermal Spitz's (desmoplastic) nevus (ECN-BLITZ). Epithelioid combined nevus is thought to be a fitting nosologic designation for all of these lesions.

Adolescent↗

Pyoderma gangrenosum. A comparison of typical and atypical forms with an emphasis on time to remission. Case review of 86 patients from 2 institutions.

Pyoderma gangrenosum (PG) is an idiopathic, inflammatory, ulcerative disease of undetermined cause. The diagnosis is based on clinical and pathologic features and requires exclusion of conditions that produce ulcerations. An atypical bullous variant (atypical pyoderma gangrenosum, APG) exists with clinical features similar to those of Sweet syndrome. Because PG is a rare disease, few large case series have been reported. Pyoderma gangrenosum was first recognized as a unique disease entity in the first half of the 20th century. Cumulative knowledge of PG is based on a handful of case series and multiple individual case reports. To augment that knowledge, we present our experience with a large number of patients over a significant time. We performed a retrospective analysis of the medical records of 86 patients with PG who were evaluated and treated over 12 years at 2 university-based dermatology departments. The mean (+/- standard deviation) age of onset of PG and APG, respectively, was 44.6 +/- 19.7 years and 52.2 +/- 15.3 years. Lower extremity involvement was most common in PG, whereas upper extremity involvement was most common in APG. Associated relevant systemic diseases were seen in 50% of patients. Inflammatory bowel disease was the most common association in patients with PG, whereas hematologic disease or malignancy was most common in those with APG. Although a few patients were managed with local measures or nonimmunosuppressive treatment, the majority required oral corticosteroid therapy, often with systemic immunosuppressive treatment. PG patients required a mean 11.5 +/- 11.1 months of treatment to achieve remission compared with 9.0 +/- 13.7 months for patients with APG. Five patients (5.8%) had disease that was extremely refractory to multiple intensive therapies. The prognosis and disease associations for PG and APG appear to be different. Compared with PG, APG is more often associated with hematologic disease or malignancy, and remits more quickly.

Adolescent↗

The role of recovering physicians in 19th century addiction medicine: an organizational case study.

An elaborate network of inebriate homes, inebriate asylums, nationally franchised private addiction treatment institutes, and proprietary home cures for addiction arose on the American landscape between 1850 and 1900. The pinnacle of the movement to professionalize America's first addiction treatment field was the founding of the American Association for the Cure of Inebriety in 1870 and its publication of the first issue of the Journal of Inebriety in 1876. One of the most contentious issues among the various branches of this new professional field was the question of the use of "reformed men" as physicians, managers and attendants within treatment institutions. This article describes the employment of recovering physicians within one 19th century addiction treatment franchise--the Keeley Institutes--and documents the nature of the professional debate stirred by what was then a controversial practice.

Alcoholism↗

Atypical Spitz nevi/tumors: lack of consensus for diagnosis, discrimination from melanoma, and prediction of outcome.

The biological nature of Spitz nevi/tumors and their diagnostic distinction from, or relationship to, melanoma remain unresolved issues. In this report, a series of 30 melanocytic lesions removed from 28 patients, including atypical Spitz nevi/tumors and metastasizing Spitzoid tumors/melanomas, were evaluated by a panel of dermatopathologists to evaluate interobserver diagnostic concordance and to assess the prognostic power of histological criteria. For inclusion in the study, each lesion had to display some criteria for the Spitz nevus, and in addition one of the following was required: (1) definitive clinical outcome such as metastasis or death of disease, or (2) long-term follow-up if the patient remained disease free. Each lesion was reviewed independently and blinded as to the clinical data by 10 pathologists, who categorized them as (1) typical Spitz nevus/tumor, (2) atypical Spitz nevus/tumor, (3) melanoma, (4) tumor with unknown biological potential, or (5) other melanocytic lesion. There was limited discussion of criteria before the review. Evaluation of 17 Spitzoid lesions yielded no clear consensus as to diagnosis; in only one case did six or more pathologists agree on a single category, regardless of clinical outcome. Notably, however, some lesions that proved fatal were categorized by most observers as either Spitz nevi or atypical Spitz tumors. Conversely, seven or more pathologists scored 13 lesions as melanoma. These results illustrate (1) substantial diagnostic difficulties posed by many Spitz tumors, especially those with atypical features, even among experts, and (2) the lack of objective criteria for their distinction from melanoma and for gauging their malignant potential. Nevertheless, our observations do suggest that a biological relationship exists between the Spitz nevus/tumor and melanoma.

Adolescent↗

Treatment of unfavorable myelodysplastic syndrome with all-trans-retinoic acid and subcutaneous Ara C.

Thirty patients with high-risk myelodysplastic syndrome, defined as > 5% bone marrow blasts, were treated with a combination of oral all-trans-retinoic acid (45 mg/m2 daily) and subcutaneous AraC (10 mg/m2) on days 1-14 of each 28-35 day cycle repeated for 2-6 cycles. Complete remission lasting 9, 12, and 15 months was achieved in three patients. Partial and minor response did not translate into meaningful clinical improvement, like complete responders. Overall incidence of leukemia transformation and survival of this cohort of patients was no different from the expected outcome for a group of patients with similar characteristics.

Adult↗