Basal-Cell Carcinoma Occurring in Cutaneous Infundibular Cysts: Report of 2 Cases and Review of the Literature.

Cysts lined by stratified squamous epithelium indistinguishable from the epidermis, referred to as epidermoid cysts, epidermal inclusion cysts, and infundibular cysts, are the most common type of cyst occurring in the skin. They are invariably benign, and malignant neoplasms arising within the wall of such cysts are distinctly uncommon. Even basal-cell carcinoma, which is the most common cutaneous malignant neoplasm of the skin, has rarely been reported to occur in association with epidermoid cysts. The authors report their experience studying 2 patients with basal-cell carcinoma arising in association with an epidermoid cyst. These cases highlight the need to examine, histopathologically, tissue from this common and usually benign lesion. The authors also review the medical literature.

Clear-cell melanocytic lesions with balloon-cell and sebocyte-like melanocytes: a unifying concept.

Melanocytes may assume unique shapes and sizes but rarely have clear cytoplasm. We studied 28 melanocytic lesions that contained clear-cell melanocytes of the balloon-cell and sebocyte-like types. Clear-cell melanocytes were found more commonly in females (64%) than in males (36%), with predominance in females younger than 50 years (79%) and predominance in males older than 50 years (67%). They were distributed evenly throughout the body but were not found on acral sites. Clear-cell melanocytes were most prevalent in congenital nevi (18 or 72%) but were also found in 5 Clark nevi, 2 Meischer nevi, 1 Unna nevus, 1 atypical intra-epidermal proliferation, and 1 melanoma. The clear cells were distributed diffusely in 86% of the lesions and focally in 14%. The overall percentage of clear-cell melanocytes was 56%. The percentage of balloon cells was 57% and sebocyte-like melanocytes 32%. Clear cells with morphologic features of both balloon cells and sebocyte-like melanocytes, that is, intermediate cells, were present in all lesions with an overall percentage of 12%. The presence of melanocytes of both the balloon-cell and sebocyte-like types and the finding of clear-cell melanocytes with intermediate features in all lesions lends support to the theory that balloon-cell and sebocyte-like melanocytes may represent morphologic expressions of the same alteration in melanogenesis.

[Laparoscopic approach of achalasia. Long-term clinical and functional results and quality of life]. / Abordaje laparoscópico de la acalasia. Resultados clínicos, de calidad de vida y funcionales a largo plazo.

INTRODUCTION: Laparoscopic Heller myotomy has become the gold standard procedure for patients with achalasia. This study evaluates the clinical status, quality of life, and functional outcomes after laparoscopic Heller myotomy. MATERIAL AND METHODS: We analyzed patients who underwent laparoscopic Heller myotomy with an associated anti-reflux procedure from October 1998 to December 2010. Before surgery, we administered a clinical questionnaire and as of 2002, we also evaluated quality of life using a specific questionnaire (GIQLI). In 2011, we performed a follow up for all available patients. We administered the same clinical questionnaire and quality of life test as before surgery and performed manometry and 24-hour pH monitoring. According to the length of follow up, patients were divided into 3 groups. Group 1 with a follow-up between 6 and 47 months; group 2 follow-up between 48 and 119 months, and group 3 with a follow-up of more than 120 months). Moreover, 27 patients had already been evaluated with this same protocol in 2003. Pre- and postoperative data were compared for the 3 groups and for patients who completed follow up in 2003 and 2011. RESULTS: Ninety-five patients underwent laparoscopic Heller myotomy. Seventy-six (80%) were available for follow-up. Mean follow-up was 56 months (range 6-143). Global improvement in dysphagia was 89%. Total DeMeester score decreased in the 3 groups. GIQLI scores improved after surgery, reaching normal values. Manometric determinations showed normal LES pressures after myotomy in the 3 groups. Ten percent of overall 24-hour pH monitoring was abnormal. The group of patients followed up in 2003 and in 2011 showed no impairment in the variables studied in the long term. CONCLUSIONS: Long-term follow up of the laparoscopic approach to achalasia showed good results concerning clinical status and quality of life, with normal sphincteric pressures and a low incidence of gastroesophageal reflux.

Basal cell carcinoma with myoepithelial differentiation.

A lesion from the left cheek of a 48-year-old man showed a neoplasm composed primarily of cells with eccentric crescent-shaped nuclei and abundant, homogenous, eosinophilic cytoplasm resembling signet ring cells. Immunohistochemical studies showed the cells to stain positively for pan cytokeratin and smooth muscle actin, indicating myoepithelial differentiation (MED). Foci of conventional basal cell carcinoma were present, and cells with MED were also admixed within some of the aggregations of basal cell carcinoma. On the basis of these findings, we interpreted this lesion to represent basal cell carcinoma with MED. A review of the existing literature of basal cell carcinomas with similar morphologic features is also presented.

[Surgical treatment of achalasia: Better than dilations?]. / Tratamiento quirúrgico de la acalasia: ¿mejor que las dilataciones?

Achalasia is an infrequent esophageal disease that severely impairs the quality of life of affected individuals. The etiology of this entity is not well defined and its main clinical features are dysphagia and regurgitation. The treatment of achalasia continues to be palliative and is aimed at providing functional and symptomatic relief through opening of the lower esophageal sphincter. The present article describes and evaluates the medical and surgical treatments most commonly used in clinical practice after the introduction of minimally invasive surgery, which has represented an important addition to the therapeutic alternatives. Currently, the most appropriate initial option is laparoscopic surgery, while pneumatic dilatation and botulinum toxin injection should be reserved for selected patients.

Seasonal variation of transient acantholytic dyskeratosis (Grover's disease).

BACKGROUND: Grover's disease (GD), or transient acantholytic dermatosis, is a pruritic, papulovesicular eruption characterized histopathologically by acantholysis with or without dyskeratosis. The origin of GD is unknown. Suggested causes include sweating, heat, immobilization occlusion, external beam and ultraviolet radiation, and xerosis. GD has also been found to occur in association with other diseases. OBJECTIVE: Our aim was to assess whether GD exhibits seasonal variation and, if so, to determine whether any inferences can be drawn from its seasonal variation regarding its cause. METHODS: We identified 385 patients who fulfilled both clinical and histopathologic criteria for GD among 423,106 patients diagnosed at the Ackerman Academy of Dermatopathology in New York City during the period from July 1, 1999 through June 30, 2004. By design, no hospitalized patients were studied. RESULTS: A diagnosis of GD was given to 0.09% of biopsy specimens at the Ackerman Academy of Dermatopathology. GD was diagnosed approximately 4 times more commonly in winter than in summer, although the number of biopsies was constant. The average age of GD patients was 64 years with a male/female ratio of 1.95:1. The most common histopathologic type of GD was pemphigus vulgaris. GD was suspected clinically in 54% of patients. LIMITATIONS: This study did not assess hospitalized patients with GD or GD patients who lived outside the northeastern United States. Because the data assessed resided in a commercial dermatopathology laboratory, patients assessed in almost all cases had insurance coverage. Patients without insurance likely were not included in the study. CONCLUSIONS: The diagnosis of GD constitutes a higher proportion of biopsies in the winter than in the summer and therefore, by inference, occurs more frequently in the winter. In the winter, elderly men whose skin is naturally xerotic sweat less and are exposed to low ambient humidity. Rather than being caused by sweating and heat, GD arises against a backdrop of an intact but xerotic epidermis with decreased sweat production and is likely related to impaired epidermal integrity.

Diagnostic value of potent acid inhibition in gastro-oesophageal reflux disease.

Gastro-oesophageal reflux disease (GORD) is defined as 'Chronic symptoms or mucosal damage produced by abnormal reflux of gastric contents into the esophagus'. The Genval Workshop Report defines that GORD exists when the frequency of heartburn is equal to or greater than 2 days/week and that it is one of the most common gastrointestinal conditions in the general population. Endoscopy is the most recommendable exploratory procedure in a patient with symptoms of GORD, fundamentally heartburn and regurgitation. However, 50-75% of the patients with symptoms compatible with GORD have normal endoscopy. Thus, endoscopy does not appear to be indispensable in a large group of patients with GORD. Endoscopy is therefore the gold standard for the diagnosis of reflux oesophagitis (histopathological changes in the oesophageal mucosa), but there is no gold standard for the diagnosis of non-erosive GORD. Twenty-four-hour pH monitoring has come to be considered the most sensitive and specific test in the diagnosis of GORD, but a significant proportion of patients (about 25%) have symptoms compatible with GORD and have 24-h pH monitoring results that can be considered normal. Besides, demonstrating the presence of acid reflux alone does not prove that it is the cause of suspected GORD-related signs or symptoms. Therefore, despite 'positive' pH studies, there is a significant number of patients failing to respond to therapy, mainly ear, nose and throat complaints, supposed as manifestations of gastro-oesophageal reflux disease. Despite 24-h oesophageal pH testing being an excellent diagnostic tool, it has no utility in routine clinical settings and hence its availability should be limited to tertiary care settings. With the demonstration that antisecretory treatment with high doses of proton pump inhibitors (PPIs) for 1 week or 2 weeks achieves significant improvement or even remission of the symptoms of GORD, it not surprising that it has been proposed as a diagnostic test for the disease. For patients with symptoms compatible with GORD without alarm symptoms or other suspected complications of GORD, a short course of empiric PPI therapy gives valuable information about the presence of GORD. The PPI test is a simple, sensitive and cost-effective tool, but it has insufficient specificity for use as an objective criterion alone. The use of PPIs both as a diagnostic test (1-2 weeks) and as a diagnostic-therapeutic test (1-4 months) has a moderate usefulness and may be used especially in those environments in which there are difficulties in performing the objective test.

Granular parakeratosis: pathologic and clinical correlation of 18 cases of granular parakeratosis.

BACKGROUND: Granular parakeratosis (originally termed axillary granular parakeratosis) is an idiopathic, benign, nondisabling cutaneous disease that manifests with intertriginous erythematous, brown or red, scaly or keratotic papules and plaques. It presents in all age groups and has no established clinical associations. OBJECTIVES: We wanted to assess the following: (1) the incidence of granular parakeratosis in biopsy specimens; (2) the differential diagnosis submitted with specimens requisition of granular parakeratosis; and (3) variations in its histopathology. METHODS: Between July 1, 1999 and December 31, 2003, 363,343 specimens were submitted to The Ackerman Institute of Dermatopathology in New York. A computer search was done of these specimen records and cases diagnosed with granular parakeratosis were reviewed. A thickened stratum corneum with retention of keratohyalin granules was considered diagnostic of granular parakeratosis. RESULTS: Eighteen of 363,343 specimens (0.005%) were diagnosed with granular parakeratosis. The diagnosis was confirmed by re-review of specimens. All lesions were located in the axillae. All patients were adults; most were women. In only one instance was the correct clinical diagnosis of granular parakeratosis submitted with a biopsy requisition of it. Granular parakeratosis is rare, if its incidence among biopsy specimens is representative of its true prevalence. The failure to include granular parakeratosis on biopsy requisition forms of granular parakeratosis specimens indicates that dermatologists are not familiar with it. Variations of the histopathology of the 18 cases occurred but did not correlate with the clinical impressions included on biopsy requisition forms.

Effect of long-term acid gastric inhibition on bacterial translocation in cirrhotic rats.

BACKGROUND: Bacterial translocation (BT) related to intestinal bacterial overgrowth (IBO) plays an important role in the pathogenesis of bacterial infections in cirrhosis. Inhibition of acid gastric secretion promotes IBO and might favor BT. We evaluated the effect of long-term inhibition of acid gastric secretion on BT in cirrhotic rats. METHODS: Cirrhotic rats with and without ascites induced by oral CCl4 and controls were randomized to treatment with a daily subcutaneous injection of placebo, ranitidine (50 mg/kg), or pantoprazole (8 mg/kg) during 2 weeks. Continuous pH-metry was performed for 2 h before and at the end of treatment; thereafter, a laparotomy to obtain samples of blood, mesenteric lymph nodes, ascites, spleen, liver, and cecal stools was performed. RESULTS: Ranitidine and pantoprazole increased gastric pH as compared with placebo (P<0.001). However, antisecretory drugs increased the incidence of BT only in ascitic rats treated with ranitidine (P<0.05) or pantoprazole (P=0.07) when compared with placebo-treated ascitic rats or cirrhotic rats without ascites treated with the same drug. Cirrhotic ascitic rats treated with pantoprazole showed a trend toward an increased incidence of IBO (P=0.08), a higher ileal malondialdehyde level (P<0.01), and an increased production of tumor necrosis factor-α (P<0.05). CONCLUSION: Although inhibition of acid gastric secretion increased gastric pH in all animals, the incidence of BT increased only in ascitic rats, and it was associated with a trend toward an increase in IBO incidence, a higher ileal malondialdehyde level, and an increased production of serum tumor necrosis factor-α. Therefore, antisecretory drugs should be carefully administered to cirrhotic ascitic patients.

Twenty-two years' survival of metastatic gastrinoma evidenced recently by somatostatin-receptor-specific scintigraphy.

An 18-year-old male presented in 1979 with a gastrinoma of unknown primary origin. Massive upper-digestive haemorrhage led to total gastrectomy, at which histology evidenced liver metastases, confirmed 9 months later at reoperation for an intestinal occlusion. Postoperative morphological evidence of liver metastases was repeatedly negative using abdominal ultrasound and computerized tomography (CT) scans and magnetic resonance imaging (MRI), but a recent somatostatin-receptor-specific scintigraphy (Octreoscan) was positive only in the liver area. Twenty-two years after diagnosis, the primary tumour has not been identified, the patient leads a normal life, and his circulating gastrin levels, although still elevated at 317-550 pg/ml (normal < 127 pg/ml), have fallen over recent years from > 1000 pg/ml. We discuss the relevance of the described prognostic factors.

Abordaje laparoscópico de la acalasia. Resultados clínicos, de calidad de vida y funcionales a largo plazo / Laparoscopic approach of achalasia. Long-term clinical and functional results and quality of life

INTRODUCCIÓN: La miotomía laparoscópica se ha convertido en el tratamiento de elección en los pacientes con acalasia. Este estudio evalúa a largo plazo el estado clínico, de calidad de vida y los resultados funcionales posteriores a la miotomía de Heller laparoscópica. MATERIAL Y MÉTODOS: Se analizó a los pacientes diagnosticados de acalasia primaria e intervenidos mediante una miotomía de Heller laparoscópica con un procedimiento antirreflujo desde octubre de 1998 hasta diciembre de 2010. Previamente a la intervención, se evaluó la sintomatología de los pacientes mediante un cuestionario específico (escala DeMeester) y desde el 2002 se evaluó la calidad de vida mediante un test validado (GIQLI). En el año 2011 se reevaluó el estado clínico y de calidad de vida, y además se realizó una manometría y una pHmetría de 24h. En función del tiempo de seguimiento, los pacientes fueron divididos en 3 grupos (grupo 1: seguimiento entre 6 y 47 meses; grupo 2: seguimiento entre 48 y 119 meses y grupo 3, seguimiento superior a 120 meses). Además, 27 pacientes de la serie fueron evaluados con el mismo protocolo en 2003 y reevaluados en 2011. RESULTADOS: Noventa y cinco pacientes fueron incluidos en el estudio. El 80% (n = 76) estuvieron disponibles para realizar el seguimiento. El tiempo de seguimiento medio fue de 56 meses (rango 6-143 meses). La mejoría global de la disfagia fue del 89%. Los valores estudiados mediante la escala de DeMeester descendieron significativamente en los 3 grupos y los índices de calidad de vida se normalizaron posteriormente a la cirugía. Las determinaciones manométricas mostraron la normalización mantenida en la presión del esfínter esofágico inferior en todos los grupos. Se detectó una incidencia de reflujo gastroesofágico del 10%. En el subgrupo de pacientes analizados prospectivamente en 2003 y 2011, no se demostró empeoramiento de los resultados a largo plazo. CONCLUSIONES: La miotomía de Heller laparoscópica mejora la clínica y la calidad de vida de los pacientes con acalasia primaria manteniendo las presiones del esfínter esofágico inferior normales a largo plazo, con una baja tasa de incidencia de reflujo gastroesofágico

INTRODUCTION: Laparoscopic Heller myotomy has become the gold standard procedure for patients with achalasia. This study evaluates the clinical status, quality of life, and functional outcomes after laparoscopic Heller myotomy. MATERIAL AND METHODS: We analyzed patients who underwent laparoscopic Heller myotomy with an associated anti-reflux procedure from October 1998 to December 2010. Before surgery, we administered a clinical questionnaire and as of 2002, we also evaluated quality of life using a specific questionnaire (GIQLI). In 2011, we performed a follow up for all available patients. We administered the same clinical questionnaire and quality of life test as before surgery and performed manometry and 24-hour pH monitoring. According to the length of follow up, patients were divided into 3 groups. Group 1 with a follow-up between 6 and 47 months; group 2 follow-up between 48 and 119 months, and group 3 with a follow-up of more than 120 months). Moreover, 27 patients had already been evaluated with this same protocol in 2003. Pre- and postoperative data were compared for the 3 groups and for patients who completed follow up in 2003 and 2011. RESULTS: Ninety-five patients underwent laparoscopic Heller myotomy. Seventy-six (80%) were available for follow-up. Mean follow-up was 56 months (range 6-143). Global improvement in dysphagia was 89%. Total DeMeester score decreased in the 3 groups. GIQLI scores improved after surgery, reaching normal values. Manometric determinations showed normal LES pressures after myotomy in the 3 groups. Ten percent of overall 24-hour pH monitoring was abnormal. The group of patients followed up in 2003 and in 2011 showed no impairment in the variables studied in the long term. CONCLUSIONS: Long-term follow up of the laparoscopic approach to achalasia showed good results concerning clinical status and quality of life, with normal sphincteric pressures and a low incidence of gastroesophageal reflux

Are all melanomas the same? Spitzoid melanoma is a distinct subtype of melanoma.

BACKGROUND: Although the majority of melanomas demonstrate high rates of mutations in B-RAF or N-RAS that result in constitutive activation of the mitogen-activated protein kinase-signaling pathway, emerging data suggest molecular differences among melanoma subtypes. In this study, the authors evaluated the contribution of B-RAF and N-RAS mutations to the pathogenesis of Spitzoid melanomas. METHODS: In total, 33 Spitzoid melanomas were analyzed for clinical and pathologic characteristics as well as for hot-spot mutations in the B-RAF and N-RAS genes. In the majority of patients (28 of 33 melanomas), the tumors were confined to the skin with no evidence of metastasis (average follow-up, 32.5 mos). There were five metastasizing melanomas (5 of 33 tumors) with regional or systemic spread. RESULTS: Of 33 Spitzoid melanomas, only 1 showed the V600E mutation in the B-RAF gene (1 of 33 tumors; 3%). It was noteworthy that none of the metastatic Spitzoid melanomas (0 of 5 tumors; 0%), of which 2 resulted in fatal outcomes, demonstrated mutations in B-RAF or N-RAS. CONCLUSIONS: In contrast to the majority of cutaneous melanomas, activating hot-spot mutations in B-RAF or N-RAS were not involved in the pathogenesis of Spitzoid melanoma. These data suggested that Spitzoid melanoma is a distinct form of melanoma with unknown genes and/or signaling pathways involved in its development.