Search

The perinucleolar compartment (PNC) is a small nuclear body that plays important role in tumorigenesis. PNC prevalence correlates with poor prognosis and cancer metastasis. Its expression in pediatric Ewing sarcoma (EWS) has not previously been documented. In this study, we analyzed 40 EWS tumor cases from Caucasian and Hispanic patients for PNC prevalence by immunohistochemical detection of polypyrimidine tract binding protein and correlated the prevalence with dysregulated microRNA profiles. EWS cases showed staining ranging from 0 to 100%, which were categorized as diffuse (≥77%, n = 9, high PNC) or not diffuse (

Background: Surgical care is an essential component to achieve universal health coverage. An increase in children's surgical burden is recognized worldwide, but changes in the epidemiological profile associated with global geographic heterogeneity are to be expected. The aims of this study are to analyse the prevalence and epidemiological changes of surgical activity in a tertiary center in Mexico. Methods: Ambispective cohort study. Institutional records were reviewed searching for children’s surgeries in the period comprising 2001-2017. The variables analyzed were age, sex, number of surgeries per subject, and mortality rate; regarding the interventions, kind of inward stay, kind of surgery (ICD 9 CM), ward or department responsible, and perioperative mortality rate (POMR). Results: Of the 104,578 institutional admissions, 37% were surgical procedures of preschool-aged children (30.2%), and infants (27.3%). These tended to decrease, while for adolescents to increase. A total of 84,859 surgeries were performed (1.36 per operating room/day), averaging 4.992 per year, with a marginal tendency to increase. 65.8% of procedures required hospitalization. The systems more frequently intervened were: musculoskeletal system (20.1%, tending to increase), digestive system (19.5%), and cardiovascular system (11.3%). The overall mortality rate of surgical admissions was 5.5%, with POMR of 1.54%. Conclusion: The institutional epidemiological profile has changed, and relevant trends were identified. We corroborated the presence of global geographic heterogeneity with analogous hospitals

There are no pathognomonic histopathological features to distinguish acute graft-vs-host disease (aGVHD) from skin drug reactions (SDRs) in pediatric patients with multiple drug regimens that have received blood transfusions and/or transplants. We aimed to determine if the addition of apoptosis markers is helpful to distinguish aGVHD from SDRs in these patients.Skin biopsy specimens from patients with a clinical diagnosis of aGVHD or SDRs were evaluated for the presence of apoptotic bodies, satellitosis, interface damage, vasculitis, and inflammatory infiltrate on HE stain. Information was completed with apoptotic markers (transferase-mediated dUTP nick end-labeling [TUNEL], bcl-2, and caspase-3).The skin biopsy specimens of 32 patients with aGVHD and 11 with SDRs were included for study. Only the number of apoptotic keratinocytes per 10 high-power fields (hpf) showed a significant difference between both groups (P = 0.02); the presence of ≥4 apoptotic keratinocytes per 10 hpf was identified as the optimal cut-off point to discriminate aGVHD from SDRs. No SDRs cases had follicular apoptotic cells. TUNEL, bcl-2, and caspase-3 determination showed no difference between both groups.The presence of ≥4 apoptotic keratinocytes per 10 hpf (in aGVHD) and the absence of follicular apoptotic cells (in SDRs) might be a useful marker to distinguish between them.

Background Tuberculosis is considered an emerging disease worldwide; in the last 10 years, its incidence has increased to more than 9.6 million cases of active tuberculosis. In 2014, it resulted in 1.5 million patient deaths. However, oral presentation with bone involvement occurs in less than 3% of all reported cases and rarely arouses clinical suspicion on initial presentation. Case presentation A 15-year-old Mexican girl who had a previous diagnosis of neurofibromatosis presented to our hospital with pain and swelling in the region of the left mandibular body since November 2011. A clinical examination revealed pain in the mandibular region, a mass of soft consistency that seemed to involve bone, and a fistula with discharge of intraoral purulent material. Additionally, tachycardia and hyperthermia were observed. The left submental and submandibular regions had a 12-cm-diameter swelling, which was well-delineated and nonerythematous. The final diagnosis was established by real-time polymerase chain reaction. Conclusions The final diagnosis of rare cases of tuberculous osteomyelitis in the jaw can be established by deoxyribonucleic acid (DNA) identification of Mycobacterium tuberculosis in the lesion. Simple and fast complementary diagnosis by real-time polymerase chain reaction is a fundamental approach to establishing early and effective pharmacological and surgical treatment.

Indeterminate cell histiocytosis is a rare disorder involving altered homing mechanisms of the cutaneous histiocytic/dendritic system. It has been described predominantly in adults, with less than a dozen cases in children. A 13-year-old adolescent girl presented with a 4-year history of asymptomatic erythematous nodules and plaques, measuring from 1 to 5 cm in diameter, that were located mainly on the trunk and proximal portions of her limbs. A skin biopsy showed dermal diffuse infiltration of histiocytic cells. Most of the histiocytic cells were strongly positive for S100 protein. No Birbeck granules were found. Treatment with topical steroid was ineffective. After 6 months of pure coal tar and 5% 5-fluorouracil cream, an almost total clearing of lesions was observed. An accurate diagnosis of this condition is mandatory in order to avoid unnecessary treatments. Conservative management is also discussed.

Benign cephalic histiocytosis (BCH) is best understood as a form of non-Langerhans cell histiocytosis, specifically as an early mononuclear variant of juvenile xanthogranuloma (JXG). However, the progression of BCH into JXG in the same patient has only been reported once before. We describe the case of a 2-year-old girl with asymptomatic, large, ill-defined infiltrated flat plaques over both cheeks, in addition to isolated papules. A punch biopsy of a plaque revealed dermal infiltration by vacuolated and scalloped histiocytes positive for CD68 KP-1, and that lacked expression of CD1a and S-100 protein, favoring macrophages over Langerhans cells. Electron microscopy study showed comma-shaped intracytoplasmic bodies in the histiocytic cells leading to the diagnosis of BCH. One year later, after an episode of varicella-zoster infection, the flat plaques over the cheeks became large reddish-yellow nodules, and in a second biopsy appeared to progress to JXG. Virus-related mechanisms of progression are discussed.

Polypoid arteriovenous malformations, which are localized in the colon, are extremely rare in adults, with only 7 cases published to date. Here we present the case of a 6-year-old girl with a jejunal polypoid tumor that a sonogram and a computed tomographic scan have shown to resemble intussusception. Histologically, numerous large ectatic veins intermixed with small-caliber arteries, venules, arterioles, and capillaries were observed in the intestinal wall. A retrospective computed tomographic scan 3-dimensional angiographic reconstruction demonstrated that this may be a diagnostic characteristic. Clinical and morphologic comparisons with previously reported cases were discussed.

INTRODUCTION: Primary central nervous system (CNS) melanocytic tumors are extremely uncommon in children. Annual incidence is 1 per 10 million population and carry a poor prognosis. Herein we present two cases treated at our institution, a leading pediatric oncology center in Mexico. METHODS: Our first patient was a 1 year-old female who began with non-convulsive status epilepticus and no fever history. Right frontoparietal leptomeningeal enhancement was evident on MRI, and a primary leptomeningeal melanoma was diagnosed according to biopsy. Radiotherapy did not improved her condition and further neurologic deterioration ensued. The second patient was a 6 year old male with a history of headache and vomiting, and left sided hemiparesis was evident at admission. MRI showed an ill-defined right temporal lobe lesion with mass effect and leptomeningeal involment. Immature cells were isolated on cerebrospinal fluid (CSF) and a primary malignant melanocytic lesion was diagnosed on brain biopsy. Later he developed refractory status epilepticus and died of septic shock. DISCUSSION: Although melanocytes are normally present in the nervous system, primary melanocytic tumors are extremely uncommon, being less than 0.1% of all CNS tumors in children. Three mayor variants are distinguished: diffuse melanocytosis, meningeal melanocytoma and malignant melanoma. Complete surgical resection is not always possible, while the efficacy of radiotherapy and chemotherapy remain controversial. CONCLUSION: Our experience shows that these lesions are extremely rare in our population and cannot be safely resected. Diagnosis is made by biopsy exclusively and neurologic deterioration eventually occurs despite radiotherapy.

Dermal dendrocyte hamartomas are extremely rare; only two examples have been described with clinical features different from our cases and with incomplete immunohistochemical characterization. We report three female patients presenting a medallion-shaped, well-defined, slightly atrophic and asymptomatic congenital lesion. All 3 patients showed a fusiform-cell proliferation. Immunohistochemistry was positive for CD34, factor XIIIa, and fascin. Electron microscopy showed typical features of dermal dendrocytes. We believe that the lesions described represent a new, clinically and histopathologically distinct lesion originating in dermal dendrocytes. We propose to name it medallion-like dermal dendrocyte hamartoma.

Background Silvery hair and severe dysfunction of the central nervous system (neuroectodermal melanolysosomal disease or Elejalde syndrome) characterize this rare autosomal recessive disease. Main clinical features include silver-leaden hair, bronze skin after sun exposure, and neurologic involvement (seizures, severe hypotonia, and mental retardation). Large granules of melanin unevenly distributed in the hair shaft are observed. Abnormal melanocytes and melanosomes and abnormal inclusion bodies in fibroblasts may be present. Differential diagnosis with Chediak-Higashi syndrome and Griscelli syndrome must be done. Observations We studied pediatric patients with silvery hair and profound neurologic dysfunction. Immune impairment was absent. Age of onset of neurologic signs ranged from 1 month to 11 years; the signs included severe muscular hypotonia, ocular alterations, and seizures. Mental retardation since the first months of life was noted in 4 cases. Psychomotor development was normal in 3 cases, but suddenly the patients presented with a regressive neurologic process. Four patients died between 6 months and 3 years after the onset of neurologic dysfunction. One patient showed characteristic ultrastructural findings of Elejalde syndrome. Conclusions Elejalde syndrome is different from Chediak-Higashi and Griscelli syndrome and is characterized by silvery hair and frequent occurrence of fatal neurologic alterations. Psychomotor impairment may have 2 forms of presentation: congenital or infantile. Although Elejalde syndrome and Griscelli syndrome are similar, the possibility that they are 2 different diseases, although probably allelic related, is suggested.