Thoracic lipoblastoma in a 6-year-old African male: a case report.

Lipoblastoma is a very infrequent tumor, characteristic of early childhood. The thoracic location is infrequent, with isolated reports to date. We present the case of a 6-year-old male patient with a right thoracic tumor of months of evolution that was surgically removed by right anterolateral thoracotomy and in which the diagnosis of classic well-differentiated lipoblastoma was histologically confirmed. The patient evolved favorably and was discharged. He is currently under follow-up and without recurrence 1 year after surgery. This is, to our knowledge, the first thoracic lipoblastoma reported in an African pediatric patient. The importance of knowing the clinical, semiological, and intraoperative characteristics of this tumor becomes even more important, as in our case, in the context of international cooperation, where in many cases, there is no possibility of performing pre-operative imaging studies or subsequent genetic studies.

El lipoblastoma es un tumor muy infrecuente, característico de la primera infancia. La localización torácica es infrecuente, con reportes aislados hasta la fecha. Presentamos el caso de un paciente varón de 6 años con una tumoración torácica derecha de meses de evolución que fue extirpada quirúrgicamente mediante toracotomía anterolateral derecha y en la que se confirmó histológicamente el diagnóstico de lipoblastoma clásico bien diferenciado. El paciente evolucionó favorablemente y fue dado de alta. Actualmente se encuentra en seguimiento y sin recidiva un año después de la cirugía. Este es, hasta donde sabemos, el primer lipoblastoma torácico reportado en un paciente pediátrico africano. La importancia de conocer las características clínicas, semiológicas e intraoperatorias de este tumor cobra aún más importancia, como en nuestro caso, en el contexto de la cooperación internacional, donde en muchos casos no existe la posibilidad de realizar estudios de imagen preoperatorios ni estudios genéticos posteriores.

Pediatric Rectosigmoid Atypical Juvenile Polyps Presenting With Rectal Prolapse and Acute Bleeding: A Case Report and a Comprehensive Literature Review.

Rectosigmoid solitary juvenile polyps are benign lesions, relatively frequent in childhood. The clinical debut of a pediatric polyp with bleeding is relatively frequent, but there are very few reports of rectal prolapse of polyps. We present the case of a 7-year-old female patient with no previous history who presented with rectal prolapse of a polyp with acute bleeding. An urgent endoscopic examination was performed and 2 rectosigmoid polypoid lesions were found and resected. The anatomopathological study showed that these were 2 hamartomatous polyps with mild dysplasia. The patient is asymptomatic and is being followed up. The literature concerning rectal prolapse of polyps in the pediatric population is scarce. In a pediatric patient with a rectal prolapse, this entity should be considered in the differential diagnosis.

Superior Vesical Fissure as an Incomplete Form of Bladder Exstrophy: A Case Report with Clinico-Pathological Correlation and a Comprehensive Literature Review.

Multiple variants of classic bladder exstrophy have been described, all of them infrequent. Superior vesical fissure is a mild variant of this pathology in which genital involvement is scarce or absent. To date, there are only isolated reports of this entity. We report a full-term female patient of Arabian descent with a clinical and radiological diagnosis of superior vesical fissure that was surgically corrected in our center with a favorable evolution. Histological study, supported by immunohistochemical techniques, showed squamous and transitional epithelium and discrete chronic inflammation. Our literature review identified 26 reports of superior vesical fissure (including ours), with high heterogeneity in terms of clinical characterization and associated malformations and with only two histological reports. The clinical evolution of the patients reported in the literature was favorable, with lower morbidity and mortality than in classical forms of bladder exstrophy.

Sea-blue histiocytosis.

A 78-year-old woman with hypertrophic cardiomyopathy underwent a septal myomectomy and valve replacement. In the immediate postoperative period she developed shock of mixed etiology and died. At autopsy, hepatomegaly and splenomegaly were identified, with PAS and Giemsa positive intracellular ceroid granular deposits. Sea-blue histiocytosis is an extremely rare, chronic and benign deposit disease. It is characterized by hepatosplenomegaly, thrombocytopenia and lymphadenopathy. The presence of ceroid substance in granules in PAS and Giemsa stains should establish the diagnosis of suspicion.

Congenital Meatal Urethral Stenosis in a Female Patient: A Case Report.

We present the case of a 6-year-old girl who presented with alterations in the voiding stream. On physical examination, a very small urethral meatus was identified at the expense of a membrane. The renovesical ultrasound showed no alterations. An uroflowmetric study was performed, showing a bladder outlet obstruction pattern. The urethral meatus was calibrated and a ventral meatotomy was performed. The histological study of the resected membrane showed a transitional urethral mucosa with chronic focal inflammation and discrete hyperplasia. The patient evolved favorably, with resolution of the symptoms and no notable complications. This is, to the best of our knowledge, the first reported case with a histological study of a congenital meatal urethral stenosis. In the presence of lower urinary tract obstruction, this entity should be considered in the differential diagnosis. Surgical treatment is curative.

Coagulation Profile: Alterations and Diagnostic Yield in Pediatric Acute Appendicitis: A Prospective Validation Study.

Background: The literature regarding alterations in the coagulation profile in pediatric acute appendicitis (PAA) is scarce and mainly limited to retrospective studies. Evidence on the diagnostic yield of coagulation parameters is limited to fibrinogen. Patients and Methods: This is a prospective study with 151 patients divided into two groups: patients with nonsurgical abdominal pain (NSAP) in whom the diagnosis of PAA was excluded (n = 53) and patients with a confirmed diagnosis of PAA (n = 98). In 93 patients (62%), a coagulation study was obtained at the time of diagnosis and international normalized ratio (INR), activated partial thromboplastin time (aPTT), d-dimer, platelets, mean platelet volume, and platelet-to-lymphocyte ratio were analyzed. The PAA group was further classified into complicated (n = 19) and non-complicated PAA (n = 40). Quantitative variables were compared between groups using the Mann-Whitney U test. Diagnostic performance of the coagulation profile was evaluated with the area under the receiver operating characteristic (ROC) curves. Results: Patients with NSAP had lower median levels of INR, fibrinogen and d-dimer than those with PAA. Moreover, patients with complicated PAA had higher median values of INR and fibrinogen. None of the patients needed specific treatment for the correction of coagulopathy. Fibrinogen was the parameter with the highest diagnostic yield for distinguishing between NSAP and PAA (area under the curve [AUC], 0.74; 95% confidence interval [CI], 0.65-0.85), as well as between complicated versus non-complicated PAA (AUC, 0.71; 95% CI, 0.57-0.86). Conclusions: This study found a moderate extrinsic pathway coagulopathy in patients with PAA, especially in complicated PAA. Fibrinogen is a parameter with moderate diagnostic yield for the diagnosis of PAA.

Cooperation and Scientific Contribution: The Search for a Common Nexus.

International cooperation in pediatrics and pediatric surgery entails important barriers such as sociocultural differences, language difficulties, lack of infrastructure and resources, and short duration of campaigns. In this work, we share our personal experience in relation to the scientific publication of works carried out in pediatric international cooperation, and we make a critical reflection on the aspects to be considered for this field to develop in the future.

Giant Perineal Plexiform Neurofibroma in an 8-Year-Old African Male.

The perineal presentation of plexiform neurofibroma is exceptional, with only two cases reported to date.We present an 8-year-old African male with a large perineal tumor of years of evolution. He had no associated symptoms. Café au lait stains were observed on examination, without other findings of relevance. The patient had no preoperative radiological studies. Partial excision of the lesion was performed. Histopathological study of the specimen revealed a plexiform neurofibroma.The lack of diagnostic suspicion due to the atypical nature of the location, the anatomical complexity of surgical resection and the potential urological and rectal involvement make this lesion a diagnostic-therapeutic challenge. Among the differential diagnoses, schwannoma, congenital lipoma, hamartoma and lipoblastoma should be considered.

Gastric-phenotype Mucinous Carcinoma of the Fallopian Tube with Secondary Ovarian Involvement in a Woman with Peutz-Jeghers Syndrome: A Case Report.

Peutz-Jeghers syndrome is an autosomal dominant condition characterized by the association of hamartomatous polyps in the digestive tract, mucocutaneous pigmentation, family history, and infrequently tumors of the female genital tract with one of the most characteristic being the gastric-type endocervical adenocarcinoma. We present the case of a 75-year-old woman with a history of gastrointestinal polyps and cancer of the pancreas and breast, diagnosed with Peutz-Jeghers syndrome, who clinically debuted with a primary adnexal tumor. However, on histologic examination it was found to be a gastric-phenotype primary mucinous carcinoma tubal in origin, associated to tubal mucinous metaplasia and secondary ovarian involvement. One of her daughters had a confirmed genetic diagnosis of Peutz-Jeghers syndrome and presented with mucinous metaplasia of the tubal mucosa in the pathological study of a prophylactic hysterectomy specimen. Another of her daughters died from an ovarian juvenile granulosa cell tumor, she did not have a genetic diagnosis of Peutz-Jeghers syndrome. This case intends to highlight the rarity of gastrointestinal-type mucinous carcinomas of the ovary and fallopian tube (similar to gastric-type endocervical adenocarcinoma) in Peutz-Jeghers syndrome and emphasize the importance of genetic counseling of these patients as well as the adequate sampling of surgical specimens for early detection and treatment.

Extensive Keloid and Hypertrophic Mixed Scarring Pattern in Ear Lobes of a 14-Year-Old African Female: A Case Report.

Background Massive earlobe scarring/keloid formation can occur after ear piercing in individuals of African descent. Case report: A 14-year-old African girl with pierced ears in childhood presented with two progressively growing and disfiguring tumors on both earlobes. The maximum diameter of each lesion was 5.5 centimeters, and the weight of each lesion was approximately 20 grams. Histologically, there was a mixed pattern of keloid and hypertrophic scarring. Discussion: Massive keloids can occur after ear piercing in childhood. It is unclear why some individuals develop these massive keloids.