Fetus in fetu (FIF) is an exceedingly rare congenital anomaly characterized by the presence of a partially developed fetus within the body of its twin. It is a rare entity with fewer than 200 cases documented worldwide. Typically presenting as an abdominal mass in early childhood, this condition remains a diagnostic challenge due to its infrequency and varied clinical manifestations. We present the case of a 1-year-old female child referred to the pediatric surgery department due to an abdominal mass detected by her parents. Physical examination revealed a firm, ill-defined mass in the right upper abdomen. Ultrasonography delineated a heterogeneous, calcified mass in the right upper quadrant. Laboratory investigations (complete blood count, viral markers), including tumor markers (AFP, beta-hCG), were within normal limits. A midline laparotomy was performed, and intraoperative findings revealed a well-encapsulated mass adherent to the retroperitoneum, adjacent to the liver. Careful dissection was carried out to preserve vital structures, and the mass was successfully removed without rupture. Histopathological examination confirmed the diagnosis of fetus in fetu, displaying a spectrum of tissues reminiscent of embryonic development, including skin, adnexal structures, brain tissue, mature cartilage, adipose tissue, bone marrow elements, and a partially developed vertebral column. After the surgical resection, the patient experienced an uneventful postoperative recovery. At one year of follow up the patient has shown no signs of recurrence. FIF is characterized by diagnostic challenges, often requiring postoperative histological confirmation. Histopathological examination confirmed the presence of FIF. Surgical excision, is crucial for favorable outcomes, especially in preventing potential complications. Comprehensive histopathological evaluation remains imperative for accurate diagnosis.
Abstract Background: Autosomal recessive hypercholesterolemia (ARH), a genetic disorder of the affecting lipid metabolism. We present a child with this disorder wo was long undiagnosed, for many years. Clinical Description: An 8-year-old girl presented with multiple soft, painless, progressively increasing since the age of 2 years. Although lipid profiles had been found to be abnormal, she was not evaluated nor treated till 8 years of age. On examination, the swellings were present over upper and lower limbs and buttocks. Management and Outcome: Low-density lipoprotein (LDL) levels were elevated. Histopathology of the lesions confirmed xanthoma with immunohistochemistry being positive for CD 68 and ki67 index - 15%–20%. Next-generation sequencing homozygous mutation involving intron 15 of chromosome variant c2312-1G > A , causing loss of function variant in gene LDL receptor. The child was treated with gradually increasing doses of atorvastatin, with periodic echocardiography. There was progressive lowering of LDL over 6 months. Conclusion: Ignorance among pediatricians regarding this rare entity of ARH may result in lack of initiation of treatment for years, which may lead to detrimental cardiovascular complications in later life. Genetic analysis and prompt treatment can help in improving lipid parameters.
Congenital pulmonary airway malformations (CPAM) are a spectrum of cystic and non-cystic anomalies arising from abnormal airway development in utero, with an incidence of 1 in 25,000 to 35,000 births. CPAM can present prenatally or postnatally with respiratory distress, recurrent infections, or occasionally as an incidental finding. This case series aims to highlight the clinical, radiological, and histopathological characteristics of CPAM through three pediatric cases, which include types 1, 2, and 3 CPAM.
Thymomas are not so common tumors that are encountered in day-to-day pathology reporting. The WHO system was proposed in 2015. Although, through its detailed reporting, the WHO elaborates all subtypes and morphological clinches to diagnosis, it was important to ascertain its reproducibility in our day-to-day reporting.The aims of the study were (1) to study the interobserver agreement, concordance rates, and variability in the classification of a large number of thymomas received in our department as per the WHO 2015, (2) to correlate the WHO subtype with Masaoka-Koga stage, and (3) to study the variations in demography of thymomas in Indian patients as compared to those reported in the literature.This retrospective study was done at a tertiary care teaching hospital with huge surgical oncology patient load, also pertaining to the cardiothoracic surgeries. It is predominantly an interobserver agreement design to study the reproducibility of the WHO 2015 classification on thymic epithelial tumors.Four pathologists have independently reviewed histopathology slides of 65 cases of thymomas and classified them into predefined categories. Kappa statistics was applied to the observations.There was a substantial interobserver agreement in overall classification of thymomas with a Cohen's kappa score of 0.66. A better score was achieved for the classification of Group B thymomas. The WHO subtypes correlate well with the Masaoka-Koga staging system, and this finding is statistically significant. This article also presents the clinical details of a large number of thymoma cases.The new WHO classification has good reproducibility among pathologists in thymoma reporting.
'/%3e%3cuse xlink:href='%23a' transform='rotate(23.036 .093 25.536)'/%3e%3cuse xlink:href='%23a' transform='rotate(45.87 1.273 16.18)'/%3e%3cuse xlink:href='%23a' transform='rotate(69.945 .996 12.078)'/%3e%3cuse xlink:href='%23a' transform='rotate(20.66 -19.689 31.932)'/%3e%3c/svg%3e)
'%3e%3ccircle r='20' fill='%23008'/%3e%3ccircle r='17.5' fill='%23fff'/%3e%3ccircle r='3.5' fill='%23008'/%3e%3cg id='d'%3e%3cg id='c'%3e%3cg id='b'%3e%3cg id='a' fill='%23008'%3e%3ccircle r='.875' transform='rotate(7.5 -8.75 133.5)'/%3e%3cpath d='M0 17.5.6 7 0 2l-.6 5L0 17.5z'/%3e%3c/g%3e%3cuse xlink:href='%23a' transform='rotate(15)'/%3e%3c/g%3e%3cuse xlink:href='%23b' transform='rotate(30)'/%3e%3c/g%3e%3cuse xlink:href='%23c' transform='rotate(60)'/%3e%3c/g%3e%3cuse xlink:href='%23d' transform='rotate(120)'/%3e%3cuse xlink:href='%23d' transform='rotate(-120)'/%3e%3c/g%3e%3c/svg%3e)