Atypical goblet cell hyperplasia occurs in CPAM 1, 2, and 3, and is a probable precursor lesion for childhood adenocarcinoma.

Congenital pulmonary airway malformation (CPAM) is a developmental disorder. Types 1-2-3 are the more common ones. Atypical goblet cell hyperplasia (AGCH) in CPAM might be a precursor lesion for pulmonary adenocarcinomas. In nine out of 33 CPAM cases, types 1-3 showed foci of goblet cell proliferations. As these cells completely replace normal epithelium, we prefer to name these proliferations AGCH. In 5 cases, adenocarcinomas were seen (AC). All cases were analyzed for proteins possibly being associated with CPAM development: fibroblast growth factor 10 (FGF10) and receptor 2 (FGFR2), forkhead box A1 (FOXA1) and A2 (FOXA2), MUC protein 5AC (MUC5AC), human epidermal growth factor receptor 2 (erbB2, HER2/neu), hepatocyte nuclear factor 4α (HNF4α), SOX2, and Ying Yang protein 1 (YY1). By next generation sequencing, AGCH and adenocarcinomas were evaluated for driver mutations. Expression for FGF10, FGFR2, FOXA1, and FOXA2 was seen in CPAM epithelium and stroma, but not differently in AGCH and AC. SOX2 was positive in CPAM epithelium and AGCH, however weakly in AC. YY1 and MUC5AC showed more intense staining in AGCH and AC than in CPAM epithelium. HER2 was intensely expressed in AC and less intensely in AGCH, but not in CPAM epithelium. KRAS mutation in exon 2 was detected in all AGCH and AC, but was absent in CPAM epithelia. AGCH can arise in CPAM types 1-3. Oncogenic KRAS mutation seems to be the oncogenic driver already in AGCH, proving its role as a precursor lesion for adenocarcinoma. It might upregulate HER2 at the protein level. YY1 seems to be involved in carcinogenesis.

Peutz-Jeghers syndrome: need for early screening.

Peutz-Jeghers syndrome (PJS) is an autosomal dominant cancer-predisposing condition characterised by intestinal hamartomatous polyps and distinct melanin depositions in skin and mucosa. Small intestinal cancer in patients with PJS usually presents by the third decade. A 7-year-old-PJS boy presented with recurrent episodes of colicky abdominal pain and melena requiring repeated blood transfusions. Abdominal CT scan revealed multiple jejunal polyps with jejunoileal intussusception. On exploration, the intussuscepted bowel was resected along with its mesentery and anastomosed. Simultaneously, multiple enterotomies with resection of palpable polyps were performed. The resected bowel showed well-differentiated stage 2A adenocarcinoma with clear resected margins. Postoperatively, the complaints were relieved. On follow-up, he was asymptomatic and is now on yearly cancer surveillance. This is probably the youngest reported case of small bowel cancer in PJS.

Adenocarcinoma gástrico asociado a Helicobacter pylori en pediatría / Gastric adenocarcinoma associated with Helicobacter pylori in the pediatric setting

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From newborn to toddler: report of two cases of congenital granular cell tumor.

A congenital granular cell tumor (CGCT) is an uncommon benign soft tissue lesion that usually arises from the alveolar ridge in newborns. It can severely interfere with the respiratory and feeding systems, if left untreated. We present the cases of 2 newborn infants with protruded intraoral tumors that severely compromised breastfeeding. The tumors were removed by water laser without the need for suturing or pain medication. Histopathologic assessment was performed for definitive diagnosis, and the children were followed until full primary dentition was observed without any sign of CGCT recurrence.

Fetal lung interstitial tumor (FLIT): A proposed newly recognized lung tumor of infancy to be differentiated from cystic pleuropulmonary blastoma and other developmental pulmonary lesions.

The differential diagnosis of congenital lung lesions includes a variety of pulmonary malformations, and uncommon or rare neoplasms such as the pleuropulmonary blastoma (PPB) and congenital peribronchial myofibroblastic tumor (CPMT). Although most of the congenital lesions have a predominantly cystic appearance, the exceptions of a more solid process are the type 3 congenital cystic adenomatoid or pulmonary airway malformation (CCAM-CPAM) and the CPMT. The clinical and pathologic features of a unique solid or mixed solid/cystic lung mass composed of immature interstitial mesenchyme in association with irregular airspace-like structures mimicking abnormal incompletely developed lung are presented in this report of 10 infants (7 males, 3 females) whose tumor-like lesions were detected in the prenatal period to 3 months of age (median, 1-day old). A lobectomy was done in all 10 infants and 1 infant received adjuvant chemotherapy. One of the surgical resections occurred as an ex utero, antenatal procedure because of fetal ascites. There have been no reported recurrences in those patients with greater than 12 months of follow-up ranging from 15 to 182 months (9 cases). Because of the morphologic resemblance of this mass-like lesion to fetal lung at 20 to 24 weeks gestation (as though any further pulmonary development was arrested in these localized lesions), we are proposing the designation of fetal lung interstitial tumor (FLIT) whose pathogenetic relationship, if any, to type 1 (cystic) pleuropulmonary blastoma remains uncertain to date.

[Hereditary non-polyposis colorectal cancer associated with hereditary gastric cancer. A case report.]. / Cáncer colorrectal hereditario no polipósico asociado a cáncer gástrico hereditario. Informe de un caso.

The information regarding the association of gastric cancer and type 2 Lynch syndrome is limited. Previous studies have reported that both entities may be present in the same individual in less than 5% of the cases. The most frequent form of hereditary colorectal cancer is the Lynch syndrome or Hereditary Non-polyposic Colorectal Cancer, which is associated with germ-line mutation mostly of two genes, MLH1 and MSH2, which account for almost 90 percent of all identified mutations. The hereditary diffuse gastric cancer syndrome is caused by a germ-line mutation in the E-cadherin (CDH1) gene; only about 50 families with this syndrome have been reported. We present a case report of a patient who was diagnosed with both syndromes.

Pancreatic carcinoma in a neonate.

The clinical and radiographic findings in a neonate with adenocarcinoma of the head of the pancreas are presented. CT revealed the mass to be well defined, with an inhomogeneous density before and after intravenous contrast enhancement. Ultrasound revealed the mass to be mainly densely echogenic with small, irregular echo-free areas. The portal vein was intimately applied to the posterior aspect of the mass. The literature concerning adenocarcinoma of the pancreas in the pediatric age group is reviewed.