Hemangiomas are the most common soft-tissue tumors of infancy, occurring in approximately 5 to 10 percent of one-year-old children. Despite the frequency of these tumors, their pathogenesis is not completely understood, and the best approach to their management remains controversial. Research on angiogenesis, an improved knowledge of the classification of the vascular anomalies of infancy, advances in imaging techniques, recently identified complications, and new therapeutic approaches, such as treatment with interferon and lasers, have altered our understanding of this common childhood problem and the way we approach it. The term hemangioma has traditionally been applied to a variety of vascular . . .

https://www.nejm.org/doi/pdf/10.1056/NEJM199907153410307

Hemangiomas in Children

Colorectal carcinoma is one of the most common cancers and one of the leading causes of cancer-related death in the United States. Pathologic examination of biopsy, polypectomy and resection specimens is crucial to appropriate patient managemnt, prognosis assessment and family counseling. Molecular testing plays an increasingly important role in the era of personalized medicine. This review article focuses on the histopathology and molecular pathology of colorectal carcinoma and its precursor lesions, with an emphasis on their clinical relevance.

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Colorectal carcinoma: Pathologic aspects

A practice guideline from the American College of Medical Genetics and Genomics and the National Society of Genetic Counselors: referral indications for cancer predisposition assessment

Background: Although high-risk mutations in identified major susceptibility genes (DNA mismatch repair genes and MUTYH) account for some familial aggregation of colorectal cancer, their population prevalence and the causes of the remaining familial aggregation are not knownMethods: We studied the families of 5,744 colorectal cancer cases (probands) recruited from population cancer registries in the United States, Canada, and Australia and screened probands for mutations in mismatch repair genes and MUTYH We conducted modified segregation analyses using the cancer history of first-degree relatives, conditional on the proband's age at diagnosis We estimated the prevalence of mutations in the identified genes, the prevalence of HR for unidentified major gene mutations, and the variance of the residual polygenic componentResults: We estimated that 1 in 279 of the population carry mutations in mismatch repair genes (MLH1 = 1 in 1,946, MSH2 = 1 in 2,841, MSH6 = 1 in 758, PMS2 = 1 in 714), 1 in 45 carry mutations in MUTYH, and 1 in 504 carry mutations associated with an average 31-fold increased risk of colorectal cancer in unidentified major genes The estimated polygenic variance was reduced by 30% to 50% after allowing for unidentified major genes and decreased from 33 for age <40 years to 05 for age ≥70 years (equivalent to sibling relative risks of 51 to 13, respectively)Conclusions: Unidentified major genes might explain one third to one half of the missing heritability of colorectal cancerImpact: Our findings could aid gene discovery and development of better colorectal cancer risk prediction models Cancer Epidemiol Biomarkers Prev; 26(3); 404-12 ©2016 AACR

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Prevalence and Penetrance of Major Genes and Polygenes for Colorectal Cancer

Successful treatment of vascular anomalies has eluded the physician until now, despite various treatments utilised. Bleomycin has been successfully used in intralesional injection treatment of cystic hygromas and haemangiomas, based specifically on a high sclerosing effect on vascular endothelium. In a prospective study of 95 patients, the effectiveness of intralesional bleomycin injection (IBI) treatment in haemangiomas and vascular malformations was evaluated and documented. Complete resolution or significant improvement occurred in 80% of all patients treated. Complete resolution occurred in 49% of haemangiomas, 32% of venous malformations, and 80% of cystic hygromas. Significant improvement occurred in 38% of haemangiomas, 52% of venous malformations, 13% of cystic hygromas and 50% of lymphatic malformations. Of the six patients who presented with a painful lesion, four experienced complete resolution and two had significant improvement to treatment. Local complications encountered were superficial ulceration occurring in 2 patients, and cellulitis in 1 of the 95 patients. Systemic complications were flu-like symptoms in three patients and partial, transient hair loss in two patients. None of the patients presented with haematological toxic effects or signs of pulmonary involvement (fibrosis, hypertension). IBI is an effective treatment in haemangiomas and vascular malformation lesions, obviating the need for invasive primary surgery or systemic treatment regimens in 80% of cases, and allowing for limited need of secondary surgical or adjunctive procedures in cases with a moderate result.

Intralesional bleomycin injection (IBI) treatment for haemangiomas and congenital vascular malformations.

Aim: Report of a series of 12 cases of juvenile polyposis coli. Methods: The study period was from 1995 to 2005. All the patients were treated by total colectomy with rectal mucosectomy and endorectal ileoanal pullthrough with or without ileal pouch formation. Covering ileostomy was avoided in all the cases. Time taken for the surgery, postoperative complications and continence were documented. Results: The mean operating time was 4.2 h (range: 4-5 h). The mean duration of hospital stay was 16.3 days (range: 15-18 days). The most common postoperative complication was pouchitis and perianal excoriation. Initially, all the patients were passing stools at an interval of 2 h, and after 3 weeks, the frequency has reduced to 6-8 stools per day. In the follow-up after 3 months, the frequency was 3-5 per day with minimal soiling. Conclusions: Single-stage total colectomy with rectal mucosectomy and endorectal ileoanal pull-through without covering ileostomy and pouch formation is a safe and definitive treatment for juvenile polyposis coli if the patient selection is appropriate.

Juvenile polyposis syndrome

1195 cases of various type of haemangioma were treated with various steroid modalities. The response to intralesional steroids was excellent in strawberry and mixed haemangiomas. The response to oral steroids was also good in these two types. The response of cavernous variety was poor with either modality used alone but with combined modality, a moderate response can be obtained.

Role of steroid in childhood haemangioma : A 10 years review

Infantile hemangioma is a common disease. Steroids have been used for its treatment; however, intralesional steroids cause pain and other problems. A treatment modality that can avoid these problems is desirable. The authors evaluated the role of topical steroids as an alternative to intralesional steroids in the treatment of superficial hemangioma. Inclusion criteria were <2 superficial type ofhemangiomas <5 cm. The topical steroid mometasone furoate was applied twice daily. Intralesional triamcinolone acetonide was injected at monthly intervals using a 24-gauge needle at doses of 1 to 2 mg/kg. Forty-five (86.5%) patients responded to treatment with the topical steroids, of which 50% had excellent and 36.5% had good response. In the intralesional group, the response rate was 95.7%, of which 63.8% had excellent and 31.9% had good response. Complications in the topical steroid group were mild itching and irritation (19.2%) and hypopigmentation (7.6%). Complications in the intralesional group were pain (100%), bleeding (17%), infection (17%), cutaneous atrophy (8.5%), cushingoid facies (2.1%), and growth retardation (2.1%). Topical steroids are a reasonably good alternative to intralesional steroids as an initial choice for treating superficial hemangioma.

Evaluation of topical steroids in the treatment of superficial hemangioma.

Buts: La fistule tracheo-oesophagienne congenitale avec atresie de l'oesophage (TEF avec EA) n'est pas une malformation rare du nouveau-ne. L'approche classique pour la reparation de TEF avec EA est une thoracotomie droite avec une approche extra-pleurale, ligature de la veine azygos, identification et ligature de la fistule tracheo-oesophagienne, identification du cul de sac superieur de l'oesophage et anastomose termino-terminale. Cette etude est realisee pour apprecier si la ligature de la veine azygos est necessaire durant la reparation de TEF avec EA. Methodes: Nous avons etudie 50 cas dans les 2 dernieres annees (janvier 2003 janvier 2005). Pour 25 enfants (groupe A). La reparation etait faite avec preservation de la veine azygos alors que 25 enfants (groupe B) etaient operes avec une approche classique avec ligature et section de la veine azygos. Les resultats postoperatoires sont compares dans les 2 situations. Resultats: Les infections pulmonaires postoperatoires etaient plus frequentes chez les enfants operes avec l'approche classique (56% dans le groupe B et 12% dans le groupe A) et ceci est significatif. Les complications a type de fistule anastomotiques etaient, elles, legerement plus hautes dans le groupe B, mais ceci n'etait pas significatif. La mortalite etait similaire dans les 2 cas. Les bebes du groupe A etaient hemodynamiquement plus stable que ceux du groupe B. Conclusion: La preservation de la veine azygos maintient un drainage veineux normal du mediastin et diminue la stase thoracique postoperatoire et les complications a type de pneumonie ainsi la veine azygos doit etre preservee autant que possible.

Is Ligation of Azygos Vein Necessary in Primary Repair of Tracheoesophageal Fistula with Esophageal Atresia

Objectives To evaluate the efficacy of intralesional triamcinolone in enhancing regression in various type of hemangiomas. Setting Outpatients department of a teaching hospital Subjects: One hundred and five patients with rapidly growing surface hemangioma. The age ranged from 1 month to 15 months (mean 7 months). Intervention Intralesional triamcinolone administered at monthly intervals, ranging from single injection to 7 injections (mean 3.6 injections). Main outcome measure Regression of the hemangioma. Results The overall response rate was 88.6% (excellent 51.4%, and good 37.2%). Maximum response was observed in children below 1 year of age (> 90%), lesions over the face (92.8%) and strawberry hemangiomas (99.9%). Conclusion Intralesional administration of triamcinolone devoid of systemic side effects and an effective initial modality for rapidly growing hermangiomas.

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S. C. Gopal

Papers

Juvenile polyposis syndrome

Role of steroid in childhood haemangioma : A 10 years review

Evaluation of topical steroids in the treatment of superficial hemangioma.

Is Ligation of Azygos Vein Necessary in Primary Repair of Tracheoesophageal Fistula with Esophageal Atresia

Local steroid therapy in cutaneous hemangiomas.