SPORADIC AND HEREDITARY FORMS OF MULTIPLE PRIMARY MALIGNANT TUMORS IN CHILDREN

2019 ◽  
Vol 65 (4) ◽  
pp. 565-574
Author(s):  
Alina Malakhova ◽  
Tatyana Kazubskaya ◽  
V. Kozlova ◽  
Tatyana Kondrateva ◽  
Yevgeniy Trofimov ◽  
...  

The article presents the features of the clinical manifestation of multiple primary sporadic and heritable malignant tumors in 104 children who observed at Pediatric Oncology and Hematology Research Institute of «NN Blokhin's National Medical Research Center of Oncology» from 1998 to 2012. The age of patients at the time of occurrence of the second malignant tumor ranged from 2.8 to 28 years and averaged 15.6 years. The second neoplasia occurred significantly more frequently in the group of primary hemoblastosis, compared with the group of solid tumors - 8.7% versus 3.4%, respectively (p <0.0001). In children with multiple primaries, were found tumors that occur in common the most frequency. Retinoblastoma patients have an increased risk of developing sarcoma. Patients with Hodgkin's lymphoma have an increased risk of developing leukemia. In surviving children after treatment of the first neoplasia the thyroid, bone tissue and breast have a specific risk for the development of metachronous cancer and are target organs for control. Children with new hematopoietic tumors may be candidates for metachronous development of leukemia and bone tissue tumors. The using methods Next Generation Sequencing (NGS) and Multiplex Ligation-dependent Probe Amplification (MLPA) revealed germinal mutations in 12 children with of multiple primary tumors. The mutations in the TP53, RB1, CHEK2, FANCN/PALB2, MLH1, PMS2 genes identified in patients were associated with hereditary syndromes and an increased risk of developing second tumors, among which: sarcomas, brain tumors, hematopoietic tumors were the most frequent. It was shown that the second tumors can appear at the any age. Children who survived the treatment of the first tumor in later life should be monitored annually. Clinical management of children with multiple primary tumors requires a multidisciplinary approach.

2021 ◽  
Vol 13 (2) ◽  
pp. 36-43
Author(s):  
A. A. Zagidullina ◽  
V. Kh. Kharbediya ◽  
A. Z. Dzampaev ◽  
D. V. Nisichenko ◽  
S. N. Mikhailova

Background. Retinoblastoma is a malignant intraocular tumor developing from the retinal neuroectoderm and diagnosed primarily in young children. This type of cancer is associated with a high risk of multiple primary tumors emerging after treatment completion. Multiple primary tumors are two or more independent tumors developing in one patient. Treatment of this disease is challenging.Objective – to evaluate the impact of risk factors on the efficacy of therapy for multiple primary tumors and to analyze treatment outcomes.Materials and methods. A 2-year-old boy was diagnosed with bilateral retinoblastoma (OD – stage T3bN0M0 and OS – stage T3cN0M0). He received special treatment from September 2005 to November 2006. In 2012, the patient underwent cataract surgery: the lens was removed, then an intraocular lens was installed, and laser dissection of the posterior capsule of the lens was performed. Six years later, in August 2018, the patient was diagnosed with osteosarcoma. The boy received combination organ-sparing therapy according to the EURAMOS-1 treatment protocol for osteosarcoma. During therapy, he developed a secondary tumor, namely osteoblastic osteosarcoma. Both the boy and his father were found to have a mutation in the RB1 gene.Results. Currently, patient’s condition is satisfactory; he has no complains. The boy is in remission for 2 years.Conclusion. The development of secondary tumors depends on the genetic factors, type of treatment for primary tumor, and environmental factors. Therefore, it is extremely important to assess risk factors for multiple primary tumors at the moment of primary retinoblastoma detection. The results of such assessment will help to choose an optimal treatment strategy.


1927 ◽  
Vol 23 (12) ◽  
pp. 1288-1288

The author reports that 9,371 autopsies performed between 1920 and 1926 at the Pathologoanat. The author reports that from the total number of cases of malignant tumors of 1.123 (11.98%) in the Institute of Pathology and Anatomy of Prof. Kiml in Prague 14 multiple cancers were found. Besides 4 cases in which the primary origin of the tumor was questionable, 10 cases (0,107% of all autopsies and 0.89% of malignant tumors) remain, where the primary plurality may be considered as reliable or at least very probable.


2021 ◽  
pp. 014556132110280
Author(s):  
Tong Sha ◽  
Yang Yu ◽  
Kang Liu ◽  
Bin Sun ◽  
Wei Zhang

Multiple primary tumors of the parotid gland refer to the occurrence of 2 or more primary tumors in the ipsilateral or bilateral parotid gland metachronously or synchronously, which do not originate from postoperative recurrence or metastasis. Multiple primary tumors of the parotid gland, especially synchronous benign and malignant tumors, are extremely uncommon. A rare case of multiple Warthin tumors with epithelial–myoepithelial carcinoma of the ipsilateral parotid gland is reported as follows.


2019 ◽  
Vol 65 (3) ◽  
pp. 321-329
Author(s):  
David Zaridze ◽  
Anush Mukeriya

Smoking not only increases the risk of the development of malignant tumors (MT), but affects the disease prognosis, mortality and survivability of cancer patients. The link between the smoking of cancer patients and increased risk of death by all diseases and oncological causes has been established. Mortality increases with the growth of the smoking intensity, i.e. the number of cigarettes, smoked per day. Smoking is associated with the worst general and oncological survivability. The statistically trend-line between the smoking intensity and survivability was observed: each additional unit of cigarette consumption (pack/year) leads to the Overall Survival Reduction by 1% (p = 0.002). The link between smoking and the risk of developing second primary tumors has been confirmed. Smoking increases the likelihood of side effects of the antitumor therapy both drug therapy and radiation therapy and reduces the treatment efficacy. The smoking cessation leads to a significant improvement in the prognosis of a cancer patient. Scientific data on the negative effect of smoking on the prognosis of cancer patients have a major clinical importance. The treatment program for cancer patients should include science-based methods for the smoking cessation. The latter is fundamentally important, taking into account that the smoking frequency among cancer patients is much higher than in the population.


2016 ◽  
Vol 31 (4) ◽  
pp. 461-465 ◽  
Author(s):  
Valentina Zampiga ◽  
Rita Danesi ◽  
Gianluca Tedaldi ◽  
Michela Tebaldi ◽  
Ilaria Cangini ◽  
...  

Li-Fraumeni syndrome (LFS) is an autosomal dominant disorder occurring at a young age that predisposes individuals to multiple forms of cancer and to a heterogeneous spectrum of malignancies. We describe the clinical history of a patient who had 5 primary malignant cancers and a familiar history consistent with LFS. We analyzed the genomic DNA of the proband and her relatives by next-generation sequencing (NGS) technology using an enrichment protocol for the simultaneous sequencing of 94 genes involved in hereditary cancers. Genetic analysis of the proband revealed a TP53 germline mutation in exon 5 determining a nucleotide alteration at codon 175 (R175H), a hot spot mutation site related to LFS and a reported pathogenic mutation. The proband daughter's and brother's DNA did not carry the TP53 mutation but they had some rare variants in common with the proband, in addition to other variants with a still unclear role. In conclusion, we identified a TP53 mutation in a patient with multiple primary tumors and a family history characterized by a severe susceptibility to cancer. The genetic analysis by targeted NGS led to the identification of the genetic background and to the exclusion of a cancer risk for the family members. Targeted NGS represents an efficient approach for the identification of mutations in families with a heterogeneous phenotype.


Author(s):  
Jennifer Seyffert ◽  
Alysa Herman ◽  
Brian Katz ◽  
Daniel Rivlin

<p>Merkel cell carcinoma (MCC) is an aggressive and uncommon neuroendocrine tumor which clinically presents as a rapidly growing solitary nodule or plaque located in sun exposed areas on the head, neck, and extremities. Merkel cell carcinomas can be UV-induced or result from viral infection with the Merkel cell polyomavirus (MCV). We present a unique case of bilateral symmetric Merkel cell carcinomas located on the dorsal hands and briefly discuss options for genomic investigation to delineate if the tumors are multiple primary tumors or result of metastatic disease.</p>


2018 ◽  
Vol 9 ◽  
Author(s):  
Mathias Cavaillé ◽  
Flora Ponelle-Chachuat ◽  
Nancy Uhrhammer ◽  
Sandrine Viala ◽  
Mathilde Gay-Bellile ◽  
...  

1994 ◽  
Vol 56 (3) ◽  
pp. 320-323 ◽  
Author(s):  
Andreas Jovanoic ◽  
Ignaz G. H. van der Tol ◽  
Engelbert A. J. M. Schulten ◽  
Pieter J. Kostense ◽  
Nico de Vries ◽  
...  

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