ORPHAN TUMORS: EXPERIENCE OF DIAGNOSIS AND TREATMENT OF NEUROECTODERMAL TUMORS OF THE INTRATHORACIC LOCALIZATION
Keywords:
primitive neuroectodermal tumor, immunohistochemical study, neoadjuvant chemotherapy, adjuvant chemotherapy, combined treatmentAbstract
Summary. Objective: to summarize and analyze the
results of diagnosis and treatment of patients with intrathoracic peripheral primitive neuroectodermal tumor (PNET) for the assessment of prognostic indicators and the effectiveness of various treatment protocols
of patients. Object and methods: the data of observations of 12 patients with a primary PNET of the chest
cavity, two of them with a rare localization inside the
lungs. Histological examination was complemented by
cytogenetic and immunohistochemical methods for refined diagnostic. All patients underwent radical surgical
removal of tumors; in 11 of them treatment was started with neoadjuvant chemotherapy (CT); adjuvant CT
was administered according to the indications. The duration of recurrence-free period and survival of the patient were evaluated after treatment. Results: prognostic sign for patients with tumors of neuroectodermal histogenesis were formulated. The efficacy of neoadjuvant
CT in all clinical studies waqs noted. The most efficacy was the complex treatment with neoadjuvant and adjuvant CT to be used as a method of choice for this pathology. Conclusion: the most convincing confirmation
of the diagnosis of PNET is based on the definition of
tissue markers for immunohistochemical study. Treatment tactics of patients with PNET should be based on
an integrated approach; at the same time, surgical technique is a key in operable tumors.
References
Давыдов МИ, Мачаладзе ЗО, Полоцкий БЕ и др. Мезенхимальные опухоли средостения (обзор литературы). Сиб онкол журн 2008; 1 (25): 64–74.
Захарычев ВД, Борисюк БО, Кобзев ОИ. Примитивные нейроэктодермальные опухоли грудной полости: результаты лечения. Онкология 2012; 14 (2): 157–60.
Карташова ОМ, Анненкова ИВ, Карташов МВ. Лучевая диагностика периферической примитивной нейроэктодермальной опухоли у детей. Детская больница 2010; 3: 47–52.
Пугачев КК, Кузьмин ИВ, Махсон АН и др. Периферическая примитивная нейроэктодермальная опухоль (Аскина). Вест травматол ортопед 1999; 2: 55–62.
Семенова АИ. Саркома Юинга и периферические примитивные нейроэктодермальные опухоли (клиника, диагностика, лечение). Практ онкол 2005; 6 (4): 234–9.
Семенова АИ. Саркома Юинга: характеристика заболевания, особенности диагностики, лечебная тактика. Практ онкол 2010; 11(1): 45–52.
Франк ГА. Проблемы морфологической классификации и диагностики опухолей мягких тканей. Практ онкол
; 5 (4): 231–6.
De Alava BE, Gerald WL. Molecular biolodgy of the Ewings sarcoma. Primitive neuroectodermal tumor family. J Clin Oncol 2000; 18: 204–8.
Barker LM, Pendergrass TD, Sanders JE, et al. Survival after recurrence of Ewing’s sarcoma family of tumors. J Clin Oncol 2005; 23 (19): 4354–61.
Cavazzana AO, Nifo V, Roberts J, et al. Peripheral neuroepithelioma: a light microscopic, immunocytochemical diagnosis of olfactory neuroblastoma. J Neuropathol Exp Neorol 1985; 44: 18–31.
Cotteril SJ, Abrens S, Paulussen M, et al.Prognostic factors in Ewings tumor of bone: analisis of 975 patients from the European Intergroup Cooperative Ewings Sarcoma Study Group. J Clin Oncol 2010; 18: 3108–15.
Demir A, Dagoglu N, Gunluoglu MZ, et al. Surgical treatment and prognosis of primitive neuroectodermal tumors of the thorax. J Thorac Oncol 2009; l4: 185–92.
Dunst J, Sauer R, Burgers JMV, et al. Radiation therapy in Ewing’s sarcoma: an update of the CESS 86 trial. Cancer
; 67: 2818–25.
Evans RG, Nesbit ME, Gehan EA, et al. Multimodal therapy for the management of localized Ewing’s sarcoma of pelvic and sacral bones: a report from the second intergroup study. J Clin Oncol 1991; 9 (7): 1173–80.
Gachechiladze M, Skarda J, Ibrahim M, et al. Primitive neuroectodermal tumor (PNET) of the lung in adult woman. World J Surg Oncol 2014; 12: 374.
Hancorn K, Sharma A, Shackcloth M. Primary extraskeletal Ewing’s sarcoma of the lung. Interact Cardiovasc Thorac Surg 2010; 10: 803–4.
Kinsella TJ, Lichter AS, Miser J, et al. Local treatment of Ewing’s sarcoms: radiaton, therapy versus surgery. Cancer Treat Rep 1984; 68: 695–701.
Llombart-Bosch A, Contesso G, Peydro-Olaya A, et al. Histology, immunohistochemistry, and electron microscopy of small round cell tumors of bone. Semin Diagn Pathol 1996; 13 (3): 153–70.
Moll R, Lee I, Gould VE. Immunocytochemical analysis of Ewing’s tumors: patterns of expression of filaments and desmo- somal proteins indicate cell type heterogeneity and pluripotential differentiation. Am J Pathol 1987; 127: 288–304.
Neef H. The role of surgery in diagnosis and treatment of mediastinal malignance. Lung 2010; 168 (Suppl 1): 1153–61.
Picci P, Bobling T, Bacci G, et al. Chemotherapy-induced tumor necrosis as a prognostic factor in localized Ewing’s sarcoma of the extremities. J Clin Oncol 2009; 15: 1553–60.
Polonowski JM, Brasnu D, Roux FX, et al. (1992) Esthesioneuroblastoma: complete tumor response after induction chemotherapy. Ent J 1992; 69: 743–5.
Takahashi D, Nagayama J, Nagatoshi Y. Primary Ewing’s sarcoma family tumors of the lung: a case report and review of the literature. Jpn J Clin Oncol 2007; l37: 874–7.
Turc-Carel C, Philip I, Berger MP, et al. Chromosome study of Ewing’s sarcoma (ES) cell lines. Consistency of a reciprocal translocation t(11;22)(q24;q12). Cancer Genet Cytogenet 1984; 12: 1–19.
Weissferdt A, Moran CA. Primary pulmonary primitive neuroectodermal tumor (PNET): a clinicopathological and immunohistochemical study of six cases. Lung 2012; 190 (6): 677–83.
Weiss SW, Goldblum JR. Primitive neuroectodermal tumors and related lesions. Soft Tissue Tumors. SW Weiss, JR Goldblum, eds. Maryland Heights: Mosby Elsevier, 2008, 945–87.
Workman ML, Soukup SW, Neely JE, et al. Biologic characteristics of four Ewings’s sarcomas. Cancer Genet Cytogenet
; 15: 215–25.
