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Meningioma, Angiomyolipoma, Ependymoma and Sarcoma: Report of a Case and Review of the Literature

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Meningioma, Angiomyolipoma, Ependymoma and Sarcoma: Report of a Case and Review of the Literature Volume 13, No 1 International Journal of Radiation Research, January 2015 A novel case of quartet tumor: meningioma, angiomyolipoma, ependymoma and sarcoma: report of a case and review of the literature C. Yavas 1, P. Karabagli 2, G. Yavas 3, H.Acar 4* , O. Ata 5 1Konya Training and Research Hospital, Department of Radiation Oncology Konya, Turkey 2Selcuk University, Department of Pathology Konya, Turkey 3Selcuk University, Department of Radiation Oncology Konya, Turkey 4Selcuk University, Department of Medical Genetics Konya, Turkey 5Selcuk University, Department of Medical Oncology Konya, Turkey ► Case report ABSTRACT * Corresponding author: Mulple primary tumors in a single paent are relavely rare when four or Dr. Song Hilal Acar, more disnct lesions are considered. Herein, we report a case of woman with Fax: +90 332 4461608 four different primary tumors: meningioma, renal angiomyolipoma, spinal E-mail: [email protected] ependymoma and high-grade so ssue sarcoma. There was no family history and hereditary syndrome. The genec analysis was completely normal. To Revised: April 2014 est of our knowledge, the present paent is the "rst case in the literature, Accepted: May 2014 who presented with these four types of tumors. Int. J. Radiat. Res., January 2015; Keywords: Angiomyolipoma, ependymoma, meningioma, sarcoma. 13(1): 73-78 DOI: 10.7508/ijrr.2015.01.010 INTRODUCTION ed the incidence of a second primary cancer ran es from 3% to 7% with the hi her percent- (,-5) The phenomenon of multiple primary a e representin the older a e roups . Here- neoplasms in the same individual was described in we report a case with four primary tumors, all irstly by Warren and Gates (1) . Recently, the involvin different or an systems. Downloaded from ijrr.com at 0:18 +0330 on Saturday October 2nd 2021 [ DOI: 10.7508/ijrr.2015.01.010 ] early detection and the advances in therapy for mali nant diseases have contributed to prolon ed survival of patients, resultin in an CASE REPORT increment of multiple primary tumors. However, the reports of multiple primary tumors are still A 59-year-old woman admitted to the hospi- uncommon. They appear more frequently in the tal, with a complaint of pro ressive weakness on upper di estive tract, respiratory system, head her lower e.tremities over a 6-month history in and neck re ion, or uro enital system. The inci- 0anuary 2011. 1he denied tobacco usa e or dence ran es from 2% to 10% (2) . Amon those alcohol consumption. 1he was a housewife livin with multiple primary mali nancies, double in a small villa e. There was no family history. cancers is commonly seen, while triple cancers The patient had a past history of menin ioma. occur in 0.5% of patients, and quadruple or The mass located in the ri ht parieto-occipital quintuple cancers occur in only less than 0.1% of re ion 2.52221.5 cm in si3e, the irst of her the population (3) . Autopsy series have estimat- cancers. It was curatively resected in November Yavas et al./ A novel case of quartet tumor 2004. Pathological examination revealed that bundles. Pathologic examination revealed an the tumor characterized was due to the angiomyolipoma which was her second tumor. formation of multiple, discrete, concentrically The tumor composed of adipose tissue, smooth laminated, calcareous bodies (psammoma muscles and blood vessels (i ure 2). After bodies). It was diagnosed as psammomatous sur ery the patient refused any further meningioma, grade I (WHO, 2000) (i ure 1). In dia nostic work and treatment so she did not o 2010 she admitted to the hospital with left lank to her routine follow-up until (a year later) the pain which was increased pro ressively within 3 development pro ressive weakness on her months. The physical e.amination was lower e.tremities. unremarkable. 8ltrasound demonstrated a Neurolo ical e.amination revealed a bilateral hyperechoic mass on the left kidney. The CT hemiparesis (3/5). Lumbar ma netic resonance revealed a hetero eneous mass in the left ima in (MRI) revealed an intadural and kidney measurin 82523 cm. rossly; the tumor e.tramedullary mass located in the level of L2- was conined within the renal capsule. A radical L3. 7n T1-wei hted ima es, the mass appeared left nephrectomy was performed. The major isointense whereas on T2-wei hted ima es, it histolo ical components of the tumor were was hyperintense relative to the normal cord. A characteri3ed by abnormally thick -walled subtotal e.cision of the mass was then vessels, mature fat cells, and smooth muscle performed, which was followed by transient improvement. Histolo ical indin s indicated a dia nosis of tanycytic ependymoma rade II (WH7, 2007). The tumor characteri3ed by markedly elon ated spindle shaped cells, which were immunopositive for 1-100 protein and lial ibrillary acidic protein (i ure 3). 1ince the tumor subtotally resected, a total dose of 5000 cGy radiotherapy (RT) with 2 Gy/fraction was planned. RT ield was deined as the preoperative tumor volume (determined by T1 ma netic resonance ima in (MRI) scan) plus 2 Figure 1. The tumor characterized by the abundance of psammoma bodies (H&E ×40). Downloaded from ijrr.com at 0:18 +0330 on Saturday October 2nd 2021 [ DOI: 10.7508/ijrr.2015.01.010 ] Figure 3. The tumor consistent of elongated cells with highly fibrillary processes, forming a fascicular architecture (H&E ×40). The tumor characterized by Figure 2. Renal angiomyolipoma showing mature fat markedly elongated spindle shaped cells, which were cells, blood vessels, and smooth muscle cells (H&E immunoposive for S-100 protein and glial " rillary ×100). acidic protein. Int. J. Radiat. Res., Vol. 13 No. 1, January 2015 74 Yavas et al./ A novel case of quartet tumor cm mar in a dose of 50Gy was applied. We thou ht that she mi ht have had a enetic Three months after the RT, she came to syndrome which was associated with multiple routine follow-up. 1he had no complaint about primary tumors. Thus conventional cyto enetic her three other tumors althou h she had a mass analysis and FI1H (luorescence in situ hybridi- on her ri ht luteal re ion. Physical e.amination 3ation) with P53 ene were done. Aoth cyto e- revealed a 10 cm i.ed irre ular mass located on netic analysis and FI1H analyses showed a nor- her ri ht buttock. We inspected her previous mal karyotype and p53 ene. plannin tomo raphy for spinal ependymoma Durin follow-up the patient was admitted to however; there was no mass on her ri ht luteal the hospital with shortness of breath and severe re ion. MRI revealed a 15210 cm mass on the cou h. A contrast enhanced CT revealed ri ht luteal re ion. We performed position multiple nodules in her lun s, which were emission tomo raphy (PET)-CT, and it identiied as metastases. The patient was demonstrated 10215 cm hetero enous hospitali3ed. However; her dyspnea was hypermetabolic mass with a standardi3ed pro ressively increased and 15 months after the uptake (18E) value of ,3.81. The tumor e.cised completion of the last RT, the patient died of from ri ht luteal area characteri3ed by an respiratory failure. e.treme de ree of pleomorphism with numerous typical and atypical mitotic i ures and a fascicular rowth pattern. Hemorrha e and necrosis were e.tensive in tumor. Immuno- DISCUSSION histochemically tumor cells were positive for Eimentin, but ne ative for CD,5, AE1/AE3, 1MA, Multiple primary tumors in a patient are 1-100, Desmin and Myo enin. Histolo ical and e.tremely low conditions but have increase in immunohistochemical indin s indicated dia no- frequency in recent decades. This may be due to sis of hi h rade sarcoma (i ure ,). Durin the the medical improvements in both dia nostic operation the tumor was disinte rated so the and therapeutic strate ies, which eventually sur ical mar ins could not be determined. We increase the overall survival time of the patents planned a dose of 66-70 Gy RT to the tumor bed, with multiple mali nancies. 7n the other hand, with 5 cm and 2 cm mar ins in craniocaudal ra- as the a e increased, the risk of developin a dial directions respectively; however she could tumor increases as well. We report a patient not tolerated the doses above 62 Gy. who developed four different tumorsC a menin- ioma, an iomyolipoma, tanycytic ependymoma and hi h rade sarcoma. To best of our knowled e, the present patient is the irst case in the En lish literature, who presented with these four types of tumors. In reviews of the literature with respect to Downloaded from ijrr.com at 0:18 +0330 on Saturday October 2nd 2021 [ DOI: 10.7508/ijrr.2015.01.010 ] the multiple primary mali nancies, it is interest- in that, the 0apanese population seems to have hi her likelihood of developin multiple mali - nancies. This may be due to lon er life span, ene susceptibility or advancement in the ield of the oncolo y. Table 1 summari3es the report- ed cases of four or more primary mali nancies in the literature durin 1981-2011 (6-27) . Figure 4. .naplasc tumor cells are arranged haphazardly in sheets and necroc areas (H%E '40). Althou h the appearance of four and more pri- Immunohistochemically tumor cells were posive for mary tumors in one patient is not very common, 0imenn, ut negave for 1243, .E14.E3, SM., should not be considered as such a rare event. S-100, 2esmin and Myogenin. As it is seen in the table, most of the cases were 75 Int.
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