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Primary mediastinal germ cell tumor - successful curative resection following chemotherapy - a case report

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Primary mediastinal germ cell tumor - successful curative resection following chemotherapy - a case report
  Case ReportPrimary mediastinal germ cell tumor - successful curative resection followingchemotherapy - a case report Saulat Fatimi 1 , Sadaf Sheikh 2 , Muhammad Usman Aziz 3 , Omar Aftab 4 Department of Surgery 1,2,4 , Final year Medical Student 3 , Aga Khan University Hospital, Karachi.   Abstract Primary mediastinal germ cell tumours are relative-ly rare and account for only a small portion of all the medi-astinal tumours. We present a case of a primary mediastinalgerm cell tumour in a 14 years old Afghani boy. Introduction Germ cell tumors account for approximately 1% of all the malignancies occurring in men, with testis being themost common site of srcin. Extra gonadal germ cell tumorscan occur in the midline of the body from the pineal glandthrough to the mediastinum, retro peritoneum and sacrum.The different subtypes of this entity are made up of semino-mas and nonseminomatous germ cell tumors (i.e., embryon-al carcinoma, choriocarcinoma, yolk sac tumors, mixedgerm cell tumors, mature and immature teratomas). Germcell tumors are primarily treated with Cisplatin based 188J Pak Med Assoc  chemotherapy and the surgical resection is reserved for residual masses. Case Report A 14 years old Afghani patient presented with a 6-8months history of dyspnea and left breast tenderness. Healso gave one year history of weight loss, anorexia, malaiseand non-productive cough. There was no history of bone pain.On physical examination, bilateral gynaecomastiawith left breast tenderness and multiple nodules were pres-ent. The breathing was harsh with decreased breath soundson the left side and trachea was shifted to the right. Genitalexamination showed normal external meatus and skin, righttesticular swelling which was mobile and non-tender andleft testicle was almost atrophied. Heart sounds were nor-mal.Initial laboratory investigations revealed hemoglo- bin of 11 G/dl, hematocrit of 34, a white blood cell count of 8700/dL with 60.5% neutrophils and 26% lymphocytes,ESR of 40mm first hour creatinine of 0.7 mg/dl, LDH of 1796 IU, calcium of 8.5 mg/dl, β -HCG of 9.3 mIU/ml andalpha-fetoprotein of 9600 IU/ml.LFTs showed bilirubin of 0.40 mg/dl, albumin of 3.33, SGPT of 14 and ALP of 333 (Table).Chest X-ray (Figure 1) showed a mass in the leftchest abutting the heart and raised left hemidiaphragm sec-ondary to phrenic nerve involvement.CT scan of the chest (Figure 2) showed a mediasti-nal mass measuring 10.4x7.9x16 mm and occupying lefthemithorax. The tumour was in the proximity to the heartand great vessels. The heart was pushed to the right sideacross midline with accentuated pericardial fluid trapped between the heart and described mass. It had well definedmargins and left paratracheal lymphadenopathy was pres-ent.After a month, Video Assisted Thoracoscopy(VATS) was done which revealed a gelatinous mass reach-ing upto the chest wall between 2nd and 5th Inter-costalspaces which was haemorrhagic. The biopsy of the massshowed a mixed germ cell tumor (Yolk sac tumour) with ter-atomatous component. The patient was started onChemotherapy consisting of Cisplatin, Etopside and Figure 2. CAT scan of the tumor is being shown. Notice the proximity of the tumor to the heartand great vessels.Figure 1. Chest X-ray is being shown. Notice the mass in the left chest abutting the heart andraised left hemidiaphragm secondary to phrenic nerve involvement. Tumor MarkerPreChemotherapyPostChemotherapyNormal values AFP (IU/ml) β hCG(mIU/ml)LDH (IU)>300009.317969.65<23460.5-5.5 IU/ml0-5mIU/ml253-548IU Table. Temporal assessment of tumour markers. Vol. 56, No. 4, April 2006189  Dexamethasone and received 8 cycles in total. After theshrinkage of tumor size, the residual mass in the medi-astinum which was involving the phrenic nerve, pericardi-um, lung and portion of left ventricle, posterolateral thora-cotomy was done and the entire tumor along with pericardi-um was removed (Figure 3). Post operative course wasuneventful and the patient was discharged on 7th Post oper-ative day. Discussion Mediastinal tumours are rarely observed in the clin-ical setting. Mean age of presentation is 35 years and nospecific sex predilection has been observed. Clinical pic-ture is vague and hence clinical diagnosis remains a dilem-ma. However, almost all tumours can be picked up on chestradiograph. 1 Mediastinum is documented to be the mostcommon extra gonadal site of germ cell tumours while 95%of all the germ cell tumours are located in the gonads. Theyhave been shown to srcinate from intratubular testicular germ cells and can either be seminomatous or non-semino-matous. Extragonadal germ cell tumours, which are a lesscommon variant (5%), have been hypothesized to arisefrom migration of the germ cells along the urogenital ridgeand most of them being the non-seminomatous variant. 2 However, primary mediastinal germ cell tumours remain arelatively rare clinical entity. Primary mediastinal germ celltumors such as uchoriocarcinomas, teratomas with yolk saccomponent and embryonal carcinomas have been reportedin the literature. Cases of co-existing primary gonadal andextra-gonadal germ cell tumours have also been cited. 3 Workup of mediastinal mass consists of CT imaging of thechest, a biochemical assessment of tumour markers andfine needle aspiration or core biopsy. Testicular ultrasoundhas also been practiced to rule out co existing germ celltumours in the testis and the mediastinum. 1,2 Earlier literature had shown that FNA did not proveto be a good diagnostic tool for mediastinal germ celltumours so management approach was not based on it. 3 Based on the tumour markers and histologic assessment, pri-mary germ cell tumour was suspected. Surgical resection of mediastinal germ cell tumours is recommended to followchemotherapy after normalization of markers. 2 Sternotomyand posterolateral thoracotomy have been used before for anterior and anterosuperior mediastinal masses. 2 Mariel EGels et al had successfully shown thoracotomy to be a suc-cessful postchemotherpay intervention in disseminated nonseminomatous testicular germ cell tumours. 6 Same approachhas been used successfully for resection of intrathoracic pri-mary germ cell tumour. 3 In light of the cited literature VATSand Thoracotomy were performed after normalization of thetumour markers in the presentation. The tumor mass in themediastinum which was involving the phrenic nerve, peri-cardium, lung and portion of left ventricle were completelyresected via thoracotomy.The surgery was unremarkable andno major complications were observed. Though multiple primary tumours remain a rather uncommon clinical observation, it is a potential risk in indi-viduals with germ line mutations and epimutations in theDNA repair genes. Suter C M et al very recently studied twoindividuals with multiple primary tumours and elucidatedan epimutation involving allele-specific and mosaic hyper-methylation of the DNA mismatch repair gene MLH1. 7 Asimilar genetic aberration in our patient may be responsiblefor the co existing primary tumours.Hence, we support and recommend the workupcomprising CT imaging of the chest, and temporal biochem-ical assessment of tumour markers for mediastinal germ celltumours. References 1.Whooley BP, Urschel JD, Antkowiak JG, Takita H. Primary tumors of themediastinum J Surg Oncol 1999; 70:95-9.2.Walsh GL, Taylor GD, Nesbitt JC, Robert J. Amato Intensive Chemotherapyand Radical Resections for Primary Nonseminomatous Mediastinal Germ cellTumors Ann. Thorac Surg 2000; 69: 337-43.3.Okur E, Halezeroglu S, Somay A, Atasalihi A. Unusual intrathoracic locationof a primary germ cell tumor. Eur J Cardio-thorac Surg 2002; 22:651-3.4.Dorota Plewicka, Wojceich Stefanek, Jan Luczyc-Wyhowski Epidermoid cystof the testis . Case Rep Clin Pract Rev 2003, 4, 266-68.5.Szymendera JJ, Zborzil J, Sikorowa L, Lenko J, Kaminska JA, Gadek AEvaluation of five tumor markers (AFP, CEA, hCG, hPL and SP1) in moni-toring therapy and follow-up of patients with testicular germ cell tumors.Oncology 1983;40:1-10.6.Mariel E. gels, Hoekstra HJ, Sleijfer DT, Nijboer AP, Molenaar WN, Ebels T.Thoracotomy for Post chemotherapy Resection of Primary Residual Tumor Mass in Patients with Nonseminomatous Testicular Germ Cell tumors. Chest1997; 112; 967-73.7.Suter CM, Martin DI, Ward RL Germline epimutation of MLH1 in individu-als Figure 3. The resection of the actual tumor is being shown. Notice the anterior basilar segment of left lower lobe attached to the tumor which is also resected with the mass. The underlying heart isalso seen. 190J Pak Med Assoc
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