scholarly journals MON-LB040 Challenge in Diagnosing and Treating of Mediastinal Paraganglioma

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Basil Alomair ◽  
Anwar Jammah

Abstract Paraganglioma is a rare type of neuroendocrine tumor of the autonomicnervous system.1 It is extremely rarely present in mediastinum (less than2%).2Case report We report a 43 years-old woman, she was referred to our clinic with ahistory of high blood pressure (BP) for two years, accompanied by spellsof sweating, headache, anxiety and palpitation. She was provisionaldiagnosed with essential hypertension and was on four medications onmaximum dose.The patient declined any previous surgical history, and review of systemswas unremarkable. On examination, she was conscious and alert. Her vitalsigns were within normal limit. She reported to our clinic with homereadings of BP ranging from 150-180 mmHg systolic and 90-100 mmHgdiastolic.Initial investigations of basic chemistry, renal profile, hormonal profile,aldosterone, and renin were within the normal range.Serum Normetanephrine was 800 ng/L (normal < 180 ng/L), 24 hoursurine of Normetanephrine was 5205 microgram/24 hours (normal < 600microgram/24 hours), and 24 hours urine Metanephrine was withinnormal. CT scan of the adrenal and MRI abdomen showed normal adrenalglands and no mass in the abdomen. MIBG scan was normal for the wholebody. An unusual location of the Paraganglioma was suspected, andfurther images were carried on. A CT chest showed 4x4 cm posteriormediastinal mass in area of Aortopulmonary window, adherent toposterior wall of Aorta and pulmonary artery. Positron EmissionTomography (PET) scan was done prior to the surgery.Surgical resection of the mass with reconstruction of both pulmonaryartery and pericardium was done without any complications. Moreover,histopathology confirmed the diagnosis of Paraganglioma.The patient was followed up with a CT scan six months post-operativelyas an outpatient, along with 24-hour urine Metanephrine and Normetanephrine. All labs and imaging were normal. The patient hadanother Normetanephrine measurement twelve months later and it wasnormal. Now, she has been followed for seven years with no moresymptoms and normal BP readings. All of her antihypertensive agentswere discontinued. ConclusionWe experience an unusual location of Paraganglioma at the mediastinum,which is representing less than 2% of all Paraganglioma. However, in thepresence of characteristic of Paraganglioma symptoms, a thoroughassessment should be carried out and such location of mediastinumshould be suspected and investigated.References:1. Institute NC. Pheochromocytoma and Paraganglioma Treatment(PDQ®)-Health Professional Version. https://www.cancer.gov/types/pheochromocytoma/hp/pheochromocytoma-treatment-pdq. Published 2019. Accessed February 1, 2020.2. Paraganglioma: An Uncommon Cause of Mediastinal Mass. -PubMed - NCBI. https://www.ncbi.nlm.nih.gov/pubmed/32000513.Accessed February 1, 2020.

2012 ◽  
Vol 15 (3) ◽  
pp. 170
Author(s):  
Hee Moon Lee ◽  
Dong Seop Jeong ◽  
Pyo Won Park ◽  
Wook Sung Kim ◽  
Kiick Sung ◽  
...  

A 54-year-old man was referred to our institution with hemoptysis and hoarseness of 1 year's duration. A computed tomography (CT) scan showed an anterior mediastinal mass (2.5 cm x 1.0 cm), which was diagnosed as thymoma. The tumor was resected under a sternotomy. The tumor had invaded the anterior wall of the ascending aorta. With the patient under cardiopulmonary bypass, the aortic wall invaded by the mass was resected, and arterial reconstruction was performed with patch material. The tumor was revealed to be a tumor of neuronal origin. The patient's postoperative course was uneventful. The patient was discharged on postoperative day 9. One year after the operation, a follow-up chest CT evaluation showed no specific complications or recurrence.


2003 ◽  
Vol 61 (1) ◽  
pp. 91-94 ◽  
Author(s):  
Oswaldo Ignácio de Tella Jr ◽  
Marco Antonio Herculano ◽  
Mirto Nelso Prandini ◽  
João Norberto Stavile ◽  
Antonio de Pádua Furquim Bonatelli

We present CT scan, MRI and histopathologic findings of two patients harboring a rare type of meningioma. In the first case, a 52 year-old male patient, a large parasellar mass invading the cavernous sinus, infiltrating the infratemporal fossa and extending as low as C2 was founded. The tumor was isointense on T1, enhanced strongly with gadolinium injection, and was hyperintense on T2. In the second case, a 19-year-old male patient, a large high density temporal right mass was disclosed by CT scan. Both patients were taken to surgery. In the first case, only a partial removal was possible to be accomplished due to a severe intra operative bleeding. In the second case, the tumor was totally removed. Both showed characteristic pathologic findings of a meningioma resembling a chordoma. Meningioma is a relatively common intracranial tumor, occurring most frequently in adults, showing a wide variety of growth patterns. We described a pattern that had a peculiar chordoma-like appearance. The pathological findings and the differential diagnosis from chordoma are discussed.


2008 ◽  
Vol 97 (1) ◽  
pp. 42-49 ◽  
Author(s):  
D. Hoem ◽  
D. Jensen ◽  
S. Steine ◽  
T. E. Thorsen ◽  
A. Viste ◽  
...  

Background and Aims: Insulinoma is a very rare type of islet cell tumour, but nevertheless the most common endocrine tumour of the pancreas. We aimed at reviewing our clinical experience with this tumour type and to assess whether organ culture could be obtained from surgically resected insulinoma material. Material and Methods: All patients with insulinomas (6 men and 10 women) referred to Haukeland University Hospital between 1986 and 2006 were included in the study. Median age of onset was 53 years (range 21–74). Biochemical diagnosis was established during a 72 h fast test. Imaging and localization of the tumours were performed with intra-operative ultrasonography, endoscopic ultrasonography, CT-scan and/or transcutaneous ultrasonography. For six patients, organ cultures were set up from tumour tissue fragments. Results: The annual incidence of insulinoma was 0.8 per million. The patients generally presented with non-specific, episodic symptoms, which often were mistaken for cardiovascular, neurological or diabetic disease and in some cases delayed the diagnosis with several years. Two patients had diabetes prior to the diagnosis of insulinoma. Patient weight gain was probably due to increased food intake, compensating for the hypoglycemia. Intra-operative ultrasonography detected all tumours correctly, whereas 73% were detected by endoscopic ultrasonography and 38% by CT scan. Five insulinomas were located in the head, eight in the body and three in the tail of the pancreas. All were removed by open-access surgery, eleven cases by resection and five by enucleation. One tumour was malignant with liver metastases and two patients had tumours defined as borderline. Insulinoma tissue fragments developed into spheroids during the first week of culturing and insulin secretion into the media was demonstrated. Conclusions: Insulinomas are rare and diagnostically challenging tumours. Intra-operative ultrasonography was superior to other imaging modalities to locate the lesion. In organ culture, insulinomas readily form spheroids which may be used to yield insight into beta-cell biology.


2021 ◽  
Vol 2 (2) ◽  
Author(s):  
Wenny Supit

Abstract: Basosquamous carcinoma (BSCC) is a rare type of skin cancer with an incidence of less than 2% of all skin cancers and the risk of local recurrence ranges between 15% and 50%. We reported a male, aged 57 years old, working as a civil employee, came to the eye clinic with a recurrent BSCC in the right lower palpebral since a month ago. The patient was previously diagnosed as BSCC and had undergone a surgery to remove cancer cells in 2017. However, in 2019, a scan of the axial incision of the head revealed a tumor mass in the right infraorbital area with T4N0Mx. The hypodense structured tumor mass showed increased contrasting. The opera-tive management performed was extensive excision and deep excision with rotational flaps and drainage attached. Histopathological examination of the tumor tissue and excision margins, optic nerve tissue, as well as infraorbital bone tissue indicated a BSCC. Diagnosis of BSCC was confirmed based on anamnesis, physical examination, ophthalmic examination, head CT scan with contrast, and histopathological examination. This case report was aimed to explore BSCC especially in palpebra due to the lack of data of similar cases as well as the potential for diagnosis and promising management.Keywords: basosquamous carcinoma (BSCC), palpebral, reccurent  Abstrak: Karsinoma basoskuamosa (BSCC) merupakan jenis kanker kulit yang langka dengan kejadian kurang dari 2% dari semua jenis kanker kulit namun dengan risiko kekambuhan lokal berkisar 15% dan 50%. Kami melaporkan kasus seorang laki-laki berusia 57 tahun, bekerja sebagai ASN, datang ke klinik mata dengan BSCC palpebra kanan bawah berulang sejak satu bulan lalu. Pasien sebelumnya didiagnosis dengan BSCC dan telah menjalani operasi untuk mengangkat sel kanker pada tahun 2017. Namun, pada tahun 2019, pemindaian kepala sayatan aksial menunjukkan adanya massa tumor di area infraorbital kanan dengan T4N0Mx. Massa tumor berstruktur hipodens dengan kontras yang meningkat. Manajemen operatif dilakukan eksisi ekstensif dan eksisi dalam dengan flap rotasi dan drainase terpasang. Pemeriksaan histopatologik dilakukan terhadap jaringan tumor dan margin eksisi, jaringan saraf optik, serta jaringan tulang infraorbital dengan simpulan suatu BSCC. Pada kasus ini diagnosis BSCC ditegakkan berdasarkan anamnesis, pemeriksaan fisik, pemeriksaan oftalmologi, pemeriksaan penunjang berupa CT scan kepala dengan kontras, dan pemeriksaan histopatologik. Laporan kasus ini bertujuan untuk mendalami BSCC khususnya di palpebra karena minimnya data mengenai kasus yang serupa, serta potensi diagnosis dan penatalaksanaan yang menjanjikannya.Kata kunci: karsinoma basoskuamosa (BSCC), palpebra, rekuren


1983 ◽  
Vol 18 (5) ◽  
pp. 581-584 ◽  
Author(s):  
Joseph D. Awotwi ◽  
Jaime Zusman ◽  
William W. Waring ◽  
Robert C. Beckerman

2020 ◽  
Vol 11 ◽  
pp. 204062072097761
Author(s):  
Lena Horvath ◽  
Andreas Seeber ◽  
Christian Uprimny ◽  
Dominik Wolf ◽  
David Nachbaur ◽  
...  

Combined 18F-fluoro-deoxyglucose ([18F]FDG) positron emission tomography and computed tomography ([18F]FDG-PET/CT) is increasingly used for the diagnostic and therapeutic management of hematologic and non-hematologic malignancies. Here, we describe a unique case of a patient presenting with very severe aplastic anemia and a mediastinal mass showing disseminated hypermetabolic lesions of the bones after receiving granulocyte colony-stimulating factor (G-CSF), highly suspicious for disseminated metastatic lesions. A 71-year-old patient presented with a 3 week history of dyspnea and fatigue. Blood tests showed severe pancytopenia and iliac crest bone marrow biopsy revealed an extensively hypoplastic bone marrow. Diagnostic work-up by histology, conventional cytogenetics and flow cytometry confirmed the diagnosis of very severe aplastic anemia. Besides blood transfusions, the patient was treated with G-CSF. Furthermore, computed tomography revealed a suspect mass in the anterior mediastinum, presenting with moderate glucose metabolism in the subsequent [18F]FDG-PET/CT scan. In addition, multiple disseminated and highly metabolic bone lesions of primarily the ribs were detected, suspicious of malignant bone infiltration. Since physiologic bone marrow activation by G-CSF-stimulation could not be ruled out, G-CSF therapy was interrupted to repeat the PET/CT scan 10 days later. On the second [18F]FDG-PET/CT the moderately hypermetabolic mediastinal mass persisted. However, the initially FDG-avid bone lesions almost completely resolved, rendering the diagnosis of G-CSF-induced bone marrow hypermetabolism very likely without the need for further invasive diagnostic procedures. The mediastinal mass was thereafter histologically verified as thymoma. Interpretation of [18F]FDG-PET/CT in patients with aplastic anemia may be complicated by the frequent therapeutic use of G-CSF. With G-CSF, islets of residual bone marrow activity can be visualized on [18F]FDG-PET/CT images that might be misinterpreted as malignant bone infiltration. Repeating PET/CT scan after G-CSF discontinuation can prevent unnecessary invasive diagnostic procedures in these patients.


2017 ◽  
Vol 4 (2) ◽  
pp. 823
Author(s):  
Bishal Gautam ◽  
Sanjeev Devgarha ◽  
R. M. Mathur ◽  
Anula Sisodiya

Pulmonary blastomas are a rare aggressive neoplasm comprising 0.25-0.5% of all primary lung tumours. Morphologically they mimic foetal lung tissue before 4 months gestation. Study reports a case of 23-year-old male patient who came with heaviness in chest and breathing difficulty since one and a half months. On CT scan of chest, findings are suggestive of anterior mediastinal mass extending till right hila with cardiac displacement and compression. Histopathology confirmed the presence of pulmonary blastoma.


2010 ◽  
Vol 23 (1) ◽  
pp. 80-83
Author(s):  
Khandaker Abu Talha ◽  
ATM Mosharef Hossain ◽  
Khan Asaduzzaman ◽  
Amer Wahed ◽  
Farhana Selina ◽  
...  

We present a lady with scalp swelling and headache. CT scan of brain showed a right parietal bony lesion without any dural or parenchymal invasion. Tumour was removed. Bony gap was repaired by acrylic bone cement. Post operative recovery was uneventful. Biopsy report of tumour was primary calvarial meningioma. This is a very rare type of CNS tumour. Only very few cases were reported before. TAJ 2010; 23(1): 80-83


Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 5200-5200 ◽  
Author(s):  
Jacqueline Baptista ◽  
Eris Tollkuci ◽  
Sunita Nathan ◽  
John J. Maciejewski ◽  
Melissa L. Larson ◽  
...  

Abstract T-cell acute lymphoblastic leukemia (ALL) is a very challenging disease to attain durable remission once relapses. We treated a 40 year-old female with T-cell ALL, normal cytogenetic who relapsed twice after her matched related donor allogeneic stem cell transplantation (allo-SCT) with Nelarabine achieving third complete remission prior to second allo-SCT. She was initially treated with HyperCVAD x 5 cycles with IT chemo (negative cytopathology). After achieving complete remission (CR), she received PK-directed IV Busulfan and Fludarabine myeloablative conditioning followed by 12/12 HLA-MRD allo-SCT with 11.06 x 106 CD34+cells/kg. GVHD prophylaxis was Tacrolimus and mini-Methotrexate (5 mg/m2/dose). She engrafted on time. She never had GVHD. On day+ 100, bone marrow biopsy revealed relapsed disease involving 60% of marrow space. Patient was treated with one cycle augmented HyperCVAD resulted in second CR. She developed elevated transaminases, liver biopsy was consistent with NASH and drug-induced liver injury, negative for GVHD. She was given alpha interferon 3 million units MWF in attempt to induce GVHD/GVL while pending insurance approval for donor lymphocyte infusion. She developed full donor chimerism as well as extensive chronic GVHD involving skin, liver, skin, lung, eyes, vagina, and mouth after interferon. She was treated only with topical steroid therapies and remained in CR until day+266. On Day+266, she was again found to have relapse of her disease with significant leukocytosis and circulating blasts. CT scan showed recurrent large anterior mediastinal mass. She was treated with Cytoxan (500 mg/m2) and Etoposide (100 mg/m2 x 5 days), complicated with neutropenic fevers before receiving outpatient Nelarabine. She recovered with progressive leukemia, which was treated with a single dose of Cytoxan 600mg/m2. She developed diffuse alveolar hemorrhage, treated in the MICU with high dose steroids and antimicrobials. She was discharged to outpatient clinic and given Nelarabine at 1500 mg/m2 on days +1, +3, +5, every 3 weeks for 2 cycles. Her clinical condition and peripheral counts improved with disappearing peripheral blasts after the initial cycle of Nelarabine. She received second cycle and subsequent restaging studies showed remission marrow (CR-3) with complete resolution of mediastinal mass on CT scan. She developed mild neuropathy after Nelarabine. She is currently inpatient receiving her for second MRD allo-SCT. This case is noteworthy and clearly illustrates lack of GVL effect in T-cell ALL and an efficacy of two cycles of Nelarabine even in the setting of refractory T-cell ALL relapsing twice after allogeneic SCT. Disclosures No relevant conflicts of interest to declare.


Sign in / Sign up

Export Citation Format

Share Document