Adjuvant radiotherapy in a case of atypical meningioma after gross total resection: an unresolved issue

We present a case of a 48-year-old man diagnosed with parasagittal atypical meningioma (AM) involving biparietal bones with intracranial and extracranial extension up to galea aponeurotica of the scalp. The patient underwent Simpson’s grade 2 resection (GTR (gross total tumour resection) with coagulation of dural attachment). Currently, in AMs, the role of adjuvant radiotherapy is controversial after GTR. Here, through this case, we have discussed in detail issues related to tumour origin, that is, primary versus secondary extradural meningioma and controversial topics regarding the role of adjuvant radiotherapy in the management of AMs. We have presented our radiation treatment strategy addressing the high-risk zones related to tumour extension in this case.

Atypical presentation of intravascular leiomyomatosis mimicking advanced uterine sarcoma: modified laterally extended endopelvic resection with preservation of pelvic neural structures

Intravascular leiomyomatosis is a rare, benign tumour of myometrial smooth muscle. Despite being non-invasive, these tumours can proliferate aggressively within vascular structures including pelvic vessels, the vena cava and the heart. We discuss a 77-year-old woman presenting with a 9 cm uterine mass extending into the right adnexa and ovarian vein. Following hysteroscopic biopsy, palliative radical surgical resection was performed for suspected stage IV leiomyosarcoma. Tumour extension into the pelvic sidewall and obturator fossa indicated a modified laterally extended endopelvic resection combined with skeletonisation and preservation of the pelvic neurovasculature, ultimately providing a curative procedure with minimal functional neurological morbidity. We present this unusual case to assist in the development of a consensus for optimal case management where formal guidelines are not yet available. We summarise current understanding of intravascular leiomyomatosis and highlight the value of advanced surgical techniques using knowledge of complex ontogenetic and pelvic neuroanatomy in its management.

Primary cardiac B cell lymphoma in an immunocompetent patient

Primary cardiac lymphoma is a rare entity of extranodal lymphoma and is observed with increasing frequency in immunocompromised hosts. However, a considerable proportion of cardiac lymphomas still occur in immunocompetent patients. We report the case of a 55-year-old immunocompetent Japanese man with a large amount of pericardial fluid and the presentation of heart failure secondary to primary cardiac B cell lymphoma, which was diagnosed by cytological examination of pericardial fluid and imaging. The right atrium, right ventricle and pericardium were affected by the tumour, which encased the mid/distal portion of the right coronary artery (RCA). Pretreatment optical coherence tomography of the RCA demonstrated no tumour extension into the vascular structure but a focal mural thrombus. We initiated chemotherapy (steroid therapy then COP at half dose/R-CHOP/R-CHASE) [COP (C: Cyclophosphamide, O: Oncovin, P: Prednisolone) R-CHOP (R: Rituximab, C: Cyclophosphamide, H: Doxorubicin Hydrochloride, O: Oncovin, P: Prednisolone) R-CHASE (R: Rituximab, C: Cyclophosphamide, HA: high dose Cytarabine, S: Steroid, E: Etoposide)]with administration of low-dose aspirin to prevent possible ischaemic events. The patient had a good clinical course without adverse events except for transient pericarditis.

Spinal Tumor Surgery- Our Experiences of 35 Cases in a Peripheral Hospital in Bangladesh

Introduction: Surgical outcome of spinal tumour varies depending on a number of factors such as: site of the tumour, compression within the spinal canal, the histological characteristics of the tumour, the neurological progression and initial response to corticosteroid therapy, patient’s age, comorbidity, tumour extension, involvement of neighboring structures and organs etc. Materials & Methods: The 35 patients with spinal tumour underwent surgery by our team in 10 years (January 2009 - December 2018) were reviewed retrospectively. Results: Analysis of the surgical outcome of our spinal tumour patients was done on different variables like age, sex, presenting symptoms, neuroimaging, comorbidities etc. The aim of surgery was decompression of the spinal cord, total removal of the tumour when possible and spinal stabilization when needed. Out of our 35 patients with spinal tumour, extradural tumour comprises 8, intradural extramedullary tumour 25 and intramedullary tumour 2. Conclusion: The aim of this study is to analyze the data to made conclusion for more effective strategy as per site, size, type, resectibility and histological variety to establish and effective treatment protocol and prevention of per-operative and post-operative complications. Intradural extramedullary tumour can be radically resected with no mortality and minimal peri-operative morbidity. But resection of intramedullary spinal tumour is difficult, hazardous and usually incomplete, so needs much more skilled and meticulous surgical hands. Medicine Today 2021 Vol.33(1): 38-41

Clear Cell Sarcoma of Kidney in Adult with Extensive Invasion of Pelvic Veins: Dual Aberration

Clear Cell Sarcoma of Kidney (CCSK) is a highly malignant renal tumour. The mean age of diagnosis is between 12-36 months. Due to heterogeneous histological appearance and age group affection similar to wilms tumour, it often leads to misdiagnosis. It has a tendency to metastasise distantly to bone and its therapeutic response differs from other childhood kidney tumours. So use of Immunohistochemical (IHC) markers becomes essential in many cases in differentiating CCSK from other paediatric renal neoplasms. It is extremely rare in adults, till now only 26 adult cases have been reported in the medical literature. Here, the case of a 49-year-old male presenting with haematuria and pedal oedema is reported. On radiological examination, he had a large left renal mass with tumour extension to Left Renal Vein (LRV) and Inferior Vena Cava (IVC) as thrombi. Histology and Immunohistochemistry (IHC) study revealed CCSK ruling the other differentials. This case is presented for its rarity in adult patients, unusual clinical feature of widespread vascular invasion rather than bone metastasis, simulating Renal Cell Carcinoma (RCC) and aggressive clinical behaviour. The diagnostic challenges faced by pathologist and clinicians further necessitate the proper diagnosis of the tumour for better management of such cases.

Intracardiac extension of chondroblastic osteosarcoma

Intravascular tumour extension invading the intracardiac space is rarely seen with osteosarcoma. We present a patient with a history of previously resected pelvic osteosarcoma who was later found to have a local recurrence with continuous intravascular extension from the right femoral vein to the right atrium. Preoperative imaging studies initially described extensive thrombus burden, and a multidisciplinary approach involving open and percutaneous thrombectomy was planned. Intraoperative inspection and pathological analysis revealed unresectable malignant solid tumour rather than thrombus. Though exceedingly rare, the possibility of metastatic tumour must be considered when planning treatment strategies for these patients.

Pegvisomant in combination or pegvisomant alone after failure of somatostatin analogs in acromegaly patients: an observational French ACROSTUDY cohort study

Objective After surgery, when somatostatin analogs (SAs) do not normalise IGF-I, pegvisomant (PEG) is indicated. Our aim was to define the medical reasons for the treatment of patients with PEG as monotherapy (M) or combined with SA, either as primary bitherapy, PB (PEG is secondarily introduced after SA) or as secondary bitherapy, SB (SAs secondarily introduced after PEG). Methods We retrospectively analysed French data from ACROSTUDY. Results 167, 88 and 57 patients were treated with M, PB or SB, respectively, during a median time of 80, 42 and 70 months. The median PEG dose was respectively 15, 10 and 20 mg. Before PEG, the mean IGF-I level did not differ between M and PB but the proportion of patients with suprasellar tumour extension was higher in PB group (67.5% vs. 44.4%, P = 0.022). SB regimen was used preferentially in patients with tumour increase and IGF-I level difficult to normalise under PEG. In both secondary regimens, the decrease of the frequency of PEG’s injections, compared to monotherapy was confirmed. However, the mean weekly dose of PEG between M and PB remained the same. Conclusions The medical rationale for continuing SAs rather than switching to PEG alone in patients who do not normalise IGF-I under SAs was a tumour concern with suprasellar extension and tumour shrinkage under SA. A potential explanation for introducing SA in association with PEG appears to be a tumour enlargement and difficulties to normalise IGF-I levels under PEG given alone. In both regimens, the prospect of lowering PEG injection frequency favoured the choice.

Childhood Liver Tumours

Childhood liver tumours are a heterogeneous group of neoplasms encompassing individually rare histological varieties. This chapter discusses the two most frequent subtypes—hepatoblastoma and hepatocellular carcinoma. In relation to hepatoblastoma, it covers the biology, epidemiology, clinical presentation, diagnosis, and discusses the different treatment strategies including risk-adapted treatment and orthotopic liver transplantation. The chapter reviews the renewed histological classification of paediatric primary liver tumours, the revised PRETEXT (pre-treatment extent) system to describe tumour extension, and the novel risk-stratification system for hepatoblastoma. It also reviews the treatment results and the prognosis of patients with hepatoblastoma. Finally, the chapter discusses the even rarer tumour, hepatocellular carcinoma.

Pharyngeal closure after a total laryngectomy: mechanical versus manual technique

ObjectiveTo verify the main advantages and drawbacks of mechanical suturing for pharyngeal closure after total laryngectomy versus a manual suturing technique.MethodsA retrospective review was carried out of 126 total laryngectomies performed between 2008 and 2018. Manual closure was performed in 80 cases (63.5 per cent) and mechanical suturing was performed in 46 cases (36.5 per cent).ResultsMechanical suturing was used significantly more frequently in patients with: glottic tumours (p = 0.008), less local tumour extension (p = 0.017) and less pre-operative morbidity (p = 0.014). There were no significant differences in the incidence of pharyngocutaneous fistula between the manual suture group (16.3 per cent) and the mechanical suture group (13.0 per cent) (p = 0.628). None of the patients treated with mechanical suturing had positive surgical margins. Cancer-specific survival for the mechanical suture group was higher than that for the manual suture group (p = 0.009).ConclusionMechanical suturing of the pharynx after total laryngectomy is an oncologically safe technique if used in suitable cases.