Primary non-Hodgkin's lymphoma of the thyroid, type MALT: a case report from the Adolphe SICE General Hospital, Pointe Noire (Congo - Brazzaville)

Primary lymphomas of the thyroid are rare tumours, representing less than 5% to 15% of thyroid cancers. These lymphomas develop on a pre-existing thyroid disease, notably Hashimoto's thyroiditis, or as a secondary site of a disseminated lymphoma. This pathology is aggressive and presents as a painful, rapidly evolving goitre with signs of compression. The most common histological types are non-Hodgkin's lymphoma type B. We report a case of thyroid non-Hodgkin's lymphoma of the MALT type in a 71 year old woman with a specific history; the positive diagnosis was made thanks to a histological study of the surgical specimen completed by an immunohistochemical study on paraffin sections. The postoperative clinical course after six years was favourable, with no local recurrence or distant metastasis. Keywords: Goiter; Primary Lymphoma; Thyroid; MALT; Immunohistochemistry

A case report on asymptomatic and biochemically silent pheochromocytoma in adrenal incidentaloma

Pheochromocytoma are rare tumours originating from the chromaffin tissue. The clinical manifestations are variable and are not specific as pheochromocytoma and often imitate other diseases. The diagnosis is established by measurement of catecholamines and their metabolites in urine or plasma and by radiographic studies for localisation. Surgical removal of the tumour is the preferred treatment. 45 years old female presented with adrenal incidentaloma of about 6.1×6.2×5.4 cm well defined heterogenous lesion with internal cystic areas seen in right adrenal region abutting upper pole of right kidney. Biochemical investigations for adrenal hormones including plasma aldosterones, cortisols, plasma metanephrines, 24 hrs urinary metanephrines and VMA were found to be normal. Proceeded with adrenelectomy and histopathology of the specimen revealed pheochromocytoma as diagnosis. Pheochromocytoma leads to high mortality and morbidity rates if untreated. Fractionised metanephrines and catecholamines in a 24 hrs urine analysis is the preferred biochemical test. In a biochemically silent pheochromocytoma imaging modalities are used to identify and locate the tumour. Adequete alpha and beta blockade should be ensured before tumour removal. Surgery is recommenced irrespective of size and normal biochemical study to prevent complications.

Whole Genome Sequencing and Custom Liquid Biopsy Markers in Sarcomas

Background: Sarcomas are rare tumours with heterogeneous clinical behaviour including varying rates of metastasis. Clinical treatment and follow-up rely on crude grading systems with uncertain accuracy for individual patients. Whole genome sequencing (WGS) detects both structural variation and single nucleotide variants and may thus add important diagnostic/prognostic information. Liquid biopsies (LB) may potentially identify hematogenous spread and treatment response rate but further evaluation in sarcomas is needed. Methods: In this study we explore the performance of individualized LB in four patients with different types of sarcomas. Fresh frozen tumour tissue was sequenced using WGS and whole transcriptome sequencing. Three putative driver variants or one fusion gene were selected per case and custom digital droplet PCR (ddPCR) assays were designed, evaluated on tumour DNA and used to assess levels of cell free tumour DNA in plasma taken prior to surgery. Results: In LB, ddPCR identified three variants in one patient with metastatic disease. The remaining three patients had negative LBs and were without disease at follow-up (>18 months after surgery). Conclusions: WGS is a powerful tool to detect all types of genetic changes in sarcoma and can facilitate clinical diagnosis/classification while custom LB may add prognostic information.

Object Detection Improves Tumour Segmentation in MR Images of Rare Brain Tumours

Tumour lesion segmentation is a key step to study and characterise cancer from MR neuroradiological images. Presently, numerous deep learning segmentation architectures have been shown to perform well on the specific tumour type they are trained on (e.g., glioblastoma in brain hemispheres). However, a high performing network heavily trained on a given tumour type may perform poorly on a rare tumour type for which no labelled cases allows training or transfer learning. Yet, because some visual similarities exist nevertheless between common and rare tumours, in the lesion and around it, one may split the problem into two steps: object detection and segmentation. For each step, trained networks on common lesions could be used on rare ones following a domain adaptation scheme without extra fine-tuning. This work proposes a resilient tumour lesion delineation strategy, based on the combination of established elementary networks that achieve detection and segmentation. Our strategy allowed us to achieve robust segmentation inference on a rare tumour located in an unseen tumour context region during training. As an example of a rare tumour, Diffuse Intrinsic Pontine Glioma (DIPG), we achieve an average dice score of 0.62 without further training or network architecture adaptation.

Proton therapy with a fixed beamline for skull-base chordomas and chondrosarcomas: outcomes and toxicity

Aim This study presents an analysis (efficacy and toxicity) of outcomes in patients with skull-base chordomas or chondrosarcomas treated with a fixed horizontal pencil proton beam. Background Chordomas (CAs) and chondrosarcomas (CSAs) are rare tumours that are usually located near the base of the skull and very close to the brain's most critical structures. Proton therapy (PT) is often considered the best radiation treatment for these diseases, but it is still a limited resource. Active scanning PT delivered via a fixed pencil beamline might be a promising option. Methods This is a single-centre experience describing the results of proton therapy for 31 patients with CA (n = 23) or CSA (n = 8) located near the base of the skull. Proton therapy was utilized by a fixed pencil beamline with a chair to position the patient between May 2016 and November 2020. Ten patients underwent resection (32.2%), 15 patients (48.4%) underwent R2 resection, and 6 patients had unresectable tumours (19.4%). In 4 cases, the tumours had been previously irradiated. The median PT dose was 70 GyRBE (relative biological efficacy, 1.1) [range, 60 to 74] with 2.0 GyRBE per fraction. The mean GTV volume was 25.6 cm3 [range, 4.2–115.6]. Patient demographics, pathology, treatment parameters, and toxicity were collected and analysed. Radiation-induced reactions were assessed according to the Common Terminology Criteria for Adverse Events (CTCAE) v 4.0. Results The median follow-up time was 21 months [range, 4 to 52]. The median overall survival (OS) was 40 months. The 1- and 2-year OS was 100%, and the 3-year OS was 66.3%. Four patients died due to non-cancer-related reasons, 1 patient died due to tumour progression, and 1 patient died due to treatment-related injuries. The 1-year local control (LC) rate was 100%, the 2-year LC rate was 93.7%, and the 3-year LC rate was 85.3%. Two patients with CSA exhibited progression in the neck lymph nodes and lungs. All patients tolerated PT well without any treatment interruptions. We observed 2 cases of ≥ grade 3 toxicity, with 1 case of grade 3 myelitis and 1 case of grade 5 brainstem injury. Conclusion Treatment with a fixed proton beam shows promising disease control and an acceptable toxicity rate, even the difficult-to-treat subpopulation of patients with skull-base chordomas or chondrosarcomas requiring dose escalation.

P-OGC92 Esophagogastric Lymphomas; Lymphomas outside Lymphoid tissue

Background Primary gastric lymphomas are rare tumours and account for 5% of all gastric neoplasms. Esophaphageal lymphomas are even rarer, accounting for 1% of all oesophageal tumours. Incidence of these tumours have been increasing over time and there is increasing need to understand this tumour subtype. This is a single centre study of esophagogastric lymphomas over a period of 15 years. Methods Between 2005 to 2020; patients who were diagnosed of having esophagogastric lymphomas, were retrospectively studied. Their clinical records were assessed for age, gender, clinical signs and symptoms, histological type, association with H pylori, LDH levels, stage at diagnosis, treatment type, Endoscopy and clinical follow up and survival. Results 66 patients were diagnosed of having lymphoma, out of which 4 (6%) were oesophageal and 62 (93.93%) were gastric. Median age was 77 years (Range 41-102 years), 39 were male (59%) and 27(41%) were female. Anaemia was the commonest sign (53%), followed by nausea/vomiting (45%), weight loss (44.5%), abdominal pain (40.6%) and dysphagia (24%). 15.6% patients were found to have H pylori and 10% of patients had Barrett’s. Diffuse Large B Cell Lymphomas were commonest tumours (28.33%) followed by MALToma’s (26.6%). 60% patients were T3/4 at the time of diagnosis and 9.7% had other OG malignancies. 53% patients received antibiotics, 61% received chemotherapy, 16% received chemoradiotherapy and 16% received surgical resection. Median survival was 3 years (range 0-14). Conclusions Esophagogastric lymphomas are successfully treated with chemotherapy with promising survival. The stage at diagnosis and presence of comorbidities are limiting factors in overall disease prognosis.

Case report: tricuspid valve myxoma: an uncommon presentation of a common tumor

Amongst the benign cardiac tumors atrial Myxomas are commonest. Tricuspid valve attachment is an uncommon site for cardiac Myxoma. We present a case of an 18 years old girl who presented to us with history of shortness of breath, abdominal distension, leg swelling, palpitations and dizziness for two months. On Echocardiography there was a large pedunculated mass attached to tricuspid valve. She underwent surgical excision of the mass. Biopsy confirmed the Myxoma. In conclusion, Myxoma originating from tricuspid valve are rare tumours which can cause tricuspid valve obstruction and right heart failure symptoms due to their location. In these cases shortness of breath, embolisation and symptoms of venous congestion are commonly observed. Key Words: Case report, Myxoma, right heart, cardiac CT, tricuspid valve. Continuous...

Fetus diagnosed with cervical teratoma delivered by exit procedure (Extra uterine intrapartum treatment)

Congenital cervical teratomas are rare tumours of germ cells that should be diagnosed antenatally by ultrasound during anomaly scan or even earlier. The incidence of teratomas of the head and neck is 3-5%. We are presenting a case of rare cervical teratoma.1 Congenital cervical tumours are often clinically dramatic, though basically benign. Prognosis is favourable only if the airway is quickly stabilized and necessarily surgical procedure is planned and executed effectively.2 Case presentation: A 34-year-old female presented at 32 weeks of gestation, at Al Amal National Hospital where an ultrasound examination revealed a single viable cephalic fetus with a huge irregular heterogeneous anterior neck mass suspected as congenital cervical neck teratoma. The delivery was planned at 37 weeks gestation. The team was assembled for EXIT procedure which includes the obstetricians, neonatologist, anthologist, ENT surgeons. At 37 weeks of gestation, the child was maintained on maternal circulation after caesarean section until successfully intubated (Extra Uterine Intrapartum Treatment (EXIT) was performed at Al Amal National Hospital after liaison with the anesthetist, neonatologist and the pediatric surgeon. A female fetus weighing 3.8 kg was delivered and intubated immediately. Unfortunately, the newborn died at 48hours of age. Conclusion: Prenatally diagnosed head and neck teratomas can result in early neonatal death if the delivery was not planned correctly with right multidisciplinary team. In this case EXIT is the procedure of choice.

Artificial intelligence in oncology: current applications and future perspectives

Artificial intelligence (AI) is concretely reshaping the landscape and horizons of oncology, opening new important opportunities for improving the management of cancer patients. Analysing the AI-based devices that have already obtained the official approval by the Federal Drug Administration (FDA), here we show that cancer diagnostics is the oncology-related area in which AI is already entered with the largest impact into clinical practice. Furthermore, breast, lung and prostate cancers represent the specific cancer types that now are experiencing more advantages from AI-based devices. The future perspectives of AI in oncology are discussed: the creation of multidisciplinary platforms, the comprehension of the importance of all neoplasms, including rare tumours and the continuous support for guaranteeing its growth represent in this time the most important challenges for finalising the ‘AI-revolution’ in oncology.

Gallbladder Neuroendocrine Tumors (NETs): Is there a need for a specific approach?

Neuroendocrine tumors (NETS) of the Gallbladder or the biliary tree are rare. Most of the current guidelines and protocols are derived from the experience of managing Lung small cell neuroendocrine carcinoma (SCNEC) or gastrointestinal (GI) NETS. But the overall outcome of Gallbladder NETS (GB NETS) seems worse than similarly staged lung NETS and adenocarcinoma of the gallbladder. This may be due to its rarity and lack of literature for a focused approach towards its treatment. Hence the need for a specifically designed approach might help improve the results of treatment for these rare tumours. We share our experience of two patients with GB NETS and their 5-year outcome.