Extradural thoracic nerve root hemangioblastoma approached by a combined posterior thoracic spine and video-assisted thoracoscopic surgery: A case report

Background: Hemangioblastomas commonly occur in the posterior fossa and are typically attributed to sporadic or familial Von Hippel–Lindau disease. Spinal hemangioblastomas, found in 7–10% of patients, are usually located within the cord (i.e., intramedullary). Here, a 58-year-old male presented with a purely extradural hemangioblastoma involving a spinal root that was surgically excised. Case Description: A 58-year-old male was admitted with a progressive paraparesis and incomplete sensory deficit. The magnetic resonance imaging documented a solid dumbbell-shaped lesion that extended through the left T3-T4 foramen resulting in nerve root and spinal cord compression. Following arterial embolization and lesion excision by both neurosurgeons and thoracic surgeons, the patient’s deficits improved. The postoperative computed tomography scan documented complete tumor removal, and the neuropathology revealed a hemangioblastoma. Conclusion: Here, we describe a 58-year-old male with a purely extradural thoracic foraminal T3-T4 dumbbell-shaped hemangioblastoma successfully treated by both embolization and surgical excision.

Pigmented epithelioid melanocytoma (PEM) of the spine with compression fracture: case report

Background Pigmented epithelioid melanocytoma (PEM) is a sporadic type of pigmented melanocytic tumor with uncertain malignant potential. PEM arises as a solitary neoplasm that predominantly occurs spontaneously in otherwise healthy patients. Due to its rarity, a gold standard treatment regimen does not exist; however, symptomatic cases should be managed with radiotherapy and surgery. Case presentation A 28-year-old Thai female presented with a sudden onset of back pain and weakness of the lower extremities during the postpartum period. Magnetic resonance imaging demonstrated abnormal soft tissue formation from T4 to T7; it extended to the vertebral bodies, left neural foramina, and posterior columns of T6 and T7. The patient underwent complete tumor debulking, decompressive laminectomy from T4 to T8, and posterior instrumentation from T3 to T10. The histopathology and immunohistochemistry suggested PEM. The patient fully resolved back pain after surgery. Nevertheless, as the patient re-presented with a neurological deficit a few months after the operative intervention, it was decided to perform a surgical resection via an en bloc vertebrectomy. At the one-year follow-up, although the patient reported continued improvement of her back pain, there was no motor power improvement. Conclusions Spinal cord compression due to PEM is uncommon, especially in adults. Early diagnosis and treatment provide a good prognosis and help to regain lost neurological functions. Complete tumor removal and decompression of the spinal cord must be considered as a treatment strategy. Perioperative radiotherapy and chemotherapy have also been highlighted as treatment modalities for spinal tumors. With our reported case, early operative intervention coupled with radiotherapy produced satisfying outcomes.

Carbon ion radiotherapy with complete tumor regression for primary malignant melanoma of female urethra orifice: a case report

Primary malignant melanoma of the female urethra (PMMFU) is extremely rare, accounting for 0.2% of all melanomas, and fewer than 200 cases have been reported worldwide. Because of the small number of clinical cases and unclear biological characteristics, there is no uniform and standard treatment protocol. We herein describe the treatment of PMMFU using carbon ion radiotherapy. The radiotherapy was delivered at 60.8 Gy (RBE) in 16 fractions, once daily, five times per week. The patient achieved complete tumor disappearance within 1 year after carbon ion radiotherapy and remained disease-free thereafter. She developed acute grade 1 radiation dermatitis and urethritis, which resolved quickly; no other toxic effects were observed. At the time of this writing, her survival duration was 33 months. This case demonstrates that carbon ion radiotherapy may be a good option for primary genitourinary mucosal malignancies.

Congenital orbital teratoma: a case report with preservation of the globe and 18 years of follow-up

Background Congenital orbital teratomas are extremely rare, usually benign neoplasms, comprised of cells originating from all three germ cell layers. Clinically the tumor appears solid, most of the times is intraconal and presents as a rapidly growing mass leading to a massive unilateral axial proptosis, chemosis, exposure keratopathy, markedly distended eyelids and often, loss of vision. To prevent these complications, tumor excision usually involves enucleation or even orbital exenteration. Case presentation We report a case of a 1-day old infant who presented with dramatic proptosis at birth due to a true congenital orbital teratoma. We describe the clinical findings, the preoperative neuroimaging, the surgical management which included complete tumor resection with preservation of the globe to allow for optimal orbital growth, the histopathological evaluation, and the clinical course during 18 years of follow up. Conclusion Every effort to salvage the globe should be made to achieve the best possible orbito-facial development. Furthermore, the value of prompt surgical management with a less invasive transconjunctival globe sparing procedure can be appreciated in our case.

ATR inhibition enables complete tumour regression in ALK-driven NB mouse models

High-risk neuroblastoma (NB) often involves MYCN amplification as well as mutations in ALK. Currently, high-risk NB presents significant clinical challenges, and additional therapeutic options are needed. Oncogenes like MYCN and ALK result in increased replication stress in cancer cells, offering therapeutically exploitable options. We have pursued phosphoproteomic analyses highlighting ATR activity in ALK-driven NB cells, identifying the BAY1895344 ATR inhibitor as a potent inhibitor of NB cell growth and proliferation. Using RNA-Seq, proteomics and phosphoproteomics we characterize NB cell and tumour responses to ATR inhibition, identifying key components of the DNA damage response as ATR targets in NB cells. ATR inhibition also produces robust responses in mouse models. Remarkably, a 2-week combined ATR/ALK inhibition protocol leads to complete tumor regression in two independent genetically modified mouse NB models. These results suggest that NB patients, particularly in high-risk groups with oncogene-induced replication stress, may benefit from ATR inhibition as therapeutic intervention.

Effect of Low-molecular Pectin and Metformin on Paclitaxel Toxicity in Rats with Walker Carcinosarcoma

Recently, researchers have been particularly interested in the use of herbal agents in combination therapy of tumors with a spectrum of regulatory effects, low toxicity, and high bioavailability, as well as drugs that indirectly affect the growth and metastasis of tumor cells. The article presents the results of experiments on the study of the combined effect of metformin, low molecular weight pectin and cytostatic agent — paclitaxel on the transplantable tumor — Walker’s carcinosarcoma 256, in order to reduce the toxic effect of the chemotherapy drug. With the combined administration of pectin and paclitaxel (10 mg/kg), metformin and paclitaxel (10 mg/kg), inhibition of tumor growth was observed. On the 25th day, the death of 50% of the animals was observed in the first group, and in the remaining 50%, complete tumor regression was observed. With a single administration of paclitaxel at a single dose of 10 mg / kg of live weight, 62.5% of the animals died in the first 4 days. The combination of drugs (pectin + metformin + paclitaxel) at various single doses of paclitaxel (10, 20, and 25 mg/kg) had an antagonistic effect, showing worse results than the use of each drug with a cytostatic alone (death of animals in the period from 1 to 6 days). Thus, the experimental results demonstrated a clear toxicity reduction effect at high doses of paclitaxel in combination with pectin or metformin.

Photodynamic therapy of the experimental tumors of different morphological types with liposomal boronated chlorin е6

The article summarizes the results of studies of the effectiveness of photodynamic therapy using a new domestic photosensitizer liposomal borated chlorin e6 (LBC) after its parenteral administration (intraperitoneal and intravenous). Antitumor efficacy was evaluated in rats with M-1 sarcoma and PC-1 alveolar liver cancer and mice with B16 melanoma and Ehrlich’s carcinoma, which were transplanted subcutaneously into the thigh area of the animals. The aim of the study was to determine the optimal regimes of photodynamic therapy that would allow achieving the maximum antitumor effect up to 21 days after the photodynamic therapy. The therapy was carried out under the control of the accumulation of the photosensitizer in the tumor and surrounding tissues of the thigh by selecting the doses of the drug and the parameters of laser radiation (energy density and power density). The effectiveness of therapy was assessed by the inhibition of tumor growth, by the percentage of animals with complete tumor regression, by the absolute growth rate in animals with continued tumor growth compared to controls. The results of our studies have shown that the domestic photosensitizer liposomal borated chlorin e6 has high antitumor activity in vivo. In an experimental study of the photosensitizer under certain PDT modes, the maximum antitumor effect (complete tumor regression in 100% of animals) was obtained up to 21 days after PDT in all tumor models used.

Transpupillary laser thermotherapy of retinoblastoma

Introduction. Despite the fact that transpupillary thermotherapy (TTT) is one of the main local methods of small retinoblastoma (RB) destruction, only a few studies have been published on the use of this method, and they are devoted only to certain aspects of the application of TTT.Purpose of the study – to evaluate the effectiveness of TTT in the treatment of children with RB.Material and methods. In the period from 2011 to 2020, 177 children (224 eyes, 1156 tumors) with RB were treated by TTT. Of these, 99 (56 %) patients were boys, 78 (44 %) – girls. The mean age at the time of treatment was 16.8 months (from 0 to 86 months). Bilateral RB was observed in 128 (72.3 %) patients, monolateral – in 49 (27.7 %). In 51 (28.8 %) cases, TTT was performed on an only eye. TTT was performed on eyes that had RB of groups A (n = 43; 19 %), B (n = 81; 36 %), C (n = 31; 14 %), D (n = 63; 28 %), E (n = 6; 3 %). In total, 1156 tumors were treated by TTT. 488 (42 %) tumors were localized post-equatorially (of which 27 were located juxtapapillary, 23 – in the macular zone, 22 – paramacular). 668 (58 %) foci had pre-equatorial localization (on the middle and far periphery of the fundus). The number of foci in one eye varied from 1 to 48 (mean – 5). The mean tumor thickness was 1.1 mm (from 0.2 to 4.5), the mean base diameter was 2.2 mm (from 0.3 to 13.4). TTT was performed using a diode laser with the following parameters: wavelength – 810 nm, spot diameter – 1200 microns, power from 200 to 800 mW (mean – 350 mW), exposure-from 3 to 15 s in the application mode, and continuous in the scanning mode.Results. Complete tumor regression after TTT was achieved in 92 % of cases (1064 tumors). Incomplete regression of the tumor with stabilization was achieved in 0.7 % (8 tumors). The average number of TTT sessions to achieve full regression was 1.7 (from 1 to 10). Complete tumor regression after 1 TTT session was achieved in 54 % of cases (622 tumors), after 2 sessions – in 11 % (132 tumors), after 3 sessions – in 7 % (85 tumors), after 4 or more sessions– in 19 % (225 tumors). In 7 % of cases (82 tumors), due to the progression of the tumor, other treatment methods (brachytherapy, cryotherapy, stereotactic radiosurgery) were applied. 209 (93 %) eyes were preserved. 15 (7 %) eyes were enucleated due to continued tumor growth, total retinal detachment, vitreous hemorrhage, or subatrophy of the eyeball. The mean follow-up after TTT was 35.5 months (from 3 to 112 months).Conclusion. TTT is a highly effective method of RB treatment and can be used for destruction of small primary foci of both post-equatorial and pre-equatorial localization, residual tumors after inefficiency of other local methods. TTT is also effective in the treatment of large cavitary tumors located in functionally significant areas of the retina.

Adolescent Plaque Brachytherapy for Large Choroidal Metastasis from Lung Carcinoid Tumor

The purpose of this publication is to report a child with choroidal metastasis from a primary lung carcinoid tumor treated with 125-iodine plaque brachytherapy while achieving complete tumor regression. A 15-year-old girl with a history of stage IV lung carcinoid tumor developed painless progressive visual loss in her right eye. Ophthalmological exam documented a unilateral solitary large choroidal mass with complete exudative retinal detachment. Planned palliative treatment with plaque brachytherapy was performed utilizing a total dose of 45 Gy to the tumor apex. No tumor regression was noted after 6 months of follow-up. Ten months after primary therapy, re-treatment with a total dose of 90 Gy to the tumor apex was performed due to tumor progression. Complete regression was achieved after secondary treatment without evidence of recurrence after 18 months of follow-up. In conclusion, high-dose plaque brachytherapy may induce complete tumor regression in select adolescent patients with choroidal metastasis from lung carcinoid tumor.

Roles of Surgery in the Treatment of Patients With High-Risk Neuroblastoma in the Children Oncology Group Study: A Systematic Review and Meta-Analysis

Purpose: Neuroblastoma is the most common extracranial solid tumor in children, and most patients are at high risk when they are initially diagnosed. The roles of surgery and induction chemotherapy in patients with high-risk neuroblastoma have been a subject of much controversy and debate. The objective of the current study was to assess the roles of surgery in high-risk neuroblastoma.Method: The review protocol was prospectively registered (PROSPEROID: CRD42021253961). The PubMed, Embase, Cochrane, and CNKI databases were searched from inception to January 2020 with no restrictions on language or publication date. Clinical studies comparing the outcomes of different surgical ranges for the treatment of high-risk neuroblastoma were analyzed. The Mantel–Haenszel method and a random effects model was utilized to calculate the hazard ratio (95% CI).Results: Fourteen studies that assessed 1,915 subjects met the full inclusion criteria. Compared with the gross tumor resection (GTR) group, complete tumor resection (CTR) did not significantly improve the 5-year EFS [p = 1.0; HR = 0.95 (95% CI, 0.87–1.05); I2 = 0%], and the 5-year OS [p = 0.76; HR = 1.08 (95% CI, 0.80–1.46); I2 = 0%] of patients. GTR or CTR resection had significantly better 5-year OS [p = 0.45; HR = 0.56 (95% CI, 0.43–0.72); I2 = 0%] and 5-year EFS [p = 0.15; HR = 0.80 (95% CI, 0.71–0.90); I2 = 31%] than subtotal tumor resection (STR) or biopsy only; however, both CTR or GTR showed a trend for more intra and post-operative complications compared with the STR or biopsy only [p = 0.37; OR = 1.54 (95% CI, 1.08–2.20); I2 = 0%]. The EFS of the patients who underwent GTR or CTR at the time of diagnosis and after induction chemotherapy were similar [p = 0.24; HR = 1.53 (95% CI, 0.84–2.77); I2 = 29%].Conclusion: For patients with high-risk neuroblastoma, complete tumor resection and gross tumor resection of the primary tumor were related to improved survival, with very limited effects on reducing intraoperative and postoperative complications. It is necessary to design strong chemotherapy regimens to improve the survival rate of advanced patients.Systematic Review Registration:https://www.crd.york.ac.uk/PROSPERO/, PROSPEROID [CRD42021253961].