Melanotic Neuroectodermal Tumor of Infancy at Skull: Rare and Rapid-Growing Tumor but Histologically Benign

2021 ◽  
pp. 1-6
Author(s):  
Vich Yindeedej ◽  
Lisa Kittisangvara

<b><i>Introduction:</i></b> Melanotic neuroectodermal tumor of infancy (MNTI) is a rare and rapid-growing tumor. However, a neurosurgeon should not overlook this entity when differential diagnosing rapid-growing skull tumor because its histology nature is just benign, and the prognosis is much better than other malignant tumors. <b><i>Case Presentation:</i></b> We reported the case of a 5-month old male presenting with progressive rapid-growing skull tumor which became 10 cm in diameter in only 5 months compared to the normal head circumference at birth. At first, we thought of malignant skull tumor and performed only biopsy to establish diagnosis. But, when the pathology revealed benign MNTI, we performed preoperative tumor embolization and then radical surgery. Good result was observed. <b><i>Discussion:</i></b> Skull MNTI is the second most common location after the maxilla. Even advanced imaging nowadays cannot distinguish MNTI from other malignant tumors definitely. Urgent biopsy is recommended to establish diagnosis of this benign tumor first. Preoperative angiography with tumor embolization is recommended when feasible, followed by craniotomy with radical resection.

2021 ◽  
Vol 20 (4) ◽  
pp. 168-177
Author(s):  
A. V. Lopatin ◽  
A. Yu. Kugushev ◽  
N. S. Grachev ◽  
S. A. Yasonov ◽  
N. I. Ponomareva ◽  
...  

Melanotic neuroectodermal tumor of infancy is a rare neoplasm that predominantly involves cranial bones and tends to occur during the first year of life. About 500 cases have been described in the literature to date; 6% of them have been reported to be malignant. Treatment for these malignant tumors was not documented and often turned out to be ineffective.Here we report a case of a child aged 2 years and 4 months who presented with a rapidly growing mass in the maxillary region spreading through the orbit into the anterior cranial fossa. The patient’s parents gave consent to the use of their child’s data, including photographs, for research purposes and in publications. He was treated at the Russian Children’s Clinical Hospital from July 2018 to November 2019. The child underwent chemoradiation and staged surgical removal of the tumor. Treatment with ICE and radiation therapy led to a significant reduction of the tumor volume and enabled us to perform cytoreductive surgery with the removal of the mass in the maxilla. Further treatment according to the CWS 2009 guidance for high-risk patients with NRSTS (NonRhabdomyosarcoma Soft Tissue Sarcoma) and radiation therapy resulted in further regression of intraorbital and intracranial components of the tumor and we performed a radical resection of the residual tumor conglomerate. Investigations during the course of treatment revealed no signs of metastatic involvement. The behavior of malignant melanotic neuroectodermal tumors of infancy is unpredictable, that is why in case of the massive involvement of the facial bones when surgery is associated with a high risk of functional impairment or cosmetic deformity, one should consider preoperative chemotherapy to reduce tumor size and intraoperative blood loss. Moreover, chemotherapy in combination with resection makes it possible to minimize the risk of local relapse or metastasis. 


2019 ◽  
Vol 5 (1) ◽  
Author(s):  
Masaki Nio ◽  
Motoshi Wada ◽  
Hideyuki Sasaki ◽  
Hiromu Tanaka ◽  
Masatoshi Hashimoto ◽  
...  

Abstract Background Although cancer occurrence following surgery for biliary atresia has gradually increased, the development of cholangiocarcinoma in a native liver survivor of biliary atresia is extremely rare. Case presentation A 3-month-old female patient with the correctable type of biliary atresia underwent a cystoduodenostomy. At 16 years of age, she underwent multiple surgeries including lysis of intestinal adhesions, ileostomy, and gastrojejunostomy at another hospital. At 54 years of age, she underwent lithotomy at the porta hepatis, resection of the residual cystic bile duct with gallbladder, and hepaticojejunostomy in Roux-en-Y fashion. As she approached the age of 63, her computed tomography scan showed no liver tumors. In the following year, she developed cholangiocarcinoma at the porta hepatis and underwent chemotherapy. However, the cancer progressed, and she died before she reached the age of 64 years. Conclusions Cholangiocarcinoma is extremely rare in patients with biliary atresia. However, physicians should follow up patients with biliary atresia as closely as possible, as malignant tumors secondary to biliary atresia may increase in number in the near future because of the growing number of long-term survivors with biliary atresia.


2020 ◽  
Author(s):  
Jie Liu ◽  
Shudong Yang ◽  
zhen yu ◽  
Tao Bian

Abstract Background: We describe a rare case of metastatic ameloblastoma in a 68-year-old Chinese woman diagnosed with ameloblastoma of the right mandible in 1974. Case presentation: Despite radical resection, she experienced repeated local recurrences, including asymptomatic bilateral pulmonary nodules, cough, and hemoptysis diagnosed as non-malignant inflammatory cell infiltration. The patient developed an aggravated cough, palpitations, and dyspnea and was admitted to our respiratory department in September 2018. Chest computed tomography revealed multiple bilateral lung nodules, masses, and soft tissue opacities. She was diagnosed with advanced metastatic ameloblastoma in bilateral lungs via bronchoscopic biopsy, with a misdiagnosis time of 32 years. Because her systemic condition was poor, systemic chemotherapy and radiotherapy were not recommended. Dyspnea significantly improved after interventional bronchoscopic therapy and tumor electroexcision; she died of respiratory failure 1 year after diagnosis. Conclusions: Clinicians should carefully explore the medical histories of patients with surgical histories of ameloblastoma and subsequent unexplained pulmonary metastatic tumors to avoid misdiagnosis.


2019 ◽  
Vol 2 (1) ◽  
pp. 18-22
Author(s):  
Alexandru Papuc ◽  
Ioan Mihai Japie ◽  
Traian Ciobanu ◽  
Octavian Nutiu ◽  
Dragos Radulescu ◽  
...  

AbstractThe GCT is an aggressive benign tumor with metastatic potential, most often within the lungs in 2-3% of the patients. It makes about 5% of total bone tumors and about 15% of total benign bone tumors.The maximum incidence occurs between 30 and 40 years old, most frequently affecting the long bones epiphysis (distal femur, proximal tibia, and distal radius).We report the case of a 50-year-old female, with no previous medical history, admitted in the emergency department (ED) for significant pain and functional impairment of the left knee. Clinical examination and imaging tests established the diagnosis of distal femoral tumor.The patient underwent surgical segmental resection of the tumor within oncological limits and subsequent arthroplasty with cemented modular tumoral prosthesis was performed.Even if the GCT is a benign tumor, it has an aggressive behavior and malignancy potential with an important impact on quality of life. Due to localization, this type of tumor can quickly manifest clinically, which allows an early diagnosis and a less invasive surgical technique.


2020 ◽  
Vol 87 (11-12) ◽  
pp. 76-79
Author(s):  
P. G. Yakovlev

Objective. To determine the operative volume of surgical treatment in total radical resection of the bladder in autonomous regime and to estimate the survival indices in patients with low-differentiated cancer of the bladder depending on time of conduction of radical total resection of the bladder. Materials and methods. Retrospectively 522 histories of the diseases were analyzed in the patients with diagnosis of low-differentiated cancer of the bladder, including 190, in whom radical total resection of the bladder was performed in 1998 - 2016 yrs period. The survival indices were determined. Using Kaplan-Meier method, comparative analysis of the survival indices in patients was done, to whom radical total resection or salvage resection of the bladder performed. Results. Total radical resection of the bladder in autonomous regime was done in 24.7% patients with low-differentiated cancer of the bladder or in 67.9% patients, to whom total resection of the bladder was performed. The indices of total 3-, 5- and 10-yr survival of the patients have constituted 96, 93 and 45%, accordingly. The survival indices in the patients after organ-preserving treatment and further salvage total resection of the bladder for locally progression of cancer were better, than in the patients, to whom the treatment consisted of primary radical total resection of the bladder, but the difference have appeared statistically nonsignificant (p=0.286). Average period between conduction of organ-preserving treatment and salvage total resection of the bladder have constituted 34 mo. Conclusion. Radical total resection of the bladder in autonomous regime constitutes the extended method of treatment of muscularly-invaded low-differentiated cancer of the bladder (performed in 24.7% of all the patients) and guarantees satisfactory late follow-up results while primary or postponed performance.


2012 ◽  
Vol 9 (1) ◽  
pp. 54-57 ◽  
Author(s):  
P R Sthapit ◽  
S Marasini ◽  
U Khoju ◽  
G Thapa ◽  
B P Nepal

Background Ocular trauma is an important cause of blindness and ocular morbidity throughout the world. Prevalence studies help to define the target group, plan strategies and thereby lessen trauma related blindness in the country. Objective To identify the risk factors for ocular trauma, those at risk, describe the visual acuity at time of clinical presentation, and assess the nature and severity of ocular trauma in patients presenting to Dhulikhel Hospital’s emergency department and eye OPD with ocular trauma. Methods It is a descriptive and prospective study of all the patients with ocular trauma presenting to Dhulikhel Hospital from December 2009 to December 2010. A complete history and detailed ophthalmologic evaluation was done and noted down. Results There were 112 subjects with ocular trauma. Mean age was 28.43±16.36 years with a range of one year to 74 years. Males predominated with 72.3% frequency (p<0.001). Incidence peaked in third decade of life (p<0.01). Ocular trauma peaked in months of September and October. Presenting visual acuity better than 6/12 was noted in 83.9% of cases whereas 2.7% of cases had visual acuity of <3/60. Household (56.3%, p<0.001) was the commonest place of injury with wooden objects (19.6%) being the commonest object of injury. Most of the injuries were mechanical (85.7%, p>0.001). ConclusionBlunt injuries were more common in young males. Household and workplace were common location of ocular injuries. Public awareness and strict legislation to use personal protective devices can help to reduce the occurrence of ocular injury.http://dx.doi.org/10.3126/kumj.v9i1.6264 Kathmandu Univ Med J 2011;9(1):54-7


2020 ◽  
pp. 014556132092556
Author(s):  
Przemyslaw Krawczyk ◽  
Antoni Bruzgielewicz ◽  
Kazimierz Niemczyk

Nonepithelial tumors of the larynx are relatively rare neoplasms of the head and neck. The chondrosarcoma, which develops commonly from cricoid and thyroid cartilage, stands for 0.2% of the laryngeal malignant neoplasms. The rhabdomyoma is even more uncommon benign tumor developing from the laryngeal striated muscles. The clinical manifestation and the treatment options depend on the histopathological evaluation, tumor localization, and its size. In presented case, the simultaneous occurrence of benign and malignant tumors of mesenchymal origin in the patient’s larynx was provoking hoarseness, globus sensation, and dysphagia. To the best of authors’ knowledge, no other case of the concomitance of rhabdomyoma and chondrosarcoma of the larynx have been reported in the literature.


1989 ◽  
Vol 30 (6) ◽  
pp. 591-595
Author(s):  
L. Ekelund ◽  
L. Athlin

The diagnostic utility of extremely low field magnetic resonance (MR) imaging was evaluated in 25 patients with focal hepatic masses, including 17 with primary (n=7) or secondary (n= 10) malignant neoplasms and 8 with benign lesions (6 hemangiomas). The findings were compared with the results of computed tomography (CT). Out of 16 patients with malignant tumors demonstrated by both modalities, the diagnostic information from MR imaging was equal to or better than that from CT in 6 patients and inferior to CT in 10. Shortcomings of MR were mainly due to low signal-to-noise ratio and poor spatial resolution, resulting in an image quality inferior to that obtained at higher field strengths. Considering these facts, together with the long imaging times required, low field MR cannot be recommended for general use in the evaluation of hepatic masses. On the other hand, our results indicate that this technique may be useful in establishing the diagnosis of hepatic hemangioma.


2018 ◽  
Vol 01 (02) ◽  
pp. 094-099
Author(s):  
Rajat Jain ◽  
Amit Keshri ◽  
Ravi Manogaran ◽  
Raj Kumar ◽  
Pearly Keeranghat

Abstract Background Preauricular transzygomatic surgical approach (PTZA) can be used to approach and resect tumors of infratemporal fossa (ITF) and surrounding skull base lesion. Various modifications in this approach can be used to approach various critical areas such as cavernous sinus, sphenoid sinus. Materials and Methods Clinical charts were reviewed to determine the association among pathological variables, surgical procedures, and outcomes. Results Three out of seven were malignant tumors and required pterional craniotomy and postoperative radiotherapy. 4 out of 7 were benign tumors and required craniofacial osteotomies. Conclusion PTZA is a versatile approach for tumor of ITF with or without intracranial extradural extension and cosmetically better than other approaches. This approach is better suited for lateral ITF tumors that are difficult to access through endoscopic approaches.


2020 ◽  
Vol 21 (14) ◽  
pp. 4877
Author(s):  
Kazushige Yoshida ◽  
Masanori Okamoto ◽  
Kaoru Aoki ◽  
Jun Takahashi ◽  
Naoto Saito

Osteosarcoma is one of the most common primary malignant tumors of bone. The combination of chemotherapy and surgery makes the prognosis better than before, but therapy has not dramatically improved over the last three decades. This is partially because of the lack of a novel specialized drug for osteosarcoma, which is known as a tumor with heterogeneity. On the other hand, immunotherapy has been one of the most widely used strategies for many cancers over the last ten years. The therapies related to T-cell response, such as immune checkpoint inhibitor and chimeric antigen receptor T-cell therapy, are well-known options for some cancers. In this review, we offer the accumulated knowledge of T-cell-related immunotherapy for osteosarcoma, and discuss the future of the therapy.


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