A Unique Case Report of Infant-Type Hemispheric Glioma (Gliosarcoma Subtype) with TPR-NTRK1 Fusion Treated with Larotrectinib

Pathobiology ◽  
2022 ◽  
pp. 1-8
Author(s):  
Bruna Minniti Mançano ◽  
Mariana Bisarro dos Reis ◽  
Daniel Antunes Moreno ◽  
Flávia Escremim de Paula ◽  
Carlos Roberto de Almeida Junior ◽  
...  

Herein, we present a rare case of a nine-month-old boy diagnosed with infant-type hemispheric glioma (gliosarcoma subtype) at the left frontal lobe. Following subtotal resection, the patient started chemotherapy with the BABY POG protocol. We describe the clinical diagnosis, histological characteristics, radiological features, molecular aspects, and management of this tumor. A comprehensive molecular analysis on the tumor tissue showed a <i>TPR-NTRK1</i> gene fusion. The patient was treated with a TRK inhibitor, larotrectinib, and exhibited a stable disease with residual lesion following 8 months of target therapy. The present study is the first report of an infantile gliosarcoma harboring <i>NTRK1</i> rearrangement treated with larotrectinib.

2021 ◽  
pp. 17-19
Author(s):  
B. Santhi ◽  
Manigandan Manigandan ◽  
Nishok Nishok

May-Thurner syndrome (MTS) is a venous outow obstruction disorder characterized by compression of the left common iliac vein by an overriding right common iliac artery. MTS primarily affects young to middle-aged women, although many patients remain entirely asymptomatic.We report a unique case of a 25 -year-old male who presented with MTS-related multiple varicosities in left lower limb.


2020 ◽  
Vol 2020 ◽  
pp. 1-5
Author(s):  
Natheer H. Al-Rawi ◽  
Sahar Othman ◽  
Ab Rani Samsudin

The peripheral ameloblastoma (PA), also known as extraosseous ameloblastoma, is a rare soft tissue tumor of odontogenic origin, accounting for 1–5% of all ameloblastoma. In some cases, saucerization of underlying bone is the only radiological evidence of this lesion, and PA has identical histological characteristics of intraosseous ameloblastoma. However, it is slow growing, less aggressive, and less invasive in nature. The present report describes a rare case of PA in the maxillary labial gingiva of a 37-year-old man with port-wine vascular malformation. PA was clinically diagnosed as a pyogenic granuloma, and following the surgical treatment of the lesion, its histological features were of ameloblastoma. This case illustrates the importance of including peripheral ameloblastoma in the differential diagnosis of painless exophytic gingival swelling.


2015 ◽  
Vol 12 (2) ◽  
pp. 140-142 ◽  
Author(s):  
B P Sah ◽  
S T Chettri ◽  
J N. Prasad ◽  
P P Gupta ◽  
S P Shah ◽  
...  

Foreign body ingestion is a common occurrence in children and in specific high-risk  groups. It is usually diagnosed based on a history of ingestion given by the patient  or an observer. However, children and mentally retarded adults may be unable to  give an accurate history, and a high index of suspicion must be maintained in these  groups. We report a rare case of foreign body stone in an mentally retarded adult  which presented with drooling and impaired feeds, thence enabling for high index  for suspicion.  Health Renaissance 2014;12(2): pp: 140-142


2009 ◽  
Vol 64 (suppl_1) ◽  
pp. ONS62-ONS70 ◽  
Author(s):  
Eun Jig Lee ◽  
Jung Yong Ahn ◽  
Taewoong Noh ◽  
Se Hun Kim ◽  
Tai Seung Kim ◽  
...  

Abstract Objective: The microsurgical pseudocapsule can be found in the transition zone between an adenoma and the surrounding normal pituitary tissue. We investigated the precise histology of the pseudocapsule. Furthermore, we evaluated the remission rate, the changes in pituitary function, and the recurrence rate after intensive resection of the pseudocapsule. Methods: In 616 patients with pituitary adenomas (Hardy Types I–III) over a period of 14 years, we introduced intensive resection of the microsurgical pseudocapsule to achieve complete tumor removal. A combined pituitary function test and radiological study were performed on the patients before surgery, 1 year after surgery, and at subsequent 1.5-year intervals 2 to 13 years postoperatively. Results: Microsurgical pseudocapsules were identified in 343 (55.7%) of 616 patients, and the distinct microsurgical pseudocapsules were observed in 180 (52.5%) of these patients. In the remaining 163 patients, the microsurgical pseudocapsules were incompletely developed. Tumor cluster infiltration was present in the pseudocapsule in 71 (43.6%) of these patients. Aggressive resection of the microsurgical pseudocapsule was more often required in larger tumors than in smaller ones. The presence of a pseudocapsule was slightly more frequent in prolactin-secreting tumors (70.9%) than in growth hormone-secreting (55.0%) and adrenocorticotropic hormone-secreting (40.0%) tumors. In the 243 patients of the total resection group who underwent combined pituitary function tests more than 2 times after surgery, the surgical remission rate was 99.1 % in clinically nonfunctional tumors, 88% in growth hormone-secreting, 70.6% in prolactin-secreting, and 100% in adrenocorticotropic hormone-secreting tumors. The surgical remission rate was 86.2% in the presence of a pseudocapsule and 94.3% in the absence of a pseudocapsule. Preoperative hypopituitarism improved in 140 patients (57.6%), persisted in 47 patients (19.3%), and was aggravated in 33 patients (13.6%). There was no statistical difference in improvement or deterioration of pituitary function according to the existence or absence of the pseudocapsule. The tumor recurrence rate was 0.8% in the total resection group and was 42.1 % in the subtotal resection group. Conclusion: We have shown that tumor tissue is frequently present within the pseudocapsule, suggesting that any tumor remnant in the pseudocapsule could be a source of recurrence and an obstacle to achieving complete remission. These results indicate that intensive resection of the pseudocapsule could result in a higher remission rate without deteriorating pituitary function.


2017 ◽  
Vol 08 (03) ◽  
pp. 458-460
Author(s):  
William Martin ◽  
Ravi Rajmohan ◽  
Muhittin Belirgen

ABSTRACTVertebral hemangiomas (VHs) are the most common benign vertebral neoplasm and typically are asymptomatic, only to be discovered incidentally on imaging from the fourth to fifth decade of life. Seldom do they enlarge to a point of compression, causing pain and focal neurologic deficits. We present the rare case of an 8-year-old female who presented with paraparesis after a fall. Imaging revealed a pathological fracture of the T8 vertebra with retropulsion and spinal cord compression from both fracture and epidural tumor tissue. The patient underwent an anterior and posterior removal of the tumor, decompression, and fusion. Pathological report of specimen biopsy confirmed a benign hemangioma. To the best of our knowledge, this is the same age as the youngest previously reported case of symptomatic VH and it is the longest to be recurrence-free at follow-up. The hemangioma was successfully treated with tumor removal, decompression, and fusion. No adjuvant treatment was required, and she remained asymptomatic without recurrence at her 4-year follow-up.


2014 ◽  
Vol 2014 ◽  
pp. 1-8 ◽  
Author(s):  
Saptarshi Biswas ◽  
Catherine Price ◽  
Sunil Abrol

Bullet embolism within the gastrointestinal system is extremely rare. Such bullet injuries are infrequently covered in the general literature, but the surgeon should be aware of the phenomenon. Smaller caliber bullets are more common in civilian gunshot wound (GSW) events. These bullets are able to tumble through the gastrointestinal tract and cause perforation of the intestinal lumen which is small enough to be easily missed. Bullets retained in the abdominal cavity should not be dismissed as fixed and should be carefully monitored to ensure that they do not embolize within the bowel and cause occult lesions during their migration. We present a unique case wherein a bullet caused a minute perforation in the small bowel, before migrating to the distal colon, which resulted in late presentation of sepsis secondary to peritonitis.


Author(s):  
M Maleki

Introduction: Variety of tumors could involve the skull; however, very few may occur over the midline. Some may affect venous blood flow of superior sagittal sinus. Few challenging cases are presented Material: 1-Rare case of osteoblastoma over the torcula, (headache, visual symptoms, papilledema, VI nerve palsy). 2- Rare case of metastatic liposarcoma involving midsagital sinus, partially occluding it (headache and visual blurring). 3- A huge atypical (grade2) meningioma over the vertex Method: Case #1, the tumor over the venous confluences (torcula) was removed easily, without any complications. Complete resolution of symptoms Case #2, complete en-block resection of tumor, with sacrifice of mid-sagittal portion of sinus, without any neurological sequellae. Case #3, subtotal resection, followed by radiotherapy.Discussion &Conclusion: Anterior 3rd of sagital sinus could be sacrificed (if necessary), without major consequences. However, whenever mid or posterior portion of the sinus is involved, interruption of venous flow could pose very serious complications. Occasionally, chronic compression of sinus may force increasing collateral venous return, in which case one may attempt a complete resection of the lesion, with sacrifice of part of the sinus, as in our second case. In the region of torcula, however, one should be very careful not to damage it


1996 ◽  
Vol 110 (9) ◽  
pp. 896-898
Author(s):  
Juan P. Rodrigo ◽  
Jose A. Fernandez ◽  
Juan C. Alvarez ◽  
Justo Gómez ◽  
Carlos Suárez

AbstractGiant lymph node hyperplasia (Castleman's disease) is usually reported as a solitary mediastinal tumour, although involvement of other anatomical sites and a multicentric form have been reported. We describe a rare case of Castleman's disease due to its localisation (the left infratemporal fossa) and histology (plasma-cell variant). A brief review of the main clinico-histological characteristics of Castleman's disease is also presented.


2009 ◽  
Vol 54 (3) ◽  
Author(s):  
Ki Eom ◽  
Tae Kim

AbstractWe report the case of a 52-year-old man in whom multiple conglomerated ring-enhanced lesions in the left frontal lobe were revealed on magnetic resonance imaging (MRI); further, he presented with headache. Subtotal resection of the mass was performed and the histopathological diagnosis of gemistocytic astrocytoma was made. He received postoperative radiotherapy of remnant mass. Six months post-surgery, new multiple lesions were developed on the left cerebellum and the lesion yielded radiological findings that were quite similar to those of the lesion previously observed in the left frontal lobe. Total resection was performed with the aid of neuronavigation and a live yellow 10-cm-long worm with an active scolex was found. A pathologist identified the worm as a sparganum of Spirometra mansoni. This suggests that the live worm may have moved to the ipsilateral cerebellum due to the stimulus from the surgery and radiation on the frontal lobe. Although this case presented characteristic MRI findings of sparganosis, we did not conduct a serological test; therefore, we misdiagnosed sparganosis as gemistocytic astrocytoma. To the best of our knowledge, this is the first report of the ipsilateral transtentorial migration of cerebral sparganosis.


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