scholarly journals Leiomyosarcomatous transformation of unknown pediatric primary brainstem lesion following radiotherapy

2002 ◽  
Vol 12 (6) ◽  
pp. 1-2 ◽  
Author(s):  
David John Yeh ◽  
Richard B. Hessler ◽  
Mark R. Lee

Primary leiomyosarcoma of the central nervous system is rare and has been described both de novo and following temporally remote radiotherapy for a different unrelated malignancy. The authors report the case of a 42-year-old man in whom 60Co radiation treatment had been performed for an unknown primary mass in the brainstem 25 years previously. He presented with progressive neurological deterioration after undergoing many years of conservative therapy. A stereotactic biopsy sampling procedure was performed, and examination of the left cerebral pedunculopontine lesion revealed a spindle cell neoplasm. Histopathological and immunohistochemical examination of the tumor obtained from definitive resection suggested leiomyosarcomatous transformation of ganglioglioma.

Neurosurgery ◽  
1986 ◽  
Vol 19 (3) ◽  
pp. 426-433 ◽  
Author(s):  
David S. Moss ◽  
Stephen J. Haines ◽  
Arnold S. Leonard ◽  
Louis P. Dehner

Abstract Congenital neoplasms of the central nervous system are extremely rare, although they have been well documented since the earliest reports from the mid-19th century. Medulloblastoma, other primitive neuroectodermal tumors, and various types of gliomas have comprised the majority of cases. This report describes a highly unusual infratentorial and supratentorial tumor presenting as a scalp mass in a neonate who experienced in utero distress. The supratentorial mass extended through a defect in the skull to the parietooccipital lobe, and the infratentorial aspect involved the 9th and 10th cranial nerves in the region of the cerebellopontine angle. A complex spindle cell neoplasm incorporating peripheral nerve sheath and vascular characteristics was further characterized by electron microscopy and immunohistochemistry. Based upon these studies, the tumor was interpreted as a congenital schwannoma with divergent vascular differentiation. The child has been tumor-free for approximately 2 years after the initial operative procedure.


2014 ◽  
Vol 138 (2) ◽  
pp. 274-277 ◽  
Author(s):  
Jessica A. Clevenger ◽  
Romil Saxena ◽  
Muhammad T. Idrees

Spindle cell neoplasms are rarely reported in liver allografts; most are benign and associated with Epstein–Barr virus infection. We present a case of a malignant spindle cell neoplasm arising in a liver allograft. The patient underwent orthotopic liver transplant for cirrhosis secondary to nonalcoholic steatohepatitis. After 2 years, he presented with vague abdominal complaints. Imaging studies revealed a 10-cm right hepatic lobe mass. The patient underwent right-sided hepatectomy. The tumor displayed areas of broad, relatively hypocellular fascicles, whorls, and perivascular clustering; spindle cells with mild to moderate nuclear pleomorphism; and relatively abundant eosinophilic cytoplasm. Mitotic activity ranged from 2 to 4 mitotic figures per 20 high-power fields. Immunostaining displayed positivity for epithelial membrane antigen, vimentin, CD99, BCL2, cytokeratin, and human herpesvirus 8. Interphase fluorescence in situ hybridization findings were negative for a translocation involving the SS18 gene (18q11). We believe the tumor represents the first reported case of a novel unclassified spindle cell malignant neoplasm in a liver allograft.


CytoJournal ◽  
2020 ◽  
Vol 17 ◽  
pp. 9
Author(s):  
Arpita Mathur ◽  
Anjali Sharma ◽  
Mudit Sharma ◽  
Abhishek Maurya ◽  
Anamika Yadav ◽  
...  

Scrape cellblock (SCB) is a novel technique to suggest possible primary site in fine-needle aspiration cytology (FNAC) smears from the liver, lung, and lymph nodes which are the common sites of metastasis of many primary tumors. Immunocytochemistry (ICC) on SCB averts the need of more invasive diagnostic procedures and gives a conclusive diagnosis. We present a series of four cases with unknown primary site, in which ICC was done on SCB to suggest possible primary site. Three of them were liver space-occupying lesions (SOL) and one from the periportal lymph node. In all four cases, wet-fixed smear for hematoxylin and eosin stain was prepared as routine procedure. FNAC was reported as metastatic adenocarcinoma in two and metastatic spindle cell neoplasm in one liver SOL. Periportal node was reported metastatic adenocarcinoma. Two hematoxylin and eosin-stained slides from each case with higher cellularity were used to scrape off the material to prepare SCB. ICC was put which gave conclusive diagnosis in all the cases. On ICC, two cases of metastatic carcinoma in the liver were diagnosed as metastatic neuroendocrine neoplasm from Gastrointestinal Tract and metastatic adenocarcinoma from the stomach. Spindle cell neoplasm of the liver was diagnosed as gastrointestinal stromal tumor from the stomach. Pancreatic head mass in metastatic periportal node was confirmed later by radiologic examination. SCB is a useful technique to make the best use of available material where reaspiration is difficult. ICC on SCB is of maximum utility to suggest possible primary sites in metastatic cases with unknown primary or where biopsy of the lesion is not possible.


2006 ◽  
Vol 21 (1) ◽  
pp. 1-6 ◽  
Author(s):  
Shahid M. Nimjee ◽  
Ciaran J. Powers ◽  
Ketan R. Bulsara

✓In this paper the authors review the literature concerning de novo cavernoma formation after radiation treatment. PubMed and MEDLINE database searches were performed. Data were compiled on all patients in whom de novo cavernomas formed after radiation treatment and whose cases were reported in the literature. The authors found reports in the literature of 76 patients in whom cavernomas formed de novo after radiation treatment. The mean age of the patients was 11.7 years, and the majority of these lesions occurred in males. The patients received a mean radiation dose of 60.45 Gy. The mean latency period before detection of the cavernoma was 8.9 years, and most of these lesions were detected incidentally. In symptomatic patients, the most common presenting symptoms were seizures. Thirty-seven of the patients had evidence of hemorrhage, and 54% of these required surgical intervention. De novo formation of cavernomas after radiation treatment is a relatively rare phenomenon. Patients in whom these cavernomas develop need to be followed closely because there is a propensity for the lesions to hemorrhage. Surgical intervention to treat symptomatic lesions has a favorable outcome.


2020 ◽  
Author(s):  
Claire Forde ◽  
Andrew T King ◽  
Scott A Rutherford ◽  
Charlotte Hammerbeck-Ward ◽  
Simon K Lloyd ◽  
...  

Abstract Background Limited data exists on the disease course of Neurofibromatosis Type 2 (NF2) to guide clinical trial design. Methods A prospective database of patients meeting NF2 diagnostic criteria, reviewed between 1990–2020, was evaluated. Follow-up to first vestibular schwannoma (VS) intervention and death was assessed by univariate analysis and stratified by age at onset, era referred and inheritance type. Interventions for NF2-related tumours were assessed. Cox regression was performed to determine the relationship between individual factors from time of diagnosis to NF2-related death. Results Three-hundred-and-fifty-three patients were evaluated. During 4643.1 follow-up years from diagnosis to censoring 60 patients (17.0%) died. The annual mean number of patients undergoing VS surgery or radiotherapy declined, from 4.66 and 1.65 respectively per 100 NF2 patients in 1990-1999 to 2.11 and 1.01 in 2010-2020, as the number receiving bevacizumab increased (2.51 per 100 NF2 patients in 2010-2020). Five patients stopped bevacizumab to remove growing meningioma or spinal schwannoma. 153/353 (43.3%) had at least one neurosurgical intervention/radiation treatment within 5 years of diagnosis. Patients asymptomatic at diagnosis had longer time to intervention and better survival compared to those presenting with symptoms. Those symptomatically presenting <16 and >40 years had poorer overall survival than those presenting at 26-39 years (P=0.03 and P=0.02 respectively) but those presenting between 16-39 had shorter time to VS intervention. Individuals with de novo constitutional variants had worse survival than those with de novo mosaic or inherited disease (P=0.004). Conclusion Understanding disease course improves prognostication, allowing for better informed decisions about care.


2010 ◽  
Vol 120 (S3) ◽  
pp. S119-S119
Author(s):  
Joshua M Levy ◽  
Christian P Hasney ◽  
Paul L Friedlander ◽  
Michael S Ellis ◽  
Mary A Fazekas-May

Author(s):  
Nithya J. ◽  
Banumathy M. ◽  
Radha A.

Solitary nerve sheath tumor such as Benign schwannomas arising in the pelvic retro peritoneum is infrequently reported. Retroperitoneal location accounts for 0.3-3.2% of primary schwannomas. We report a case of benign retroperitoneal pelvic schwannoma in pregnancy that was incidentally diagnosed when it presented with Preterm premature rupture of membranes and mechanical obstruction for labour. She underwent caesarean section and delivered a healthy baby. She was evaluated in the postoperative period by computerized tomography (CT) imaging studies and CT guided fine needle aspiration cytology (FNAC) was not diagnostic. Complete surgical excision of the tumor was achieved in the postpartum period. The adjacent vascular and urinary channels sustained no injuries and she had no neurologic deficit. Histology revealed spindle cell neoplasm composed of interlacing fascicles and sheets of spindle cell with focal areas of nuclear palisading and thick walled blood vessels. Immunohistochemistry was positive for S 100 suggesting schwannoma. Retroperitoneal location of schwannomas is rare and surgery is curative. Prognosis is good, since recurrence is rare. 


2015 ◽  
Vol 35 (7) ◽  
pp. 664-670 ◽  
Author(s):  
Ezequiel D. Santos ◽  
José R. Silva Filho ◽  
Tanise P. Machado ◽  
Stefano L. Dau ◽  
Rubens Rodriguez ◽  
...  

Abstract A 4-year-old female captive-bred snake of the genus Bothrops showed swelling on the left side of the oral cavity, suggesting the development of neoplasia. The mass was removed surgically and sent for pathological examination. Two months later a new increase in volume in the same site was observed, suggesting recurrence. The lesion was completely removed and sent for pathological analysis. Histologically, the two-samples consisted of a mass with highly-cell density composed of spindle-shaped anaplastic cells arranged in interwoven bundles, distributed throughout the tissue extension and, occasionally, polygonal cells arranged in irregular fascicles. The Masson trichrome staining showed modest amount of collagen supporting the neoplastic cells. PAS-positive content was not observed in the cytoplasm of neoplastic cells. Histological and histochemical findings indicated that it was a spindle cell neoplasm, but the classification was not possible. Immunohistochemistry was requested and performed using the streptavidin-biotin-peroxidase method. The markers used were anti-vimentin, anti-PCNA, anti-EMA, anti-melan A and anti-melanosome, anti-desmin, anti-actin, anti-CD68 and anti- S100protein. The neoplastic cells were immunoreactive for vimentin and PCNA and negative for the other antibodies. The morphology characterization, histochemical and immunohistochemical analysis of neoplastic cells allowed the definitive diagnosis of oral fibrosarcoma.


2021 ◽  
Vol 8 (2) ◽  
pp. 01-07
Author(s):  
Brenda Bertado Cortes ◽  
Brian Madariaga Cortes ◽  
Massiel Zenteno Zenteno ◽  
Bayron Alexander Sandoval Bonilla

Chronyc lymphosytic inflammation with pontine perivascular enhacement responsive to steroids (CLIPPERS), described for the first time in 2010 by Pittock and collaborators, is a rare inflammatory disease of the central nervous system (SNC) characterized by the presence of punctuate or curvilinear lesions described like “salt and pepper” appearance on the magnetic resonance imaging (MRI), that enhance with Gadoliniumn administration; this lesions show an exquisite response to corticosteroid therapy. The etiology of this disease remains unknown. However, the existing articles suggest an autoimmune component, which may or may not be related to other autoinmmune, infectious or malignant pathologies. Due to the above, it is generally considered that in order to reach the diagnosis of CLIPPERS, the possibility of other more common or aggressive pathologies must first be ruled out through extensive investigation. However, if the clinical and imaging presentation are typical and there is a very high suspicion of CLIPPERS, early initiation of glucosteroid therapy may be recommended. There is no unanimous therapy plan due to the few cases reported worldwide thus far. In cases of severe relapses or atypical behavior, both clinical and radiological, it is recommended to consider a stereotactic biopsy of the lesion to integrate the definitive diagnosis. Because it is a rare disease, it is relatively unknown to first- contact medical personnel; in our particular case, this leads to referral to the neurosurgery department who, thanks to their experience, have considered a demilinizing disease as a diagnostic possibility and requested an assessment by the neurology department. Here, we report the evolution and management of three diagnosed cases of CLIPPERS at a single Third-level Medical Center in México; based on clinical, radiological and neuropathological findings; as well as highlighting the importance of lesion biopsy in selected cases.


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