scholarly journals PRIMARY EWING'S SARCOMA OF THE NASAL CAVITY IN PEDIATRIC PRACTICE

Author(s):  
Bekisheva Aigul Bekisheva Aigul ◽  
Makhneva Anna Makhneva Anna ◽  
Bulegenova Minira Bulegenova Minira ◽  
Abyov Galyjan Abyov Galyjan ◽  
Remkulova Mahabbat Remkulova Mahabbat

Introduction: Ewing's sarcoma (ES) is an aggressive tumor occuring more frequently in childhood and adolescence, mainly observed during the first three decades of life. Microscopically consists of small round cells, with a high nuclear cytoplasmic index, originating from primitive neuroectodermal cells. Most often such tumor occurs in early childhood or adolescence. [1] Ewing's sarcoma refers to tumors of the bone tissue : the limbs, ribs, and pelvic bone, but also it may occur in soft tissues, which means any localization in the human body. Among extra-skeletal localities, ES rarely occurs in the head and neck (2-3% of all ES) and extremely rare in the nasal cavity or paranasal sinuses [1,2,3]. Diagnosis the primary of ES nasal localization is complex and mainly depends on histopathological research, as visual diagnostic methods (x-ray, CT, MRI) do not identify the type of tumor. MRI examination of tumors of such localization may suggest a malignant nature, so further differential diagnosis should exclude the following: malignant lymphoma, rhabdomyosarcoma, moderately differentiated carcinomas, and ES. These tumors have common radiological features, so they require additional research [4,5]. From abovementioned, the following clinical case of primary Ewing's sarcoma in the nasal cavity in a 14-year-old girl seemed to be interesting . The patient complained on the difficulty of breathing, swelling in the right half of the nose wing. MRI showed a large neopasm of the facial skull on the right (pterygoid and subterranean fossa, right half of the nose, nasopharynx, latticed bone and paranasal sinuses). Histological examination of the samples revealed a solid neoplastic tissue consisting of monomorphic small circular or oval cells with a high nuclear-cytoplasmic index. Some exapnsion of the tumor cells around the vessels was observed, while part of epithelial lining and glandular structures were preserved in the same places. Foci of necrosis and extensive hemorrhages were visualized. The immunohistochemical study with the CD99 antibody showed a total positive membrane reaction with the closure of the stained membranes ( with the presence of membranes ring staining ). The reaction with anti Fli1 revealed total nuclear staining of tumor cells, except for the stromal cells. Thus: as primary Ewing's sarcoma affects the sinus tract very rarely, diagnosis of the tumor in this location is difficult. MRI and other visual methods in these cases are not informative, because a number of tumors have common radiological features, that make the differential diagnosis very difficult. In this regard, the pathomorphological study including immunohistochemical research is the main method of diagnosing ES.

Author(s):  
Alan Alexander ◽  
Kyle Hunter ◽  
Michael Rubin ◽  
Ambarish P. Bhat

AbstractExtraosseous Ewing’s sarcoma (EES), first described in 1969, is a malignant mesenchymal tumor just like its intraosseous counterpart. Although Ewing’s sarcomas are common bone tumors in young children, EESs are rarer and more commonly found in older children/adults, often carrying a poorer prognosis. We discuss the multimodality imaging features of EES and the differential diagnosis of an aggressive appearing mass in proximity to skeletal structures, with pathologic correlates. This review highlights the need to recognize the variability of radiologic findings in EES such as the presence of hemorrhage, rich vascularity, and cystic or necrotic regions and its imaging similarity to other neoplasms that are closely related pathologically.


2021 ◽  
Vol 59 (1) ◽  
pp. 39-42
Author(s):  
А. Bekisheva ◽  
A. Makhneva ◽  
E. Satbaeva ◽  
G. Abyov ◽  
M. Remkulova

Ewing’s sarcoma is an aggressive tumor rarely found in the head and neck region and extremely rare in the nasal cavity or paranasal sinuses (2-3% of all Ewing’s sarcomas). Purpose: The article presents a clinical diagnostic case of nasal cavity Ewing’s sarcoma finally verified only by IHC test. Results: The presented clinical case describes Ewing’s sarcoma of the nasal cavity in a 14-year-old girl and presents the clinical picture, the results of MRI and CT examination of the sinuses, histopathological and IHC tests. Adequate diagnostical tools made it possible to establish a diagnosis. Timely determined treatment tactics, taking into account the tumor localization and possible postoperative complications, led to tumor remission. At present, the patient is under dynamic observation. Conclusion: The presented clinical case confirms that a primary nasal cavity Ewing’s sarcoma diagnostic largely depends on histopathological examination since visual diagnostic techniques do not provide reliable information on the tumor type.


1998 ◽  
Vol 56 (4) ◽  
pp. 803-807 ◽  
Author(s):  
PAULO HENRIQUE AGUIAR ◽  
WEI LIU CHING ◽  
HELIO LEITÃO ◽  
F. ISSA ◽  
GUILHERME LEPSKI ◽  
...  

Cerebral hemiatrophy or Dyke-Davidoff-Masson syndrome is a condition characterized by seizures, facial asymmetry, contralateral hemiplegia or hemiparesis, and mental retardation. These findings are due to cerebral injury that may occur early in life or in utero. The radiological features are unilateral loss of cerebral volume and associated compensatory bone alterations in the calvarium, like thickening, hyperpneumatization of the paranasal sinuses and mastoid cells and elevation of the petrous ridge. The authors describe three cases. Classical findings of the syndrome are present in variable degrees according to the extent of the brain injury. Pathogenesis is commented.


BMC Cancer ◽  
2018 ◽  
Vol 18 (1) ◽  
Author(s):  
Kohei Arimizu ◽  
Gen Hirano ◽  
Chinatsu Makiyama ◽  
Mioko Matsuo ◽  
Takakazu Sasaguri ◽  
...  

1999 ◽  
Vol 38 (6) ◽  
pp. 613-614 ◽  
Author(s):  
Hirofumi SAKURAI ◽  
Akiko KOBAYASHI ◽  
Tetsurou ICHIKAWA ◽  
Hiroshi HASHIMOTO

1997 ◽  
Vol 15 (2) ◽  
pp. 583-588 ◽  
Author(s):  
D C West ◽  
H E Grier ◽  
M M Swallow ◽  
G D Demetri ◽  
L Granowetter ◽  
...  

PURPOSE To determine the feasibility of detecting Ewing's sarcoma (ES) or peripheral primitive neuroectodermal tumor (PNET) through a reverse-transcriptase polymerase chain reaction (RT-PCR) of the t(11;22)(q24;q12) fusion transcript in blood and bone marrow samples from patients with these neoplasms. PATIENTS AND METHODS Peripheral-blood (PB) and/or bone marrow aspirate (BM) samples were obtained from 28 patients with ES or PNET at initial presentation or at relapse. Patients were divided into two groups: newly diagnosed patients with nonmetastatic disease and those with metastatic/relapsed disease. RNA was extracted from fractionated BM and PB samples, and RT-PCR was performed for the EWS/HumFLI1 fusion mRNA was transcribed across the t(11;22) breakpoint. RESULTS Among the 16 patients with nonmetastatic disease, three of 16 were RT-PCR positive for EWS/HumFLI1 RNA in BM and three of 10 were positive in PB. The total number of nonmetastatic patients who were positive in either PB or BM was four of 16 (25%). Among patients with metastatic/relapsed disease, two of six were positive in BM and five of 10 were positive in PB. The total fraction of patients with metastatic/relapsed disease that was positive in either BM or PB was six of 12 (50%). CONCLUSION In this study, we show that it is possible to amplify the EWS/HumFLI1 RNA by RT-PCR from the BM and PB of a subset of patients with both nonmetastatic and metastatic ES or PNET, which implies that occult tumor cells are present at these sites. The true biologic and clinical meaning of this information is unknown. However, it does suggest a possible application of RT-PCR for the monitoring of residual disease in patients who are undergoing therapy for ES or PNET. This approach may permit early identification of patients who may benefit from alternative therapy or who may be spared possible overtreatment.


2014 ◽  
Vol 7 (2) ◽  
pp. 76-79
Author(s):  
Vishal Prasad ◽  
Chandrakala Srinivas ◽  
Sheetal Krishnappa ◽  
Suresh T Narayan Rao ◽  
Harshitha T Rajanna

ABSTRACT Objective To highlight an interesting and a rare case of two rhinoliths in a single nasal cavity of an elderly male patient, mimicking fungal sinusitis or malignancy. To emphasize that rhinolith has to be considered in the differential diagnosis of unilateral nasal mass even in the elderly. Case report A 55-year-old man presented with a right-sided nasal obstruction, foul-smelling nasal discharge and a mass in the right nasal cavity of 6 months duration. Endoscopic examination revealed a gritty, blackish brown mass filling the entire right nasal cavity. A clinical differential diagnosis of malignancy or fungal sinusitis was made. CT scan of paranasal sinus was suggestive of fungal sinusitis following which patient was posted for biopsy. Intraoperatively, patient was found to have two separate masses in the right nasal cavity which were removed. We found a septal perforation adjoining the area where the rhinolith was impacted, with septum pushed laterally and adherent to middle turbinate. However, there were no features suggestive of malignancy. Histopathological examination also ruled out malignancy and confirmed a vegetable foreign body with calcification around an acellular material. Conclusion Rhinolith, though rare should be considered in the differential diagnosis of unilateral nasal mass in the elderly. How to cite this article Prasad V, Srinivas C, Krishnappa S, Rao STN, Rajanna HT. Two Rhinoliths in a single nasal Cavity in an Elderly Patient Mimicking fungal sinusitis. Clin Rhinol An Int J 2014;7(2):76-79.


1976 ◽  
Vol 44 (5) ◽  
pp. 608-612 ◽  
Author(s):  
Pete M. Fitzer ◽  
William R. Steffey

✓ The authors present a case in which primary Ewing's sarcoma of the right petrous pyramid in a 9-year-old girl showed no uptake on a 99mTc-pertechnetate nuclide angiogram. Intense uptake was present on a 99mTc-polyphosphate bone scan, but a static brain scan was only minimally abnormal. The diagnosis and treatment of Ewing's sarcoma are reviewed.


2010 ◽  
Vol 2010 ◽  
pp. 1-4 ◽  
Author(s):  
Detlef Brehmer ◽  
Randolf Riemann

Rhinoliths are mineralised foreign bodies in the nasal cavity that are a chance finding at anterior rhinoscopy. Undiscovered, they grow appreciably in size and can cause a foul-smelling nasal discharge and breathing problems. Giant nasal stones are now a very rare occurrence, since improved diagnostic techniques, such as endoscopic/microscopic rhinoscopy, now make it possible to identify foreign bodies at an early stage of development. We report the case of a 37-year-old patient who, at the age of 5-6 years, introduced a foreign body, probably a stone, into his right nasal cavity. On presentation, he complained of difficulty in breathing through the right nostril that had persisted for the last 10 years. For the past four years a strong fetid smell from the nose had been apparent to those in his vicinity. Under general anaesthesia, the stone was removed in toto from the right nasal cavity. The possible genesis of the rhinolith is discussed, our case compared with those described in the literature, and possible differential diagnoses are considered.


2005 ◽  
Vol 13 (1) ◽  
pp. 88-92 ◽  
Author(s):  
BPB Tow ◽  
MH Tan

We report a case of limited stage Ewing's sarcoma which was initially treated as chronic osteomyelitis for 3 years. A 24-year-old man presented with a one-week history of pain in the right arm and fever, with histology suggestive of osteomyelitis of the affected humerus. He developed multiple relapses of pain and fever; each episode responded to antibiotic treatment. A second biopsy was performed 3 years later and confirmed a diagnosis of Ewing's sarcoma. Despite a 3-year delay in diagnosis, the disease remained localised. This case report highlights an atypical facet of the natural history of Ewing's sarcoma: a response to antibiotic and anti-inflammatory agents, and the limited stage of the disease despite a misdiagnosis of 3 years. This suggests the possibility that anti-inflammatory agents exert an inhibitory effect on the tumour growth. We also highlight the newer histologic and immunologic staining used in the diagnosis of Ewing's sarcoma.


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