scholarly journals Disseminated Glioneuronal Tumor: A rare presentation in children

2021 ◽  
Vol 8 (10) ◽  
pp. 1747
Author(s):  
Neha Kumari ◽  
Namrata S. Patil ◽  
Ananya Mukherjee ◽  
Sushma U. Save

Gliomas, though most common pediatric central nervous system tumor, can manifest as disseminated glio-neuronal tumor, a rare variant in children. Clinical presentation depends on its location, type and age of child. We are presenting 8 years old male child with fever, projectile vomiting and severe headache which woke him up from deep sleep for 1 month. He had positive meningeal signs and raised intracranial tension with cerebrospinal fluid picture suggestive of partially treated meningitis. There was no improvement even on adequate duration of intravenous antibiotics and had appearance of new onset false localizing signs, MRI brain showed features of cryptococcal meningitis for which India ink staining was negative. As clinical picture was unlike of meningitis, repeat 3 tesla MRI brain was done. Expert neuro-radiologist’s opinion was in favor of disseminated glio-neuronal tumor which was confirmed on histopathological examination. Child underwent laminectomy in TATA memorial hospital and advised palliative care. Child succumbed at home within 6 months of illness.

Author(s):  
Sameer Peer ◽  
Vivek Murumkar ◽  
Karthik Kulanthaivelu ◽  
Chandrajit Prasad ◽  
Shilpa Rao ◽  
...  

Abstract Background Diffuse leptomeningeal glioneuronal tumor (DLGNT) has been recently described in the literature. The complete neuroimaging spectrum and histopathological characteristics of this entity are yet to be elucidated. In an endemic region, diffuse leptomeningeal enhancement on neuroimaging with associated communicating hydrocephalus is usually suggestive of infective meningitis and the patients are started on empirical anti-microbial therapy. However, it is important to consider other differential diagnosis of leptomeningeal enhancement in such cases, particularly if the clinical condition does not improve on anti-microbial therapy. An early diagnosis of a neoplastic etiology may be of particular importance as the treatment regimens vary considerably depending on the underlying disease condition. Case presentation In this case report, we describe a case of DLGNT with high-grade histopathological features which was initially managed as tubercular meningitis based on the initial neuroimaging findings. Due to worsening of the clinical course and subsequent imaging findings at follow-up, a diagnosis of DLGNT was considered and subsequently proven to be DLGNT with features of anaplasia on histopathological examination of leptomeningeal biopsy specimen. Conclusion This case highlights the importance of recognizing certain subtle finding on MRI which may help in an early diagnosis of DLGNT which is crucial for appropriate treatment.


Diagnostics ◽  
2021 ◽  
Vol 11 (5) ◽  
pp. 903
Author(s):  
Antonella Cacchione ◽  
Angela Mastronuzzi ◽  
Andrea Carai ◽  
Giovanna Stefania Colafati ◽  
Francesca Diomedi-Camassei ◽  
...  

Rosette-forming glioneuronal tumors (RGNTs) are rare, grade I, central nervous system (CNS) tumors typically localized to the fourth ventricle. We describe a 9-year-old girl with dizziness and occipital headache. A magnetic resonance imaging (MRI) revealed a large hypodense posterior fossa mass lesion in relation to the vermis, with cystic component. Surgical resection of the tumor was performed. A RGNT diagnosis was made at the histopathological examination. During follow-up, the patient experienced a first relapse, which was again surgically removed. Eight months after, MRI documented a second recurrence at the local level. She was a candidate for the proton beam therapy (PBT) program. Three years after the end of PBT, the patient had no evidence of disease recurrence. This report underlines that, although RGNTs are commonly associated with an indolent course, they may have the potential for aggressive behavior, suggesting the need for treatment in addition to surgery. Controversy exists in the literature regarding effective management of RGNTs. Chemotherapy and radiation are used as adjuvant therapy, but their efficacy management has not been adequately described in the literature. This is the first case report published in which PBT was proposed for adjuvant therapy in place of chemotherapy in RGNT relapse.


2013 ◽  
Vol 88 (6 suppl 1) ◽  
pp. 89-92 ◽  
Author(s):  
Ilner de Souza e Souza ◽  
Mayra Carriijo Rochael ◽  
Rogério Estevam Farias ◽  
Roberto Bezerra Vieira ◽  
Janaina Silva Tirapelle Vieira ◽  
...  

Nodular fasciitis is a benign tumor, resulting from reactive proliferation composed of fibroblastic/myofibroblastic cells. Due to its rapid growth and high cellularity it may be mistaken for sarcoma. Despite the possibility of spontaneous regression, excision is the treatment of choice. A 24-year-old female patient presented with a nodule on the zygomatic region with 3 months of evolution. Excisional biopsy was performed. Histopathological examination associated with immunohistochemical markers HHF35, AML and Ki-67 allowed diagnostic confirmation. The main relevance of the case presented is its rare location, suggesting its inclusion among the differential diagnoses of tumor lesions on the face.


2012 ◽  
Vol 52 (186) ◽  
Author(s):  
S Acharya ◽  
DK Uprety

Primary vaginal carcinoma in uterovaginal prolapse is a rare entity. We report a case of an 84-years-old lady, who presented with long standing vaginal ulcer in association with third degree uterovaginal prolapse. Incisional biopsy was taken from the ulcer. Histopathological examination showed a large cell keratinizing squamous cell carcinoma. Keywords: Squamous cell carcinoma, uterovaginal prolapse, vaginal carcinoma.


Author(s):  
Smita Singh ◽  
Jyoti Garg ◽  
Kusha Sharma ◽  
Kiran Agarwal

Intra-abdominal masses in children are usually malignant. Benign tumours at this location are not seen frequently. It is even rarer to find solitary myofibromas intra-abdominally as these tumours are known to have predilection for the head and neck region. We present an unusual case of solitary myofibroma with abdominal localisation in a six-year-old male child who presented with abdominal distension since six months. Computed Tomography (CT) abdomen revealed a complex solid-cystic mass extending from umbilicus to right iliac fossa and measuring 9×6×4 cm. Histopathological examination revealed a tumour displaying biphasic nodular pattern with the presence of lighter staining fascicles of mature myoid cells along with darker staining and more cellular areas of smaller primitive cells. On Immunohistochemistry (IHC), tumour cells were positive for vimentin and Smooth Muscle Actin (SMA) with variable reactivity for desmin while these were negative for CD34 and Anaplastic Lymphoma Kinase 1 (ALK). Based on the histopathological and immunohistochemical findings, final diagnosis of myofibroma was made. Recognition of these lesions is extremely challenging owing to their rare presentation intra-abdominally and also because of their close morphological overlap with other spindle cell tumours commonly found at this site. This case highlights the combined role played by histopathology and IHC in making a clear distinction between different entities. It is imperative for both clinicians and histopathologists to establish the correct diagnosis as excision of the solitary myofibroma is curative in most cases and offers better clinical course than the more commonly found malignant tumours at this site.


2017 ◽  
Vol 20 (1) ◽  
pp. 3-9
Author(s):  
Ram Kumar Shrestha ◽  
Bibek KC ◽  
Gopal Sedain ◽  
Gita Sayami ◽  
Sushil Shilpakar ◽  
...  

Introduction: CNS tumor requires intraoperative decision making regarding the extent of tumor removal. Clinical examination and imaging studies are not sufficient enough to predict the biological behavior of the tumors. Squash cytology is a quick method of evaluation of cytomorphologic features prepared from smear technique and provide the preliminary diagnosis and aid in intraoperative decision making by differentiating neoplastic from non neoplastic and benign from malignant lesions. The aim of this study is compare the diagnostic accuracy of squash cytology to that of histopathological examination. Methods: This study consists of 36 specimens from both brain and spine subjected to both squash cytology and histopathological evaluation. The squash preparation and histopathological finding were later compared and diagnostic accuracy calculated. Results: Gliomas are the most common tumor encountered and the accuracy of Squash cytology obtained was 71%. In meningioma, 100% diagnostic accuracy was obtained however, there was limitation in accurately predict the subgroup of tumor by squash cytology alone. Other neoplastic lesions included in this study were Schwannoma, Oligodendroglioma, Ependymoma, mixed tumors and others. Overall, the accuracy predicted by squash cytology is found to be 77.8 % in this study. Conclusion: Squash cytology is rapid and reliable method of tissue diagnosis that aid in intraoperative decision making regarding the extent of Central Nervous System tumor excision


2019 ◽  
pp. 112067211987007
Author(s):  
Jayati Sarangi ◽  
Aanchal Kakkar ◽  
Diya Roy ◽  
Rishikesh Thakur ◽  
Chirom Amit Singh ◽  
...  

Purpose: To describe a rare presentation of a case of intraocular non-teratoid medulloepithelioma with teratoid metastases in ipsilateral intraparotid lymph nodes. Case description: A 9-year-old male child with previous history of ciliary body non-teratoid medulloepithelioma presented with a swelling in the right pre-auricular region for 1 month. Magnetic resonance imaging and positron emission tomography–computed tomography showed a right intraparotid mass with enlarged ipsilateral cervical lymph nodes. A core biopsy was taken from the lesion, which on microscopy showed a tumor composed of small round cells arranged in cords, tubules lined by multilayered cells, and in cribriform pattern. These cells were embedded in a hypocellular, loose myxoid matrix. Based on the histopathological characteristics and previous history, a diagnosis of medulloepithelioma metastastic to ipsilateral parotid gland was made. The patient underwent right total conservative parotidectomy and bilateral neck dissection. Histopathological examination revealed metastatic medulloepithelioma in five out of eight intraparotid lymph nodes, with extranodal extension into the adjacent parotid parenchyma. Foci of hyaline cartilage were identified within the tumor, leading to a diagnosis of metastatic teratoid medulloepithelioma. Conclusion: Intraparotid lymph node metastases from intraocular medulloepithelioma is a rare possibility and we recommend that the parotid should be evaluated in cases of intraocular medulloepithelioma at initial presentation as well as during the follow-up period. Also, metastasis should be considered in all pediatric patients with solitary mass lesions showing unconventional histology for a primary parotid neoplasm.


2020 ◽  
Vol 13 (7) ◽  
pp. e235064
Author(s):  
Faranak Rafiee ◽  
Sara Haseli ◽  
Seyed Hamed Jafari ◽  
Pooya Iranpour

Extramedullary haematopoiesis (EMH) is defined as haematopoiesis occurring in organs outside the bone marrow. The liver is one of the rare sites of EMH, and to the best of our knowledge, a few cases of adult EMH of the liver have been reported in the last 20 years. Here, we reported the case of a 68-year-old man with a known history of myelofibrosis presented with vague abdominal pain. An abdominal CT scan showed a hypoattenuating periportal mass encasing the portal vein. The final diagnosis of EMH was made through the histopathological examination. This is a rare presentation of EMH, which may be easily mistaken for other pathologies such as metastases. Familiarity with this type of presentation aids in correctly diagnosing it in an appropriate clinical setting.


Rare Tumors ◽  
2016 ◽  
Vol 8 (4) ◽  
pp. 169-172 ◽  
Author(s):  
Yusuke Takanashi ◽  
Shogo Tajima ◽  
Masaru Tsukui ◽  
Kazuya Shinmura ◽  
Takamitsu Hayakawa ◽  
...  

An extremely rare case of non-mucinous lepidic-predominant invasive adenocarcinoma (LPA) showing extensive aerogenous spread with a pneumonic presentation is reported. A 73-year-old woman was referred to our hospital because of an infiltrative shadow on chest xray. Chest computed tomography revealed extensive ground glass opacities in the right lower lobe, which was accompanied by infiltrative shadow with a pneumonic presentation. Invasive mucinous adenocarcinoma was presumed, and a partial resection of the right lower lobe was done. Histopathological examination revealed lepidic growth-predominant invasive adenocarcinoma with Clara type tumor cells, and there were innumerable aerogenous metastases also consisting of Clara cells. Because Alcian Blue and periodic acid-Schiff staining disclosed no mucus, the tumor was diagnosed as a non-mucinous LPA. The patient showed a poor response to 5 courses of pemetrexed, and she died one year after the diagnosis due to cancer progression. Nonmucinous LPA showed a rare presentation characterized by extensive aerogenous spread followed by a poor prognosis.


2019 ◽  
Vol 10 (02) ◽  
pp. 327-330 ◽  
Author(s):  
Vivek Mahesh Agrawal ◽  
Pramod Janardhan Giri

ABSTRACTIsolated sellar tuberculoma is a very rare condition and usually presents with headache and decreased vision. It can present with panhypopituitarism with 3rd nerve palsy. Tuberculoma mimics pituitary adenoma clinically as well as radiologically and requires endoscopic transsphenoidal surgery and histopathological examination for the final diagnosis. We present a rare case of a 40-year-old female presented with headache, decreased vision, and unilateral 3rd nerve palsy with panhypopituitarism.


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