scholarly journals Olfactory Neuroblastoma: The Adjunctive Role of Somatostatin Agonists After Surgery

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A595-A595
Author(s):  
Fernando Pazos Toral ◽  
Maria Martino ◽  
David Lobo Duro ◽  
Isabel Martinez Rodriguez

Abstract Clinical Case: A 54 years old woman who had a right-sided nasal obstruction, rhinorrhea, and sometimes purulent discharge in 2016. In 2002 she had hyperthyroidism by Graves disease and treated for two years with remission after that. The examination of the nose revealed a dark red bleeding mass filling the area from the right nasal cavity to the nasopharynx. The biochemical and serum hormonal values were in a normal range. Urine 5 -HIIAA was 2,5 mg/24h. MRI T1 and T2-weighted images revealed a mass of 4,1 x 4,2 cm, slightly higher signal in T2 and isointense in T1, that extended into the right nasal cavity and ethmoidal sinus. A biopsy was performed, which revealed neuroendocrine characteristics. Octreotide scan with SPECT showed an intense nasal uptake. Right internal maxillary artery embolization was performed. In the next two days, the tumor was resected through a right lateral rhinotomy. Histopathological examination revealed olfactory neuroblastoma, score low grade, ki67<2%. Positive immunohistochemical staining to CD56, synaptophysin, NSE, GFAP, calretinin, S-100 and chromogranin A, and negative staining to cytokeratins (CKs) (CKAPM, CKBPM, CK8/18), CD99 and Bcl-2. The resection was almost total, with no octreotide scan uptake. The post-MRI revealed a minimal residual lesion. Lanreotide treatment was introduced after surgery; the dose was 120 mg every four weeks the first year and every 8 weeks after that. No secondary effects or biochemical alterations were observed. In 2020 the octreotide scan again revealed nasal uptake showing local recurrence. This local recurrence was treated with surgery, followed by lanreotide 120 mg every 4 weeks. No residual disease has been found afterward. Discussion: A limited number of cases have been reported, but none of them were treated with somatostatin agonists. As in our case, the majority of the ON is slow-progressive and non-secreting. The patients mainly complain of local symptoms such as nasal congestion. Surgery is considered to be the first-line treatment for localized disease. In the case of a close margin of the lesion or a residual tumor, the recommendation was radiotherapy or chemotherapy for more advanced disease. We confirmed that the tumor expressed somatostatin receptors. The octreotide scan has been found helpful in the diagnosis and follow-up. We treated the patient with lanreotide with no progression of the disease for three years. Local recurrence could be treated with surgery, following by lanreotide treatment, no residual disease has been found to date. The somatostatin analogs may be a useful adjuvant therapy for stable disease without evident residual disease after surgery.

2020 ◽  
Vol 55 (1) ◽  
pp. 39-42
Author(s):  
B. B. Zhetpisbaev ◽  
E. B. Satbaeva ◽  
N. A. Ryskeldiev ◽  
N. N. Ashirov ◽  
E. S. Gorshkova

The article describes a classical case of olfactory neuroblastoma in a 33-year-old female patient diagnosed with «nasal cavity neoplasm (polyp?)» who was referred to the National Centre for Neurosurgery JSC from the ENT Department of the City Hospital No. 1. The first symptoms of the tumor occurred during her pregnancy. The aggressive tumor has invaded the anterior cranial fossa and spread into the right half of the ethmoid sinuses and the orbit. The tumor diagnostics was complicated by the prevalence of symptoms of a nasal cavity neoplasm. The “olfactory neuroblastoma” diagnosis was verified by clinical observations, MRI, and pathomorphological studies. Histological examination of the tumor showed a typical structure of olfactory neuroblastoma. IHC examination showed an expression of neuronal differentiation markers. The degree of malignancy was determined by Hyams grade


Author(s):  
Lin Chen ◽  
Jingxin Wang ◽  
Zhigang Yang ◽  
Yingkun Guo

AbstractOlfactory neuroblastoma (ONB) is a rare malignant neuroectodermal tumor of the nasal cavity. Olfactory neuroblastoma centered in the posterior right orbit with prominent orbital protrusion is even rare. Grading ONB is extremely important as individualized treatment plans must be formulated according to tumor grade. We report the case of a 67-year-old female who presented with the chief complaints of persistent nasal congestion with intermittent epistaxis and unilateral proptosis over the past five years. Radiological imaging was suggestive of a large heterogeneous mass in the right superior nasal cavity with extensions into the right medial orbit, nasopharynx, the right maxillary sinus, the anterior cranial fossa, right ethmoidal, frontal and bilateral sphenoidal sinuses, as well as into the right frontal lobe. Assessment of the radiologic features revealed the diagnostic possibility of olfactory neuroblastoma. A nasopharyngeal biopsy confirmed an olfactory neuroblastoma. Frontal osteoplastic craniotomy and excision of the intracranial part of the tumor from above and transnasal endoscopic removal of the mass in the nasal cavities, paranasal sinuses and right medial orbit from below was done. Evaluation of histopathological characteristics and immunohistochemical findings revealed a diagnosis of WHO grade IV olfactory neuroblastoma. Because of poor economic condition, the patient did not take adjuvant radiotherapy and chemoradiation and post-operative examination. We report a huge ONB centered in the posterior right orbit with prominent orbital protrusion. Magnetic resonance image and computed tomography are helpful for evaluating the appearance and the extent of ONB, as well as grading this tumor, which may aid therapeutic decisions and improve survival.


2020 ◽  
Vol 13 (10) ◽  
pp. e236802
Author(s):  
Nigel Jeronimo Cortez Santos ◽  
Isabelle Dominique Villegas Tomacruz ◽  
Frances Lina Lantion-Ang ◽  
Paula Veronica San Juan Reyes

Osteomyelitis is a rare initial presentation of HIV. We report a case of a 25-year-old, apparently well man presenting with a traumatic, pathological fracture of the right radius. He had a 2-week history of low-grade fever, swelling and purulent discharge of the radial aspect of his right forearm. Osteomyelitis, secondary bacteraemia and pneumonia were clues that led physicians to test for HIV. Multiple debridement, sequestrectomy and vacuum-assisted closure were done. Tissue cultures revealed Mycobacterium tuberculosis (TB) and methicillin-sensitive Staphylococcus aureus. He was treated successfully with 6 weeks of culture-guided intravenous oxacillin, staphylococcal decontamination and first-line anti-TB regimen (rifampicin, isoniazid, ethambutol, pyrazinamide). Antiretroviral agents were started thereafter. Successful infection control and preservation of limb functionality was achieved with a multidisciplinary team approach. To our knowledge this is the first reported case of an adult patient with HIV presenting with tuberculous and pyogenic osteomyelitis of the radial bone.


2017 ◽  
Vol 26 (4) ◽  
pp. 342-346 ◽  
Author(s):  
Sally Nguyen ◽  
Marjorie Perron ◽  
Sylvie Nadeau ◽  
Alexandre Nakao Odashiro ◽  
Marie-Noëlle Corriveau

Background. Epithelial myoepithelial carcinomas (EMCs) are rare low-grade salivary gland tumors. Here, we report the case of a 75-year-old man presenting with an oncocytic variant of EMC of the nasal cavity, initially diagnosed as an oncocytoma. Methods. Our patient underwent functional sinus surgery in 2012. On pathology, an oncocytic neoplasm was found in the right nasal cavity, characterized by fragments of uniform bland oncocytic cells with bilayered arrangement of nuclei. Immunohistochemical stains demonstrated biphasic cells: luminal epithelial and basal cell–type myoepithelial cells. The tumor was best diagnosed as an oncocytoma. In 2015, the patient presented with a recurrent right inferior turbinate lesion, compatible with oncocytic EMC. Results. The patient underwent oncological surgery and received adjuvant radiotherapy. He had no disease recurrence. Conclusion. Different variants of EMCs exist, such as oncocytic EMC. EMCs should be treated aggressively because they can be locally invasive, recur, and give rise to distant metastases.


2021 ◽  
Vol 06 (03) ◽  
pp. 188-193
Author(s):  
Prasetyo Sarwono Putro ◽  
Meutia Apriani ◽  
Muchtar Hanafi ◽  
Vania Puspitasari

Diagnosis to treatment of Juvenile Nasopharyngeal Angiofibroma (JNA) required a multidisciplinary approach. CT scan works by combining multi-slice imaging from a device that rotates around the object. The potential of missing certain parts in the scanning process can occur. Angiography was the option to cover the CT scan pitfalls. In this case, we discussed CT scan pitfalls that can be overcome by angiography through JNA case report by showing clearer picture of the JNA and its feeding artery. 14 years old child complained of nasal congestion. On physical examination, the lesion expanded the anterior side of nasal cavity. The patient underwent a synonasal CT scan without contrast. It was obtained a heterogeneous solid mass in the nasopharynx extending to the concha and right and left maxillary sinuses. However, until the preparation of angiography, the actual size of the tumor, as well as the entire vasculature, is not yet known. The angiographic features suggested that the right side (seen in the right maxillary artery) was more dominant than the left side. However, both the right and the left finding reassured that the tumor location was more dominant in the anterior nasal cavity. The posterior lesion was also seen but did not predominate in comparison to the anterior. These findings helped clinicians in planning operative action in order to evacuate the tumor.


2020 ◽  
Vol 55 (1) ◽  
pp. 33-35
Author(s):  
B. B. ZHETPISBAEV ◽  
E. B. SATBAEVA ◽  
N. A. RYSKELDIEV ◽  
N. N. ASHIROV ◽  
E. S. GORSHKOVA

The article describes a classic case of olfactory neuroblastoma in a 33-year-old female patient diagnosed with «nasal cavity neoplasm (polyp?)» who was referred to the National Centre for Neurosurgery JSC from the ENT Department of the City Hospital No. 1. The first symptoms of the tumor occurred during her pregnancy. The aggressive tumor has invaded the anterior cranial fossa and spread into the right half of the ethmoid sinuses and the orbit. The tumor diagnostics was complicated by the prevalence of symptoms of a nasal cavity neoplasm. The “olfactory neuroblastoma” diagnosis was verified by clinical observations, MRI, and pathomorphological studies. Histological examination of the tumor showed a typical structure of olfactory neuroblastoma. IHC examination showed an expression of neuronal differentiation markers. The degree of malignancy was determined by Hyams grading.


Rheumatology ◽  
2021 ◽  
Vol 60 (Supplement_1) ◽  
Author(s):  
Jessica Ellis ◽  
Keziah Austin ◽  
Sarah Emerson

Abstract Background/Aims  A 49-year-old female of Nepalese heritage was referred with right-sided headache, scalp tenderness, and a painful swelling overlying the right temple. She denied any visual or claudicant symptoms but felt systemically unwell with a fever. There were no symptoms suggestive of an inflammatory arthritis, underlying connective tissue disease or vasculitis. She was normally fit and well with no past medical history. She did not take any regular medications and denied using over the counter or illicit drugs or recent travel. On review she had a low grade fever. There was a large tender, erythematous swelling overlying the right temple. Bilaterally the temporal arteries were palpable and pulsatile. Peripheral pulses were normal with no bruits. There was no evidence of shingles (HSV) or local infection. Full systemic examination revealed no other abnormalities. Laboratory tests showed: PV 2.56, CRP 101, total white cell count 14.38 (eosinophils 0.4), albumin 33, Hb 115. Urine dip was normal. Renal function, liver function and immunoglobulins were normal. ANCA was negative. Hypoechogenicity surrounding the right frontal branch of the right temporal artery was seen on ultrasound. There were no discrete masses suggestive of cysts, abscess or tumours. Temporal artery biopsy confirmed the presence of vasculitis; histology demonstrated transmural lymphohistiocytic inflammation, disruption of the elastic lamina and intimal proliferation. Prednisolone was started at 40mg daily. Four weeks after initially presenting she was asymptomatic and her inflammatory markers had normalised. Methods  The case is discussed below. Results  Temporal arteritis, or GCA, is primarily a disease of older adults; with age 50 often used as an inclusion criteria, and is more common in Caucasian populations. Limited reports exist of GCA in younger cohorts, but these are rare. An important differential in younger patients, such as ours, is juvenile temporal arteritis. This rare localised vasculitis affects almost exclusively the temporal artery. It is typically a disease of young males, who present with non-tender temporal swelling. Systemic symptoms are unusual and inflammatory markers are normal. Clinical or laboratory evidence of organ involvement, peripheral eosinophilia or fibrinoid necrosis on histology should prompt consideration of an AAV or PAN. Incidence of GCA increases in correlation with Northern latitude, with highest rates reported in Scandinavian and North American populations. GCA is rare in Asian populations. Higher diagnostic rates in countries where physicians have increased awareness of GCA proposed as an explanation for this difference; however differences in incidence are still observed between Asian and Caucasian populations presenting to the same healthcare providers. Conclusion  GCA is an uncommon diagnosis in younger and non-Caucasian patients. Thorough investigation through ultrasound and biopsy helped increase our diagnostic confidence in this unusual case. Rheumatologists must be alert to atypical presentations in order to deliver prompt and potentially sight-saving treatment. Disclosure  J. Ellis: None. K. Austin: None. S. Emerson: None.


2021 ◽  
Vol 9 (5) ◽  
pp. 950
Author(s):  
Chiara Sodini ◽  
Elena Mariotti Zani ◽  
Francesco Pecora ◽  
Cristiano Conte ◽  
Viviana Dora Patianna ◽  
...  

In most cases, infection due to Bartonella henselae causes a mild disease presenting with a regional lymphadenopathy frequently associated with a low-grade fever, headache, poor appetite and exhaustion that spontaneously resolves itself in a few weeks. As the infection is generally transmitted by cats through scratching or biting, the disease is named cat scratch disease (CSD). However, in 5–20% of cases, mainly in immunocompromised patients, systemic involvement can occur and CSD may result in major illness. This report describes a case of systemic CSD diagnosed in an immunocompetent 4-year-old child that can be used as an example of the problems that pediatricians must solve to reach a diagnosis of atypical CSD. Despite the child’s lack of history suggesting any contact with cats and the absence of regional lymphadenopathy, the presence of a high fever, deterioration of their general condition, increased inflammatory biomarkers, hepatosplenic lesions (i.e., multiple abscesses), pericardial effusion with mild mitral valve regurgitation and a mild dilatation of the proximal and medial portion of the right coronary artery, seroconversion for B. henselae (IgG 1:256) supported the diagnosis of atypical CSD. Administration of oral azithromycin was initiated (10 mg/kg/die for 3 days) with a progressive normalization of clinical, laboratory and US hepatosplenic and cardiac findings. This case shows that the diagnosis of atypical CSD is challenging. The nonspecific, composite and variable clinical features of this disease require a careful evaluation in order to achieve a precise diagnosis and to avoid both a delayed diagnosis and therapy with a risk of negative evolution.


2021 ◽  
Vol 4 (Supplement_1) ◽  
pp. 117-118
Author(s):  
D Maillet ◽  
E Desilets ◽  
T Maniere

Abstract Background Endoscopic submucosal dissection (ESD) is an endoscopic procedure developed in Asian countries to treat early gastric cancer (EGC). Western countries have less experience with this challenging technique. Aims The goal of this study is to evaluate the effectiveness of ESD as a preliminary experience. Methods This is an unicentric retrospective study of all consecutive gastric ESD for adenomas or EGC from 07/2017 to 08/2020. The primary endpoints were en bloc and R0 resection rates. Results Nineteen patients (mean age 74.2 (54–88), sex ratio 3F/16M) and 23 lesions were included. Mean diameter was 25 mm (10–90). Treatment was previously performed in 7 cases (30.4%), by ESD (5) or EMR (2). The procedure, performed under general anaesthesia, lasted on average 148 minutes (45–412). En bloc resections were performed in 16 cases (69.6%); 5 cases (21.7%) were converted to P-EMR and there was a failure to resect the lesion because of deep invasion or perforation in 2 cases (8.7%). Pathologic examination demonstrated 2 low-grade dysplasia, 4 high-grade dysplasia and 15 adenocarcinomas: intramucosal (8), sm1 (2), sm2 (2), sm3 (1) or sm deep (2). R0 and curative resection rates were 43.5% and 39.1% respectively. The complication rate related to the procedure was 30.4% including 5 perforations and 2 delayed bleeding: all were managed endoscopically. Five patients (21.7%) underwent subsequent gastrectomy for non-curative resection (4) or failed resection (1); 3 had no residual disease on final pathology, 1 had high grade dysplasia and 1 had intramucosal adenocarcinoma. One patient went to palliative care because he was unfit for surgery. Follow-up endoscopy was completed in all 17 patients who underwent endoscopic resection (mean 10 months (2–24)). Recurrence occurred in 23.5% (4/17); all were successfully treated by another ESD. Conclusions In our preliminary experience, the rate of en bloc and R0 resection were 70% and 44%. Compared to other studies, these low en bloc and curative resection rates may be explained by technically difficult lesions during the learning curve and might improve with experience. Nevertheless, surgery has been avoided in 13/19 patients (68%) with endoscopic intervention. Funding Agencies None


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