scholarly journals Melanotic Neuroectodermal Tumor of Infancy

2018 ◽  
Vol 142 (11) ◽  
pp. 1358-1363 ◽  
Author(s):  
Brian S. Soles ◽  
Allecia Wilson ◽  
David R. Lucas ◽  
Amer Heider
Keyword(s):  
Differential Diagnosis ◽  
Local Recurrence ◽  
Soft Tissues ◽  
Malignant Neoplasm ◽  
Neck Region ◽  
Neuroectodermal Tumor ◽  
Growth Potential ◽  
Incomplete Resection ◽  
The Central Nervous System ◽  
Blue Cell

Context.— Melanotic neuroectodermal tumor of infancy, albeit rare and generally regarded as benign, is an important tumor to recognize because of its rapid growth, potential for local recurrence, and small round blue cell morphology, which can lead to misdiagnosis of a malignant neoplasm. Objective.— To review its clinical presentation and immunomorphologic findings, and discuss common entities in the differential diagnosis. Data Sources.— The study involved PubMed searches, including multiple review articles, case studies, retrospective studies, selected book chapters, and University of Michigan cases. Conclusions.— Melanotic neuroectodermal tumor of infancy most commonly occurs in the bones of the head and neck region during the first year of life, but it can also present in other locations, including the central nervous system, testes, ovaries, and subcutaneous soft tissues. Histologically, it is composed of a biphasic population of cells, consisting of epithelioid melanin-producing cells and primitive neurogenic cells in a fibrocollagenous stroma. These microscopic findings, especially in small biopsies, can lead to a broad differential diagnosis that includes malignant small round blue cell tumors and malignant melanoma. Melanotic neuroectodermal tumor of infancy commonly has an infiltrative growth pattern, and anatomic constraints often lead to incomplete resection and local recurrence, requiring multiple surgical operations. Because melanotic neuroectodermal tumor of infancy can mimic a more aggressive and aggressively treated malignancy, recognition of this rare tumor is very crucial for pathologists.

scholarly journals Kimura disease: a rare cause of a recurrent cheek mass in a Jamaican man

2021 ◽  
Vol 2021 (4) ◽  
Author(s):  
Geoffrey Williams ◽  
Carlos Neblett ◽  
Jade Arscott ◽  
Sheena McLean ◽  
Shereika Warren ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Local Recurrence ◽  
Head And Neck ◽  
African Descent ◽  
Young Men ◽  
Neck Region ◽  
Serum Immunoglobulin ◽  
Western World ◽  
Kimura Disease ◽  
Head And Neck Region

Abstract Kimura disease (KD) is a chronic, inflammatory, benign disorder endemic to Asia that typically manifests as a triad of painless masses in the head and neck region, elevated eosinophils and serum immunoglobulin. It usually affects young men in their second and third decades of life and is rarely seen outside of the orient. This is a report of a case of KD in a young man of African descent who presented with a cheek mass. KD was not included in our differential diagnosis, and this report highlights the need to consider this entity, which can be easily missed due to its rarity in the Western world. There is no cure for the disease, and management includes medical and surgical modalities, but local recurrence or relapse is not uncommon.

scholarly journals Primitive Neuroectodermal Tumor of the Liver: A Case Report

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
Eduardo Cambruzzi ◽  
Enilde Eloena Guerra ◽  
Hamilton Cardoso Hilgert ◽  
Herbert Jorge Schmitz ◽  
Vinícius Lopes Silva ◽  
...  
Keyword(s):  
Primitive Neuroectodermal Tumor ◽  
Soft Tissues ◽  
Young Patients ◽  
Neuroectodermal Tumor ◽  
Solid Mass ◽  
Resected Liver ◽  
The Central Nervous System ◽  
Right Lobe ◽  
Upper Abdominal ◽  
The Right

Primary liver sarcomas represent a rare group of neoplasias, with angiosarcoma being the most common histological type. Primitive neuroectodermal tumor (PNET) represents a high malignant neoplasia that usually affects the central nervous system and soft tissues. An 18-year-old male patient was admitted with clinical complains of pain in the right upper abdominal quadrant. The clinical evaluation revealed a solid mass in the right hepatic lobe. On the gross examination of the resected liver specimen, the right lobe of the liver was replaced by a yellow-red solid mass measuring 21 cm in its largest dimension. On the histopathology, a tumor composed of small round blue cells with little cytoplasm and round nuclei was identified. The lesion revealed positive immunoexpression for vimentin and CD99 and negative immunostaining for desmin, CD45, cytokeratin, and neuroblastoma protein, suggesting, then, the diagnosis of PNET. Although it is an unusual tumor, it should be considered in the differential diagnosis of liver masses, especially in young patients.

scholarly journals The role of immunohistochemistry for diagnosing undifferentiated malignancy in the head and neck region

2019 ◽  
Vol 10 (1) ◽  
pp. 48-53
Author(s):  
Mohammad Sowkat Hossain ◽  
S M Mahbubul Alam ◽  
Sk Md Jaynul Islam ◽  
Wasim Selimul Haque ◽  
Shamoli Yasmin
Keyword(s):  
Head And Neck ◽  
Soft Tissues ◽  
Histopathological Examination ◽  
Malignant Neoplasm ◽  
Cervical Lymphadenopathy ◽  
Neck Region ◽  
Histopathological Diagnosis ◽  
Female Ratio ◽  
Head And Neck Region

Background: Undifferentiated tumours in the head and neck region are not uncommon. They can arise from different sites like in mucosa as well as in salivary glands, soft tissues or lymph nodes. Histopathological examination plays a central role in the diagnosis but difficulties arise with some tumours which are poorlydifferentiated due to their high inter- and intra-observer variability. In those cases, immunohistochemistry has greatly assisted to diagnose the tumours that cannot be accurately identified using routine histopathological procedures. The correct histopathological diagnosis is essential especially in case of malignant tumourwhere subsequent specific therapy is required. The aim of this study was to determine the role of immunohistochemistry for diagnosing undifferentiated malignancy in the head and neck region. Methods: This cross-sectional study was conducted during July 2014 to June 2015. A total of 35 Bangladeshi patient’s specimens of head and neck swelling were collected from two renowned laboratories in Dhaka city.These cases were diagnosed as undifferentiated tumour in histopathological examination.Standard protocol was followed for immunohistochemistry.Then primary immunohistochemical panel which included the markers for Epithelial CK (AE1/AE3),mesenchymal marker (Vimentin) and lymphoid marker (LCA) were used.The cases which were not resolved by primary immunohistochemistry panel, the second panel was applied for further sub classification (Desmin, Chromogranin, CK20, CEA, CD20, CD30, HMB45, NSE). Based on interpretation of immunohistochemical findings final diagnoses were made.Data analysis was performed using the Statistical Package for the Social Sciences for Windows version 22.0 (SPSS, Chicago, Illinois, USA). Results: A total of 35 undifferentiated tumorsof head-neck region were studied.The mean age was 46.3±17.6 years and male to female ratio was 4.8:1. The majority 13 (37.1%) patient had cervical lymphadenopathy, 11 (31.4%) had neck mass and 4(11.4%) had in nasal/sinonasal mass.Regarding histopathologicalcell types, round cell was 21 (60.0%), spindle cell 6 (17.1%), pleomorphic cells 6(17.1%) and epithelioid cells 2(5.7%). In initial histopathological examination, majority 25(71.4%) were undifferentiated malignant neoplasm, 8(22.8%) were metastatic undifferentiated carcinoma, 1(2.9%) was pleomorphic sarcoma and the remaining 1(2.9%) had malignant adnexal tumour. By application of immunohistochemistry, most (33, 94.3%) of the cases were resolved and the two cases remained unresolved. Among the resolved cases majority (15, 45.5%) were lymphoma, 4(12.1%) were metastatic carcinoma, 3(9.1%) were Ewing’s sarcoma and malignant melanoma were found in 3(9.1%) cases. Conclusion: This study supports that the immunohistochemical technique has a fundamental role in the investigation of undifferentiated tumour origin, to determine the correct guidance for treatment and improving the prognosis for head and neck tumour patients. Birdem Med J 2020; 10(1): 48-53

scholarly journals A Rare Presentation of Inflammatory Myofibroblastic Tumor in the Nasolabial Fold

2019 ◽  
Vol 2019 ◽  
pp. 1-5
Author(s):  
Hind K. Alshammari ◽  
Haya F. Alzamami ◽  
Mona Ashoor ◽  
Wasan F. Almarzouq ◽  
Haitham Kussaibi
Keyword(s):  
Inflammatory Myofibroblastic Tumor ◽  
Soft Tissues ◽  
Histopathological Examination ◽  
Benign Lesion ◽  
Neck Region ◽  
Surgical Excision ◽  
Nasolabial Fold ◽  
Rare Presentation ◽  
The Central Nervous System ◽  
Sublabial Approach

Inflammatory myofibroblastic tumor (IMT) is a benign lesion that occurs most frequently in the soft tissues and viscera. In the head and neck region, the tumor has been reported to occur in the orbit, tongue, nasopharynx, larynx, and paranasal sinuses and the central nervous system. Despite being a benign lesion, it exhibits infiltrative and destructive behaviours, making histopathological examination necessary to confirm the diagnosis. We report the case of a 38-year-old female presented with a right nasolabial fold mass, which was confirmed histologically to be an IMT. Surgical excision of the mass was achieved through a sublabial approach with an uneventful postoperative period. To the best of our knowledge, this is the first reported case of an IMT in the nasolabial fold.

scholarly journals Primitive neuroectodermal tumor of thyroid: a rare presentation

2017 ◽  
Vol 4 (1) ◽  
pp. 276
Author(s):  
Shravan S. Shetty ◽  
Dhairyasheel N. Savant ◽  
Hozefa Lokhandwala ◽  
Akanksha Chichra
Keyword(s):  
Head And Neck ◽  
Multimodal Treatment ◽  
Primitive Neuroectodermal Tumor ◽  
Soft Tissues ◽  
Neck Region ◽  
Neuroectodermal Tumor ◽  
Rare Presentation ◽  
Term Survival ◽  
Complete Surgical Excision ◽  
Response To Chemotherapy

<p>Extraskeletal Ewing’s sarcoma (EES) commonly arises in the soft tissues of trunk or extremities. EES is rare in the head and neck region; most having been documented in nasal cavity, paranasal sinuses and neck. Head and neck PNETs have an intermediate prognosis. We report the case of a 12 year old boy who presented with primitive neuroectodermal tumor of the thyroid and was treated with multimodal treatment including surgery, chemotherapy and radiotherapy. The patient is alive and fit with a functional larynx. Major drug regimens use vincristine, doxorubicin, ifosfamide or cyclophosphamide, dactinomycin and/or etoposide. Complete surgical excision is undertaken whenever possible to improve long-term survival. However, the relative radiosensitivity of tumors of the Ewing family, suggest multimodal treatment including adjuvant radiotherapy in case of positive margins or poor response to chemotherapy rather than resection with 2-3 cm margins, which would imply laryngeal sacrifice for thyroid tumors.</p>

scholarly journals Solitary Plasmacytoma of The Jaws: Therapeutically Considerations and Prognosis: A Case Report

2021 ◽  
Vol 1 (1) ◽  
Author(s):  
Mohamed Hamdy Helal ◽  
Ahmed Mohamed Bader ◽  
Mohamed A. Fouad ◽  
Malak Yousef Mohamed shoukheba
Keyword(s):  
Soft Tissues ◽  
Plasma Cells ◽  
Malignant Neoplasm ◽  
Neck Region ◽  
Extramedullary Plasmacytoma ◽  
Solitary Plasmacytoma ◽  
Upper Respiratory Tract ◽  
Head And Neck Region ◽  
Plasma Cell Neoplasm ◽  
Disseminated Disease

The plasma cell neoplasm is a rare malignant neoplasm of plasma cells that may present as Extramedullary Plasmacytoma (EMP) in soft tissues especially in the upper respiratory tract, in the bone as a Solitary Plasmacytoma of bone (SPB), or as a part of the multifocal disseminated disease as Multiple Myeloma (MM). The majority of 80% occurs in the head and neck region. In our case, a 23-year-old female patient presented with a non-tender swelling of the gingiva in the upper and lower jaws with the mobility of all teeth. The radiological examination showed severe destruction of bone around all teeth.

Ewing Sarcoma Family Tumors: Past, Present and Future Prospect

2020 ◽  
Vol 16 ◽  
Author(s):  
Adil Abbas ◽  
Alaa Mohammed Noor Samkari
Keyword(s):  
Soft Tissues ◽  
Fusion Gene ◽  
Surgical Techniques ◽  
Disease Free Survival ◽  
Clinical Aspects ◽  
Immunohistochemical Markers ◽  
The Central Nervous System ◽  
Blue Cell ◽  
Involved Field ◽  
Involved Field Radiotherapy

Abstract: Ewing’s sarcoma (ES), also known as mesenchymal primitive neuroectodermal tumor (PNET), is a malignant round blue cell tumour (MRBCT) with a varying degree of neuronal differentiation. PNET arise from the primitive nerve cells of the central nervous system (CNS) but they may occur in the bones of the extremities, pelvis, vertebral column and chest wall. Extraskeletal ES/PNET may affect the various soft tissues including those of the pelvis, paraspinal region and the thoraco - pulmonary regions Histopathological differentiation between ES, PNET and other related sarcomas is often difficult. On light microscopy, the same histopathological appearance of ES, has been termed PNET, Askin – Rosay (A – R) tumor and malignant neuropeithelioma by various other authors. Immunohistochemical distinction is also difficult due to poor tissue differentiation and poor intake of the various specific immunohistochemical markers. The most frequent translocation is t (11; 22) (q24; q12) resulting in the EWSR1-FLI1 fusion gene detected in nearly 90% of cases and is considered the whole mark of the diagnosis ES, PNET, atypical ES and A – R tumor. Therefore, ES, atypical ES, PNET and A – R tumor are currently regarded as one entity grouped together under the Ewing Family Tumors (EFT) and are treated in an identical way. EFT represent only about 3% of all pediatric malignancies. The annual incidence is between 2 and 5 cases per million children per year. The peak prevalence of the tumor is between the ages of 10 and 15 years. The incidence is higher in males than in females with a ratio of 1.3: 1. Newer groups of MRBCT are with great similarities to EFT are being recently described. These tumors; atypical EFT and Ewing’s like Sarcomas (ELS) bear similarities to EFT but has basic morphological and molecular differences. Optimal treatment requires the use of adjuvant and new-adjuvant chemotherapy (CTR), radical surgical resection and/or involved field radiotherapy (RT). The reported disease free survival (DFS) and overall survival (OS) ranges between 45 – 80% and 36 - 71% respectively. The overall prognosis for metastatic and recurrent disease remains poor. Use of newer conventional and targeted medications, improved RT delivery and surgical techniques may further improve the outcomes. The past few years has seen advances in genomics-based sarcoma diagnosis and targeted therapies. In this wide review article we provide comprehensive report of EFT and discuss the various clinical aspects and the recent advances used in the diagnosis and treatment.

scholarly journals MESENCHYMAL CHONDROSARCOMA OF THE HEAD AND NECK. AN OVERVIEW OF THE FEATURES, PROGNOSTIC FACTORS AND DIRECTIONS OF THE SEARCH FOR TREATMENT STRATEGIES. CLINICAL CASE

2020 ◽  
Vol 66 (4) ◽  
pp. 420-424
Author(s):  
Vladimir Baryshev ◽  
Feliks Sevryukov ◽  
V. Andreev ◽  
L. Kondrashova ◽  
V. Kapinus ◽  
...  
Keyword(s):  
Local Recurrence ◽  
Head And Neck ◽  
Clinical Case ◽  
Soft Tissues ◽  
Treatment Strategies ◽  
Neck Region ◽  
Mesenchymal Chondrosarcoma ◽  
Histological Structure ◽  
Long Lifetime ◽  
Methods Of Treatment

Mesenchymal chondrosarcoma is a rare highly differentiated malignant tumor with a characteristic histological structure, which affects bones or soft tissues and has a high potential for local recurrence. The small number of observations of mesenchymal chondrosarcoma in the head and neck region causes the episodic nature of studies of this problem, and the treatment and diagnostic tactic remains controversial. The article presents the results of the most extensive and systematic studies, which show that the main method of treatment for this category of patients is surgical. Neoadjuvant or adjuvant chemotherapy tends to improve overall survival. Radiation therapy is used as adjuvant treatment. The work provides a clinical case of a long lifetime of a patient with local recurrence of the disease in the absence of regional, distant metastasis and the use of all current methods of treatment for cancer.

scholarly journals Intraoral Soft Tissue Fibrosarcoma: A Case Report and Review

2014 ◽  
Vol 4 (2) ◽  
pp. 118-123
Author(s):  
Jagdish Vishnu Tupkari ◽  
Tabita Joy Chettiakandy ◽  
Dimple Padawe ◽  
Keshav Kumar ◽  
Manisha Sardar ◽  
...  
Keyword(s):  
Case Report ◽  
Soft Tissue ◽  
Head And Neck ◽  
Soft Tissues ◽  
Malignant Neoplasm ◽  
Neck Region ◽  
Soft Tissue Tumors ◽  
The Body ◽  
World Health ◽  
Head And Neck Region

ABSTRACT Adult fibrosarcoma (FS), defined by the World Health Organization as ‘malignant neoplasm composed of fibroblasts with variable collagen production and, in classical cases, a ‘herringbone’ architecture’, is a very rare soft tissue sarcoma. The earlier literature revealed that the adult fibrosarcoma was the most commonly occurring lesion, however the incidence of which has declined dramatically over the past several decades. This is due to evolution in the classification of soft tissue tumors, recognition of its subtypes and increased understanding of the many other mesenchymal and nonmesenchymal tumors. Sarcomas are the rare group of malignancies which accounts for less than 1% of all the body sarcomas of which 5 to 15% occurs in head and neck region. It involves deep soft tissues of extremities, trunk, head and neck. Among the head and neck region, intraoral adult fibrosarcomas are very rare without any sex predilection. A case of primary soft tissue FS in posterior mandible of an 13-year-old female patient is documented here, which will be an addition to the cases reported earlier in the literatures. How to cite this article Tupkari JV, Chettiakandy TJ, Padawe D, kumar k, Sardar M, Gupta N. Intraoral Soft Tissue Fibrosarcoma: A Case Report and Review. J Contemp Dent 2014;4(2):118-123.

A Melanotic Neuroectodermal Tumor of Infancy Arising from the Anterior Fontanel

1977 ◽  
Vol 63 (4) ◽  
pp. 373-380 ◽  
Author(s):  
Corradino De Pascalis ◽  
Pierpaolo Mastroiacovo ◽  
Renato Mastrangelo
Keyword(s):  
Differential Diagnosis ◽  
Local Recurrence ◽  
Neuroectodermal Tumor ◽  
Rare Tumor ◽  
Anterior Fontanel ◽  
Benign Nature

A case of local recurrence of melanotic neuroectodermal tumor of infancy (HINT), arising from the anterior fontanel in a 6-month-old female, is presented. The follow-up of 48 months after surgery confirms the benign nature of this tumor despite its local recurrence. Clinical and histopathologic characteristics of this rare tumor are discussed. A review of 12 cases of MNT involving the skull is given. It is emphasized that this entity should be kept in mind in the differential diagnosis of a mass in the craniofacial structures in infancy.

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