Primitive Neuroectodermal Tumor of the Lung

2001 ◽  
Vol 125 (3) ◽  
pp. 397-399
Author(s):  
Andrea G. Kahn ◽  
Alejandra Avagnina ◽  
Jorge Nazar ◽  
Boris Elsner
Keyword(s):  
Primitive Neuroectodermal Tumor ◽  
Neuron Specific Enolase ◽  
Middle Lobe ◽  
Neurosecretory Granules ◽  
Primitive Neuroectodermal Tumors ◽  
Ultrastructural Studies ◽  
Neuroectodermal Tumors ◽  
Right Middle Lobe ◽  
Cytoplasmic Processes

Abstract Primitive neuroectodermal tumors occur most frequently in bone and soft tissue but have been reported in other locations. Primary lung primitive neuroectodermal tumors without pleural or chest wall involvement are extremely rare. We present a case with immunohistochemical and ultrastructural studies and follow-up of the patient. An 18-year-old man presented with hemoptysis. Chest radiographs revealed a right middle lobe mass, and bronchoscopy showed an endobronchial tumor. The lesion was resected by middle lobectomy. After 2 years, a local recurrence was treated by pneumonectomy. The patient died after surgery. Histologically, the tumor was composed of uniform cells with round nuclei and scanty cytoplasm arranged in cohesive lobules with occasional rosette formation. Immunohistochemically, the tumor was positive for vimentin, CD99, neuron-specific enolase, and neurofilaments. Ultrastructural study revealed neurosecretory granules and cytoplasmic processes. Our case shows the value of immunohistochemistry and electron microscopy in the diagnosis of primitive neuroectodermal tumors in unusual locations.

scholarly journals Primitive Neuroectodermal Tumor of Axilla in an Adult: A Rare Entity

2021 ◽  
Vol 3 (2) ◽  
pp. 1-2
Author(s):  
Shravan S Shetty ◽  
◽  
Vidhi Shah ◽  
Eshpuniyani Prriya Prriya ◽  
◽  
...  
Keyword(s):  
Primitive Neuroectodermal Tumor ◽  
Subcutaneous Tissue ◽  
Neuroectodermal Tumor ◽  
Round Cell ◽  
Primitive Neuroectodermal Tumors ◽  
Neuroectodermal Tumors ◽  
Poorly Differentiated ◽  
The Right ◽  
Disease Free

Primitive neuroectodermal tumors (PNETs) are poorly differentiated small round cell neoplasms which mainly affect children and are not commonly seen in adults. Superficial primitive neuroectodermal tumors are rare and have a favourable prognosis compared to conventional deep seated tumors. We report a case of a 62 year old gentleman with a primitive neuroectodermal tumor arising from the subcutaneous tissue of the right axilla. He was treated with multimodal treatment including surgery and radiotherapy. He is alive and disease free at 2 year follow up.

Clinical Analysis of Primary Primitive Neuroectodermal Tumors in the Female Genital Tract

2014 ◽  
Vol 24 (3) ◽  
pp. 404-409 ◽  
Author(s):  
Changji Xiao ◽  
Jing Zhao ◽  
Peng Guo ◽  
Dan Wang ◽  
Dachun Zhao ◽  
...  
Keyword(s):  
Genital Tract ◽  
Female Genital Tract ◽  
Clinical Manifestations ◽  
Neuron Specific Enolase ◽  
Lower Abdominal Pain ◽  
Primitive Neuroectodermal Tumors ◽  
Pathologic Features ◽  
Neuroectodermal Tumors ◽  
Female Genital

ObjectiveThe aim of the study was to investigate the clinical manifestations, diagnosis, treatment, and prognosis of primitive neuroectodermal tumors (PNETs) in the female genital tract.MethodsFrom April 2001 to May 2013, the clinicopathologic characteristics, treatments, outcomes, and prognosis of 11 patients with PNET in the female genital tract were analyzed retrospectively at our hospital.ResultsThe location of PNET in the 11 patients presented here included vulva (2 patients), cervix (2 patients), uterus and its ligament (5 patients), and the ovaries (2 patients). Ages ranged from 18 to 59 years (median, 31 years).The main clinical manifestations of PNET in the female genital tract are irregular vaginal bleeding (6 patients), pelvic mass, uterine enlargement, and rapidly increasing vulvar mass (8 patients), and vulvar pain and lower abdominal pain (5 patients). The CA125 levels of 8 patients were elevated before the operations and reduced to normal when the diseases were controlled, while the levels increased as the tumor was progressive. Results for the most commonly used immunohistochemistry studies revealed CD99 in 11 of the 11 tumors, synaptophysin in 6 of the 7 positive tumors, and neuron-specific enolase in 6 of the 6 tumors. Ten patients underwent surgical resection. Nine of them underwent preoperative or/and postoperative combination chemotherapy. The follow-up of 10 patients were available and ranged from 1 to 145 months (median, 30.5 months), 3 of whom experiencing recurrence.ConclusionsPrimitive neuroectodermal tumor is very rare and can originate from any part of the female genital tract. The tumors had different manifestations but the same pathologic features. CA125 may be an important marker for prognosis and follow-up of PNET of the female internal genital tract.

Primitive Neuroectodermal Tumor of Axilla in an Adult: A Rare Entity

2021 ◽  
pp. 1-2
Author(s):  
Shetty Shravan S ◽  
◽  
Shah Vidhi ◽  
Keyword(s):  
Primitive Neuroectodermal Tumor ◽  
Subcutaneous Tissue ◽  
Neuroectodermal Tumor ◽  
Round Cell ◽  
Primitive Neuroectodermal Tumors ◽  
Neuroectodermal Tumors ◽  
Poorly Differentiated ◽  
The Right ◽  
Disease Free

Primitive neuroectodermal tumors (PNETs) are poorly differentiated small round cell neoplasms which mainly affect children and are not commonly seen in adults. Superficial primitive neuroectodermal tumors are rare and have a favourable prognosis compared to conventional deep seated tumors. We report a case of a 62 year old gentleman with a primitive neuroectodermal tumor arising from the subcutaneous tissue of the right axilla. He was treated with multimodal treatment including surgery and radiotherapy. He is alive and disease free at 2 year follow up

scholarly journals Primitive neuroectodermal tumor of the esophagus with metastasis in the pineal gland

2019 ◽  
Vol 07 (09) ◽  
pp. E1163-E1165
Author(s):  
Leonardo Blas Jhon ◽  
Paloma Sánchez-Fayos ◽  
Maria Jesus Martín Relloso ◽  
Daniel Calero Barón ◽  
Juan Carlos Porres Cubero
Keyword(s):  
Case Report ◽  
Pineal Gland ◽  
Disease Progression ◽  
Rare Case ◽  
Primitive Neuroectodermal Tumor ◽  
Malignant Neoplasms ◽  
Primitive Neuroectodermal Tumors ◽  
First Case ◽  
Neuroectodermal Tumors ◽  
Chemotherapeutic Treatment

AbstractPrimitive neuroectodermal tumors (PNET) are very rare tumors that belong to a family of malignant neoplasms of tiny round cells which are derived from the neural crest. This report discusses a rare case of an adult woman with esophageal PNET, confirmed by immunohistochemistry, that presented with metastasis to the pineal gland. To our knowledge, this is the first case report of a PNET with these features. Despite surgery and chemotherapeutic treatment, our case has shown disease progression.

Primitive Neuroectodermal Tumor of Cerebrum With Adipose Tissue

2001 ◽  
Vol 125 (2) ◽  
pp. 264-266
Author(s):  
Satish Krishnamurthy ◽  
Stephen Kent Powers ◽  
Javad Towfighi
Keyword(s):  
Skeletal Muscle ◽  
Central Nervous System ◽  
Nervous System ◽  
Adipose Tissue ◽  
Smooth Muscle ◽  
Primitive Neuroectodermal Tumor ◽  
Primitive Neuroectodermal Tumors ◽  
Mature Adipose Tissue ◽  
Neuroectodermal Tumors ◽  
The Central Nervous System

Abstract Primitive neuroectodermal tumors (PNETs) of the central nervous system are uncommon embryonal neoplasms, rarely occurring in adults. Differentiation into specific mesenchymal tissues, such as cartilage, bone, skeletal muscle, smooth muscle, or adipose tissue, is rare. We report a case of a 51-year-old woman with a PNET of cerebrum that showed extensive mature adipose tissue differentiation. This is the second case, to our knowledge, of PNET of cerebrum with adipose tissue elements that has been described.

scholarly journals Is Ovarian Tissue Transplantation Safe in Patients with Central Nervous System Primitive Neuroectodermal Tumors?

2020 ◽  
Vol 9 (12) ◽  
pp. 4101
Author(s):  
Thu Yen Thi Nguyen ◽  
Alessandra Camboni ◽  
Rossella Masciangelo ◽  
Jacques Donnez ◽  
Marie-Madeleine Dolmans
Keyword(s):  
Cancer Cells ◽  
Ovarian Tissue ◽  
Neuron Specific Enolase ◽  
Cancer Recurrence ◽  
Tissue Transplantation ◽  
Ovarian Tissue Transplantation ◽  
Primitive Neuroectodermal Tumors ◽  
Immunodeficient Mice ◽  
Neuroectodermal Tumors ◽  
Digital Polymerase Chain Reaction

The risk of reseeding malignancy harbored in cryopreserved and transplanted ovarian tissue has been a source of concern. This study aimed to determine the potential relationship between frozen–thawed ovarian tissue transplantation and primary cancer recurrence. Three patients with cerebral primitive neuroectodermal tumors (PNET) were included in this study. One woman gave birth to three healthy babies following reimplantation of her cryopreserved ovarian tissue, but subsequently died due to cancer relapse six years after ovarian tissue transplantation. The second subject died from progressive cancer, while the third is still alive and awaiting reimplantation of her ovarian tissue in due course. Frozen ovarian cortex from all three patients was analyzed and xenotransplanted to immunodeficient mice for five months. Main outcomes were the presence of cancer cells in the thawed and xenografted ovarian tissue at histology, immunostaining (expression of neuron-specific enolase and glial fibrillary acidic protein (GFAP)), and reverse-transcription droplet digital polymerase chain reaction (RT-ddPCR) (levels of enolase 2 and GFAP). In conclusion, no malignant cells were detected in ovarian tissue from patients with PNET, even in those who experienced recurrence of the disease, meaning that the risk of reseeding cancer cells with ovarian tissue transplantation in these patients can be considered low.

scholarly journals Fatal congenital lobar emphysema in a puerpera: a case report and literature review

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Fanyi Gan ◽  
Liang Xia ◽  
Yushang Yang ◽  
Qiang Pu ◽  
Lunxu Liu
Keyword(s):  
Left Lung ◽  
Middle Lobe ◽  
Left Hemithorax ◽  
Recurrent Pneumonia ◽  
Mediastinal Shift ◽  
Life Threatening ◽  
Right Middle Lobe ◽  
Lobar Emphysema ◽  
The Right

Abstract Background Congenital lobal emphysema (CLE) is a developmental lung abnormality usually diagnosed in the neonatal period and is rarely observed in adults. Adults with CLE are usually asymptomatic and only a small fraction may present with coughing, recurrent pneumonia and respiratory distress. In imaging studies, the most frequently affected lobe of CLE is the left upper lobe, followed by the right middle lobe. However, multilobar involvement with severe mediastinal shift is extremely rare. Case presentation We report a case of fatal CLE in a 28-year-old puerpera with postpartum respiratory failure. Chest computed tomography (CT) revealed emphysema of the right upper, middle and lower lobes resulting in adjacent atelectasis. Hyperinflation of the right upper lobe crossed the midline, leading to a deviation of the mediastinal structure to the left hemithorax and severe compression of the left lung. Conclusions Early and timely diagnosis of CLE with routine follow-up is necessary for patients. CLE, especially with multilobar involvement or mediastinal shift, could be life-threatening and should be promptly and aggressively treated to prevent severe complications.

scholarly journals Primary intracranial peripheral primitive neuroectodermal tumor in an adult patient with aphasia: a rare case report

2021 ◽  
Author(s):  
kazem ghaemi ◽  
mahdieh rajabi-moghaddam ◽  
Hamid Abbaszadeh
Keyword(s):  
Case Report ◽  
Adult Patient ◽  
Rare Case ◽  
Primitive Neuroectodermal Tumor ◽  
Chief Complaint ◽  
Peripheral Primitive Neuroectodermal Tumor ◽  
Primitive Neuroectodermal Tumors ◽  
Neuroectodermal Tumors ◽  
Rare Case Report ◽  
First Time

Primary intracranial primitive neuroectodermal tumors (PNETs) are extremely rare malignancies, affects children and adolescents with only 10 cases has been reported over 33 years old. we present a case of PNET in a 36 years old female patient with the chief complaint of aphasia for the first time

scholarly journals Incidental endobronchial hamartoma in a patient with enchondroma

2019 ◽  
Vol 12 (9) ◽  
pp. e229670
Author(s):  
Arun Kadamkulam Syriac ◽  
Amrit Ved Bhaskarla ◽  
Mohamed Elrifai ◽  
Abdul H Alraiyes
Keyword(s):  
Ct Scan ◽  
Argon Plasma Coagulation ◽  
Argon Plasma ◽  
Mesenchymal Cell ◽  
Middle Lobe ◽  
Endobronchial Lesion ◽  
Right Middle Lobe ◽  
Benign Tumours ◽  
The Right

Hamartomas are the most common type of benign tumours of the lung, constituting a small portion of all lung neoplasms. Hamartomas are rare benign tumours composed of multiple mesenchymal cell lines. Two clinical types have been defined according to the location: intraparenchymal and endobronchial, more frequently the former. We present a case of endobronchial hamartoma causing significant blockage of the right middle lobe. The finding was incidental on a CT scan of the chest done for staging purposes for a large mixed lytic and sclerotic lesion that was found within the proximal-mid portion of the tibial diaphysis. The endobronchial lesion was removed by hot electrocautery snare during bronchoscopy and identified as a hamartoma. Argon plasma coagulation was applied to the lesion’s base afterwards and the patient was to follow-up in 3 months for a repeat CT scan.

scholarly journals Renal Primitive Neuroectodermal Tumors

2012 ◽  
Vol 136 (6) ◽  
pp. 686-690 ◽  
Author(s):  
Tanner Bartholow ◽  
Anil Parwani
Keyword(s):  
Primitive Neuroectodermal Tumor ◽  
Correct Diagnosis ◽  
Age Distribution ◽  
Disease Free Survival ◽  
Neuroectodermal Tumor ◽  
Renal Tumors ◽  
Primitive Neuroectodermal Tumors ◽  
Neuroectodermal Tumors ◽  
Genetic Abnormalities ◽  
Disease Free Survival Rate

Primitive neuroectodermal tumors exist as a part of the Ewing sarcoma/primitive neuroectodermal tumor family. These tumors most commonly arise in the chest wall and paraspinal regions; cases with a renal origin are rare entities, but have become increasingly reported in recent years. Although such cases occur across a wide age distribution, the average age for a patient with a renal primitive neuroectodermal tumor is the mid- to late 20s, with both males and females susceptible. Histologically, these tumors are characterized by pseudorosettes. Immunohistochemically, CD99 is an important diagnostic marker. Clinically, these are aggressive tumors, with an average 5-year disease-free survival rate of only 45% to 55%. Given that renal primitive neuroectodermal tumor bears many similarities to other renal tumors, it is important to review the histologic features, immunostaining profile, and genetic abnormalities that can be used for its correct diagnosis.

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