Background: Peripheral neuroectodermal tumors arising from vulva are extremely rare; only 12 cases have been reported so far. These tumors belong to the Ewing sarcoma family of tumors. They occur in bones and variety of soft tissues of chest wall, pelvis, paravertebral region, and lower extremities. Being aggressive tumors, they have poor prognosis.
Case: We report a case of a 20-year-old woman who presented with a 20 x 15 x 10-cm pedunculated mass arising from the right labium majus. Judicious use of a panel of markers led to diagnosis of peripheral neuroectodermal tumor. The patient died of metastatic disease.
Conclusions: Judicious use of a panel of markers and a molecular test, if necessary, helps in confirming the diagnosis. Surgery followed by chemotherapy with or without radiation is the treatment of choice.
Download full-text PDF |
Source |
|---|---|
| http://dx.doi.org/10.1097/LGT.0b013e3181b0f8f3 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Making sense of vertebrate senses from a neural crest and cranial placode evo-devo perspective.
Trends Neurosci
January 2025
Division of Biology and Biological Engineering, California Institute of Technology, Pasadena, CA 91125, USA. Electronic address:
The evolution of vertebrates from protochordate ancestors marked the beginning of the gradual transition to predatory lifestyles. Enabled by the acquisition of multipotent neural crest and cranial placode cell populations, vertebrates developed an elaborate peripheral nervous system, equipped with paired sense organs, which aided in adaptive behaviors and ultimately, successful colonization of diverse environmental niches. Underpinning the enduring success of vertebrates is the highly adaptable nature of the peripheral nervous system, which is enabled by the exceptional malleability of the neural crest and placode developmental programs.
View Article and Find Full Text PDFMetastatic Neuroblastoma Masquerading as Ewing's Sarcoma on X-ray: An Imaging Puzzle in Pediatric Oncology.
J Orthop Case Rep
January 2025
Department of Pathology, All India Institute of Medical Sciences Bhopal, Bhopal, Madhya Pradesh, India.
Introduction: Neuroblastoma is an embryonic tumor of the peripheral sympathetic nervous system. It is the most common extracranial solid tumor of childhood and accounts for up to 15% of all pediatric cancer fatalities. The manifestation of neuroblastoma is variable depending on the location of the tumor and the presence or absence of paraneoplastic syndromes.
View Article and Find Full Text PDFPrimary ovarian peripheral primitive neuroectodermal tumor presented with breast metastasis; Case report.
Rare Tumors
January 2025
Internal Medicine Department, An Najah National University Hospital, Nablus, Palestine.
Ewing sarcoma family tumors (ESFT) pose diagnostic challenges, which largely depend on the primary site of involvement and tumor stage. Despite advancements in treatment, metastatic ESFTs remain associated with poor outcomes. This case describes a 21-year-old woman who, in July 2022, presented with a left breast mass identified through ultrasound and CT scan, along with abdominal distention.
View Article and Find Full Text PDFChallenges in Diagnosing Primary Intracranial Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor: A Case Report.
Curr Med Imaging
January 2025
Department of Radiology, Affiliated Hospital of Southwest Medical University, Sichuan, China.
Background: Primary intracranial Ewing Sarcoma/peripheral Primitive Neuroectodermal Tumor (EWS/pPNET) is exceedingly rare and easy to misdiagnose.
Case Presentation: We present a case involving a 23-year-old male who presented with headaches and vomiting. The preoperative brain imaging revealed an irregular mass in the left parietal lobe, initially misdiagnosed as meningioma.
A Case of Primary Ovarian Primitive Neuroectodermal Tumor.
Med J Islam Repub Iran
July 2024
Advanced Diagnostic and Interventional Radiology Research Center (ADIR), Tehran University of Medical Sciences, Tehran, Iran.
Primitive neuroectodermal tumors (PNET) are a family of poorly differentiated malignant neoplasms of neuroectodermal origin. According to the location of origin, PNETs could be further categorized as central or peripheral. Peripheral PNET (pPNET) is an uncommon type that accounts for 1% of all soft tissue sarcomas and occurs outside the central and sympathetic nervous systems.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!