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 ◽  
...  

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.

2004 ◽  
Vol 14 (2) ◽  
pp. 370-372 ◽  
Author(s):  
K.-J. Kim ◽  
B.-W. Jang ◽  
S.-K. Lee ◽  
B.-K. Kim ◽  
S.-L. Nam

Peripheral primitive neuroectodermal tumor (PNET) belongs to the PNET/Ewing's sarcoma family. PNET is a small round cell tumor of putative neuroectoderm origin and is the second most common sarcoma among children and young adults. It may occur anywhere in the body and within any age group; however, it is most likely to occur in the bone and soft tissues. There have been a small number of case reports of PNET arising in the ovary. We presented a case of PNET arising in the right ovary of an 18-year-old woman. The tumor was metastased to the lymph nodes of the pelvis and para-aorta at surgical staging. We had persecuted Taxol/carboplatin chemotherapy, pelvic cavity radiotherapy, and Vincristine/Actinomycink, Cyclophosphamide/Doxorubicin (VACA). She died after 10 months due to septic shock.


2018 ◽  
Vol 142 (11) ◽  
pp. 1358-1363 ◽  
Author(s):  
Brian S. Soles ◽  
Allecia Wilson ◽  
David R. Lucas ◽  
Amer Heider

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.


1996 ◽  
Vol 35 (5) ◽  
pp. 460-465 ◽  
Author(s):  
Mayumi UENO ◽  
Junzo FUJIYAMA ◽  
Tadashi IDEI ◽  
Kunishi KAWAMATA ◽  
Yukitoshi SATOH ◽  
...  

2014 ◽  
Vol 2014 ◽  
pp. 1-3
Author(s):  
Zhenhua Ma ◽  
Wei Yang ◽  
Yingmin Yao ◽  
Qingguang Liu

Human hydatid disease is a significant health problem in endemic regions caused by the larval form ofEchinococcus granulosus. In this paper, we report a case of liver hydatid cyst. The patient, a 15-year-old boy, presented with a history of intermittent upper abdominal pain of a few-month duration was referred to our hospital for investigation. Computed tomographic scan and laboratory test suggested a hydatid cyst in the right lobe of liver. The adventitia resection of hydatid cyst was smoothly performed as there was a less bloody virtual space between adventitia and outer membrane. Our diagnosis was made using an imaging approach and was confirmed during surgery. We proposed the adventitia resection of hydatid cyst could be safe and easy to perform with low risk of bleeding and bile leakage.


Author(s):  
Shravan S. Shetty ◽  
Dhairyasheel N. Savant ◽  
Hozefa Lokhandwala ◽  
Akanksha Chichra

<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>


2021 ◽  
Author(s):  
Qiaoyun Chen ◽  
Ronghui Jin ◽  
Xu Li ◽  
Qifan Wu ◽  
Gaili An

Abstract Background: Peripheral primitive neuroectodermal tumor (pPNET) is a relatively rare malignant neoplasm that usually occurs in children and young adults,associated with poor prognosis. However, standard treatment for refractory pPNET has not been determined.Case presentation: A case of a 38-year-old woman with pPNET on the right shoulder and back,she had gained up to seven-and-a-half years suvival undergoing these comprehensive treatment including surgery, radiotherapy, chemotherapy, antiangiogenic treatment. However, the tumor eventually progressed after receiving multiline treatment.With the patient's strong desire to receive immunotherapy, we finally adjusted the treatment plan to " anti-angiogenic tyrosine kinase inhibitor Lenvatinib combined with PD-1 inhibitor Toripalimab" for 2 cycles.Unfortunately,she developed grade 2-3 immune pneumonia after treatment.Conclusions: To our knowledge, this is the first report of immunotherapy combined with antiangiogenic therapy in pPNET,which provides new ideas for the treatment of refractory pPNET.


2005 ◽  
Vol 42 (6) ◽  
pp. 834-836 ◽  
Author(s):  
A. Berrocal ◽  
D. L. Montgomery ◽  
J. T. Mackie ◽  
R. W. Storts

A variety of embryonal tumors of the central nervous system, typically malignant and occurring in young individuals, are recognized in humans and animals. This report describes an invasive subdural but predominantly extramedullary primitive neuroectodermal tumor developing at the lumbosacral junction in a 6-month-old Brahman crossbred calf. The tumor was composed of spindloid embryonal cells organized in interlacing fascicles. The cells had oval to elongate or round hyperchromic nuclei, single to double nucleoli, and scant discernible cytoplasm. Immunohistochemical staining for neuron-specific enolase, synaptophysin, and S-100 protein and formation of pseudorosettes suggested neuronal and possibly ependymal differentiation.


2021 ◽  
Author(s):  
Xin-xiang Ma ◽  
Chuan-xin Zhang ◽  
Yi-fan Ren ◽  
Yu-lin Zhang ◽  
Ji-ai Zheng

Abstract IntroductionIn children, snoring is often associated with hypertrophied tonsils and adenoids. There was a case in a girl who did not suffer from hypertrophy adenoids, but from a peripheral primitive neuroectodermal tumor (pPNET) in the oropharynx. pPNET often occurs in the deep soft tissues of the trunk, paravertebral, lower limbs and retroperitoneum. It is rarely located in children's oropharynx and makes snoring.Patient concernsWe report a case of a 4-years-old girl who complained of slurred speech and snoring. Her serum showed normal results, computed tomography (CT) and magnetic resonance imaging (MRI) revealed a mass lesion in oropharynx. By using histology and immunohistochemistry, cluster of differentiation 99 (CD99), vimentin, friend leukemia integration 1 (FLI-1), integrase interactor-1 and α-smooth muscle actin were positive, but epithelial membrane antigen, erythroblast transformation-specific (ETS) related gene (ERG), NK homeobox 2.2, Wilms’ tumor 1 and S100-protein were negative. And the rearrangement of the Ewing sarcoma breakpoint region 1 (EWSR1) gene was proved in the test of fluorescent.DiagnosisShe was diagnosed as a peripheral primitive neuroectodermal tumor.Interventions and outcomeThe girl had been treated with surgery and chemotherapy. She has been well for 24 months without any signs of disease.ConclusionIn children who snore, we should not only think of hypertrophied adenoids, but also the possibility of pPNETs in oropharynx. CT scan or MRI should be conducted in time, as well as histology and immunohistochemistry to avoid misdiagnosis.


2014 ◽  
Vol 5 (02) ◽  
pp. 176-179 ◽  
Author(s):  
Bashar Abuzayed ◽  
Wiam Khreisat ◽  
William Maaiah ◽  
Saoud Agailat

ABSTRACTA 24-year-old female patient presented with complaints of nausea, vomiting and of loss of consciousness lasted for 15 minutes with left sided weakness. Neuroradiological evaluation revealed a hemorrhagic mass lesion in the right frontal lobe. The patient was operated and intraoperative findings showed a cortical-subcortical hematoma including hemorrhagic and disrupted tissue with a pathologic purple tissue on the periphery of the hematoma. Postoperative course was uneventful and postoperative histopathological examination revealed primitive neuroectodermal tumor. The patient was then referred to medical and radiation oncology clinics for further evaluation and treatment.


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