Primitive neuroectodermal tumours: Anatomic location, extent of surgery, and outcome

Kimber, Chris; Michalski, A; Spitz, L; Pierro, André Canesso

doi:10.1016/s0022-3468(98)90357-8

Cited by 53 publications

(55 citation statements)

References 6 publications

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“…Apart from thoracic region they are also known to arise in head and neck, esophagus, liver, kidneys and adrenal glands. The prognosis of pPNETs depends on the tumor site, tumor volume and metastasis (11).…”

Section: Discussionmentioning

confidence: 99%

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Primitive neuroectodermal tumor of lungs in adults: a rare series of three cases treated with upfront chemo-radiation

Purkayastha¹,

Pathak²,

Sharma³

et al. 2016

Transl. Lung Cancer Res.

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Primitive neuroectodermal tumors (PNETs) are highly malignant small round blue cell tumors of neuroectodermal origin belonging to either central nervous system, autonomic nervous system or peripheral Askin's or Ewing's group of neoplasms. The latter generally arise in soft tissues of trunk or axial skeleton in children and early adolescents. However in adults this entity is very uncommon. Of all peripheral entities, primary PNET of lungs without chest wall or pleural involvement in adults are extremely rare and have been scarcely reported in world literature as single case reports. We hereby report a series of three interesting cases of adult PNET of lung diagnosed and treated in our institute. The chief presenting complaints of these patients were of chest pain, cough and dyspnea. The cases were diagnosed on the basis of imaging and biopsy which confirmed these lesions to be of PNET histology, confirmed by immunopositivity for neuron specific enolase (NSE), synaptophysin, chromogranin, CD 99 and vimentin on immunohistochemistry (IHC). All three were deemed unresectable in view of infiltration of nearby vital organs and high chances of morbidity. They were treated with upfront chemotherapy followed by conformal radiotherapy (RT) to the residual disease to which they showed significant response both clinically and radiologically. Presently these patients are on regular follow-up for over 6 months without any evidence of progression of disease or distant metastasis.

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Section: Discussionmentioning

confidence: 99%

“…SCLCs have a history of smoking and usually present at an older age while younger age and no smoking history is associated with lung PNETs. They are extremely aggressive with a dismal prognosis as patients may present with an upfront metastatic disease to contralateral lung, bone and bone marrow (11).…”

Section: Discussionmentioning

confidence: 99%

Primitive neuroectodermal tumor of lungs in adults: a rare series of three cases treated with upfront chemo-radiation

Purkayastha¹,

Pathak²,

Sharma³

et al. 2016

Transl. Lung Cancer Res.

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“…Tek ba şı na ser vi kal böl -ge tu tu lu mu %1-5 ora nın da dır. 2 Yer le şim ye ri ve mak ros ko pik gö rü nü mü multi lo bü ler gu at ra ben ze di ğin den ti ro id be zi has ta lık -la rı nın ayı rı cı ta nı sın da GNB'nin de ha tır lan ma sı ge rek ti ği dü şün ce siy le, ser vi kal GNB ta nı sı al mış genç eriş kin yaş gru bun da ki bir ol gu li te ra tür eş liğin de su nul muş tur.…”

Section: Ayirici Taniunclassified

“…Sem pa tik do ku la rın bu lun du ğu ad re nal bez (%35), ad re nal dı şı ret ro pe ri ton (%30-35), pos te rior me di as ten (%20), bo yun (%1-5) ve pel vis te (%2-3) gö rü le bi lir ler. 2 Kli nik kit le nin çev re do ku la ra yap tı ğı ba sı etki siy le şekil le nir. Bo yun yer le şim li olan lar da en sık asemp to ma tik şiş lik gö rü lür.…”

Section: Tar Tiş Maunclassified

Cervical Ganglioneuroblastoma Mimicking Thyroid Tissue in a Young Adult: Differential Diagnosis

Keskinöz¹,

Şahin²,

Sert³

et al. 2011

Turkiye Klinikleri J Med Sci

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“…3 Head and neck PNETs have an intermediate prognosis between abdominopelvic disease (who fare much worse) and extremities. 4 Ewing's thyroid cases mostly occur in patients ≤30 years.…”

Section: Introductionmentioning

confidence: 99%

Primitive neuroectodermal tumor of thyroid: a rare presentation

Shetty

Savant

Lokhandwala

et al. 2017

Int J Otorhinolaryngol Head Neck Surg

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<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>

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Primitive neuroectodermal tumours: Anatomic location, extent of surgery, and outcome

Cited by 53 publications

References 6 publications

Primitive neuroectodermal tumor of lungs in adults: a rare series of three cases treated with upfront chemo-radiation

Primitive neuroectodermal tumor of lungs in adults: a rare series of three cases treated with upfront chemo-radiation

Cervical Ganglioneuroblastoma Mimicking Thyroid Tissue in a Young Adult: Differential Diagnosis

Primitive neuroectodermal tumor of thyroid: a rare presentation

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