Peripheral Primitive Neuroectodermal Tumor Involving the Paravertebral and Retroperitoneal Regions.

Fukumoto, Shinichi; Takamura, Kei; Nakanishi, Kyoko; Yamamoto, Makoto; Inaba, Shuichi; Makimura, Shiroh; Yoshikawa, Takashi; Terai, Tsugio

doi:10.2169/internalmedicine.36.424

Cited by 9 publications

(2 citation statements)

References 17 publications

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“…Only about 10% of the patients who formed part of the German study of soft tissue tumors suffered from PNET. Only individual case reports or descriptions of small groups of patients can be found in the medical literature [17][18][19].…”

Section: Discussionmentioning

confidence: 99%

Peripheral Primitive Neuroectodermal Tumor: Challenge for Multimodal Treatment

et al. 2001

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The primitive neuroectodermal tumor (PNET) is an extremely aggressive soft tissue neoplasm that occurs in children and adolescents. We retrospectively reviewed our therapeutic experience with a multidisciplinary approach, combining surgery, chemotherapy, and radiation therapy. Treatment of PNET was carried out in compliance with the soft tissue protocol (CWS) from the German Society of Pediatric Oncology. Biopsy‐proven diagnosis was followed by chemotherapy, which in all cases led to partial remission, allowing excision of the remainder of the tumor without mutilation. After excision, irradiation of the tumor site and two further sequences of chemotherapy were performed. When PNET of the paravertebral region caused symptoms of paralysis and immediate surgery was required, postoperative chemotherapy, a second‐look operation, and irradiation were undertaken. Between 1986 and 1998 we treated 13 patients (median age 15 years). In five patients the PNET originated from the chest wall and in eight patients from the paravertebral and retroperitoneal region. Five patients died after 20 months on average, and the remaining eight patients are in full remission after 7, 16, 46, 55, 70, 74, 75, and 115 months, respectively. Close cooperation between surgeons and their pediatric and radiotherapy colleagues is obligatory when treating PNET. Chemotherapy as the first stage is mandatory to avoid a mutilating surgical procedure and intraoperative tumor cell dissemination.

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Section: Discussionmentioning

confidence: 99%

Peripheral Primitive Neuroectodermal Tumor: Challenge for Multimodal Treatment

et al. 2001

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“…[23] Cases of kidney, myocardium, rectum, ovary, retroperitoneum, and bladder involvement have been reported. [16101214]. …”

Section: Discussionmentioning

confidence: 99%

Peripheral primitive neuroectodermal tumor of the cervical spine

Cabral¹,

Nunes²,

Melo³

et al. 2012

Surg Neurol Int

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Background:Primitive neuroectodermal tumor (PNET) is a malignant neoplasm that generally arises from bone and soft tissues, with predilection for young adults. This neural crest origin tumors share biologic and histologic features with Ewing's sarcoma (ES).Case Description:We present a case of a 22-year-old woman with history of severe progressive neck pain, without radiation, associated with paresthesia in the right arm, and palpable right posterior cervical mass. Neurological examination showed increased reflexes in all four limbs, bilateral Hoffman's sign, right Babinski's sign, and right hemi-hypoesthesia. Neuroimaging revealed a right posterior cervical lesion with heterogeneous contrast enhancement extending to the neural foramina of the atlas and axis. Patient underwent microsurgical removal of the lesion, and histopathological and immunohistochemical analysis confirmed the diagnosis of peripheral primitive PNET (pPNET). The patient had adjuvant treatment with radiotherapy and chemotherapy. After twelve months, neuroimaging showed no signs of tumor regrowth and the patient had no neurological deficits. However, three months later, the patient developed hydrocephalus and cerebrospinal fluid (CSF) was positive for neoplastic cells. No other treatment was administered and the patient died.Conclusion:pPNET is a rare malignant tumor with poor prognosis, although promising results with multimodal treatment-surgery, radiotherapy, and chemotherapy. Diagnosis requires immunohistochemical analysis, with identification of neuronal differentiation markers.

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