Spinal Primitive Neuroectodermal Tumor (PNET) in a 64-year-old male treated with surgery, radiotherapy, and chemotherapy: A case report

@#Primitive neuroectodermal tumors (PNETs) are a heterogeneous group of malignant neoplasms found primarily in childhood and early adulthood. In this paper, we described the case of a 64-year-old male with primary spinal PNET, successfully treated with surgery, craniospinal radiotherapy, and concurrent chemotherapy. This is the case of a 64-year-old male who presented with a 2-month history of bilateral lower extremity weakness and numbness associated with urinary and bowel incontinence. Work-up was done, and the spine's plain magnetic resonance imaging (MRI) revealed a heterogeneously enhancing intradural lesion with an extradural component at the right T9/T10 level, causing mild to moderate cord compression. The patient underwent laminectomy and gross total resection of the said tumor. Histology and immunohistochemistry were consistent with a primitive neuroectodermal tumor of the spine. The tumor recurred three months after the surgery, and the patient was then referred for radiation therapy with concurrent chemotherapy. Repeat spinal MRIs with three- to six-month intervals after treatment showed no tumor recurrence as of August 2021. Primary spinal PNETs are rarely found in adults, especially in the elderly. These tumors currently have no recommendations or guidelines regarding their management. Thus, most cases are presently being managed based on studies on children and central nervous system (CNS) PNETs. This paper presented a case of a successfully treated primary spinal PNET in the elderly. The management was primarily based on studies done on that of the pediatric population and CNS PNETs.

Round cell sarcoma of the kidney on a patient treated for acute promyelocytic leukemia: A case report and literature review

@#This is a report of a patient who was diagnosed with acute promyelocytic leukemia (APL) last 2007 and was given a standard chemotherapeutic regimen of anthracycline, all-trans-retinoic acid and methotrexate. The patient completed treatment and recovered. Twelve years after chemotherapy, the patient was diagnosed to have renal tumor. The patient presented with intermittent episodes of non-bothersome flank pain. He was managed as a case of renal newgrowth, left, stage II (cT2bN0M0), which was eventually found to have intermediate grade, round cell sarcoma not further classified of the kidney. Immunohistochemical studies and literature review point to a newly classified subtype of sarcoma or a primitive neuroectodermal tumor, both of which are rarely found presenting in the kidneys.

Evaluation of performance of various histological grading systems of soft tissue sarcomas and the prognosis (metastatic risk and survival rate).

Background: Histologic grade represents the most important prognostic factor for all soft tissue sarcomas and it is strongly associated with the advent of metastasis and patients survival. The main objective of this study is to test individual grading system with metastatic risk and patients survival rate (prognosis). Methods: Soft tissue sarcomas (250) were graded by FNCLCC, NCI & Mhyre Jensens grading system. Special stains & immunohistochemistry were employed whenever necessary. Results: FNCLCC system shows Grade 1 = 50 (20%), Grade 2 = 75 (30%) & Grade 3 = 125 (50%). NCI (Costa et al.) showed Grade 1 = 70 (28%) Grade 2 = 85 (34%) & Grade 3 = 95 (38%). Myhre Jensen showed Grade 1 = 84 (33%), Grade 98 (39%) & Grade 3 = 68 (27%). Undoubtedly, FNCLCC system is the best of all grading systems which is very well supported by statistical analysis in this study. Conclusions: FNCLCC grading system of soft tissue sarcomas is the best documented and tested system. This present study strongly recommends FNCLCC grading system of soft tissue sarcomas to be internationally accepted because the grading system has well defined criteria & so least possible chances of interobserver variability. The present study & few other previous studies highly recommend the mandatory use of FNCLCC grading system in histopathology report format.

Tumor neuroectodérmico primitivo do terceiro ventrículo associado a metástase leptomeníngea / Primitive Neuroectodermal Tumor of the Third Ventricle Associated with Leptomeningeal Metastases

Os tumores neuroectodérmicos primitivos (PNET) são tumores malignos, não diferenciados, raramente apresentados na idade adulta, principalmente os de localização supratentorial. Apresentamos neste artigo o caso de um paciente de 19 anos, que nos chegou transferido de outro hospital com o diagnóstico de hidrocefalia. A existência do PNET associado a carcinomatose leptomeníngea foi comprovada como causa da hidrocefalia.

Primitive neuroectodermal tumors (PNET) are malignant tumors exceptionally present in adulthood, especially those with supratentorial location. In this article, we present the case of a young man who was transfer fromanother institutionwith the diagnosis of hydrocephalus; during his stay, we corroborated the presence of supratentorial PNET associated to leptomeningeal carcinomatosis that was a cause of hydrocephalus.

Isolated Supratentorial Intraventricular Recurrence of Medulloblastoma

Medulloblastoma is a common pediatric tumor typically diagnosed before the age of fifteen. Initial therapy includes surgical resection and radiation of the entire neuro-axis. Recurrence is common and typically occurs within 2 years of initial diagnosis. Those fitting Collin's Law is considered tumor-free. We report a case of single supratentorial recurrence 13 years after initial diagnosis. Here we present a 22 year old male presenting 13 years after initial diagnosis with isolated septum pellucidum recurrence. He underwent complete resection of the tumor. Medulloblastoma is a common in the pediatric population. Late recurrence to the ventricular system is uncommon. Long term follow-up is recommended in these patients.

Giant malignant insulinoma

Insulinomas are the most common pancreatic neuroendocrine tumors. Most insulinomas are benign, small, intrapancreatic solid tumors and only large tumors have a tendency for malignancy. Most patients present with symptoms of hypoglycemia that are relieved with the administration of glucose. We herein present the case of a 75-year-old woman who presented with an acute hypoglycemic episode. Subsequent laboratory and radiological studies established the diagnosis of a 17-cm malignant insulinoma, with local invasion to the left kidney, lymph node metastasis, and hepatic metastases. Patient symptoms, diagnostic and imaging work-up and surgical management of both the primary and the metastatic disease are reviewed.

A case report on development of CNS primitive neuroectodermal tumour from astrocytoma.

CNS PNET are infrequent in children and very rare in adults. Even rarer is the development of PNET from low grade astrocytoma. We hereby report a case of PNET arising from astrocytoma. Some similar cases have been reported in the past in which radiation was considered to be the etiological factor in the development of PNET from glial neoplasms but in this case the duration of conversion does not go in favour of radiation as the cause.

Alveolar rhabdomyosarcoma in children with histomorphological review.

Rhabdomyosarcomas (RMS) are aggressive malignant neoplasm of mesenchymal origin, related to skeletal muscle lineage. These are the most common soft tissue tumors in children. The diagnosis is made by microscopic analysis and ancillary techniques like immunohistochemistry, electron microscopy, cytogenetics and molecular biology. We encountered a case of a 03 years old child who presented with a tender, reddish, soft swelling over cheek for three weeks. The FNAC was reported as a small round cell tumor, Probably Primitive Neuroectodermal Tumor (PNET). The biopsy of tumor revealed a small round cell tumor with an alveolar pattern. Tumor giant cells were absent and mitotic figures were infrequent. Hence, differentials of alveolar rhabdomyosarcoma and PNET were rendered. Immunohistochemistry (IHC) demonstrated desmin positivity. Thus, a final diagnosis of alveolar rhabdomyosarcoma was offered.

Primary Primitive Neuroectodermal Tumor of the Breast: a Case Report

Primary primitive neuroectodermal tumors (PNET) are rare malignant tumors, affecting mostly children and adolescents. Only three cases of primary breast PNETs have been reported in the medical literature, with none in Korea. We present a case of a primary PNET of the breast in a 33-year-old woman, with imaging and immunohistopathology findings.

Primitive Neuroectodermal Tumor of the Liver in a 13-year-old Boy: A Case Report / 대한소아소화기영양학회지

Primary primitive neuroectodermal tumor (PNET) of the liver is a rare disease with aggressive behavior and poor prognosis. We report a case of a PNET of the liver in a 13-year-old boy. The patient was admitted to the hospital with fever and abdominal pain. Abdominal CT and MRI revealed a 5.5 cm sized, septated, non-enhancing mass in the hepatic hilum. The patient was initially diagnosed with an inflammatory pseudotumor. Despite 9 days of antibiotic therapy, the patient's clinical symptoms did not improve. A liver biopsy was performed in the interest of formulating a differential diagnosis. This procedure revealed tumor cells positive for CD99 on immunohistochemistry. The patient was diagnosed with a PNET.

Primitive Neuroectodermal Tumor of the Liver in a 13-year-old Boy: A Case Report / 대한소아소화기영양학회지

Primary primitive neuroectodermal tumor (PNET) of the liver is a rare disease with aggressive behavior and poor prognosis. We report a case of a PNET of the liver in a 13-year-old boy. The patient was admitted to the hospital with fever and abdominal pain. Abdominal CT and MRI revealed a 5.5 cm sized, septated, non-enhancing mass in the hepatic hilum. The patient was initially diagnosed with an inflammatory pseudotumor. Despite 9 days of antibiotic therapy, the patient's clinical symptoms did not improve. A liver biopsy was performed in the interest of formulating a differential diagnosis. This procedure revealed tumor cells positive for CD99 on immunohistochemistry. The patient was diagnosed with a PNET.

Primitive Neuro Ectodermal Tumor (PNET) in infant diagnosed by histopathology and immunohistochemistry techniques: A Case Report.

Primitive Neuro Ectodermal Tumor (PNET) is rare and difficult to diagnose. A case of PNET was diagnosed based on histopathological and immunohistochemical findings. A 4-month-old infant was admitted to the hospital with a tumor on the midline of his chest wall since he was 3 days old. The tumor was fixed on the chest wall and had ill-defined margin, enlarged over time and reached more than 10 cm in diameter when he was brought to a clinician. Two small ulcers were seen on the skin overlying the tumor. It was diagnosed as soft tissue tumor suggestive of a hemangioma. The tumor was 17 x 13 x 5.5 cm in size, white colored and firm to the touch. Microscopic examination revealed malignant small round cells with round to ovoid nuclei, coarse chromatin and scanty cytoplasm. Most cells were arranged in a solid pattern with scattered Homer-Wright rosettes. The mitotic count was 7/10 HPF, and necrosis was minimal (less than 25%). On immunohistochemical examination, the cells showed weak to moderate immunoreactivity to Vimentin and CD99, but showed negative to weak positive reactivity to NSE and Chromogranin. Based on the clinical features, gross findings, histopathologic and immunohistochemical examinations, the case was diagnosed as a malignant small round cell tumor consistent with PNET / ES (Ewing’s Sarcoma). To confirm the diagnosis, cytogenetic examination is suggested.

Ewing's Sarcoma/PNET of the Talus: Report of a Case / 대한세포병리학회지

Ewing's sarcoma (ES)/PNET is common in both axial and appendicular skeletons, but is extremely rare in the talus. Here, we report a case of ES/PNET of the left talus in a 29-year-old male patient diagnosed by fine needle aspiration cytology, and review the literature on similar cases. The cytological smears were composed of individually dispersed small round cells and occasional clusters of loosely cohesive cells. The tumor cells were fragile, frequently exhibiting naked nuclei. Two distinct types of cells were observed. The light (chief) cells displayed round or slightly oval nuclei with frequent indentations, generally inconspicuous nucleoli, and a thin rim of cytoplasm, which sometimes harbored small vacuoles. The dark cells were smaller, displaying scanty cytoplasm with dense hyperchromatic nuclei, intermixed with chief cells, and often manifesting as small molded groups. However, no significant nuclear pleomorphisms or mitoses were noted. Tumor cells in the cell block revealed positive cytoplasmic glycogen, as determined by a PAS stain with diastase control, and also exhibited positive immunoreactivity for CD99.

Primitive Neuroectodermal Tumor of the Kidney / 대한비뇨기과학회지

Primitive neuroectodermal tumor (PNET), also referred to as extraskeletal Ewing's sarcoma, is a highly aggressive tumor, and it is seen typically in the soft tissues of the chest wall and paraspinal region but rarely arises in the kidney. A 27-year-old woman was presented with a left renal mass and underwent a radical nephrectomy. The pathological diagnosis was a primitive neuroectodermal tumor. Microscopically, there were sheets and lobules of cells with round to oval nuclei and coarse chromatin, as well as a Homer Wright rosette formation. Immunohistochemical analysis showed strongly positive staining of CD99 and negative staining of other neuroendocrine markers, such as synaptophysin and chromogranin.

Primary peripheral primitive neuroectodermal tumor of the ovary with an epithelial cyst : A case report and review of literature.

Peripheral primitive neuroectodermal tumor (pPNET) has some histologic resemblance to a classic central primitive neuroectodermal tumor (cPNET), however it is distinctively different from cPNET by its CD99 immunoreactivity, characteristic chromosomal translocation, t(11;22)(q24:q12) and EWS/FLI-1 chimeric mRNA. Peripheral PNETs have a predilection for soft tissues rather than for viscera. Only 15 cases of primary ovarian PNET have been reported, and only one case was proven to be pPNET. Ovarian PNET is an aggressive tumor. We report a case of a 40-year-old Thai woman with a Stage IIIb right ovarian PNET is an aggressive tumor. Despite debulking operation and vigorous adjuvant chemotherapy, the patient died of disease 6 months later. Grossly, the tumor was solid and cystic. Microscopically, the former displayed unique features mimicking cPNET, but the pPNET phenotype was validated by CD99 staining. The solid portion also contained mucin-producing gland-like structures, previously described as ependymal diffentiation. In the cystic portion, the histology demonstrated epithelial linning tissue resembling cystadenoma of borderline malignancy of the ovary. It is generally accepted that both cPNET and pPNETs can have polyphenotypic differentiation. PNETs can be originated from either totipotential germ cells, neural crest remnant or mullerian-derived cells.

A Case of Primitive Neuroectodermal Tumor of the Uterus / 대한산부인과학회잡지

Primitive neuroectodermal tumor (PNET) is a rare tumor derived from fetal neuroectodermal cells. These tumors occur in the central nervous system and peripheral locations, but the occurrence of primitive neuroectodermal tumors located in the uterus is extremely rare. We present a 53-year-old woman with primitive neuroectodermal tumor of the uterus confirmed by immunohistochemical staining. We have experienced one case of PNET of the uterus and wish to report with brief review of concerned literature.

Primitive Neuroectodermal Tumor of the Kidney with CD99 Positive Staining / 대한비뇨기과학회지

Primitive neuroectodermal tumors (PNETs) are primitive neuroblastic tumors that, unlike neuroblastomas, arise outside the autonomic nervous system, but very few cases with a renal origin have been described. The immunohistochemical staining for CD99 is useful in their diagnosis. A case of renal primitive neuroectodermal tumor, in a 61- year-old man presenting with a painless gross hematuria is reported. The immunohistochemical staining of the renal mass was strongly positive for CD99.

High Dose Chemotherapy with Autologous Peripheral Blood Stem Cell Transplantation in Patients with Medulloblastoma/Primitive Neuroectodermal Tumor / 대한소아혈액종양학회지

PURPOSE: To improve survival and/or to avoid radiotherapy, high dose chemotherapy (HDCT) with autologous peripheral blood stem cell transplantation (PBSCT) was given to patients with recurrent or high risk medulloblastoma (MB)/primitive neuroectodermal tumor (PNET) as well as patients younger than 3 years of age. METHODS: Six patients (3 recurrent, 1 high risk, 2 younger than 3 years; 5 MBs and 1 PNET) received single or double HDCT and PBSCT with or without immunotherapy using interleukin-2. Chemotherapeutic regimen in the first HDCT included cyclophosphamide (1,500 mg/m2/ day for 4 days) and melphalan (60 mg/m2/day for 3 days). Chemotherapeutic regimen in the second HDCT included carboplatin (400 mg/m2/day for 3 days), thiotepa (250 mg/ m2/day for 3 days), and etoposide (200 mg/m2/day for 3 days). RESULTS: Nine HDCTs were applied in 6 patients. Three double HDCTs were rescued with peripheral blood stem cells collected during single round leukapheresis. Rapid hematologic recovery occurred in 4 patients. Engraft failure occurred in 1 patient and delayed granulocyte recovery and platelet engraft failure occurred in 1 patient. Three patients who had minimal disease before HDCT had event free survival for 7~18 months after HDCT. Tumor relapsed 8 and 12 months after single HDCT in 2 patients among 3 patients with recurrent MB/PNET. One patient with recurrent MB died due to engraft failure and sepsis. CONCLUSION: HDCT with autologous PBSCT is expected to improve survival of patients with poor prognosis MB/PNET including younger patients less than 3 years. Subsequent trials with larger number of patients and long-term follow-up are needed.

Trilateral Retinoblastoma

Trilateral retinoblastoma is a syndrome involving midline intracranial malignancies in children with the heritable form of retinoblastoma. It is rare and usually lethal in spite of aggressive treatments. We report a case of trilateral retinoblastoma with review of the literature to gain further insight into this uncommon disease.

Primitive Neroectodermal Tumor in the Cauda Equina Region

We report a malignant peripheral primitive neuroectodermal tumor (PNET) that originated in the cauda equina in a young adult, After gross total excision of the tumor, radiotheraphy was followed. The tumor had the typical appearance of a PNET with small, round cell. It stained positively for the neuron-specific enolase(NSE) conforming its neural origin and negatively for cytokeratin, glial fibrillary acidic protein(GFAP ) and S-100 protein. Three months later, MRI showed almost total obstruction at the level o f the L/L interspace caused by a mass occupying the spinal canal and extending into the L/S foramen on the right. Sixteen months after commencement of radiotherapy, severe left sciatica developed and MRI showed involvement of the both ilii and right femur with a large soft tissue mass extending into the pelvis. Palliative radiotherapy was administered but the patient died four months later.