Congenital Supratentorial and Infratentorial Peripheral Neurogenic Tumor: A Clinical, Ultrastructural, and Immunohistochemical Study

Neurosurgery ◽  
1986 ◽  
Vol 19 (3) ◽  
pp. 426-433 ◽  
Author(s):  
David S. Moss ◽  
Stephen J. Haines ◽  
Arnold S. Leonard ◽  
Louis P. Dehner

Abstract Congenital neoplasms of the central nervous system are extremely rare, although they have been well documented since the earliest reports from the mid-19th century. Medulloblastoma, other primitive neuroectodermal tumors, and various types of gliomas have comprised the majority of cases. This report describes a highly unusual infratentorial and supratentorial tumor presenting as a scalp mass in a neonate who experienced in utero distress. The supratentorial mass extended through a defect in the skull to the parietooccipital lobe, and the infratentorial aspect involved the 9th and 10th cranial nerves in the region of the cerebellopontine angle. A complex spindle cell neoplasm incorporating peripheral nerve sheath and vascular characteristics was further characterized by electron microscopy and immunohistochemistry. Based upon these studies, the tumor was interpreted as a congenital schwannoma with divergent vascular differentiation. The child has been tumor-free for approximately 2 years after the initial operative procedure.

2010 ◽  
Vol 134 (3) ◽  
pp. 481-485 ◽  
Author(s):  
Lobna Ayadi ◽  
Abdelmajid Khabir

Abstract Angiosarcomas are rare tumors that predominantly affect adults and elderly patients. Although angiosarcomas are well described in a variety of clinical settings, they have been poorly studied in children and little is known about their biology, natural history, or optimal treatment. Childhood angiosarcomas are exceedingly rare. The head and neck region and mediastinum seem to be the preferred locations. Most tumors are high-grade tumors. Vasoformative architecture is not always obvious on light microscopy requiring the benefit of immunohistochemical study. The differential diagnosis includes Kaposi sarcoma, epithelioid hemangioendothelioma, hemangiopericytoma, and spindle cell hemangioendothelioma whose prognosis is different. Complete resection is required for patients with localized tumors. Malignant vascular tumors are rare in children in the first 2 decades of life and when they do occur they seem to be more aggressive than in adults. Pathologic diagnosis is difficult particularly in poorly differentiated angiosarcomas requiring immunohistochemical study to confirm vascular differentiation.


1990 ◽  
Vol 73 (6) ◽  
pp. 946-950 ◽  
Author(s):  
Catriona A. McLean ◽  
John D. Laidlaw ◽  
David S. B. Brownbill ◽  
Michael F. Gonzales

✓ A 75-year-old man presented with a right cerebellopontine angle tumor 11 months after complete macroscopic resection of a right acoustic neurilemoma. Histological examination of the recurrent tumor showed a malignant spindle-cell neoplasm with positive staining for S-100 protein. The patient had no stigmata of von Recklinghausen's disease. It is proposed that this recurrence represents progression from a benign to a malignant acoustic nerve-sheath tumor, an event that is extremely rare outside the clinicopathological context of neurofibromatosis.


2002 ◽  
Vol 12 (6) ◽  
pp. 1-2 ◽  
Author(s):  
David John Yeh ◽  
Richard B. Hessler ◽  
Mark R. Lee

Primary leiomyosarcoma of the central nervous system is rare and has been described both de novo and following temporally remote radiotherapy for a different unrelated malignancy. The authors report the case of a 42-year-old man in whom 60Co radiation treatment had been performed for an unknown primary mass in the brainstem 25 years previously. He presented with progressive neurological deterioration after undergoing many years of conservative therapy. A stereotactic biopsy sampling procedure was performed, and examination of the left cerebral pedunculopontine lesion revealed a spindle cell neoplasm. Histopathological and immunohistochemical examination of the tumor obtained from definitive resection suggested leiomyosarcomatous transformation of ganglioglioma.


2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii446-iii446
Author(s):  
Lacey Carter ◽  
Naina Gross ◽  
Rene McNall-Knapp ◽  
,and Jo Elle Peterson

Abstract At one month of age, a female presented with a giant congenital nevus along lower back and thighs and hydrocephalus. A ventriculoperitoneal shunt was placed. An MRI was done at six months, initially reported as normal. At eleven months of age, five months after original MRI, patient presented with dysconjugate gaze and lethargy. MRI showed new 3.8 x 3.7 x 3.4 cm right cerebellopontine angle mass extending into Meckel’s cave and foramen ovale along with leptomeningeal disease extending from the mass along the entire length of the spinal cord. Retrospective review of prior MRI revealed subtle leptomeningeal enhancement concerning for neurocutaneous melanosis (NCM). Given the leptomeningeal disease, family elected for open biopsy and debulking of lesion instead of aggressive resection. Histologically, the mass showed hypercellular spindle cell neoplasm with mitotic activity and necrosis mixed with remnants of normal cranial nerve. GFAP was negative, excluding a glioma. HMB-45, MITF, panmelanoma, and Melan-A were negative, excluding melanoma. A negative myogenin stain ruled out ectomesenchymoma. S-100 protein and SOX-10 positivity with variable loss of staining for trimethylation of histone H3 K27 were indicative of malignant peripheral nerve sheath tumor (MPNST). Given the course of the mass, trigeminal nerve MPNST was presumed. Given the poor prognosis of intracranial MPNST and NCM, family elected to forgo treatment and was discharged with hospice. She died 25 days after surgery. Cranial nerve MPNST is rare. MPNST in patients with NCM has not previously been reported to our knowledge.


2020 ◽  
Vol 8 ◽  
pp. 2050313X2098117
Author(s):  
Esra Nsour ◽  
Ali Al Khader ◽  
Bushra Al-Tarawneh

Small cell carcinoma is a malignant neuroendocrine tumor with aggressive clinical behavior. Histologically, the tumor is characterized by the proliferation of small, round, blue cells. Here, we present the case of a 50-year-old man with a 1-month history of enlarging chest wall mass. Microscopic examination of the lesion revealed a highly cellular neoplasm composed of closely packed, atypical spindle cells with scant cytoplasm, inconspicuous nucleoli, and brisk mitotic activity. The hemangiopericytoma-like vascular pattern was prominent. Areas showing a fibrosarcoma-like fascicular pattern were also observed. The tumor was immunohistochemically positive for TTF1, synaptophysin, and chromogranin, confirming small cell carcinoma. Further investigations revealed a lung origin and widespread metastases. The tumor in this case closely mimicked synovial sarcoma or malignant peripheral nerve sheath tumor. Small cell carcinoma demonstrates a hemangiopericytoma-like pattern that can mimic sarcoma histologically. This is a serious pitfall that can significantly affect the speed of diagnosis and management.


2010 ◽  
Vol 120 (S3) ◽  
pp. S119-S119
Author(s):  
Joshua M Levy ◽  
Christian P Hasney ◽  
Paul L Friedlander ◽  
Michael S Ellis ◽  
Mary A Fazekas-May

PEDIATRICS ◽  
1958 ◽  
Vol 21 (1) ◽  
pp. 94-105
Author(s):  
F. H. Top

Evidence is presented from data covering the period 1940 to 1952 which corroborates the conclusion of previous studies that prior tonsillectomy probably adversely affects the occurrence of brainstem paralysis (bulbar and bulbospinal) in poliomyelitis. Neither this study nor any preceding studies relating to this problem have proved the contention. On the basis that the hypothesis is correct, an attempt is made to find an answer by studying the incidence of the common paralysis of cranial nerves (VII, IX and X and XI) in bulbar and bulbospinal cases of poliomyelitis on the basis of presence or absence of tonsils. Rates of incidence of paralysis of cranial nerves, not adjusted for age, indicate a decidedly higher proportion of paralysis of the facial nerve (VII) among nontonsillectomized patients whereas tonsillectomized persons are preportionately more affected by palatal and pharyngeal paralysis (nerves IX and X). Paralysis of the facial nerve appears from two studies to occur more commonly at earlier ages, particularly in the age group 0 to 4 years. However, age adjustment did not erase, although it did somewhat lower, the TR/TP ratio. This finding lends credence to a real difference but can only be applied to this study, as Paffenbarger in a smaller study found no significant difference in frequencies of paralysis of the facial nerve between groups with tonsils removed and tonsils present, and Southcott, also in a small study, found paralysis of the facial nerve more common among tonsillectomized patients with bulbar (includes bulbospinal) involvement. The differences noted for palatal and pharyngeal paralyses (nerves IX and X) in the unadjusted rates as between tonsillectomized and nontonsillectomized patients remain statistically different and in some instances significant when corrections for age are made. The results of this study are suggestive but give no entirely satisfactory explanation for the differences noted. Various explanations previously offered are cited and briefly discussed. Perhaps more definitive studies in animals along the approach suggested by Southcott will prove more fruitful, namely, labelling virus by some radioactive element in order to trace the route it takes to the central nervous system.


1961 ◽  
Vol 38 (2) ◽  
pp. 315-322
Author(s):  
J. E. TREHERNE

1. The influx of sodium and potassium ions into the central nervous system of Periplaneta americana has been studied by measuring the increase in radioactivity within the abdominal nerve cord following the injection of 24NA and 42K. into the haemolymph. 2. The calculated influx of sodium ions was approximately 320 mM./l. of nerve cord water/hr. and of potassium ions was 312 mM./l. of nerve cord water/hr. These values are very approximately equivalent to an influx per unit area of nerve cord surface of 13.9 x 10-2 M cm. -2 sec.-1 for sodium and 13.5 x 10-12 M cm. -2 sec.-1 for potassium ions. 3. The relatively rapid influxes of these ions are discussed in relation to the postulated function of the nerve sheath as a diffusion barrier. It is suggested that a dynamic steady state rather than a static impermeability must exist across the sheath surrounding the central nervous system in this insect.


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