Melanotic Schwannoma, a Deceptive Misnomer for a Tumor With Relative Aggressive Behavior: A Series of 7 Cranial and Spinal Cases

2020 ◽  
Vol 28 (8) ◽  
pp. 850-858
Author(s):  
Ayushi Sahay ◽  
Sridhar Epari ◽  
Priyanka Gupta ◽  
JayantSastri Goda ◽  
Prakash Shetty ◽  
...  
Keyword(s):  
Mitotic Activity ◽  
S100 Protein ◽  
Residual Tumor ◽  
Biological Behavior ◽  
Ki 67 ◽  
Histological Features ◽  
P16 Protein ◽  
Initial Surgery ◽  
Age Range ◽  
Post Therapy

The authors present in this article a series of 7 intracranial/spinal cases of melanotic schwannomas that highlight the aggressive nature of these tumors. The series comprises 2 males and 5 females, age range 19 to 50 years, with spinal/paraspinal location in 4/7 (57%), and intracranial (along the trigeminal nerve) location in 3/7 (43%). There was no association with Carney’s complex. All the cases showed similar histology of epithelioid to spindled cytomorphology with vesicular nuclei (including prominent nucleoli) and conspicuous intracytoplasmic melanin pigment. Mitotic activity was seen in 3/7 cases (43%), 2 of which showed atypical forms. Immunohistochemically, all the cases were positive for S100 protein, HMB-45, melan-A and p16 protein; while negative for PDL1. Ki-67 labeling index was >5% in cases with mitotic activity. Two cases were asymptomatic (after 2.5 and 5 years postsurgery), 2 cases (one was mitotically active, while the other had no mitosis) had recurrence 6 months and 3.5 years after initial surgery, respectively, probably suggesting that mitosis alone may not be a robust predictor of biological behavior. These patients were treated with various adjuvant modalities and were alive for 4 years and 3 years of post-therapy period, respectively. Three patients were offered adjuvant radiotherapy, based on presence of aggressive histological features or significant residual tumor. One showed good clinical response. This series highlights the variability of clinical behavior of these neoplasms belying a deceptively bland nomenclature and also highlights the lack of correlation between histological features and biological behavior.

Transition from meningeal melanocytoma to primary cerebral melanoma

2004 ◽  
Vol 101 (3) ◽  
pp. 528-531 ◽  
Author(s):  
Florian Roser ◽  
Makoto Nakamura ◽  
Almuth Brandis ◽  
Volkmar Hans ◽  
Peter Vorkapic ◽  
...  
Keyword(s):  
Malignant Melanoma ◽  
Mitotic Activity ◽  
S100 Protein ◽  
Tumor Resection ◽  
Clinical Signs ◽  
Biological Behavior ◽  
Subtotal Resection ◽  
Ki 67 ◽  
Content Type ◽  
First Case

✓ The authors describe the first case of an intracranial transition of a melanocytoma into a primary malignant melanoma within a short time. A 37-year-old woman presented with progressive brainstem syndrome due to a tumor, originally diagnosed and treated 12 years earlier, that extended from the petroclival area to the anterior craniocervical junction. The histological workup following subtotal tumor resection of the initial tumor had revealed the typical features of a fibrous melanocytic meningioma without increased proliferation. Ten years after the patient had completed treatment for the melanocytic meningioma, control neuroimaging demonstrated growth of the residual tumor with compression of the brainstem. Another neurosurgical intervention revealed a dark tumor of hard consistency. At this time immunohistochemical examinations demonstrated melanocytic features (expression of vimentin, S100 protein, and melan A) of the lesion with focally increased proliferation (5% of Ki-67—positive cells) but no higher mitotic activity. Clinical signs of deterioration along with imaging-confirmed tumor progression precipitated another operation within 7 months. A neuropathological examination revealed epithelial and anaplastic changes and indicated that the MIB-1 indices were greater than 25%. Pleomorphic changes and a focal high mitotic activity led to the diagnosis of a primary cerebral malignant melanoma. The patient's later clinical course consisted of a rapid diffuse meningeal spread of the lesion throughout the entire brain and spine. Despite whole-brain and stereotactic radiation therapy as well as chemotherapy, the patient died 4 months after the last neuropathological diagnosis. Although grossly resembling a meningioma, melanocytomas lack the former's histological and immunohistochemical features. The biological behavior of a melanocytoma is variable and recurrence may happen after subtotal resection, but intracranial transition into a malignant melanoma has not been observed previously.

scholarly journals Virškinimo trakto stromos navikų diagnostika Valstybiniame patologijos centre 2003–2004 metais

2006 ◽  
Vol 4 (2) ◽  
pp. 0-0
Author(s):  
Mindaugas Plečkaitis ◽  
Ugnius Mickys ◽  
Darius Dasevičius
Keyword(s):  
High Risk ◽  
Gastrointestinal Stromal Tumors ◽  
Proliferative Activity ◽  
Biological Behavior ◽  
Cell Type ◽  
Ki 67 ◽  
Histological Features ◽  
Stromal Tumors ◽  
Cd 117 ◽  
The Mean

Mindaugas Plečkaitis1, Ugnius Mickys2, Darius Dasevičius21 Vilniaus greitosios pagalbos universitetinė ligoninė,Šiltnamių g. 29 LT-04130, Vilnius2 Valstybinis patologijos centras,Baublio g. 5, LT-08406 VilniusEl. paštas: [email protected]; [email protected] Tikslas Išanalizuoti GIST atvejus, diagnozuotus Valstybiniame patologijos centre 2003–2004 m., apibendrinti histologinius šių navikų požymius, įvertinti histologinių požymių sąsajas su piktybinės biologinės elgsenos rizikos grupėmis. Medžiaga ir metodai Įvertinti pacientų amžių, pasiskirstymą pagal lytį, navikų lokalizaciją, daugybiškumą, metastazes, recidyvus. Papildomai įvertinti histologinius naviko požymius: histologinį piešinį (mišrus, šeivinis, epitelioidinis), organoidinių struktūrų formavimą, ritmines naviko ląstelių branduolių lygiavimosi (palisading) struktūras, ląstelių perinuklearinę vakuolizaciją, naviko miksoidinę stromą, koaguliacinę nekrozę, įsiskverbimą į savąjį gleivinės dangalą (lamina propria), dydį, mitozių kiekį, imunoprofilį. Remiantis navikų dydžiu ir mitozių kiekiu 50-yje didelio padidinimo regos laukų (pagal C.D.M. Fletcher), GIST suskirstyti į piktybinės biologinės elgsenos rizikos grupes. Rezultatai Ištirta 50 GIST atvejų. Ligonių amžiaus vidurkis 64,38 ± 14,26 metų, mediana – 68 metai. Moterų buvo 36, vyrų – 14. Nustatytos 7 (14%) GIST metastazės. Dauginių GIST diagnozuoti 4 atvejai (8%). Šių navikų recidyvų per tirtą laikotarpį nebuvo. Maždaug pusė pavienių navikų rasta skrandyje (45,7%), rečiau – plonojoje žarnoje (15,2%), rečiausiai – storojoje žarnoje (4,3%). Mišrių ir šeivinių GIST kiekis beveik nesiskyrė (atitinkamai 46,2% ir 43,6%), rečiau pasitaikė epitelioidinių navikų (10,3%). GIST dydis svyravo nuo 0,5 cm iki 25 cm (vidurkis 6,4 ± 5,3 cm; mediana 4,9 cm). Dauguma mūsų imunotipuotų GIST buvo teigiami CD 117 ir CD34 žymenims, o pusė navikų – teigiami a-lygiųjų raumenų aktinui. Didesnė dalis į rizikos grupes mūsų suskirstytų pavienių navikų (41,7%) buvo didelės piktybinės biologinės elgsenos rizikos. Koaguliacinė nekrozė buvo 86,7%, o naviko plitimo į savąjį gleivinės dangalą požymiai – 60% didelės piktybiškumo rizikos pavienių GIST. Ki-67 proliferacinis aktyvumas >10% naviko ląstelių populiacijos buvo būdingas didelės rizikos GIST, o Ki-67 proliferacinis aktyvumas <5% nekoreliavo su GIST piktybinės biologinės elgsenos rizikos grupėmis.. Išvada Tyrimo rezultatai parodė, kad naviko koaguliacinės nekrozės, plitimas į savąjį gleivinės dangalą, Ki-67 proliferacinis aktyvumas >10% naviko ląstelių populiacijos gali būti papildomi histologiniai kriterijai tikslesnei GIST prognozei nustatyti, nes yra būdingesni didelės rizikos GIST. Reikšminiai žodžiai: gastrointestininės stromos navikai, CD117, piktybinės biologinės elgsenos rizika Diagnostics of gastrointestinal stromal tumors in the National Centre of Pathology 2003–2004 Mindaugas Plečkaitis1, Ugnius Mickys2, Darius Dasevičius21 Vilnius University Emergency Hospital,Baublio str. 5, LT-08406 Vilnius, Lithuania2 National Center of Pathology,Akademijos str. 4, LT-08663 Vilnius, LithuaniaE-mails: [email protected], [email protected] Objective To review GIST cases diagnosed in 2003–2004 at the Lithuanian State Centre of Pathology, to evaluate the histological findings and to correlate histological features with the risk of malignant biological behavior. Materials and methods To evaluate the patiens’ age and sex distribution, localization, ]metastases, GIST relapses and multiple tumors. Additionally, to estimate the histological features: histological pattern (spindle, epithelioid or mixed cell type), nesting, palisading, perinuclear vacuolization of tumor cells, myxoid stroma, foci of coagulative necrosis, mucosal invasion, size, mitotic rate, immunophenotype and risk of malignant biological behavior (according to C.D.M. Fletcher) of GIST. Results Fifty GIST cases were analysed. The mean age of 36 females and 14 males was 64.38 ± 14.26 years, mediana 68 years. There were found 7 metastases (14%), 4 multiple GISTs (8%) and no one relapse case in our research. 45.7% of GISTs were localised in the stomatch, 15.2% in small intestine and 4.3% in large intestine. 46.2% of GISTs were of mixed type, 43.6% of spindle cell type and 10.3% of epithelioid type. The mean size of GISTs was 6.4 ± 5.3 cm, mediana 4.9 cm. Most of GISTs were positive to CD117 and to CD34, and one half of the tumors to a-actin. 41.7% of solitary GISTs showed a high risk of malignant biological behavior. Foci of coagulative necroses were found in 86.7% and mucosal invasion in 60% of solitary GISTs with a high risk of malignant biological behavior. Ki-67 proliferative activity >10% was characteristic of GISTs with a high risk of malignant biological behavior, whereas Ki-67 proliferative activity <5% did not correlate with the risk groups of malignant biological behavior. Conclusion The results of the research have shown that invasion of the mucosa, necrotic foci, Ki-67 proliferative activity more than 10% are characteristic of GISTs with a high risk of malignant biological behavior and should be used as additional predictors for the malignant potential of these tumors. Key words: gastrointestinal stromal tumors, CD 117, risk of malignant biological behavior

scholarly journals Improved risk-stratification for posterior fossa ependymoma of childhood considering clinical, histological and genetic features – a retrospective analysis of the HIT ependymoma trial cohort

2019 ◽  
Vol 7 (1) ◽  
Author(s):  
Stephanie T. Jünger ◽  
Martin Mynarek ◽  
Inken Wohlers ◽  
Evelyn Dörner ◽  
Anja zur Mühlen ◽  
...  
Keyword(s):  
Risk Stratification ◽  
Mitotic Activity ◽  
Posterior Fossa ◽  
Cox Regression ◽  
Residual Tumor ◽  
Histological Features ◽  
Chromosome 1Q ◽  
Risk Stratification Model ◽  
Vascular Proliferation ◽  
1Q Gain

Abstract Introduction Risk stratification of children with ependymomas of the posterior fossa in current therapeutic protocols is mainly based on clinical criteria. We aimed to identify independent outcome predictors for this disease entity by a systematic integrated analysis of clinical, histological and genetic information in a defined cohort of patients treated according to the German HIT protocols. Methods Tumor samples of 134 patients aged 0.2–15.9 years treated between 1999 and 2010 according to HIT protocols were analyzed for histological features including mitotic activity, necrosis and vascular proliferation and genomic alterations by SNP and molecular inversion probe analysis. Survival analysis was performed by Kaplan-Meier method with log rank test and multivariate Cox regression analysis. Results Residual tumor after surgery, chromosome 1q gain and structural genomic alterations were identified as predictors of significantly shorter event-free (EFS) and overall survival (OS). Furthermore, specific histological features including vascular proliferation, necrosis and high mitotic activity were predictive for shorter OS. Multivariate Cox regression revealed residual tumor, chromosome 1q gain and mitotic activity as independent predictors of both EFS and OS. Using these independent predictors of outcome, we were able to build a 3-tiered risk stratification model that separates patients with standard, intermediate and high risk, and which outperforms current stratification procedures. Conclusion The integration of defined clinical, histological and genetic parameters led to an improved risk-stratification model for posterior fossa ependymoma of childhood. After validation in independent cohorts this model may provide the basis for risk-adapted treatment of children with ependymomas of the posterior fossa.

scholarly journals TMOD-17. ONCOLYTIC POLIOVIRUS AS A PROBE FOR MECHANISMS OF IMMUNE RESISTANCE IN GLIOBLASTOMA

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii231-ii231
Author(s):  
Yuanfan Yang ◽  
Michael Brown ◽  
Matthias Gromeier ◽  
Darell Bigner
Keyword(s):  
Immune Activation ◽  
Residual Tumor ◽  
Resistance Mechanisms ◽  
Therapeutic Interventions ◽  
Preclinical Model ◽  
Tumor Diameter ◽  
Intrinsic Resistance ◽  
Ki 67 ◽  
Post Therapy

Abstract Glioblastoma is an immunologically cold tumor that employs multiple resistance mechanisms to escape activation of both innate and adaptive immunity. Transient immune activation and responses induced by immunotherapies in glioblastoma are almost always suppressed by potent intrinsic and extrinsic resistance mechanisms, limiting therapeutic efficacy. PVSRIPO is a recombinant poliovirus/rhinovirus chimera that elicits robust innate immune activation through infecting and lysing glioblastoma cells expressing the human poliovirus receptor (CD155), and through sublethal infection of tumor associated macrophages, setting the stage for lingering viral replication. We have constructed a pathological model using murine CT2ACD155 intracranial tumors stereotactically infused with a single dose of PVSRIPO (5×107 pfu) or mock control to study the response of glioblastoma in an immune competent in vivo tumor microenvironment. Tumor bearing brains were harvested before therapy and on days 2, 4, 6, 9, 12 post therapy for histology. On day 4 post therapy, histopathology showed &gt; 90% shrinkage of intracranial tumor diameter (n= 6/6). Residual tumor is surrounded by a necrotic rim on histology with profound infiltration of F4/80+ macrophages and substantially reduced expression of Ki-67 on IHC compared to controls. Other markers including CD3, CD8 and Cleaved-Caspase 3 showed no difference in tumors of both groups. Interestingly, most residual tumor progressed on day 6 post therapy and caught up with the size of control tumor by day 9. Long-term remission of tumor occurred in only 10% of mice. This model is valuable in studying the mechanisms of intrinsic resistance and the timeline of resistance to develop in order to obtain more durable immunotherapy responses in glioblastoma. Future steps are to track inflammation induced by viral infection by RNAseq, and to find potential therapeutic interventions (including blocking PD1:PD-L1 axis) to mitigate therapy resistance in this preclinical model.

Association between expression of Ki-67, parafibromin, p27, and Rb protein and the biological behavior in parathyroid tumors

2013 ◽  
Author(s):  
Keiko Ohkuwa ◽  
Chie Masaki ◽  
Junko Akaishi ◽  
Akifumi Suzuki ◽  
Takashi Uruno ◽  
...  
Keyword(s):  
Biological Behavior ◽  
Ki 67 ◽  
Rb Protein ◽  
Parathyroid Tumors

scholarly journals Changes in Cellular Regulatory Factors before and after Decompression of Odontogenic Keratocysts

2020 ◽  
Vol 10 (1) ◽  
pp. 30
Author(s):  
Slmaro Park ◽  
Han-Sung Jung ◽  
Young-Soo Jung ◽  
Woong Nam ◽  
Jung Yul Cha ◽  
...  
Keyword(s):  
Recurrence Rate ◽  
Biological Behavior ◽  
Nuclear Antigen ◽  
Epithelial Cyst ◽  
Ki 67 ◽  
Proliferation Markers ◽  
Odontogenic Keratocysts ◽  
Wide Range ◽  
Before And After ◽  
Cyst Lining

Decompression followed by enucleation, which is one of the treatments used for odontogenic keratocysts (OKCs), is frequently used in OKC lesions of large sizes. This method offers the advantage of minimizing the possibility of sensory impairment without creating a wide-range bone defect; moreover, the recurrence rate can be significantly lower than following simple enucleation. This study aimed to assess the changes in histology and expression of proliferation markers in OKCs before and after decompression treatment. A total of 38 OKC tissue samples from 19 patients who had undergone decompression therapy were examined morphologically and immunohistochemically to observe changes in proliferative activity before and after decompression. The markers used for immunohistochemistry (IHC) staining were Bcl-2, epidermal growth factor receptor (EGFR), Ki-67, P53, PCNA, and SMO. The immunohistochemistry positivity of the 6 markers was scored by using software ImageJ, version 1.49, by quantifying the intensity and internal density of IHC-stained epithelium. The values of Bcl-2, Ki-67, P53, proliferating cell nuclear antigen (PCNA), and SMO in OKCs before and after decompression showed no significant change. No correlation between clinical shrinkage and morphologic changes or expression of proliferation and growth markers could be found. There was no statistical evidence that decompression treatment reduces potentially aggressive behavior of OKC within the epithelial cyst lining itself. This might indicate that decompression does not change the biological behavior of the epithelial cyst lining or the recurrence rate.

scholarly journals Ovarian sex cord-stromal tumors: an update on clinical features, molecular changes, and management

2021 ◽  
pp. ijgc-2020-002018
Author(s):  
Rehab Al Harbi ◽  
Iain A McNeish ◽  
Mona El-Bahrawy
Keyword(s):  
Hormonal Therapy ◽  
Clinical Presentation ◽  
Therapeutic Modality ◽  
Biological Behavior ◽  
Molecular Changes ◽  
Histological Features ◽  
Stromal Tumors ◽  
Tumor Subtypes ◽  
Tumor Types ◽  
Sex Cord Stromal Tumors

Sex cord stromal-tumors are rare tumors of the ovary that include numerous tumor subtypes of variable histological features and biological behavior. Surgery is the main therapeutic modality for the management of these tumors, while chemotherapy and hormonal therapy may be used in some patients with progressive and recurrent tumors. Several studies investigated molecular changes in the different tumor types. Understanding molecular changes underlying the development and progression of sex cord-stromal tumors provides valuable information for diagnostic and prognostic biomarkers and potential therapeutic targets for these tumors. In this review, we provide an update on the clinical presentation, molecular changes, and management of sex cord-stromal tumors.

Meningeal melanocytoma

1998 ◽  
Vol 88 (1) ◽  
pp. 116-121 ◽  
Author(s):  
David B. Clarke ◽  
Richard Leblanc ◽  
Gilles Bertrand ◽  
Gilbert R. C. Quartey ◽  
G. Jackson Snipes
Keyword(s):  
Histological Examination ◽  
Posterior Fossa ◽  
Clinical Presentation ◽  
Residual Tumor ◽  
Posterior Fossa Decompression ◽  
Content Type ◽  
Meningeal Melanocytoma ◽  
Radiological Appearance ◽  
Histological Features ◽  
Fine Print

✓ Meningeal melanocytomas are rare tumors of the central nervous system that are found almost exclusively in the posterior fossa and spinal cord and whose natural history is poorly defined. In this report, the authors review the clinical presentation, radiological appearance, operative findings, and histological features in two cases of meningeal melanocytoma: one cranial and one spinal. Two women, aged 21 and 30 years, were admitted to the hospital 60 years apart: the first because of progressive paraplegia and the second because of slowly progressive hearing loss. The first patient had an extradural tumor that was treated by laminectomy, subtotal resection, and postoperative radiotherapy in 1936. Her symptoms recurred 16 years later and she underwent reoperation of the residual tumor, which was found to have an intradural component. The authors' patient, who presented 60 years later, underwent plain and enhanced computerized tomography and magnetic resonance imaging that demonstrated a large posterior fossa lesion indicative of either an acoustic neuroma or a meningioma. She underwent posterior fossa decompression but only partial excision of the tumor could be accomplished because vigorous bleeding limited the extent of the resection. Surgery was followed by radiotherapy. The residual tumor enlarged despite these measures and required repeated resection 6 months later. At the second operation the tumor was much less vascular, perhaps reflecting the effects of radiotherapy, and was removed almost entirely. The patient died 6 months later from an anticoagulant-related cerebellar hemorrhage. In both cases the lesions were jet black, and histological examination revealed melanin-containing hypercellular tumors with rare mitotic figures. Meningeal melanocytomas are being diagnosed with increased frequency in parallel with improvements in neuroimaging and clarification of histological features. Clinical presentation of patients with these tumors typically occurs in their fifth decade and women are affected twice as often as men. The posterior fossa lesions can mimic acoustic neuromas and meningiomas in location and radiological appearance; however, the internal auditory canal is normal. In the spine, meningeal melanocytomas present with the clinical features of myeloradiculopathy. Diagnosis is made intraoperatively from the gross, jet-black appearance of the tumor and from histological examination. Vascularity, size, and location may render complete resection unfeasible. Because of the tumor's propensity to recur, radiotherapy has been recommended but its role remains to be elucidated.

Nasal Chondromesenchymal Hamartoma in Children

2001 ◽  
Vol 125 (3) ◽  
pp. 400-403 ◽  
Author(s):  
Chuen Hsueh ◽  
Swei Hsueh ◽  
Frank Gonzalez-Crussi ◽  
Ta-jen Lee ◽  
Jen-liang Su
Keyword(s):  
Immunohistochemical Study ◽  
Correct Diagnosis ◽  
S100 Protein ◽  
Soft Tissue Tumors ◽  
Biological Behavior ◽  
Ultrastructural Examination ◽  
Spindle Cells ◽  
Nasal Chondromesenchymal Hamartoma ◽  
Myofibroblastic Differentiation ◽  
Malignant Soft Tissue

Abstract Hamartoma in the nasal cavity of children is especially rare. Most documented cases occurred in infants, with characteristic histologic features of a mixture of various mesenchymal tissues. McDermott et al designated it nasal chondromesenchymal hamartoma in 1998, and it has since been considered a distinct clinicopathological entity. We report 2 such examples in a full-term male newborn and a 9-month-old boy, respectively. Histologically, both cases were characterized by a mixture of various mesenchymal elements, including spindle cells, collagen fibers, and irregular islands of osseous and chondroid tissue. Immunohistochemical study showed positivity to vimentin and S100 protein. Ultrastructural examination of case 1 demonstrated fibroblastic and myofibroblastic differentiation in tumor cells. There were 11 cases of nasal chondromesenchymal hamartoma in children published to date. The tumor has a benign biological behavior, and complete resection is the treatment of choice. It is apt to be misdiagnosed because of overlapping histologic features shared with a number of benign and malignant soft tissue tumors. Awareness of this entity is essential for correct diagnosis and adequate therapy.

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