scholarly journals Atypical Central Neurocytoma With Sarcomatous Differentiation

2014 ◽  
Vol 138 (9) ◽  
pp. 1233-1237 ◽  
Author(s):  
Lakshmi Vemavarapu ◽  
Irene Czyszczon ◽  
Joseph C. Parker ◽  
Stephanie Wagner ◽  
Todd Vitaz ◽  
...  

We report a case of an intraventricular tumor with features of atypical central neurocytoma with a sarcomatous component in a 44-year-old woman who presented with headaches and vomiting. Magnetic resonance imaging revealed a 3.4-cm lobulated enhancing mass in the occipital horn of the left lateral ventricle, and the patient subsequently underwent a left occipital-parietal craniotomy for debulking. The tumor contained 2 cell populations: round cells with perinuclear halos in a fibrillary background, and spindle cells with oval nuclei arranged in interlacing fascicles with focal necrosis. The round cells had diffuse synaptophysin immunopositivity, while the spindle cells were diffusely immunopositive for vimentin and had intercellular reticulin. The mitotic activity (8 mitotic figures per 10 high-power fields) and the high Ki-67 proliferation index (15.0%) were consistent with atypical central neurocytoma with a sarcomatous component. Although different histologic variants have been described, this is the first reported case, to our knowledge, of central neurocytoma with spindle cell sarcomatous features.

2021 ◽  
pp. 106689692110704
Author(s):  
Zheng Hua Piao ◽  
Jin Ping Chen ◽  
Hai Ren Chen ◽  
Xin Cheng Zhou

The correlation of histogenesis between metaplastic thymoma and thymic sarcomatoid carcinoma is unclear. We report a case of metaplastic thymoma transformed into high-grade sarcomatoid carcinoma. A 64  ×  54  ×  32 mm anterior mediastinal mass in a 61-year-old woman microscopically consisted mainly of classic metaplastic thymoma, with the center dominated by high-grade sarcomatoid carcinoma. In some areas, both epithelial and spindle cell components of the metaplastic thymoma showed increased cellular atypia, mitotic activity, and focal necrosis and gradually transformed into the polygonal/pleomorphic and spindle cell components of sarcomatoid carcinoma. Immunohistochemically, the characteristics of the polygonal/pleomorphic sarcomatoid cells were similar to those of the epithelial component of metaplastic thymoma, while the spindle sarcomatoid cells were more similar to the spindle cells component of metaplastic thymoma. The Ki-67 index was less than 5% in the metaplastic thymoma areas but up to 70% in the sarcomatoid carcinoma area. Radical operation and postoperative radiotherapy were performed. Multifocal relapses at the pleura occurred 13 months after surgery.


2020 ◽  
Author(s):  
Lu He ◽  
Zhiwen Li ◽  
Xiangshan Fan ◽  
Jieyu Chen ◽  
Hongyan Wu ◽  
...  

Abstract BackgroundB-lymphoblastic lymphomas (B-LBLs) with a documented “double-hit” (DH) (IGH/BCL2 and 8q24/MYC rearrangement) are rare and their clinical, cytogenetic and immunophenotypic features have not been well elucidated. Here we describe an unusual case of de novo DH B-LBL.Case presentationA 39-year-old man presented with abdominal distention at admission. Radiological findings revealed a retroperitoneal lump and multiple lymphadenopathy. Histologic examination of the abdominal mass showed a predominant population of neoplastic lymphoblasts with round nuclei, which focally formed single files surrounded by fibrotic septa. These cells have high nuclear to cytoplasmic ratio, fine chromatin, and no inconspicuous nucleoli. Necrosis, apoptotic bodies and mitotic figures were common. Prominent starry-sky appearance was not observed. The typical immunophenotype of malignant cells was positive for TdT, CD99, CD10, PAX-5, Bcl2 (70%), MYC (70%), and negative for CD34,Cyclin-D1, SOX11, CD56, and CD123. The proliferation index was high with Ki-67 of 80%. Fluorescence in situ hybridization (FISH) studies demonstrated 8q24/MYC rearrangement and IGH/BCL2 gene fusion. Subsequent bone marrow biopsy showed no involvement. Normal chromosome karyotype was observed. Finally, the diagnosis of “double-hit” B-LBL was clinically rendered. The patient showed an initial response to six cycles of hyper-CVAD chemotherapy (hyper-fractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone) and was alive at the last follow-up (2020.4.10, six months after the diagnosis).ConclusionsFew guidelines regarding DH B-LBL are available in the literature or in the WHO scheme. The optimal clinical management of such patients has not been fully established attributed to the small number of reported cases. Multicenter data is warranted to better make clinical strategies on patients with DH B-LBL.


2014 ◽  
Vol 2014 ◽  
pp. 1-4
Author(s):  
Tadashi Terada

Herein reported is a case of inflammatory pseudotumor (IPT) of kidney. It is not described in WHO, AFIP, and other books. A review of the literature revealed about 35 cases. A 76-year-old man underwent nephrectomy under clinical diagnosis of renal pelvic carcinoma. Grossly, a solid tumor was seen in renal parenchyma. Microscopically, it was composed of spindle cell tissue with inflammation and many necrotizing granulomas. Epithelioid histiocytes were abundant but giant cells were few. Lymphocytes and plasma cells were also seen. The features suggested tuberculosis (TB), but Ziehl-Neelsen stains and PCR revealed no TB bacillus. Immunohistochemistry showed that the tumor spindle cells were positive for vimentin, CD68, CD45, and Ki-67 (labeling = 18%), α-smooth muscle antigen, and NSE. Focal staining of KIT (mast cells), S100 protein (Langerhans cells), and CD10 (spindle cells) was present. IgG4 was negative. The tumor spindle cells were negative for other antigens examined.


2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S42-S42
Author(s):  
A P Kanteti ◽  
E Martinez Duarte

Abstract Casestudy: Dedifferentiated solitary fibrous tumor is a rare malignant subtype of the common benign mesenchymal neoplasm with fibroblastic differentiation known as solitary fibrous tumor (SFT). These account for less than 2% of all soft masses; and about 20% of SFTs turn aggressive. They feature bland spindle cells creating a classic “pattern-less pattern” in collagenous stroma with prominent staghorn vessels. They can dedifferentiate into a biphasic tumor with low grade areas that are spindle cell rich and high-grade areas with epithelioid cells, mitotic figures and necrosis. Here we present an 84-year-old female who came from an outside institution for a pathology review. The patient’s clinical history was lacking and biopsies from the right lower and middle lobes of the lung, azygous and hilar lymph nodes were investigated. The pathology revealed multiple areas with epithelioid cells that were pleomorphic and had high nuclear to cytoplasmic ratio. These areas also contained numerous mitotic figures, necrosis and giant cells; with lymphovascular space invasion. There was a clear demarcation between the epithelioid area and an area rich in spindle cells. The spindled areas revealed a fascicular architecture with intervening sclerotic stoma and prominent rounded and dilated blood vessels. Immunohistochemistry analysis of the epithelioid and spindle cell areas were positive for STAT6. Additional testing for CD34 was diffusely positive in the spindle cell component and only focally positive in the high-grade portion of the tumor. Ki-67stain was utilized to estimate the proliferative index which was up to 80 percent in the high-grade epithelioid component and about 15% in the spindle cell portion. These morphological and histochemical findings were consistent with the diagnosis of a dedifferentiated solitary fibrous tumor.


2001 ◽  
Vol 71 (3) ◽  
pp. 116-125
Author(s):  
Norina Basa ◽  
Daniela Lazar ◽  
Remus Cornea ◽  
Sorina Taban ◽  
Melania Ardelean ◽  
...  

Alteration of β-catenin expression is involved in the development and evolution of hepatocellular carcinoma (HCC); β-catenin is able to influence tumor cell proliferation. We analyzed the immunohistochemical (IHC) expression of β-catenin on a group of 32 patients diagnosed with HCC using the anti-β-catenin monoclonal antibody (clone E247). We correlated the expression of β-catenin with the proliferation index of Ki-67 (PI Ki-67), the mitotic index (MI) and other clinical and pathological features. We observed an altered β-catenin expression in 58.38% of all HCC cases. This expression was insignificantly correlated with tumor size (]5 cm) (p = 0.683), histological grade G1-G2 (p = 0.307), vascular invasion (p = 0.299) and advanced pT stage (p = 0.453); we obtained a significantly higher MI in HCC with altered β-catenin expression (p = 0.018), as compared to HCC without overexpression (1.66 � 1.37) (p = 0.038) and a PI Ki-67 of 22.49 � 20.1 and 28.24 � 18.2, respectively in tumors with altered β-catenin expression with insignificant differences compared to HCC without overexpression (25.95 � 15.2) (p = 0.682 and p = 0.731, respectively). According to the results we obtained, aberrant β-catenin expression in HCC was correlated with a high mitotic index, therefore playing an important role in tumor progression by stimulating tumor cell proliferation; non-nuclear β-catenin overexpression can have a pathological significance in HCC, especially in cases of HCC associated with hepatitis B virus (HBV) infection.


2020 ◽  
Vol 21 (11) ◽  
pp. 1097-1102
Author(s):  
Drashti Desai ◽  
Pravin Shende

: Immunotherapy emerges as a treatment strategy for breast cancer marker, diagnosis and treatment. In this review, monoclonal antibodies (mAbs)-based passive and peptide vaccines as active immunotherapy approaches like activation of B-cells and T-cells are studied. Passive immunotherapy is mAbs-based therapy effective against tumor cells, which acts by targeting HER2, IGF 1R, VEGF, BCSC and immune checkpoints. Neuropeptide Y (NPY) and GPCR are the areas of interest to target BC metastases for on-targeting therapeutic action. Neuropeptide S (NPS) or NPS receptor 1, acts as a biomarker for Neuroendocrine tumors (NET), mostly characterized by synaptophysin and chromogranin-A expression or Ki-67 proliferation index. The protein fusion technologies arise as a promising avenue in plant expression systems for increased recombinant Ab accumulation and cost-efficient purification. Recently, mAbs-based immunotherapy effectiveness is appreciated as a novel therapeutic combination of chemotherapy and immunotherapy to reduce the side effects and improve therapeutic responsiveness. Synthetic drug resistance will be overcome by mAbs-based therapy through several clinical trials and detection methods need to be optimized for accuracy and precision. Pharmacokinetic attributes need to be accessed for preferred receptor-agonist activity without ligand accumulation.


2021 ◽  
pp. 1-6
Author(s):  
Bita Geramizadeh ◽  
Alireza Shojazadeh

<b><i>Background:</i></b> Gastrointestinal stromal tumor (GIST) of the ampulla of Vater is a rare occurrence. To the best of our knowledge, there has been no published review on this rare tumor in the English literature so far. <b><i>Summary:</i></b> In this review, we will discuss all the reported details of the published cases, including demography, clinical presentation, imaging, gross pathology and histopathology, immunohistochemical findings, treatment modalities, and outcome of cases with the diagnosis GIST from the ampulla of Vater in the last 20 years. <b><i>Key Message:</i></b> Twenty-five cases of GIST in the ampulla of Vater have been reported in the last 20 years in the English literature. GIST in the ampulla of Vater are usually small tumors (&#x3c;5 cm) in middle-age patients. The majority of the patients present with lower GI bleeding and abdominal pain. Imaging findings are not characteristic, and most of the patients without biopsy and with no histologic diagnosis were operated with the primary impression of adenocarcinoma, neuroendocrine tumor, and GIST. Perioperative tissue biopsy has been accurate in &#x3c;70% of the cases. The majority of the reported cases of GISTs in the ampulla of Vater have been low risk with spindle-cell morphology, low mitotic figures, and minimal atypia; reactive for C-KIT and DOG-1; and nonreactive for SMA, desmin, and S100. In the majority of the cases, duodenectomy with or without Whipple’s operation has been performed, and most of the cases showed good prognosis.


2021 ◽  
pp. 112-122
Author(s):  
Ricardo Fernández-Ferreira ◽  
Ildefonso Roberto De la Peña-López ◽  
Karla Walkiria Zamudio-Coronado ◽  
Luis Antonio Delgado-Soler ◽  
María Eugenia Torres-Pérez ◽  
...  

Calcitonin-negative neuroendocrine tumor (CNNET) of the thyroid is an extremely rare entity. In some of the previously reported cases within the literature, the terms “atypical medullary thyroid carcinoma,” “calcitonin-free oat cell carcinoma,” and “a distinct clinical entity” were applied to NETs without definitive evidence of calcitonin production. In the English-language literature, not only are there only few reported cases of CNNET, but the criteria for diagnosis in these cases are also controversial. Most of the current published cases were also treated surgically for local disease. We describe a case of NET of the thyroid with calcitonin, chromogranin A and thyroglobulin negativity, synaptophysin and TTF-1 positivity, and a high Ki-67 proliferation index with metastases in the cervical region as well as mediastinal adenopathies. This case was considered an unresectable thyroid carcinoma, and chemotherapy including cisplatin and etoposide was started as neoadjuvant treatment at the department of medical oncology. Total thyroidectomy plus bilateral and central cervical dissection was performed, and the patient underwent 2 cycles of adjuvant radiotherapy. Currently, the patient’s 18F-FDG-PET/CT findings show a complete response 17 months after diagnosis. In conclusion, CNNET of the thyroid is very rare and there is limited evidence regarding treatment in patients with metastases. Chemotherapy including cisplatin and etoposide as well as early aggressive surgical resection appears to positively impact patients’ survival.


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