Littoral Cell Hemangioma of the Spleen.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 3953-3953
Author(s):  
Seema Naik ◽  
Farida Chaudhri ◽  
Stephen Yang
Keyword(s):  
Platelet Count ◽  
Ct Scan ◽  
White Male ◽  
Metastatic Tumor ◽  
Low Grade ◽  
Significant Past Medical History ◽  
Ki 67 ◽  
Primary Tumors ◽  
Littoral Cell ◽  
Red Pulp

Abstract Introduction: Splenic hemangiomas are rare, range from 0.03–14% in autopsy studies. The primary tumors of the spleen are benign and originate from the vascular endothelium and rarely from the lining cells of red pulp sinuses, giving rise to Littoral cell angiomas (LCA), first described by Falk et al in 1991. It usually is an incidental benign tumor presenting as mild to moderate splenic enlargement seen at any age in both sexes. Grossly it usually has multiple distinct nodules are spongy with dark bloody spaces ranging from 0.2 to 9.0 cm in diameter. Case Report: 65 years old white male patient presented with thrombocytopenia, platelet count 84000/cmm, Hemoglobin 11.8 and WBC 5500. He had no significant past medical history. Review of systems was noncontributory. There were no other physical findings. The patient had normal upper gastrointestinal series. Colonoscopy revealed tubular adenomatous polyp. CT scan chest revealed thoracic vertebral hemangioma. CT scan of abdomen showed enlarged spleen measuring 15cm axially with multiple low attenuation areas throughout the spleen. FNA of spleen showed spindle cells positive for factor VIII and CD31. The patient had normal bone marrow biopsy as well as cytogenetics. The patient underwent splenectomy which showed littoral cell angioma with heterogeneous population of lymphocytes, histiocytes, eosinophila, neutrophils, platelets, and large polylobated cells. The patient had marked clinical improvement with normallization of platelet count post splenectomy. Discussion: Littoral cell angiomas are seen as hypo echoic areas seen on ultrasonography and CT scan of abdomen. The natural history is benign and do not recur post splenectomy. Spontaneous rupture can occur in 25% of patients. On MRI, siderosis is seen due to hemophagocytosis by littoral cells. The lesions are of variable size and solitary or multinodular located in the red-pulp of the spleen. They are composed of anastomosing vascular channels with irregular lumina featuring cyst-like spaces. The LCA cells have a dual-endothelial/histiocytic characteristics, that react with F VIII Ag and are positive for CD68 antigen. Angiostatin inhibits angiogenesis and metastatic tumor growth. The mildly atypical cells, but not frankly malignant with low Ki 67 staining and diploid DNA histogram are in accordance with a low-grade malignancy. LCA has also been associated with synchronous malignancies such as lymphoma, colonic cancer, renal cancer, ovarian cancer, pancreatic cancer, seminoma and gastric leiomyosarcoma, a long-term follow-up for these patients is recommended.

Relationship of Ki-67 proliferative index and metastatic tumor of breast cancer.

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e22190-e22190
Author(s):  
Kokoro Kobayashi ◽  
Yoshinori Ito ◽  
Akiko Ogiya ◽  
Naoya Gomi ◽  
Rie Horii ◽  
...  
Keyword(s):  
Breast Cancer ◽  
Primary Tumor ◽  
Metastatic Tumor ◽  
Labeling Index ◽  
Proliferation Index ◽  
Luminal Breast Cancer ◽  
Line Treatment ◽  
Metastatic Tumors ◽  
Ki 67 ◽  
Primary Tumors

e22190 Background: The metastatic breast tumor tends to be more aggressive with high proliferation, but this has not been proven in clinical sampling of metastatic tumors. Methods: Forty-eight patients who had histological specimens of both primary and metastatic sites of luminal breast cancer (ER and/or PgR positive and HER2 negative) were examined. We classified them as luminal A (LA) with Ki-67 labeling index of less than 14% and as luminal B (LB) with Ki-67 labeling index of more than 14%. We analyzed their overall survival (OS) and progression free survival (PFS) of 1st line treatment of each subtype of primary and metastatic tumors. Results: Subtypes of primary tumors and metastatic tumors were as follows; the primary tumor: LA; 34 patients (70.8%), LB; 14 (29.2%), metastatic tumors: LA; 21 (43.8%), LB; 27 (56.2%). Patients with LA of the primary tumor demonstrated statistically longer OS (LA; 72.5 months, LB 39.6 months, p=0.009). OS depended on the subtype of the primary tumor. In contrast, patients with LB of a metastatic tumor showed a statistically worse PFS (LA; 20.5 months, LB; 11.5 months, p=0.040). PFS of the 1st line treatment for MBC depended on the subtype of the metastatic tumor. Conclusions: The frequency of LB was increased on metastatic tumors and tended to acquire a higher proliferation index. This suggests that characterization of metastatic tumors could be better as an indicator of subsequent treatment for MBC. [Table: see text]

scholarly journals Metastatic Adrenocortical Carcinoma Co-Secreting Multiple Steroid Hormones

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A142-A142
Author(s):  
Tien-Hao Lee ◽  
Robert Galagan
Keyword(s):  
Ct Scan ◽  
Steroid Hormones ◽  
Adrenocortical Carcinoma ◽  
Exploratory Laparotomy ◽  
Surgical Pathology ◽  
Renin Activity ◽  
Low Grade ◽  
Ki 67 ◽  
Left Diaphragm ◽  
Adrenal Nodule

Abstract Background: Adrenocortical carcinoma is a rare disease which may be complicated by co-secretion of multiple steroid hormones. Clinical Case: A 53- year-old female was discovered to have 1 cm left and 1.7 cm right adrenal nodules by Chest CT scan in 2004. She had a follow-up abdominal CT scan in 2011 revealing enlargement of the left adrenal mass to 5.7 cm and a stable 1.8 cm right adrenal nodule. A laparoscopic left adrenalectomy was performed in 2012 and the surgical pathology diagnosis was benign adrenal hyperplasia. In 5/2016 the patient developed left abdominal pain and a CT scan revealed a 1.7 cm mass in the left adrenalectomy surgical bed, a 1.4 cm nodular density adjacent to the left diaphragm and the stable 1.8 cm right adrenal nodule. 6/2016 lab tests: 24 hr urinary cortisol 15 ug/24 hr (<50 μg/24hr), aldosterone 8.7 ng/dL (<31 ng/dL), renin activity 0.7 ng/ml/hr (0.5–4 ng/mL/hr) and DHEA-S 94 ug/dL (32–240 μg/dL). A re-examination of the 2012 surgical pathology resulted in an addendum diagnosis of an adrenal cortical neoplasm of indeterminate malignant potential. In 1/2018 she underwent an exploratory laparotomy with surgical resection of the 1.7 cm mass in left paracolic gutter and biopsy of numerous small retro-peritoneal and multiple liver lesions. Pathology revealed metastatic adrenocortical carcinoma with low grade mitotic activity (3 mitoses per 10 HPF) and intermediate grade Ki-67 (15–25%). 5/2018 lab results: 1. aldosterone 20 ng/dL, 2. renin activity 0.2 ng/mL/hr and 3. testosterone 34 ng/dL (<75 ng/dL). Mitotane was started in 06/2018 but was discontinued in 9/2018 due to side effects. In 3/2020 she was hospitalized for generalized weakness and was discovered to be severely hypokalemic K+ 1.5 mmol/L (3.6–5.2 mmol/L) with an aldosterone of 300 ng/dL and renin activity of 0.1 ng/mL/hr. She was treated with IV KCl to correct her hypokalemia and was discharged on oral KCl 20 meq bid and spironolactone 50 mg bid. She was readmitted to the hospital on 10/12/2020 after a near-syncopal event and lab tests revealed a K+ of 1.4, aldosterone 508 ng/dL, renin activity 0.7 ng/mL/hr, AM cortisol 13.6 μg/dL (5–20 ug/dL), testosterone 161 ng/dL, and DHEA-S 377 ug/dL, indicating co-secretion of multiple steroid hormones. Her hypokalemia was treated with IV KCl and her spironolactone dose was increased to 100 mg bid. Conclusion: This case report details the rare occurrence of an adrenocortical carcinoma which was hormonally silent but eventually metastasized and became hormonally active, co-secreting multiple steroid hormones with a predominance of aldosterone. Serial adrenal hormone lab profiles are important for optimal management of patients with this disease.

scholarly journals Antisense Oligonucleotide Targeting of Thrombopoietin Synthesis Reduces Platelet Count within the Hemostatic Range and Slows Progression of De Novo Mammary Carcinogenesis in the Mmtv-Pymt Mouse

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 989-989
Author(s):  
Toshiaki Shirai ◽  
Alexey Revenko ◽  
Justin Tibbits ◽  
Lisa M Coussens ◽  
Owen JT McCarty ◽  
...  
Keyword(s):  
Platelet Count ◽  
Intellectual Property ◽  
Property Rights ◽  
Intellectual Property Rights ◽  
De Novo ◽  
Pulmonary Metastases ◽  
Neoplastic Progression ◽  
Ki 67 ◽  
Primary Tumors ◽  
Femoral Bone Marrow

Abstract Platelets have long been proposed to play a role in fostering neoplasias, ranging from tissue repair to cancer, but clinical evidence remains limited. Since available therapautics antagonizing hemostatic platelet functions lead to bleeding, the concept of targeting platelets in the context of cancer prevention or treatment has not been pursued in controlled clinical trials. To address this, we developed a novel antiplatelet strategy by partial inhibition of thrombopoiesis. We hypothesized that if platelets played a key role in fostering neoplastic malignancies, controlled platelet count reduction within a safe range would slow platelet-dependent cancer progression without adversely affecting hemostasis. Since the MPL ligand megakaryocyte growth and development factor (thrombopoietin, MGDF) is required for maintenance of normal platelet count, and the liver is responsible only for a portion of thrombopoietin synthesis, we tested this hypothesis by targeted knock-down of hepatic thrombopoietin synthesis using an antisense oligonucleotide (ASO) in mammary cancer-prone transgenic mice. We investigated whether partial reduction of thrombopoietin sythesis through ASO knock-down was hemostatically safe and impacted kinetics or penetrance of neoplastic progression in de novo mammary carcinogenesis in MMTV-PyMT mice. We designed and generated thrombopoietin ASO-s (TPO ASO) to achieve about 50% platelet count reduction in mice by inhibition of hepatic thrombopoietin gene expression. Then MMTV-PyMT transgenic mice were subcutaneously administered one of the effective TPO ASO-s, with treatment initiated at 40 days of age and before appearance of palpable (>2.0 mm) mammary tumors. We assessed the impact of saturating dose TPO ASO, including platelet count, plasma TPO level, and number of megakaryocytes in the femoral bone marrow. Mammary tumor growth, pulmonary metastases, and overall survival (fixed end-point study based on total tumor burden) of TPO ASO-treated mice were compared with untreated controls. At study endpoints, mammary carcinomas, platelet deposition, intra-tumoral vessel density, and Ki-67 positive cells were quantitatively evaluated. As anticipated from the drug screen, TPO ASO treatment at saturating effect reduced plasma thrombopoietin levels and blood platelet count by ~50% within 4 weeks, and the number of megakaryocytes in femoral bone marrow. TPO ASO also suppressed primary tumor growth (attached figure) and impaired development of pulmonary metastases (p<0.05), resulting in increased overall survival (p<0.001). Histological analysis of primary tumors revealed that TPO ASO significantly reduced mean platelet deposition in tumor vessels (from 54% to 26%; p<0.001), reduced vessel density (from 3.1% area to 1.5% area; p<0.05) and Ki-67 positive cells (from 62/field to 36/field; p<0.05) in primary tumors. Together, these results support the notion that strategies that safely reduce platelet presence or activity within developing mammary tumors, may limit neoplastic progression coincident with reduced angiogenesis. In summary, we found that TPO ASO reduces thrombopoietin levels and platelet count within the hemostatically safe range and significantly slows neoplastic progression of spontaneous mammary carcinomas in MMTV-PyMT mice. Overall, pharmacological knockdown of hepatic TPO synthesis is a new antiplatelet strategy that may be reasonably safe and effective in various platelet-driven disorders, including cancer. Figure Figure. Disclosures Revenko: Ionis Pharmaceuticals, Inc: Employment, Other: Intellectual property rights. Monia:Ionis Pharmaceuticals, Inc: Employment, Other: Intellectual property rights. Gruber:Aronora, Inc: Employment, Other: Intellectual property rights.

Rab11 family-interacting protein 4, RAB11FIP4, is a differentially expressed gene in brain metastatic human breast cancer.

2021 ◽  
Author(s):  
Shahan Mamoor
Keyword(s):  
Breast Cancer ◽  
Metastatic Breast Cancer ◽  
Differentially Expressed Gene ◽  
Metastatic Breast ◽  
Metastatic Tumor ◽  
Clinical Problem ◽  
Interacting Protein ◽  
Primary Tumors ◽  
Free Survival ◽  
The Brain

Metastasis to the brain is a clinical problem in patients with breast cancer (1-3). We mined published microarray data (4, 5) to compare primary and metastatic tumor transcriptomes for the discovery of genes associated with brain metastasis in humans with metastatic breast cancer. We found that Rab11 family-interacting protein 4, encoded by RAB11FIP4, was among the genes whose expression was most different in the brain metastases of patients with metastatic breast cancer as compared to primary tumors of the breast. RAB11FIP4 mRNA was present at increased quantities in brain metastatic tissues as compared to primary tumors of the breast. Importantly, expression of RAB11FIP4 in primary tumors was significantly correlated with patient recurrence-free survival and distant metastasis-free survival. Modulation of RAB11FIP4 expression may be relevant to the biology by which tumor cells metastasize from the breast to the brain in humans with metastatic breast cancer.

scholarly journals A Rare Cutaneous Adnexal Tumor: Malignant Proliferating Trichilemmal Tumor

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Omer Alici ◽  
Musa Kemal Keles ◽  
Alper Kurt
Keyword(s):  
Differential Diagnosis ◽  
Elderly Women ◽  
Low Grade ◽  
Outer Root Sheath ◽  
Ki 67 ◽  
Morphological Findings ◽  
Root Sheath ◽  
Adnexal Tumor ◽  
Proliferating Trichilemmal Tumor ◽  
Malignant Proliferating Trichilemmal Tumor

Proliferating trichilemmal tumors (PTTs) are neoplasms derived from the outer root sheath of the hair follicle. These tumors, which commonly affect the scalp of elderly women, rarely demonstrate malignant transformation. Although invasion of the tumors into neighboring tissues and being accompanied with anaplasia and necrosis are accepted as findings of malignancy, histological features may not always be sufficient to identify these tumors. The clinical behavior of the tumor may be incompatible with its histological characteristics. Squamous-cell carcinoma should certainly be considered in differential diagnosis because of its similarity in morphological appearance with PTT. Immunostaining for CD34, P53, and Ki-67 is a useful adjuvant diagnostic method that can be used in differential diagnosis aside from morphological findings. In this study, we aimed to present the case of a 52-year-old female patient with clinicopathological features. We reported a low-grade malignant proliferating trichilemmal tumor in this patient and detected no relapse or metastasis in a 24-month period of follow-up.

CASE STUDY ON CIRCUMSCRIBED GLIOMAS, THEIR CLINICOPATHOLOGICAL CORRELATION IN A TERTIARY CARE CENTER

2021 ◽  
pp. 42-45
Author(s):  
Esther Alffi Papang ◽  
K. Rama
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Tertiary Care ◽  
Biological Behavior ◽  
Low Grade ◽  
Lower Grade ◽  
Who Grade ◽  
Primary Tumors ◽  
Short Period ◽  
The Impact

The histogenesis and biological behavior of primary tumors of the central nervous system(CNS) are very diverse. The majority of present gliomas as benign, slow growing lesions classied as by the WHO classicati grade I or II (Low grade gliomas) on of CNS tumors. However, a signicant fraction of gliomas develop over a short period of time and progress rapidly and are therefore classied as WHO grade III or IV(High grade gliomas). Astrocytomas are primary central nervous system tumours that can develop in adults or in children. They arise from the Astrocytes. They can be divided into diffuse that generally have a higher grade and poorer prognosis and those that are localised that tend to be of a lower grade and have a better prognosis. In this study, we outline the basic histological spectrum and features, epidemiological aspects and grade of circumscribed gliomas (localised) or other Astrocytic tumours according to WHO classication . These are the Pilocytic Astrocytoma, Pilomyxoid Astrocytoma, Subependymal giant cell Astrocytoma, Pleomorphic xanthoastrocytoma and Anaplastic astrocytoma . The knowledge of these tumours are important as they are one of the commonest cause of mortality and morbidity in both the young and old, accounting for about 60% of the glial tumours. Therefore neuropathological diagnosis and tumour characteristics will therefore profoundly inuence the impact of treatment strategies.

EPCO-17. METHYLATION ANALYSIS OF MATCHED PRIMARY AND RECURRENT IDHmt ASTROCYTOMA; AN UPDATE FROM THE GLIOMA LONGITUDINAL ANALYSIS NL (GLASS-NL) CONSORTIUM

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi5-vi5
Author(s):  
Wies Vallentgoed ◽  
Anneke Niers ◽  
Karin van Garderen ◽  
Martin van den Bent ◽  
Kaspar Draaisma ◽  
...  
Keyword(s):  
Dna Methylation ◽  
Surgical Resection ◽  
Molecular Mechanisms ◽  
Relative Proportion ◽  
Low Grade ◽  
High Grade ◽  
Primary Tumors ◽  
Genome Wide ◽  
Dna Methylation Profiling ◽  
Methylation Profiling

Abstract The GLASS-NL consortium, was initiated to gain insight into the molecular mechanisms underlying glioma evolution and to identify markers of progression in IDH-mutant astrocytomas. Here, we present the first results of genome-wide DNA-methylation profiling of GLASS-NL samples. 110 adult patients were identified with an IDH-mutant astrocytoma at first diagnosis. All patients underwent a surgical resection of the tumor at least twice, separated by at least 6 months (median 40.9 months (IQR: 24.0, 64.7). In 37% and 18% of the cases, patients were treated with radiotherapy or chemotherapy respectively, before surgical resection of the recurrent tumor. DNA-methylation profiling was done on 235 samples from 103 patients (102 1st, 101 2nd, 29 3rd, and 3 4th resection). Copy number variations were also extracted from these data. Methylation classes were determined according to Capper et al. Overall survival (OS) was measured from date of first surgery. Of all primary tumors, the methylation-classifier assigned 85 (87%) to the low grade subclass and 10 (10%) to the high grade subclass. The relative proportion of high grade tumors increased ~three-fold at tumor recurrence (32/101, 32%) and even further in the second recurrence (15/29, 52%). Methylation classes were prognostic, both in primary and recurrent tumors. The overall DNA-methylation levels of recurrent samples was lower than that of primary samples. This difference is explained by the increased number of high grade samples at recurrence, since near identical DNA-methylation levels were observed in samples that remained low grade. In an unsupervised analysis, DNA-methylation data derived from primary and first recurrence samples of individual patients mostly (79%) cluster together. Recurrent samples that do not cluster with their primary tumor, form a separate group with relatively low genome-wide DNA-methylation. Our data demonstrate that methylation profiling identifies a shift towards a higher grade at tumor progression coinciding with reduced genome-wide DNA-methylation levels.

scholarly journals Extra and intradural spinal Hemangioblastoma

2012 ◽  
Vol 11 (3) ◽  
pp. 242-244
Author(s):  
Marcelo Campos Moraes Amato ◽  
Caio César Marconato Simões Matias ◽  
João Alberto Assirati Junior ◽  
Aline Paixão Becker ◽  
Carlos Gilberto Carlotti Junior ◽  
...  
Keyword(s):  
Histopathological Examination ◽  
Correct Diagnosis ◽  
White Male ◽  
Residual Tumor ◽  
Illustrative Case ◽  
Low Grade ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease ◽  
The Central Nervous System ◽  
Spinal Hemangioblastoma

Hemangioblastomas of the central nervous system (CNS) are low-grade highly vascularized tumors that may be sporadic or associated with Von Hippel-Lindau disease. Extradural hemangioblastomas are uncommon and those located extra and intradurally are even rarer. This study uses an illustrative case and literature review to discuss the difficulties to consider the correct diagnosis and to select the best surgical approach. A 57 years-old white male patient presented with myelopathy and right C5 radiculopathy. The images showed a lobulated, hourglass shaped, highly enhanced extra/intradural lesion that occupied the spinal canal and widened the C4-C5 right intervertebral foramen. Total resection of the intradural lesion was achieved through a posterior approach, but the extradural part could only be partially removed. Complete improvement was observed after four months of follow-up and the residual tumor has been followed up clinically and radiologically. Even though the preoperative impression was of a spinal schwannoma, the histopathological examination revealed grade I hemangioblastoma as per WHO. Despite their rarity, current complementary exams allow considering the diagnosis of hemangioblastoma preoperatively. That is essential to a better surgical planning in view of the particular surgical features of this lesion.

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