Histiocytic Sarcoma Associated With Idiopathic Myelofibrosis

2004 ◽  
Vol 128 (10) ◽  
pp. 1167-1170 ◽  
Author(s):  
Masaharu Fukunaga ◽  
Hiroyuki Kato
Keyword(s):  
Tumor Cells ◽  
S100 Protein ◽  
Lymph Node Biopsy ◽  
Histiocytic Sarcoma ◽  
Idiopathic Myelofibrosis ◽  
Large Tumor ◽  
Eosinophilic Cytoplasm ◽  
Histiocytic Lymphoma ◽  
Generalized Lymphadenopathy ◽  
Multiagent Chemotherapy

Abstract We describe the case of a 39-year-old man with idiopathic myelofibrosis, who developed histiocytic sarcoma (true histiocytic lymphoma) 6 months after diagnosis. The patient developed generalized lymphadenopathy. A lymph node biopsy showed pronounced distension of the sinuses in the medulla and periphery, caused by the accumulation of large tumor cells. The tumor cells had abundant clear or eosinophilic cytoplasm. The nuclei were of various sizes and shapes, with condensed chromatin and prominent nucleoli. Some tumor cells displayed erythrophagocytosis. Immunohistochemically, the tumor cells were positive for CD68, α1-antitrypsin, CD45, CD45RO, and S100 protein, and were negative for B- and T-cell markers, CD30, CD1a, lysozyme, myeloperoxidase, factor VIII–related antigen, CAM 5.2, and HMB-45. Despite multiagent chemotherapy, the patient died of disease 25 months after diagnosis. Although histiocytic sarcomas are very rare, their recognition may be important for clinical and prognostic reasons.

scholarly journals Sporadic hemangioblastoma of the kidney: a clinicopathologic study of three cases and a literature review

2021 ◽  
Vol 49 (7) ◽  
pp. 030006052110277
Author(s):  
Yanmei Xu ◽  
Xuehua Ma ◽  
Yong Ma ◽  
Juan Li ◽  
Renya Zhang ◽  
...  
Keyword(s):  
Tumor Cells ◽  
S100 Protein ◽  
Neuron Specific Enolase ◽  
Renal Tumors ◽  
Vascular Networks ◽  
Uniform Size ◽  
Clinicopathologic Study ◽  
Eosinophilic Cytoplasm ◽  
Lipid Vacuoles ◽  
Cluster Of Differentiation

Much attention has been paid to renal hemangioblastoma, but there are still challenges in its differential diagnosis. Three cases (2 men, 1 woman; age: 40–56 years) presented with renal tumors. The tumors were surrounded by a thick fibrous capsule, well-demarcated from the surrounding renal parenchyma, and composed of sheets or nests of polygonal to short spindle-shaped tumor cells with a rich capillary network. In cases 1 and 3, the large polygonal tumor cells contained abundant pale or eosinophilic cytoplasm, and some possessed intracytoplasmic lipid vacuoles. In case 2, tumor cells were characterized by a uniform size, mild, clear, or lightly stained cytoplasm, and typical "clear cell" appearance. Immunohistochemistry revealed that the polygonal stromal cells were strongly and diffusely positive for α-inhibin, neuron-specific enolase (NSE), S100 protein, and vimentin. Cluster of differentiation (CD)10 and paired box gene (PAX)8 were positive, while epithelial membrane antigen (EMA) and cytokeratin (CK) were focally positive in case 3. CD34 and CD31 outlined the contours and distribution of the vascular networks. Renal hemangioblastoma is rare and prone to misdiagnosis; more attention should be paid to the morphological features and reasonable application of immunohistochemistry in the diagnosis of hemangioblastoma.

Canine Laryngeal Rhabdomyoma

1985 ◽  
Vol 22 (6) ◽  
pp. 533-539 ◽  
Author(s):  
D. J. Meuten ◽  
M. B. Calderwood Mays ◽  
R. C. Dillman ◽  
B. J. Cooper ◽  
B. A. Valentine ◽  
...  
Keyword(s):  
Light Microscopy ◽  
Tumor Cells ◽  
Striated Muscle ◽  
Muscle Cells ◽  
Large Tumor ◽  
Dark Cells ◽  
Eosinophilic Cytoplasm ◽  
Laryngeal Tumors

Three canine laryngeal tumors were diagnosed as oncocytomas by light microscopy, but were determined to be rhabdomyomas following ultrastructural and immunocytochemical examination. Tumors consisted of large eosinophilic cells interspersed with smaller dark cells. Large tumor cells had a granular, intensely eosinophilic cytoplasm. Scattered through the tumors were a few elongated cells with cytoplasmic cross striations and multiple nuclei. Tumor cells from all three dogs contained numerous mitochondria and bundles of myofibrils with electron-dense Z-lines typical of striated muscle cells. Intracellular myoglobin and desmin were detected in the tumors by immunocytochemistry. Comparisons are made with a previous report of canine laryngeal oncocytomas.

scholarly journals Sporadic Hemangioblastoma of the Kidney: A clinicopathologic study of 3 cases and review of the literature

2020 ◽  
Author(s):  
Yanmei Xu ◽  
Xuehua Ma ◽  
Yong Ma ◽  
Juan Li ◽  
Renya Zhang ◽  
...  
Keyword(s):  
Tumor Cells ◽  
S100 Protein ◽  
Vascular Networks ◽  
Uniform Size ◽  
Von Hippel Lindau ◽  
Clinicopathologic Study ◽  
Eosinophilic Cytoplasm ◽  
The Central Nervous System ◽  
Lipid Vacuoles ◽  
Vhl Disease

Abstract Background: Hemangioblastoma is a benign tumor of unknown histogenesis that mainly occurs in the central nervous system (CNS) associated with von Hippel-Lindau (VHL) disease. Much attention has been paid to the renal hemangioblastoma, but there are still some challenges in the differential diagnosis.Case presentation: Here, we describe three cases of sporadic renal hemangioblastoma with no clinical features of VHL diseases. All the three patients (male: 2; female: 1) were 40-56 years old. In all cases, the tumors were surrounded by a thick fibrous capsule and well-demarcated from the surrounding renal parenchyma. Tumors were composed of sheets or nests of polygonal to short spindle tumor cells and a rich capillary network. In case 1 and case 3, the large polygonal tumor cells contained abundant pale or eosinophilic cytoplasm, and some of the cells possessed intracytoplasmic lipid vacuoles. In case 2, tumor cells were characterized by uniform size, mild, clear or lightly stained cytoplasm and typical "clear cell" appearance. In the views of immunohistochemistry, the polygonal stromal cells were strongly and diffusely positive for α-inhibin, NSE, S100 protein, and vimentin. CD10 and PAX8 were positive, while EMA and CK showed focally positive in case 3. CK8/18, HMB45, MelanA, CgA, Syn, SMA, Desmin and CD56 were all negative. CD34 and CD31 outlined the contours and distribution of vascular networks in tumors. Conclusions: Renal hemangioblastoma is rare and prone to be misdiagnosed. More attention should be paid to the morphological features and reasonable application of immunohistochemistry for the diagnosis of hemangioblastoma.

scholarly journals Extragastrointestinal stromal tumor of the abdominal subcutaneous tissue: Report of a very rare case at an unusual site

2017 ◽  
Vol 45 (3) ◽  
pp. 1273-1278 ◽  
Author(s):  
Xue He ◽  
Nannan Chen ◽  
Li Lin ◽  
Congyang Wang ◽  
Yan Wang
Keyword(s):  
Tumor Cells ◽  
Subcutaneous Tissue ◽  
Dermatofibrosarcoma Protuberans ◽  
Pathological Examination ◽  
Chinese Han ◽  
Unusual Site ◽  
Stromal Tumors ◽  
Activating Mutations ◽  
Eosinophilic Cytoplasm ◽  
Exon 11

Extragastrointestinal stromal tumors (EGISTs) are rare tumors that arise outside the digestive tract. We report a case of an EGIST arising in the subcutaneous tissue of the abdominal wall, which at this site can often be misdiagnosed as dermatofibrosarcoma protuberans. The tumor was surgically resected from a 72-year-old male Chinese Han patient, and pathological examination revealed spindle-shaped tumor cells with eosinophilic cytoplasm and an oval nucleus. Immunohistochemically, the tumor cells showed strong cytoplasmic positivity for CD34, c-KIT (CD117), and DOG1. Tests for activating mutations of GISTs showed that the tumor cells carried an in-frame deletion (NP_000213.1:p.Lys550_Gln556del) in exon 11 of c-KIT (CD117). Thus, an EGIST should be considered in patients with abdominal subcutaneous tumors with an epithelioid, spindle-shaped, or mixed morphology. Immunohistochemistry of c-KIT (CD117) and DOG1 and genetic testing for activating mutations are recommended to aid in the differential diagnosis of subcutaneous tumors. In short, although EGISTs are rare in the abdominal subcutaneous tissue, pathologists must be aware of their possibility.

Plexiform Cellular Schwannoma in Infancy and Childhood: A Clinicopathological Study of Seven Cases of an Underrecognized Nerve Sheath Tumor with a Tendency Toward Local Recurrence

2021 ◽  
pp. 106689692110522
Author(s):  
Meng Sun ◽  
Mengyuan Shao ◽  
Jiahan Liu ◽  
Lu Zhao ◽  
I Weng Lao ◽  
...  
Keyword(s):  
Local Recurrence ◽  
Growth Pattern ◽  
S100 Protein ◽  
Maximum Diameter ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Thoracic Spinal ◽  
Infancy And Childhood ◽  
Eosinophilic Cytoplasm ◽  
Cellular Schwannoma

Plexiform cellular schwannoma (PCS) is very rare, and it is not completely understood. We present our experience with 7 additional cases of PCS in infancy and childhood to further characterize its distinctive clinicopathological features. There were 5 females and 2 males with a mean age of 28 months (ranging, 2 months to 8 years). The involved sites included the left forearm ( n = 2), sacrococcygeal region ( n = 2), retroperitoneum ( n = 1), thoracic spinal canal and thoracic cavity ( n = 1), and neck ( n = 1). Tumor sizes ranged from 3 to 13 cm in maximum diameter (mean, 7.1 cm). Histologically, all tumors consisted of abundant spindle cells arranged in a multinodular or plexiform growth pattern, possessing elongated, hyperchromatic nuclei and pale eosinophilic cytoplasm with indistinct cell margins. Mitotic figures were easily identified, with a mean count of 4 per 10 consecutive high power fields (HPF). Immunohistochemically, all tumors were strongly and diffusely positive for S100 protein, SOX10 and H3K27me3. The Ki-67 index ranged from 5% to 30% (mean, 15%). Follow-up (available in 6 cases) revealed that 5 patients experienced local recurrence and were treated by re-excision. There was no evidence of recurrence and metastasis in 3 patients, and the other 2 were alive with the disease. In conclusion, PCS is an uncommon nerve sheath tumor predominantly occurring in infants and children, featuring a plexiform or multinodular growth pattern and exhibiting a tendency toward local recurrence. PCS is easily mistaken as malignant peripheral nerve sheath tumor (MPNST) due to its locally aggressive behaviors and worrisome features, including hypercellularity, hyperchromatism and high proliferative activity. Increased awareness of its potential occurrence and greater familiarity with its characteristic features are helpful for both clinicians and pathologists to avoid misdiagnosis and unnecessary overtreatment.

Clear Cell Sarcoma of Tendons and Aponeuroses: A Review

2007 ◽  
Vol 131 (1) ◽  
pp. 152-156 ◽  
Author(s):  
Daniel C. Dim ◽  
Linda D. Cooley ◽  
Roberto N. Miranda
Keyword(s):  
Clear Cell ◽  
Current Knowledge ◽  
S100 Protein ◽  
Clear Cell Sarcoma ◽  
Prognostic Indicators ◽  
Gene Transcript ◽  
Poor Overall Survival ◽  
Regional Lymph Nodes ◽  
Cell Sarcoma ◽  
Eosinophilic Cytoplasm

Abstract Clear cell sarcoma of tendons and aponeuroses, also referred to as malignant melanoma of soft parts, is a rare malignancy derived from neural crest cells. It usually presents in the distal lower extremities of young adults, frequently attached to tendons or aponeuroses. It behaves like a high-grade soft tissue sarcoma and is associated with poor overall survival. Magnetic resonance imaging studies of the lesion reveal T1 hypointensity, T2 hyperintensity, and gadolinium uptake. Grossly, the tumor is usually circumscribed with a histologic pattern of uniform polygonal to fusiform cells with clear to pale eosinophilic cytoplasm divided into variably sized clusters by fibrous septa. Immunohistochemical studies in most cases show that the neoplastic cells are positive with HMB-45 and react with antibody against S100 protein. Most cases show a reciprocal cytogenetic translocation t(12;22)(q13;q12) that creates a unique chimeric fusion EWSR1/ATF1 gene transcript. Metastasis occurs mainly to regional lymph nodes and lungs. Poor prognostic indicators include a tumor size equal to or more than 5 cm, presence of metastasis, and necrosis. The mainstay of treatment is wide excision of the tumor. The use of sentinel lymph node biopsy may become an important procedure in detecting occult regional metastasis and guiding the extent of surgery. The beneficial effects of adjuvant chemotherapy and radiotherapy have not been fully evaluated. This article provides a short overview of the current knowledge of clear cell sarcoma of tendons and aponeuroses.

scholarly journals A Case of Iris Melanocytoma Demonstrating Diffuse Melanocytic Proliferation with Uncontrolled Intraocular Pressure

2017 ◽  
Vol 8 (1) ◽  
pp. 190-194 ◽  
Author(s):  
Mami Kusunose ◽  
Yuji Sakino ◽  
Yoshihiro Noda ◽  
Tsutomu Daa ◽  
Toshiaki Kubota
Keyword(s):  
Electron Microscopy ◽  
Intraocular Pressure ◽  
Tumor Cells ◽  
Trabecular Meshwork ◽  
Light And Electron Microscopy ◽  
Secondary Glaucoma ◽  
Pigment Dispersion ◽  
Large Tumor ◽  
History Of ◽  
Ring Melanoma

We report a rare case with histologically proven melanocytoma of the iris that demonstrated diffuse melanocytic proliferation with uncontrolled secondary glaucoma and investigate the etiology of the intraocular pressure elevation. The patient was a 78-year-old man with a history of darkened iris of his left eye. The intraocular pressure was 39 mm Hg. A slit-lamp examination showed a diffuse darkened iris, and a gonioscopic examination revealed open angle with circumferential heavy pigmentation. There was no pigment dispersion of the anterior chamber and no pigment deposition of the cornea. We suspected malignant ring melanoma in the left eye and enucleated it. The globe was examined with light and electron microscopy. Light microscopy revealed the presence of heavily pigmented tumor cells in the iris, ciliary body, trabecular meshwork, and Schlemm’s canal. A bleached preparation showed large tumor cells with central and paracentral nuclei without mitosis. Electron microscopy of the trabecular meshwork revealed melanin-bearing tumor cells invading the intertrabecular spaces, and the melanin granules were not phagocytosed in the trabecular cells. The mechanical obstruction of the aqueous flow by the tumor cells may be a major cause of secondary glaucoma in eyes with iris melanocytoma presenting diffuse proliferation.

scholarly journals Preoperative Nomogram for Predicting Sentinel Lymph Node Metastasis Risk in Breast Cancer: A Potential Application on Omitting Sentinel Lymph Node Biopsy

2021 ◽  
Vol 11 ◽  
Author(s):  
Xi’E Hu ◽  
Jingyi Xue ◽  
Shujia Peng ◽  
Ping Yang ◽  
Zhenyu Yang ◽  
...  
Keyword(s):  
Breast Cancer ◽  
Lymph Node ◽  
Sentinel Lymph Node ◽  
Lymph Node Biopsy ◽  
Curve Analysis ◽  
Axillary Lymph Nodes ◽  
Axillary Lymph ◽  
Large Tumor ◽  
Invasive Approach ◽  
Decision Curve Analysis

BackgroundSentinel lymph node (SLN) biopsy is feasible for breast cancer (BC) patients with clinically negative axillary lymph nodes; however, complications develop in some patients after surgery, although SLN metastasis is rarely found. Previous predictive models contained parameters that relied on postoperative data, thus limiting their application in the preoperative setting. Therefore, it is necessary to find a new model for preoperative risk prediction for SLN metastasis to help clinicians facilitate individualized clinical decisions.Materials and MethodsBC patients who underwent SLN biopsy in two different institutions were included in the training and validation cohorts. Demographic characteristics, preoperative tumor pathological features, and ultrasound findings were evaluated. Multivariate logistic regression was used to develop the nomogram. The discrimination, accuracy, and clinical usefulness of the nomogram were assessed using Harrell’s C-statistic and ROC analysis, the calibration curve, and the decision curve analysis, respectively.ResultsA total of 624 patients who met the inclusion criteria were enrolled, including 444 in the training cohort and 180 in the validation cohort. Young age, high BMI, high Ki67, large tumor size, indistinct tumor margins, calcifications, and an aspect ratio ≥1 were independent predictive factors for SLN metastasis of BC. Incorporating these parameters, the nomogram achieved a robust predictive performance with a C-index and accuracy of 0.92 and 0.85, and 0.82 and 0.80 in the training and validation cohorts, respectively. The calibration curves also fit well, and the decision curve analysis revealed that the nomogram was clinically useful.ConclusionsWe established a nomogram to preoperatively predict the risk of SLN metastasis in BC patients, providing a non-invasive approach in clinical practice and serving as a potential tool to identify BC patients who may omit unnecessary SLN biopsy.

Two for One: B-Cell Lymphomas with Features of Marginal and Follicular Lymphomas

2018 ◽  
Vol 139 (2) ◽  
pp. 84-88 ◽  
Author(s):  
Alexey Glazyrin ◽  
Chirag Patel ◽  
Lara Kujtan ◽  
Sheshadri Madhusudhana
Keyword(s):  
Bone Marrow ◽  
Follicular Lymphoma ◽  
Tumor Cells ◽  
Marginal Zone ◽  
Lymph Node Biopsy ◽  
Low Grade ◽  
Circulating Cells ◽  
Diagnosis And Classification ◽  
Follicular Lymphomas ◽  
Normal Architecture

Low-grade follicular lymphomas are genetically characterized by the translocation t(14; 18)(q32;q21) with BCL2 gene rearrangements. Marginal zone lymphomas are often associated with translocations or transcriptional deregulations of the MALT gene. We report 2 cases of lymphomas which harbor both the t(14;18)(q32;q21) translocation and MALT gene upregulation. Patients presented with numerous circulating atypical lymphocytes. Lymph node biopsy in both cases on HE staining demonstrated vague nodularity readily highlighted by CD10, CD23, or BCL6. Staining with CD20 and BCL2 demonstrated monotonous diffuse effacement of normal architecture with tumor cells without obvious follicular structures. Morphologically, tumor cells were consistent with centrocytes. Bone marrow biopsy demonstrated a combined peritrabecular and interstitial distribution of the tumor cells. These cases present substantial difficulties for diagnosis and classification. Clinical and morphological features were mostly consistent with follicular lymphoma, with a few features more often seen in marginal zone lymphomas (leukemic presentation, no CD10 in circulating cells, interstitial location of tumor cells in bone marrow); therefore, these cases were finally classified as follicular lymphoma grade I. Both patients were treated with standard chemotherapy regimens for follicular and nongastric MALT lymphomas with a good response to date.

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