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.

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 Immunohistochemical Evaluation of a Malignant Intestinal Carcinoid in a Dog

2003 ◽  
Vol 40 (2) ◽  
pp. 212-215 ◽  
Author(s):  
T. Sako ◽  
E. Uchida ◽  
M. Okamoto ◽  
E. Yamamoto ◽  
Y. Kagawa ◽  
...  
Keyword(s):  
Tumor Cells ◽  
Neuron Specific Enolase ◽  
Clinical History ◽  
Intestinal Bleeding ◽  
Neoplastic Cells ◽  
Protein Gene Product ◽  
Eosinophilic Cytoplasm ◽  
Multiple Metastases ◽  
History Of ◽  
Cytokeratin 13

An intestinal carcinoid with multiple metastases was identified in a 5-year-old male Shih Tzu with a clinical history of anemia, fatigue, anorexia, vomiting, intermittent diarrhea, intestinal bleeding, and progressive emaciation. There was a yellowish-white mass 15 mm in diameter in the anterior jejunum and white nodules consistent with metastases in many organs. Histopathologically, the mass consisted of neoplastic cells arranged in lobules, trabeculae, or closely interdigitating islands of cells. Neoplastic cells were generally polygonal with round hyperchromatic nuclei, modest amounts of eosinophilic cytoplasm, and eosinophilic cytoplasmic granules. Mitoses were common. Rosette formations of tumor cells were apparent in metastatic tumors. Immunohistochemically, tumor cells stained positive for cytokeratin 13, synaptophysin, protein gene product 9.5, neuron-specific enolase, chromogranin A, calcitonin gene-related peptide, serotonin (5-HT), and Leu-7. Serum 5-HT concentrations for this dog were increased 10-fold compared with those of normal dogs. All findings were consistent with a diagnosis of a malignant intestinal carcinoid.

scholarly journals Ovarian female adnexal tumor of probable Wolffian origin – Case report

Open Medicine ◽  
2021 ◽  
Vol 16 (1) ◽  
pp. 899-903
Author(s):  
Ljiljana Vučković ◽  
Aleksandra Klisic ◽  
Mirjana Miladinović
Keyword(s):  
Case Report ◽  
Tumor Cells ◽  
Epithelial Membrane Antigen ◽  
Neuron Specific Enolase ◽  
Diagnostic Algorithm ◽  
Accurate Diagnosis ◽  
Histopathological Analysis ◽  
Eosinophilic Cytoplasm ◽  
Adnexal Tumor ◽  
Adnexal Tumors

Abstract Background During embryonic development in women, a regression of temporary embryonic structures – mesonephric (Wolffian) ducts occurs. Adnexal tumors of Wolffian duct origin (FATWO) are rare. Case report We presented the case of a 64-year-old female patient who was diagnosed with FATWO. After the surgical treatment, the uterus with bilateral adnexal structures was submitted for histopathological analysis. The left ovary was occupied by a tumor measuring 80 × 60 × 50 mm, with smooth, shiny, whitish surface. Tumor cells were medium-sized, relatively uniform, round, and polygonal, with eosinophilic cytoplasm and centrally laid nucleus with fine chromatin, organized into solid, trabecular, and tubular formations. Tumor cells were positive for pancytokeratin (CK), CK7, CD10, neuron-specific enolase (NSE), synaptophysin, calretinin, progesterone, estrogen, and epithelial membrane antigen (EMA). Conclusion This case adds a report of a rare tumor to the literature. We must think of it in the differential diagnostic algorithm to make an accurate diagnosis for selecting the best treatment modality.

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 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 Glial Fibrillary Acidic Protein (GFAP) in Oligodendrogliomas: A Reflection of Transient GFAP Expression by Immature Oligodendroglia

1986 ◽  
Vol 13 (4) ◽  
pp. 307-311 ◽  
Author(s):  
V. Jagadha ◽  
W.C. Halliday ◽  
L.E. Becker
Keyword(s):  
White Matter ◽  
Glial Fibrillary Acidic Protein ◽  
Tumor Cells ◽  
Intermediate Filaments ◽  
Intermediate Filament ◽  
Immunohistochemical Staining ◽  
Neuron Specific Enolase ◽  
Acidic Protein ◽  
Gfap Expression ◽  
S 100

ABSTRACT:Fourteen pure oligodendrogliomas were studied by light- and electronmicroscopy and immunohistochemistry to examine glial fibrillary acidic protein (GFAP) positivity in the tumors. To compare the immunohistochemical staining patterns of neoplastic oligodendroglia and immature oligodendroglia, myelination glia in the white matter of eight normal brains from children under 6 months of age were studied. The tumors possessed light microscopic and ultrastructural features characteristic of oligodendrogliomas. Microtubules were found in the cytoplasm of nine tumors on electronmicroscopy. In one, intermediate filaments and microtubules were observed in occasional tumor cells with polygonal crystalline structures in the cytoplasm. Using the peroxidase-antiperoxidase technique, all specimens were stained for GFAP, vimentin, S-100 and neuron-specific enolase (NSE). In nine tumors, variable numbers of cells with an oligodendroglial morphology reacted positively for GFAP. All tumors were positive for S-100 and negative for vimentin and NSE. The myelination glia in the eight normal brains stained positively for GFAP but not for vimentin. Vimentin is expressed by developing, reactive and neoplastic astrocytes. Thus, GFAP positivity combined with vimentin negativity in both neoplastic and immature oligodendroglia suggests that GFAP positivity in oligodendrogliomas may reflect the transient expression of this intermediate filament by immature oligodendroglia.

scholarly journals Spinal cord hemangioblastomas with a focus on clinical presentation, diagnosis, and treatment at a tertiary care hospital of Karachi, Pakistan: A retrospective chart review

2021 ◽  
Vol 12 ◽  
pp. 24
Author(s):  
Syed Sarmad Bukhari ◽  
Muhammad Ehsan Bari ◽  
Nasir Ud Din ◽  
Zubair Ahmad
Keyword(s):  
Spinal Cord ◽  
Stromal Cells ◽  
Tertiary Care ◽  
Neuron Specific Enolase ◽  
Retrospective Chart Review ◽  
Vascular Tumors ◽  
Care Hospital ◽  
Mitotic Figures ◽  
Cluster Of Differentiation

Background: Hemangioblastomas are benign neoplasms that consist of stromal cells and small blood vessels. They are highly vascular tumors and can arise throughout the central nervous system. This study aims to provide an overview of our experience with this rare tumor’s presentation, radiology, histopathology, and outcomes as literature regarding this pathology is sparse from our country. Methods: The study is a retrospective review of cases that were histopathology proven cases of spinal cord hemangioblastomas. The clinical characteristics of these patients were examined, and their presentation was recorded. The radiology was also reviewed to describe classic appearance on magnetic resonance imaging. A detailed review of immunohistochemistry was also performed and outcome was described. Results: A total of 25 cases of spinal hemangioblastomas were found in our records in the period of 2001–2019. There were 20 males (80%) and only 5 female patients (20%). Gross tumor fragments ranged in size from 0.24 cm2 to 10.5 cm2 (mean 3.28 ± 2.65). Histologically, tumor was composed of nests of large stromal cells with clear to vacuolated cytoplasm separated by thin-walled capillaries. Focal intratumoral hemorrhage was noted. No significant cytological atypia or mitotic figures were noted. Immunohistochemical stains were performed to confirm the diagnosis and exclude other tumors. Inhibin was tested in 20 cases and it was positive in 16 cases (80%). Neuron-specific enolase was positive in 6/8 cases. Cluster of differentiation (CD) CD68 was positive in 6/6 cases and vimentin in 4/4 cases. Glial fibrillary acidic protein (GFAP) and epithelial membrane antigen were performed in 14 and 8 cases, respectively, and all were negative. Cytokeratin AE1/AE3 was negative in 13/13 cases. CD34 highlighted vasculature in the 8 cases in which it was performed and was negative in tumor cells. Follow-up was available in 17 out of 25 cases and ranged from 12 months to 216 months (mean 61.8 ± 60.6 months). Recurrence occurred in 2 out of 17 (11.7%) patients for whom follow-up information was available. Conclusion: Our experience shows that spinal cord hemangioblastomas can be surgically removed in most cases with a low risk of recurrence. Most patients in our study were male and unlike other studies, none of our cases showed GFAP positivity.

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