Metastatic Malignant Paraganglioma: A Case Report

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S69-S70
Author(s):  
Suhalika Sahni ◽  
Vikas Mehta
Keyword(s):  
Liver Biopsy ◽  
Tumor Cells ◽  
Metastatic Disease ◽  
Neurofibromatosis Type ◽  
Ki 67 ◽  
Malignant Paraganglioma ◽  
Fibrous Stroma ◽  
Von Hippel Lindau ◽  
Sustentacular Cells ◽  
Eosinophilic Cytoplasm

Abstract Objectives Paragangliomas are rare neuroendocrine tumors that arise from chromaffin cells with the ability to secrete catecholamines. The estimated combined prevalence is between 1:6,500 and 1:2,500. Paragangliomas can have a benign or a malignant course with incidence of malignant paraganglioma estimated to be 93/400 million. The majority of paragangliomas are sporadic but may be related to syndromes like multiple endocrine neoplasia type 2, von Hippel-Lindau disease, and neurofibromatosis type 1. Methods We present a unique case of a 25-year-old female who presented with a hepatic mass. Further workup showed an enhancing hypervascular mass along with lesions within the bilateral lungs, liver, spleen, and sclerotic osseous metastatic disease at cervical thoracic lumbar and sacral spine. A liver biopsy was performed, which was consistent with the diagnosis of a metastatic paraganglioma. Results The liver biopsy showed a tumor consisting of epithelioid and polygonal tumor cells with eosinophilic cytoplasm present in a vascularized fibrous stroma in a sinusoidal pattern. In certain areas, the tumor cells show marked pleomorphism with large cells with macronuclei and bizarre shapes. On immunohistochemistry (IHC), the tumor cells were strongly and diffusely positive for CD56 and vimentin and focally positive for synaptophysin and chromogranin. S100 highlighted sustentacular cells. Ki-67 was very low, staining less than 2%. In addition, the tumor cells showed loss of expression of SDHB by IHC. Conclusion Metastatic malignant nonsecretory paraganglioma is an extremely rare disease. The most unique pathological aspect of paragangliomas is that tissue acquired from different organ appears the same histologically and yet different in terms of prognosis. Metastatic hepatic paraganglioma in our patient offers interesting insights to appreciate the rare metastatic nature of paraganglioma to the liver. The elusive nature of the metastatic disease in paraganglioma poses a challenge to clinicians since the diagnosis of metastatic hepatic paraganglioma is usually clinically undiagnosed.

Multifocal Pancreatic Neuroendocrine Neoplasms In Tuberous Sclerosis: A Rare Case

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S77-S77
Author(s):  
N C Jadhav ◽  
D L Gang
Keyword(s):  
Tuberous Sclerosis ◽  
Neurofibromatosis Type ◽  
Abdominal Ultrasound ◽  
Biologically Active ◽  
Pancreatic Tumors ◽  
Neuroendocrine Neoplasms ◽  
Ki 67 ◽  
Pancreatic Neuroendocrine Neoplasms ◽  
Von Hippel Lindau ◽  
Multifocal Disease

Abstract Casestudy: Pancreatic neuroendocrine neoplasms (PanNEN) are rare accounting for 2-5% of pancreatic tumors. Although mostly sporadic, 10-20% are associated with inherited syndromes, notably MEN-1, Von Hippel- Lindau disease, neurofibromatosis type 1, and tuberous sclerosis (TS). When compared to sporadic cases, PanNEN in hereditary syndromes occur at a younger age, are often multifocal, cystic, and may show characteristic microscopic patterns. TS is an autosomal dominant multi-system disorder with mutations involving TSC1 or TSC2 genes which function as tumor suppressors by inhibiting mTORC1 kinase. PanNEN is observed in 1.5-1.8% of patients with TS and no surveillance guidelines for the assessment of pancreatic lesions are established. Compared to other syndromes, PanNEN associated with TS are solitary. To our knowledge, only two cases of multifocal PanNEN in TS patients have been reported. We present a case of a 67-year-old gentleman with a history of TS also affecting two daughters. He presented to the emergency department with severe abdominal pain. Abdominal ultrasound suggested acute appendicitis and an incidental 2.0 cm solid lesion was noted in the head of the pancreas. Follow-up MRI revealed two additional non-cystic masses in the pancreatic tail. Endoscopic ultrasound-guided biopsy of a tail lesion revealed monomorphic tumor cells with stippled chromatin without cytologic atypia. Immunohistochemical staining was positive for synaptophysin and chromogranin. Ki-67 labelling index was under 1%. Diagnosis of a well-differentiated neuroendocrine tumor (G1) was made. The patient denied symptoms of the carcinoid syndrome and no biologically active hormones were detected. Gallium PET scan revealed multiple foci of radiotracer uptake throughout the pancreas in addition to those described on MRI. Although PanNEN are rare in TS, malignant behavior has been reported. This case reinforces the importance of early detection through active surveillance, especially as surgical options may be limited in multifocal disease.

scholarly journals A Rare Primary Tumor of the Thyroid Gland: A New Case of Leiomyoma and Literature Review

2018 ◽  
Vol 12 ◽  
pp. 117955491881353
Author(s):  
Yanling Zhang ◽  
Heng Tang ◽  
Huaiyuan Hu ◽  
Xiang Yong
Keyword(s):  
Thyroid Gland ◽  
Tumor Cells ◽  
Immunohistochemical Staining ◽  
Smooth Muscle Actin ◽  
Frozen Sections ◽  
Ki 67 ◽  
Eosinophilic Cytoplasm ◽  
S 100 ◽  
Painless Mass ◽  
The Right

Primary leiomyomas of the thyroid are very rare. We here report a case of a 53-year-old woman with a painless mass at the right thyroid, revealed by physical examination. The patient underwent a lobectomy. Frozen sections showed a spindle cell tumor of the thyroid gland. The nuclei of some of the tumor cells were obviously enlarged and deeply stained. Pseudocapsule invasion was observed in small foci. Samples showed neither mitosis nor necrosis and the nature of the tumor was difficult to determine. Paraffin sections showed a well-circumscribed nodular composed of intersecting fascicles of spindled to slightly epithelioid cells with eosinophilic cytoplasm and blunt-ended, cigar-shaped nuclei. We observed no significant nuclear atypia, mitotsis, or necrosis. Immunohistochemical staining showed the tumor cells to be positive for α-smooth muscle actin and h-caldesmon but negative for TG, TTF1, PAX8, S-100, CT, CK, and CD34. The ki-67 index was very low (<1%). Primary thyroid leiomyoma is rare and difficult to diagnose using frozen sections. Diagnosis requires immunohistochemical staining. Leiomyoma may be mistaken for other thyroid tumors also characterized by spindle cells.

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 An ectopic adrenocortical oncocytic adenoma in the liver highly mimicking hepatocellular carcinoma: case report and literature review

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Jingci Chen ◽  
Xueshuai Wan ◽  
Yao Lu ◽  
Wenze Wang ◽  
Dachun Zhao ◽  
...  
Keyword(s):  
Hepatocellular Carcinoma ◽  
Tumor Cells ◽  
Correct Diagnosis ◽  
Specific Antigen ◽  
Final Diagnosis ◽  
Arterial Phase ◽  
Adrenocortical Adenoma ◽  
Ki 67 ◽  
Eosinophilic Cytoplasm ◽  
Oncocytic Adenoma

Abstract Background Ectopic adrenocortical tissue is a lesion usually found incidentally during autopsy or inguinal surgery. Here, we demonstrate an extremely unusual case of intrahepatic adrenocortical adenoma which highly mimicks hepatocellular carcinoma (HCC) and brings challenges for clinicians and pathologists. The diagnostic pitfalls have been discussed in detail to provide clues for guiding differential diagnosis and future treatment. Case presentation A 44-year-old man was admitted into our hospital for evaluation of a hepatic mass identified during routine examination. Enhanced CT revealed its margin displayed apparent enhancement in arterial phase, but hypointensity in portal and delayed phase. HCC was suspected and partial hepatectomy was performed. Microscopically, cells were arranged in solid sheets. Most of the tumor cells were large, polygonal, had prominent nucleoli and were rich in eosinophilic cytoplasm. Pleomorphic nucleus was frequently found. Focally, smaller cells were found with small nuclei and granular cytoplasm. Immunohistochemically, tumor cells were negative for Arg-1, glypican-3 (GPC3), hepatocyte specific antigen (HSA), and positive for synaptophysin (Syn), α-inhibin, and Melan A. The Ki-67 index was 1 %. The final diagnosis was ectopic adrenocortical oncocytic adenoma and the patient was uneventful after the surgery. Conclusion Intrahepatic adrenocortical adenoma in the liver can hardly be diagnosed through radiology and little experience in pathology has been reported. In the present case, massive oncocytic changes and huge pleomorphism add greatly to the difficulties of making correct diagnosis. This lesion should be carefully kept in mind and a combination of markers is suggested for differentiating from HCC.

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.

Prognostic subset of metastatic and non-metastatic luminal B breast cancer (LBBC) subtype based on Ki67 index.

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. e12570-e12570
Author(s):  
Lalnun Puii ◽  
Lalram Sangi ◽  
Hrishi Varayathu ◽  
Samuel Luke Koramati ◽  
Beulah Elsa Thomas ◽  
...  
Keyword(s):  
Breast Cancer ◽  
Metastatic Disease ◽  
Ki67 Index ◽  
Breast Cancer Patients ◽  
Luminal A ◽  
Her2 Positive ◽  
Ki 67 ◽  
Luminal B ◽  
Therapeutic Implications ◽  
Significant Difference

e12570 Background: Gene expression profiling for breast cancer has classified ER positive subtype into luminal A and luminal B. Luminal B breast cancer (LBBC) have a higher proliferation and poorer prognosis than luminal A tumors. Ki-67 index is the commonly used proliferation marker in breast cancer; however Ki67 expression can also be used to identify a subset of patients among LB with a favorable prognosis. This study attempts to verify this subset of LBBC patients based on DFS and PFS in non-metastatic and metastatic patients respectively. Methods: We retrospectively analyzed 80 IDC breast cancer patients diagnosed in 2013-2016 with complete follow-up till January-2021. We defined LBBC as ER+, PR+ or PR- , HER2+ or HER2- with a Ki67 index >20%. PFS was considered as the endpoint in patients presenting with metastatic disease whereas DFS was used in non-metastatic disease. The cut-off for ki67 was calculated using an X-tile plot (version 3.6.1, Yale University) by dividing Ki67 data into two populations: low and high, with randomized 1:1 “training” and “validation” cohorts. Results: Median age was 51.5 years. 18.7% (n=15) presented with metastasis at the time of diagnosis and their overall median PFS was found to be 25.8 months. The incidence of HER2 positive LBBC was found to be 15% (n=12) and none of them were found to be presented with metastasis. Survival and frequency of various sub groups in our study are enlisted in the given table. We estimated a Ki67 cut-off of 30% in patients with upfront metastatic disease and PFS was found to be higher in <30% compared to a Ki67 index >30% (38.9 months vs 19.7 months, p-0.002). Overall median DFS was not achieved in non-metastatic group (Mean DFS: 64.7 months) where as a statistically significant difference was observed in the survival of HER2 positive (median DFS: 53.5 months, mean DFS: 50.9) than HER2 negative patients (median DFS not achieved, mean: 66.97 months) ( p-0.021). We obtained a Ki67 cut-off of 32% in non- metastatic group and mean DFS was found to be higher in Ki67<32% (69 months) compared to Ki67>32% (61.4 months), however it failed to exhibit a statistically significant relationship ( p-0.373). Conclusions: Our study indicates that a subset of patients exists within metastatic and non-metastatic LBBC with differing prognosis based on Ki67. Larger studies are further required to confirm the findings and therapeutic implications.[Table: see text]

scholarly journals Circulating tumor cells: silent predictors of metastasis

F1000Research ◽  
2017 ◽  
Vol 6 ◽  
pp. 1445 ◽  
Author(s):  
LanLan Zhou ◽  
David T. Dicker ◽  
Elizabeth Matthew ◽  
Wafik S. El-Deiry ◽  
R. Katherine Alpaugh
Keyword(s):  
Tumor Cells ◽  
Circulating Tumor Cells ◽  
Metastatic Disease ◽  
Metastatic Breast ◽  
Prognostic Indicator ◽  
Disease Recurrence ◽  
Curative Intent ◽  
Early Detection Of Disease ◽  
Mere Presence ◽  
Intent To Treat

Circulating tumor cells (CTCs) were added to the arsenal of clinical testing in 2004 for three cancer types: metastatic breast, prostate, and colorectal cancer. CTCs were found to be an independent prognostic indicator of survival for these three diseases. Multiple enrichment/isolation strategies have been developed and numerous assay applications have been performed using both single and pooled captured/enriched CTCs. We have reviewed the isolation techniques and touched on many analyses. The true utility of a CTC is that it acts as a “silent” predictor of metastatic disease. The mere presence of a single CTC is an indication that disease has spread from the primary site. Comments and suggestions have been set forth for CTCs and cell-free DNA to be used as a screening panel for the early detection of disease recurrence and metastatic spread, providing the opportunity for early intervention with curative intent to treat metastatic disease.

Correlation between DNA Content, Expression of Ki-67 Antigen of Tumor Cells and Immunophenotype of Lymphocytes from Malignant Pleural Effusions

Tumor Biology ◽  
1998 ◽  
Vol 19 (3) ◽  
pp. 196-204 ◽  
Author(s):  
Jan Sikora ◽  
Grzegorz Dworacki ◽  
Magdalena Trybus ◽  
Halina Batura-Gabryel ◽  
Jan &Zdot&;dot;eromski
Keyword(s):  
Tumor Cells ◽  
Dna Content ◽  
Pleural Effusions ◽  
Ki 67 ◽  
Malignant Pleural Effusions

scholarly journals Prognostic Significance of Substance P/Neurokinin 1 Receptor and Its Association with Hormonal Receptors in Breast Carcinoma

2021 ◽  
Vol 2021 ◽  
pp. 1-11
Author(s):  
Riffat Mehboob ◽  
Syed Amir Gilani ◽  
Amber Hassan ◽  
Sadaf ◽  
Imrana Tanvir ◽  
...  
Keyword(s):  
Breast Carcinoma ◽  
Substance P ◽  
Tumor Cells ◽  
Prognostic Significance ◽  
Positive Staining ◽  
Ki 67 ◽  
Neurokinin 1 Receptor ◽  
Group A ◽  
Neurokinin 1 ◽  
Group B

Expression and immunolocalization of Substance P (SP)/Neurokinin-1 Receptor (NK-1R) in breast carcinoma (BC) patients and its association with routine proliferative markers (ER, PR, HER2/neu, and Ki-67) were evaluated. A cross-sectional study was performed on 34 cases of BC. There were 23 cases of group A (grade III), 8 of group B (grade II), and only 3 cases of group C (grade I). All samples were then processed for SP and NK-1R immunohistochemistry for few cases. 14/23 cases (61%) of group A, 7/8 cases (88%) of group B, and 2/3 (67%) cases of group C were SP positive. Overall, strong staining (≥10% tumor cells), labeled as “+3,” was observed in 9/14 (64.2%) cases of group A and 1/8 (12.5%) cases of group B. Moderate staining labelled as “+2” (in ≥10% tumor cells) was observed in 3/14 (21.4%) cases of group A and 4/8 (50%) cases of group B. Weak positive staining “+1” was observed in only 2/14 (14.28%) cases of group A, 2/8 (25%) cases of group B, and all 2/2 (100%) cases of group C. SP and NK-1R are overexpressed in breast carcinomas, and there is significant association between the grade of tumor and their overexpression.

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