Rare aggressive solid pseudopapillary neoplasm of the ovary with metastatic disease following surgical resection

Extra-pancreatic solid pseudopapillary neoplasms (SPNs) are rare tumours with an overall favourable prognosis and low malignant potential. SPNs with metastatic spread, distant lymph node metastasis and extrapancreatic origin are exceedingly rare. Significant controversy regarding the treatment and the management of metastatic disease exists and, currently, there are no standardised guidelines or treatment recommendations for the use of adjuvant therapy. In this case report, the authors present a patient with widely metastatic SPN of likely ovarian origin with the invasion of the inguinal lymph nodes and multiple abdominal metastatic deposits. Using the currently available literature, the authors discuss treatment options for metastatic SPN of the ovary and highlight the need for continued research in this important field.

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Solid Pseudopapillary Neoplasm of the Pancreas: A Rare Entity With Unique Features

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2016-0322-rs ◽

2017 ◽

Vol 141 (7) ◽

pp. 990-995 ◽

Cited By ~ 23

Author(s):

Peyman Dinarvand ◽

Jinping Lai

Keyword(s):

Tumor Cells ◽

Malignant Potential ◽

Nuclear Staining ◽

Rare Entity ◽

Solid Pseudopapillary Neoplasm ◽

Cytoplasmic Vacuoles ◽

Clinical Presentations ◽

Low Malignant Potential ◽

Hyaline Globules ◽

Definite Treatment

Solid pseudopapillary neoplasm of the pancreas is a rare entity with low malignant potential and excellent overall prognosis. It has nonspecific clinical presentations such as abdominal pain and nausea, with vague radiologic features. Histologic features of this neoplasm are usually specific. The tumor shows minimally cohesive, uniform, monotonous cells lining delicate capillary-sized blood vessels, described as pseudopapillary architecture. Other features including hyaline globules, cytoplasmic vacuoles, and nuclear grooving are frequently present. Use of a select panel of immunostains always helps pathologists to differentiate this tumor from other circumscribed tumors of the pancreas. Recently, β-catenin, CD10, and E-cadherin have been shown to be very important in the diagnosis of solid pseudopapillary neoplasm. Nuclear staining of tumor cells by β-catenin and membranous presentation of CD10 is seen in almost 100% of cases. Tumor cells can be partially positive for synaptophysin and chromogranin. This tumor has a low malignant potential, and definite treatment is surgical resection.

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Molecular Targets and Novel Therapeutic Avenues in Soft-Tissue Sarcoma

Current Oncology ◽

10.3747/co.27.5631 ◽

2020 ◽

Vol 27 (11) ◽

pp. 34-40 ◽

Cited By ~ 2

Author(s):

A. Elkrief ◽

T. Alcindor

Keyword(s):

Soft Tissue ◽

Soft Tissue Sarcoma ◽

Metastatic Disease ◽

Treatment Options ◽

Molecular Targets ◽

First Line ◽

Standard Chemotherapy ◽

Disease Outcomes ◽

Rare Tumours ◽

Line Chemotherapy

Soft-tissue sarcoma (sts) represents a heterogeneous group of rare tumours, and a significant number of affected patients will develop metastatic disease. Outcomes in the population with metastatic disease are generally poor, especially after progression on standard chemotherapy. The advent of personalized medicine has permitted oncologists to offer targeted treatment, thus addressing the limited treatment options and poor prognosis after progression on first-line chemotherapy. In this review, we delineate the existing data and therapeutic successes with respect to existing and emerging molecular targets in sts and options for immunotherapy in sts. Our review also summarizes emerging clinical trials that are currently recruiting patients.

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Ovarian papillary serous tumors of low malignant potential (Serous borderline tumors): A long term follow-up study, including patients with microinvasion, lymph node metastasis, and transformation to invasive serous carcinoma

Cancer ◽

10.1002/(sici)1097-0142(19960715)78:2<278::aid-cncr14>3.0.co;2-t ◽

1996 ◽

Vol 78 (2) ◽

pp. 278-286 ◽

Cited By ~ 121

Author(s):

Alexander W. Kennedy ◽

William R. Hart

Keyword(s):

Malignant Potential ◽

Serous Carcinoma ◽

Borderline Tumors ◽

Node Metastasis ◽

Follow Up Study ◽

Low Malignant Potential ◽

Long Term Follow Up ◽

Serous Tumors

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Current controversies in the biology and management of ovarian tumors of low malignant potential

The Women s Oncology Review ◽

10.3109/14733400600604448 ◽

2007 ◽

Vol 6 (1-2) ◽

pp. 15-25

Author(s):

William E. Winter ◽

Douglas N. Brown ◽

Charles A. Leath

Keyword(s):

Malignant Potential ◽

Ovarian Tumors ◽

Low Malignant Potential

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Hypoxia-inducible Factor 2α: A Key Player in Tumorigenesis and Metastasis of Pheochromocytoma and Paraganglioma?

Experimental and Clinical Endocrinology & Diabetes ◽

10.1055/a-1526-5263 ◽

2021 ◽

Author(s):

Nicole Bechmann ◽

Graeme Eisenhofer

Keyword(s):

Extracellular Matrix ◽

Signaling Pathways ◽

Metastatic Disease ◽

Therapeutic Intervention ◽

Treatment Options ◽

Hypoxia Inducible Factor ◽

Germline Mutations ◽

Driver Mutations ◽

Mesenchymal Transition ◽

Therapeutic Relevance

AbstractGermline or somatic driver mutations linked to specific phenotypic features are identified in approximately 70% of all catecholamine-producing pheochromocytomas and paragangliomas (PPGLs). Mutations leading to stabilization of hypoxia-inducible factor 2α (HIF2α) and downstream pseudohypoxic signaling are associated with a higher risk of metastatic disease. Patients with metastatic PPGLs have a variable prognosis and treatment options are limited. In most patients with PPGLs, germline mutations lead to the stabilization of HIF2α. Mutations in HIF2α itself are associated with adrenal pheochromocytomas and/or extra-adrenal paragangliomas and about 30% of these patients develop metastatic disease; nevertheless, the frequency of these specific mutations is low (1.6–6.2%). Generally, mutations that lead to stabilization of HIF2α result in distinct catecholamine phenotype through blockade of glucocorticoid-mediated induction of phenylethanolamine N-methyltransferase, leading to the formation of tumors that lack epinephrine. HIF2α, among other factors, also contributes importantly to the initiation of a motile and invasive phenotype. Specifically, the expression of HIF2α supports a neuroendocrine-to-mesenchymal transition and the associated invasion-metastasis cascade, which includes the formation of pseudopodia to facilitate penetration into adjacent vasculature. The HIF2α-mediated expression of adhesion and extracellular matrix genes also promotes the establishment of PPGL cells in distant tissues. The involvement of HIF2α in tumorigenesis and in multiple steps of invasion-metastasis cascade underscores the therapeutic relevance of targeting HIF2α signaling pathways in PPGLs. However, due to emerging resistance to current HIF2α inhibitors that target HIF2α binding to specific partners, alternative HIF2α signaling pathways and downstream actions should also be considered for therapeutic intervention.

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Comparison of clinicopathological features and long-term prognosis between mixed predominantly differentiated-type and pure differentiated-type early gastric cancer

BMC Cancer ◽

10.1186/s12885-021-07962-x ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Yutaka Okagawa ◽

Tetsuya Sumiyoshi ◽

Hitoshi Kondo ◽

Yusuke Tomita ◽

Takeshi Uozumi ◽

...

Keyword(s):

Gastric Cancer ◽

Early Gastric Cancer ◽

Vascular Invasion ◽

Survival Rates ◽

Malignant Potential ◽

Clinicopathological Features ◽

Node Metastasis ◽

Group 5 ◽

Long Term Prognosis

Abstract Background Recent studies have shown that mixed predominantly differentiated-type (MD) early gastric cancer (EGC) might have more malignant potential than pure differentiated-type (PD) EGC. However, no study has analyzed all differentiated-type EGC cases treated endoscopically and surgically. This study aimed to compare the differences in clinicopathological features and long-term prognosis between MD- and PD-EGC. Methods We evaluated all patients with differentiated-type EGCs who were treated endoscopically and surgically in our hospital between January 2010 and October 2014. The clinicopathological features and long-term prognosis of MD-EGC were compared with those of PD-EGC. Results A total of 459 patients with 459 lesions were evaluated in this study; of them, 409 (89.1%) and 50 (10.9%) were classified into the PD and MD groups, respectively. Submucosal invasion was found in 96 (23.5%) patients of the PD group and in 33 (66.0%) patients of the MD group (p < 0.01). The rates of positive lymphatic and vascular invasion and ulceration were significantly higher in the MD group than in the PD group (p < 0.01). The proportion of patients with lymph node metastasis was also significantly higher in the MD group than in the PD group (5 (10%) vs 6 (1.5%), p < 0.01). The 5-year overall and EGC-specific survival rates in the PD group were 88.3 and 99.5%, respectively, while they were 94.0 and 98.0% in the MD group, respectively. Conclusions MD-EGC has more malignant potential than PD-EGC. However, the long-term prognosis of MD-EGC is good and is not significantly different from that of PD-EGC when treated appropriately.

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