Aggressive Variant of a Solid Pseudopapillary Neoplasm: A Case Report and Literature Review

2014 ◽  
Vol 138 (7) ◽  
pp. 974-978 ◽  
Author(s):  
Bailey A. Reindl ◽  
Douglas W. Lynch ◽  
Ali D. Jassim
Keyword(s):  
Adolescent Girl ◽  
Cellular Differentiation ◽  
Clinical Course ◽  
Solid Pseudopapillary Neoplasm ◽  
Proliferative Index ◽  
Young Females ◽  
Pathologic Features ◽  
Extensive Necrosis ◽  
Aggressive Variant ◽  
Solid Pseudopapillary Neoplasms

Solid pseudopapillary neoplasm, a lesion of uncertain cellular differentiation, is an unusual tumor of the pancreas with an indolent clinical course that typically arises in young females. We report a case of solid pseudopapillary neoplasm arising in a 17-year-old adolescent girl who presented with progressive abdominal pain. The patient underwent surgical resection of an 18 × 14 × 8-cm pancreatic mass that displayed the usual histologic features of a solid pseudopapillary neoplasm in addition to prominent nuclear atypia, increased proliferative index, and extensive necrosis. These unusual histologic findings are rare and are of particular interest owing to the dramatically decreased survival time displayed in this case. Although precise pathologic criteria suggesting a high risk for aggressive behavior of solid pseudopapillary neoplasms are uncertain, recognition of the unusual pathologic features displayed in this case may be useful in the prediction of potentially more aggressive neoplasms that portend a poorer prognosis.

Necrotizing Meningoencephalitis of Maltese Dogs

1995 ◽  
Vol 32 (3) ◽  
pp. 230-235 ◽  
Author(s):  
I. H. Stalis ◽  
B. Chadwick ◽  
B. Dayrell-Hart ◽  
B. A. Summers ◽  
T. J. Van Winkle
Keyword(s):  
Cerebrospinal Fluid ◽  
White Matter ◽  
Clinical Course ◽  
Clinical Signs ◽  
Lateral Ventricles ◽  
Pathologic Features ◽  
Cerebrospinal Fluid Examination ◽  
History Of ◽  
Extensive Necrosis

The clinical and pathologic features of five young Maltese dogs with a necrotizing meningoencephalitis were studied and compared with published reports of the necrotizing meningoencephalitis of Pug dogs. The ages of the Maltese dogs ranged from 9 months to 4 years. Four dogs were male, and one was female. The dogs had a history of seizures with or without other neurologic signs for 3 days to 20 weeks prior to death. Cerebrospinal fluid examination in three dogs revealed a pleocytosis and elevated levels of protein. At necropsy, the cerebrum was asymmetrically swollen in four dogs, with a loss of distinction between the gray and white matter and mild to moderate asymmetrical dilation of the lateral ventricles. Histologically, there was extensive necrosis and nonsuppurative inflammation of the cerebral gray and white matter, overlying meninges, and adjacent thalamus and hippocampus. The 4-year-old dog had the longest duration of clinical signs and had little inflammation but extensive atrophy of affected areas, with astrocytosis. The clinical course and pathologic changes in these Maltese dogs are indistinguishable from those in reported cases of necrotizing meningoencephalitis of Pug dogs, indicating that this lesion is probably not unique to Pug dogs.

scholarly journals Multidisciplinary treatment of advanced or recurrent solid pseudopapillary neoplasm of the pancreas: three case reports

2022 ◽  
Vol 8 (1) ◽  
Author(s):  
Kiyonori Tanoue ◽  
Yuko Mataki ◽  
Hiroshi Kurahara ◽  
Tetsuya Idichi ◽  
Yota Kawasaki ◽  
...  
Keyword(s):  
Pancreatic Tumor ◽  
Clinical Course ◽  
Case Reports ◽  
Combined Therapy ◽  
Multidisciplinary Treatment ◽  
Solid Pseudopapillary Neoplasm ◽  
Case Presentation ◽  
Young Females ◽  
Long Time ◽  
Arterial Chemoembolization

Abstract Background Solid pseudopapillary neoplasm (SPN) is a rare pancreatic tumor that predominantly affects young females. Prognosis is excellent; however, 10–15% of patients show metastasis at the time of surgery or develop tumor recurrence after pancreatectomy. Case presentation We reviewed the clinical course of three patients with advanced or recurrent SPN and subsequently underwent multidisciplinary treatment at our institution between 2002 and 2019. The primary tumor was resected in all three patients, and metastases were also resected if indicated. Intensive combined therapy, including re-resection, chemotherapy, ablation, arterial chemoembolization, and radiation therapy, allowed all patients to survive for a long time. The literature review showed that resection seems to be more effective than other treatments for metastatic SPN. Conclusions Multidisciplinary treatment, including resection, may improve the prognosis of patients with SPN with recurrence or metastasis.

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

2020 ◽  
Vol 13 (12) ◽  
pp. e238136
Author(s):  
Bradley S Kushner ◽  
Deyali Chatterjee ◽  
Chet Hammill
Keyword(s):  
Metastatic Disease ◽  
Treatment Options ◽  
Malignant Potential ◽  
Solid Pseudopapillary Neoplasm ◽  
Node Metastasis ◽  
Distant Lymph Node Metastasis ◽  
Rare Tumours ◽  
Low Malignant Potential ◽  
Important Field ◽  
Solid Pseudopapillary Neoplasms

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.

scholarly journals Clinicopathologic Diagnostic Approach to Aggressive Variant Prostate Cancer

2019 ◽  
Vol 144 (1) ◽  
pp. 18-23 ◽  
Author(s):  
Varsha Manucha ◽  
John Henegan
Keyword(s):  
Prostate Cancer ◽  
English Language ◽  
Treatment Strategies ◽  
Castration Resistant Prostate Cancer ◽  
Ongoing Research ◽  
Castration Resistant ◽  
Current Review ◽  
Pathologic Features ◽  
Neuroendocrine Carcinomas ◽  
Aggressive Variant

Context.— Aggressive variant prostate cancer (AVPCa) develops in a subset of patients with metastatic castration-resistant prostate cancer. The clinical and histologic overlap of AVPCa with other neuroendocrine carcinomas of the prostate has resulted in a lack of consensus on its terminology and treatment. Objective.— To review AVPCa to familiarize pathologists with this entity so they can actively participate in the detection, ongoing research, and evolving management of AVPCa. Data Sources.— The English language literature was reviewed. Conclusions.— The current review summarizes the pathologic features of AVPCa, describes how it has been defined clinically, and discusses how biomarkers may inform treatment strategies in the future.

scholarly journals Pancreatic Solid Pseudopapillary Neoplasm: Key Pathologic and Genetic Features

2020 ◽  
Vol 144 (7) ◽  
pp. 829-837 ◽  
Author(s):  
Stefano La Rosa ◽  
Massimo Bongiovanni
Keyword(s):  
Good Prognosis ◽  
Low Grade ◽  
Solid Pseudopapillary Neoplasm ◽  
Prognostic Features ◽  
Molecular Alterations ◽  
Immunohistochemical Markers ◽  
Molecular Features ◽  
Pubmed Database ◽  
Genetic Features ◽  
Solid Pseudopapillary Neoplasms

Context.— Solid pseudopapillary neoplasm of the pancreas is a low-grade malignant tumor generally associated with a good prognosis. Solid pseudopapillary neoplasms show peculiar morphologic features, but sometimes the differential diagnosis with other pancreatic neoplasms (ie, pancreatic neuroendocrine tumors) can be a challenging task, especially in cytologic or biopsy specimens. In these cases immunohistochemistry is a useful tool, but the diagnostic utility of several proposed immunohistochemical markers is questionable. In recent years, despite several attempts to characterize the pathogenetic, molecular, and prognostic features of solid pseudopapillary neoplasms, they still remain unclear. Objective.— To give the reader a comprehensive update on this entity. Data Sources.— The PubMed database (US National Library of Medicine) was searched using the following string: pseudopapillary tumor [AND/OR] neoplasm [AND/OR] pancreas. All articles written in English were included. In addition, because a heterogeneous terminology has been used in the past to define solid pseudopapillary neoplasms, the reference lists of each paper selected in the PubMed database were also reviewed. Conclusions.— This review gives a comprehensive update on the pathologic, clinical, and molecular features of solid pseudopapillary neoplasms, particularly addressing issues and challenges related to diagnosis. In addition, we have tried to correlate the molecular alterations with the morphologic and clinical features.

Small renal neoplasms: diagnostic imaging, pathologic features, and clinical course.

Radiology ◽  
1986 ◽  
Vol 158 (1) ◽  
pp. 113-117 ◽  
Author(s):  
N S Curry ◽  
S I Schabel ◽  
W L Betsill
Keyword(s):  
Diagnostic Imaging ◽  
Clinical Course ◽  
Renal Neoplasms ◽  
Pathologic Features

Clear Cell Variant of Solid Pseudopapillary Neoplasm of the Pancreas: A Report of a Rare Variant and Review of the Literature

2019 ◽  
Vol 27 (5) ◽  
pp. 535-540
Author(s):  
Cristina Costales ◽  
Arjun Mehta ◽  
Sujit Kulkarni ◽  
Brent K. Larson
Keyword(s):  
Rare Variant ◽  
Clear Cell ◽  
Cell Tumor ◽  
Solid Pseudopapillary Neoplasm ◽  
Review Of The Literature ◽  
Pathologic Features ◽  
Cell Variant ◽  
Important Variant ◽  
Clear Cell Variant

The clear cell variant of solid pseudopapillary neoplasm (ccSPN) of the pancreas was first described in 2006. In this article, we report a case of this rare variant and review the few published reports. Both the current and previous reports show that ccSPN has several morphologic differences from conventional SPN, including clear vacuoles, fewer pseudopapillary formations, more solid/diffuse architecture, less hemorrhage, and fewer cholesterol clefts. Some of these features peculiar to ccSPN, such as solid/diffuse architecture, have been proposed to suggest aggressive behavior, though reports of ccSPN are rare and often have limited clinical follow-up. ccSPN also appears to occur more frequently in males than conventional SPNs. These clinical and pathologic features lead to unique set of differential diagnostic considerations for ccSPN, including metastatic renal cell carcinoma, perivascular epithelial cell tumor, and clear cell variants of other carcinomas. These unique features, atypical differential, and uncertain prognostic ramifications all make ccSPN an important variant to be aware of and report.

VARIANT OF RARE HERMANSKY — PUDLAK SYNDROME ASSOCIATED WITH GRANULOMATOUS COLITIS: DIAGNOSTICS, CLINICAL COURSE AND TREATMENT

2018 ◽  
Vol 40 (1) ◽  
pp. 73-78 ◽  
Author(s):  
L Y Lozynska ◽  
A Plawski ◽  
M R Lozynska ◽  
I Vytvytskyi ◽  
R Y Lozynskyi ◽  
...  
Keyword(s):  
Crohn’S Disease ◽  
Crohn's Disease ◽  
Clinical Course ◽  
Molecular Genetic ◽  
Granulomatous Colitis ◽  
Vdr Gene ◽  
Adequate Treatment ◽  
Pathologic Features ◽  
Paraovarian Cyst ◽  
Hermansky Pudlak Syndrome

Aim: To study the relationship between the genotype and the phenotype in the patients with Hermansky — Pudlak syndrome (HPS) associated with granulomatous colitis; to monitor clinical course of the disease for adequate treatment, cancer surveillance and genetic counseling. Materials and Methods: The diagnosis of HPS is established by physical examination, chest X-ray, computed tomography, endoscopic examination with biopsy, and laboratory tests, including histology, baseline laboratory blood, urine and feces tests, determination of ASCA-C and ANCA antibodies using an ELISA. Molecular genetic testing for HPS gene mutations, R702W, G908R, L1007fs and P268S mutations in NOD2 gene, and TaqI variant of the VDR gene were carried out. Results: We report 2 cases of HPS from unrelated families. Both were complicated by inflammatory bowel disease with pathologic features of Crohn’s disease refractory to antibiotics and corticosteroids. One patient (family 1) with Ashkenazi Jewish ancestry had pathogenic variant of the HPS-4 gene in exon 8, mutation P268S of NOD2 genes and “Tt” genotype of TaqI variant of the VDR gene. Another patient (family 2) carried two mutations P268S and G908R of NOD2 gene, and had a large paraovarian cyst diagnosed. No consistent success with the standard medical therapy, used for treating granulomatous colitis, associated with HPS, in presented cases was achieved. Patients needed surgical interventions at a young age and a long-term surveillance of the probable development of tumors and other complications. Azathioprine at 2 mg/kg/day and mesalazine 3 g/day were used with some positive effect for prevention of Crohn’s disease postoperative recurrence. Conclusion: The occurrence of perianal lesions, the histopathological findings and the results of the molecular genetic analysis confirmed the mutations P268S and G908R of NOD2 gene in these cases suggest that HPS was truly associated with Crohn’s disease variant with early onset and severe course. The search for the molecular causes of the disease in some individuals may help in the development of new therapeutic and surgical approaches, as well in the improvement of understanding of premalignant inflammatory conditions in a large bowel.

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