Clinicopathological and immunohistochemical study of 29 cases of solid-pseudopapillary neoplasms of the pancreas in patients under 20 years of age along with detailed review of literature

Abstract Background Pancreatic Solid Pseudopapillary Neoplasms (SPNs) are rare low-grade malignant tumors with a marked preponderance for young females. Objective was to describe the morphology, differential diagnosis, and prognosis of SPNs in patients under 20 years of age and present a detailed review of literature. Methods A total of 29 cases in patients under 20 years of age reported as SPN during the period January 2014 to December 2019, were included in the study. These included 19 resection specimens, 4 incision biopsies and 6 cases received as blocks for second opinion. Hematoxylin and eosin (H&E) slides as well as immunohistochemistry (IHC) slides of all cases were retrieved and reviewed by the authors. TFE3 and Progesterone Receptor were performed retrospectively. Results Twenty-eight of the 29 patients were females. Ages of patients ranged from 12 to 19 years. Nineteen cases were resections. Tail was the commonest location. Mean tumor size was 9.5 cm. In 89.5% cases, tumor was confined to the pancreas. In 2 cases, distant metastasis was present. In 2 cases, extension beyond pancreas was seen. Solid and pseudopapillary areas were seen in all cases while other features were variable. Beta catenin and Cyclin D1 were positive in most cases while TFE3 was positive in 57% cases. Progesterone Receptor (PR) was positive in all 13 cases in which it was performed. Follow up was available in 14 patients. Follow up period ranged from 3 to 70 months. Twelve were alive and well without recurrence or metastasis while 2 were alive with recurrence and metastasis to liver and omentum respectively. Conclusions Although many studies on SPNs have been published, surgeons, oncologists and even pathologists in this part of the world are often not aware of these rare tumors leading to inaccuracies and delays in diagnosis. In addition, this paper focusses on the interesting observation that the majority of SPNs diagnosed in our department during study period occurred in patients under 20 years of age (29 versus 21 in patients over 20). However, clinico-epidemiological, morphologic and prognostic features were similar in both age groups. Possibility of SPNs should always be considered in case of pancreatic neoplasms occurring in patients under 20 years of age as well. We believe that this is a very interesting and helpful study for the clinicians as well as the pathologists.

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Pancreatic Solid Pseudopapillary Neoplasm: Key Pathologic and Genetic Features

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2019-0473-ra ◽

2020 ◽

Vol 144 (7) ◽

pp. 829-837 ◽

Cited By ~ 4

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.

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Epidemiological Study of Adamantinoma from US Surveillance, Epidemiology, and End Results Program: III Retrospective Analysis

Journal of Oncology ◽

10.1155/2020/2809647 ◽

2020 ◽

Vol 2020 ◽

pp. 1-8

Author(s):

Mahmut Nedim Aytekin ◽

Recep Öztürk ◽

Kamil Amer

Keyword(s):

Marital Status ◽

Survival Time ◽

Survival Rates ◽

Age Groups ◽

Tumor Location ◽

Cancer Surveillance ◽

Survival Duration ◽

Low Grade ◽

The Mean

Objective. Adamantinomas are rare low-grade malignant bone tumors. This study aims to describe the demographic characteristics and survival rates of patients suffering from adamantinomas. Methods. The National Institute of Cancer Surveillance, Epidemiology, and Recent Results (SEER) database was used, and patients diagnosed with adamantinoma between 1973 and 2016 were screened. Patients were classified according to sex, age, race/ethnicity, and marital status, and also tumors were classified according to year of diagnosis, laterality, type of treatment, and follow-up. Results. The mean age of patients was 30.8 ± 16.7 (range: 4–75). A total of 92 patients were identified; of these, 43 were females and 49 were males. The mean follow-up period was 138.1 ± 90.3 (range: 1–156) months. Mean survival duration was 287.8 ± 15.4 (95% CI: 257.7–317.9) months. Five- and ten-year survival rates were 98.8% and 91.5%, respectively. Besides, survival time was also observed to be independent of gender, age groups, race, marital status, tumor location, and year of diagnosis. Conclusion. Adamantinoma is a very rare bone tumor that affects the long bones in lower extremities and is more common in men. Five- and 10-year survival prognoses are reasonably satisfactory. Also, survival time is independent of variables such as gender, age, and tumor location.

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IgM-Secreting Diffuse Large B-Cell Lymphoma (DLBCL) Is a Poor Prognostic Subset within the Non-Germinal-Centre-Type (GC-type): An Italian Multicentre Study

Blood ◽

10.1182/blood-2020-135909 ◽

2020 ◽

Vol 136 (Supplement 1) ◽

pp. 30-31

Author(s):

M. Christina Cox ◽

Luigi Marcheselli ◽

Gerardo Musuraca ◽

Maria Cantonetti ◽

Carlo Visco ◽

...

Keyword(s):

Prognostic Factor ◽

Board Of Directors ◽

B Cell Lymphoma ◽

Independent Prognostic Factor ◽

Biological Study ◽

Low Grade ◽

Advisory Committees ◽

Bulky Disease ◽

Prognostic Features

BACKGROUND: In 2014 we identified a new subset of DLBCL, defined as "IgM-secreting" (Cox MC & Di Napoli A , PLOS One 2014). This was characterised by poor prognostic features and outcome as well as frequent central nervous (CNS) system localizations. Furthermore, IgM-secretion, was an independent prognostic factor in multivariate analysis. Here we report on the largest series of IgM-secreting-DLBCL, from a multicentre Italian study. METHODS: The observational and biological study was approved by the Ethical Committee of the AUO Sant'Andrea, Italy. Enrolment criteria were: DLBCL with an associated IgM paraprotein diagnosed between 1st January 2010 and 31st December 2018 (IgM-secreting). Data were collected both prospectively and retrospectively from 17 Centres participating in the study. In addition, histopathology samples were centrally revised for immunohistochemistry (IHC) and FISH analyses. The control group (CTRL) consisted in a series of consecutive DLBCL, without an associated IgM-paraprotein (diagnosed between 01/01/2013 and 30/06/2016, enrolled in the Lymphoma Registry of the Lazio region (ReLLi Network). Last follow-up was carried out on 31st December 2019. RESULTS: 569 DLBCL cases were enrolled: 102 (17.9%) were IgM-secreting; 48 (8.4%) had a non-IgM paraprotein (IgA, IgG, or other), and 414 (72.7%) had no associated paraprotein (CTRL). IgM-secreting cases within the consecutive DLBCL patients enrolled in the ReLLi Registry were 41/466 (8.8%, 95CI 6.4-11.7%) while non IgM-paraprotein DLBCL cases were 11/466 (2.4%, 95CI 1.2-4.2%). The median level of IgM paraprotein was 17gr/L (range: <1-84gr/L); 83/102 (81.3%) were IgMk and 23/102 (22.5%) IgML respectively. The IgM-secreting group differed from the CTRL because the following characteristics were significantly more frequent: 1] age>60 (p=.001); 2] advanced stage (p<.001); 3] PS≥2 (p=.001); 4] LDH>UNL (p=.008) ; 5] ≥2 Extra-nodal sites involved (p<.001) ; 6] IPI 3-5 (p<.001); 7] central nervous system (CNS) involvement at diagnosis or relapse (p<.001); 8] lower rate of complete remission(CR) at the end of induction immunochemotherapy (p<.001). Conversely, no differences were observed for: sex, B-symptoms, HCV and HBV status, bulky disease, age≥80 years, and for transformation from low-grade lymphoma. PATHOLOGICAL AND MOLECULAR FEATURES: Paraffin tissue from 74 CTRL and 69 IgM-secreting was suitable for immunohistochemistry (IHC). The non-GCB subtype, based on Hans algorithm, was prevalent in the IgM-secreting (p=.005). No difference in BCL2 expression alone or in MYC and BCL2 double expression was observed within groups. In 48/63(76%; 95CI: 64-86%) IgM-secreting cases, both the IgM heavy and the corresponding kappa or lambda light chain protein expression were detected in the cytoplasm of the neoplastic clone. FISH analyses for MYC, BCL2 and BCL6 genes rearrangements performed in 25 IgM-secreting cases with either expression of MYC protein or a GC-phenotype showed no evidence of double or triple hits (DH/TH). TREATMENT: in the IgM-secreting group more patients were treated with RCOMP and with less intensive approach than the CTRL (p<.001). SURVIVAL: The median follow-up time was 46 months (95CI= 44-49; range 18-101) with 130 events and an incidence rate x100 person/year of 7.22 (95%CI 6.08-8.58) and a 5-yr OS of 76% (95CI 72-79%). The 5-PFS was 61% (95CI 57-65%). In univariable analysis age>60, B-symptoms, bulky disease, IPI >low risk and IgM-secreting IgM showed a worse survival (all with p<0.001). Also, the IgM-secreting group, showed a worse survival compared to the DLBCL with an associated IgG/IgA paraprotein (p<0.001). Adjusting in multiple Cox regression, IgM-secreting with IPI, gender, bulky and B-symptoms, maintain a higher risk of death either in the all cohort (HR 1.93, 95CI 1.34-2.78, p<0.001) or in patients with age<80 (HR 1.71, 95CI 1.16-2.54, p=0.007). Noteworthy, a survival sub-analysis showed that the 12/69 (17.4%) IgM-secreting with a GC-type had a better OS (9=0.008) and PFS (p=0.002) compared to the 57/69 (82.6%) IgM-secreting with a non-GC-type. CONCLUSION: Our data confirm that IgM-secreting DLBCL: 1) represents a sizable proportion of non-DH DLBCL; 2) have poor prognostic features and 3) have mostly a non-GC phenotype. Furthermore, IgM secretion appears to be an independent prognostic factor for both PFS and OS. Studies to define the biological features of this new subset are ongoing. Disclosures Cantonetti: Mundipharma: Consultancy; Takeda: Consultancy; Vifor: Consultancy; Roche: Consultancy. Re:BerGenBio ASA: Research Funding. Abruzzese:Incyte: Consultancy, Membership on an entity's Board of Directors or advisory committees; Novartis: Consultancy, Membership on an entity's Board of Directors or advisory committees; Pfizer: Consultancy, Membership on an entity's Board of Directors or advisory committees; Bms: Honoraria.

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Ovarian seromucinous tumors: clinicopathological features of 10 cases with a detailed review of the literature

Journal of Ovarian Research ◽

10.1186/s13048-021-00796-y ◽

2021 ◽

Vol 14 (1) ◽

Author(s):

Romana Idrees ◽

Nasir Ud Din ◽

Sabeehudin Siddique ◽

Saira Fatima ◽

Jamshid Abdul-Ghafar ◽

...

Keyword(s):

Who Classification ◽

Borderline Tumor ◽

Review Of Literature ◽

Stromal Invasion ◽

Detailed Review ◽

Mean Size ◽

Ovarian Involvement ◽

Female Genital

Abstract Background The 2014 WHO Classification of ovarian neoplasms introduced a new entity of seromucinous tumors associated with endometriosis. These tumors encompassed a spectrum from benign to malignant and included seromucinous cystadenoma/ cystadenofibroma, seromucinous borderline tumor/atypical proliferative seromucinous tumor and seromucinous carcinoma. However, the 2020 WHO Classification of Female Genital Tumours removed seromucinous carcinomas as a distinct entity and recategorized them as Endometrioid carcinomas with mucinous differentiation. Here we describe clinico-morphologic features of seromucinous tumors recategorizing cases originally diagnosed as seromucinous carcinoma in light of 2020 WHO classification and present detailed review of literature. Methods Slides of seromucinous tumors were reviewed. Special emphasis was given to evaluation of stromal invasion. Follow-up was obtained. Results Ten cases were diagnosed. Mean age was 40 years. Four cases were bilateral. Mean size was 19 cm. Grossly; luminal papillary projections were seen in 6 cases. Tumors demonstrated a papillary architecture with papillae lined by stratified seromucinous epithelium showing nuclear atypia. Stromal invasion was seen in 4 cases. Six cases were reported as borderline seromucinous tumors and 4 cases originally diagnosed as seromucinous carcinoma were recategorized as endometrioid carcinoma with mucinous differentiation on review. Endometriosis was seen in 4 cases. CK7, PAX8 and ER were positive in 7/7 cases. Two cases showed extra-ovarian involvement. Follow up was available in 7 cases. Six patients were alive and well at follow up ranging from 8 to 46 months. Six patients received chemotherapy postoperatively. One patient with carcinoma died of disease 18 months postoperatively. Conclusion In our series, 4 cases were originally diagnosed as seromucinous carcinomas. However, these were recategorized in light of the 2020 WHO Classification of Female Genital tumors as endometrioid carcinomas with mucinous differentiation. Six cases were diagnosed as seromucinous borderline tumors. Thus, majority of cases were borderline in agreement with published literature.

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Orbital Non Hodgkin’s Lymphoma (NHL): A Large Monocentric Study of Initial Characteristics, Natural History and Prognostic Factors.

Blood ◽

10.1182/blood.v104.11.1378.1378 ◽

2004 ◽

Vol 104 (11) ◽

pp. 1378-1378

Author(s):

Meunier Jerome ◽

Lumbroso Livia ◽

Dendale Remy ◽

Vincent-Salomon Anne ◽

Asselain Bernard ◽

...

Keyword(s):

Prognostic Factors ◽

Natural History ◽

Cell Lymphoma ◽

Malignant Tumors ◽

Univariate Analysis ◽

Bone Marrow Involvement ◽

B Cell Lymphoma ◽

Low Grade ◽

High Grade

Abstract Orbital NHL (ocular and/or adnexal involvement) represents less than 1% of all lymphomas but about half of malignant tumors of the eye and/or ocular adnexae. We therefore reviewed all patients treated at the Institut Curie between 1970 and 2003 for a NHL exhibiting initial orbital localization to define their initial characteristics, natural history and prognostic factors. Among 172 patients, 145 cases with completed datas were selected for the study. Pathological review according to the WHO classification showed 52 cases of MALT-type lymphoma (36%), 32 lymphoplasmocytic lymphomas (22%), 14 patients with lymphocytic lymphoma, 7 cases of follicular lymphoma, 13 unspecified low grade lymphomas [namely, 118 cases (82%) of low grade NHL], 22 patients with diffuse large B-cell lymphoma (15%), 2 cases of mantle cell lymphoma, 2 Burkitt’s lymphomas and one T-lymphoblastic lymphoma [namely, 27 cases (18%) of high-grade NHL]. Initial characteristics were: median age 66 years (range 3–96), sex ratio M/F 0.6, B symptoms 6%, PS≥2 in 4% of patients, stages III–IV 31.7%, bone marrow involvement 12%, elevated LDH in 18% and IPI 0-1/2/3/4-5 in 92/28/13 and 1 cases, respectively. Anatomic localizations were intra-orbital in 39 patients (27%), conjunctival in 38 (26%), eyelid in 9 cases, lachrymal in 8 and other in 8 cases. Treatment of selected patients consisted of abstention in 2 cases, surgical complete resection in 5 cases, mono or polychemotherapy alone (CT) in 4 cases, and radiotherapy alone in 98 cases (68%) or with CT in 36 cases (25%). With a median follow-up of 90 months (range 3–314), the 5-year relapse-free (RFS) and overall (OS) survivals were 64% and 79% for the low-grade NHL, and 43% and 50% for the high-grade NHL. Prognostic factors were determined for the 118 low-grade patients. In univariate analysis, age greater than 59 years, elevated IPI score and elevated LDH level were prognostic for lower RFS and OS. In multivariate analysis, age greater than 59 years was the only prognostic factor for both lower RFS and OS (Figure 1). In conclusion, with a median follow-up of 7.5 years, our large monocentric cohort of patients represents one of the most important series that defines the initial characteristics, natural history and prognostic factors of NHL with initial orbital localization. Figure Figure

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Osteomas of the craniofacial region: A case series and review of literature

Indian Journal of Plastic Surgery ◽

10.4103/0970-0358.121982 ◽

2013 ◽

Vol 46 (03) ◽

pp. 479-485 ◽

Cited By ~ 9

Author(s):

Sagar Gundewar ◽

Deepak S. Kothari ◽

Nitin J. Mokal ◽

Amol Ghalme

Keyword(s):

Surgical Approach ◽

Clinical Presentation ◽

Age Groups ◽

Case Series ◽

Surgical Excision ◽

Frontal Bone ◽

Review Of Literature ◽

Operative Complications ◽

Histological Confirmation

ABSTRACT Objective: 0 To discuss the clinical presentation, diagnosis and management of osteomas involving the craniomaxillofacial region. Materials and Methods: This study was conducted from June 2004 to March 2012 at our institute. A total of 12 cases between the ages of 10 and 50 years were managed with surgical excision and reconstruction. The criteria used to diagnose osteoma included radiographic and clinical features and histological confirmation of the specimen. The total follow-up period ranged from 6 to 24 months. Results: Out of 12 osteomas, 10 were peripheral and 2 were centrally located. Mandible involvement was seen in six patients, four involved the orbit, one the frontal bone and one the frontal bone with the skull base. All patients undergoing excision and reconstruction had a favourable aesthetic and functional outcome. There were no recurrences and no post-operative complications. Conclusion: Osteomas affect all age groups with no sex predilection and are usually clinically asymptomatic till they become large in size. Surgical excision and appropriate reconstruction is the mainstay of management. Surgery is indicated when lesion is symptomatic or actively growing and the surgical approach for exposure of the lesion should be case specific.

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Diagnostic Accuracy of Xeromammography

Tumori Journal ◽

10.1177/030089168106700510 ◽

1981 ◽

Vol 67 (5) ◽

pp. 447-453 ◽

Cited By ~ 1

Author(s):

Valter Castiglione ◽

Adelaide D'Antona ◽

Luigi Dellafiore ◽

Luisa Ferrario ◽

Guido Luzzatti ◽

...

Keyword(s):

Diagnostic Accuracy ◽

Malignant Tumors ◽

Age Groups ◽

Disease Process ◽

Diagnostic Errors ◽

Benign Tumors ◽

Radiologic Investigation ◽

Pathologic Processes ◽

Sclerosing Adenosis

The analytical study of the case material has allowed a precise evaluation of the distribution by age groups of the different pathologic processes of the breast, of the localization and dimensions of malignant tumors, as well as the diagnostic accuracy for those cases histologically ascertained and those with a follow-up. The diagnostic accuracy and the consequent reliability of the xeroradiographic method for the diagnosis of breast cancer is distinctly superior to that reported in the literature for traditional mammography, whereas for the differential diagnosis between circumscribed dysplastic manifestations and benign tumors xeromammography is not sufficiently reliable. The authors critically discuss the results and in particular the problem of false positives, which also include diagnostic errors that cannot be avoided in that they directly derive from the pathologic morphology of the disease process (plasma cell mastitis and sclerosing adenosis). As regards the problem of false negatives, they can be reduced within certain limits by resorting to other instrumental investigations. However, there are cases (1% of the malignant neoplasias histologically ascertained) that present a completely negative xeroradiographic finding. These are the limits of the radiologic investigation which cannot in any way be surmounted.

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Incidentally detected solid pseudopapillary neoplasm of pancreas in a child with an ovarian cyst: a case report

International Journal of Research in Medical Sciences ◽

10.18203/2320-6012.ijrms20201946 ◽

2020 ◽

Vol 8 (5) ◽

pp. 1899

Author(s):

Sandeep M. Reddy ◽

Siva K. Prasad ◽

Shilpa B. ◽

Baleswari G.

Keyword(s):

Ovarian Cyst ◽

Female Child ◽

World Health ◽

Low Grade ◽

Pancreas Head ◽

Complete Surgical Resection ◽

Well Differentiated ◽

Health Organization ◽

Solid Pseudopapillary Neoplasms

Solid pseudopapillary neoplasms (SPN) are the most common as well as very rare pediatric tumors of the pancreas. Most of the literature is derived from adult SPNs. As per world health organization, these tumors considered as low grade malignant with excellent survival outcomes after complete surgical resection. We report a case of incidentally detected SPN in a 16-year-old female child with an ovarian cyst. She underwent pylorus preserving pancreaticoduodenectomy and ovarian cystectomy. Histopathology revealed well differentiated SPN of the pancreas head and ovarian cystadenoma. She is symptom-free at the 6th month follow up.

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