Marginal Zone Lymphoma, Immune Dysregulation, and High-Grade Transformation
This article features a case-based approach to explore factors related to MZL progression in a patient with a complex history of autoimmunity and immune suppression. (Source: Pathology Case Reviews)
Source: Pathology Case Reviews - August 28, 2019 Category: Pathology Tags: Case Reviews Source Type: research

Posttransplant Classic Hodgkin Lymphoma: Richter Transformation or Posttransplant Lymphoproliferative Disorder?
In this report, we describe a patient whose initial CLL/SLL transformed to diffuse large B-cell lymphoma, who then received allo-SCT. Subsequent development of classic Hodgkin lymphoma proved to be a diagnostic conundrum, for which PTLD and disease progression/recurrence were both reasonable considerations. This case illustrates the diagnostic dilemma and semantic confusion faced by both pathologists and clinicians when lymphoproliferative disorders emerge within the immunologically complex interface of CLL/SLL, RT, and allo-SCT. As molecular technologies are becoming more commonplace in routine diagnostics, subpopulation ...
Source: Pathology Case Reviews - August 28, 2019 Category: Pathology Tags: Case Reviews Source Type: research

Transformation of Monoclonal B Lymphocytosis to Epstein-Barr Virus–Positive Large B-Cell Lymphoma With Intermediate Features Between Diffuse Large B-cell Lymphoma and Classic Hodgkin Lymphoma
Transformation of chronic lymphocytic leukemia (CLL) to an aggressive lymphoma, so-called Richter syndrome, usually includes diffuse large B-cell lymphoma and classic Hodgkin lymphoma. The transformation can be clonally related to the underlying CLL and is often Epstein-Barr virus (EBV) associated. Here we report the case of an 86-year-old woman with a newly identified CLL-like monoclonal B-lymphocytosis who developed diffuse lymphadenopathy. Biopsy of the left axillary lymph node showed EBV-positive large B-cell lymphoma with morphologic and immunophenotypic features intermediate between diffuse large B-cell lymphoma and ...
Source: Pathology Case Reviews - August 28, 2019 Category: Pathology Tags: Case Reviews Source Type: research

Transformed Nodular Lymphocyte-Predominant Hodgkin Lymphoma: Histologic Features and Relationship to T-Cell/Histiocyte–Rich Large B-Cell Lymphoma
We present a patient with NLPHL and transformation to DLBCL at 7 years after initial diagnosis, who ultimately developed a therapy-related myeloid neoplasm. We review the histologic spectrum of transformed NLPHL, its relationship with THRLBCL, and recent developments in its molecular pathogenesis. Cases of transformation may prove valuable in understanding complex biologic relationships between a spectrum of overlapping lymphoma entities and may ultimately help refine therapy and improve prognosis. (Source: Pathology Case Reviews)
Source: Pathology Case Reviews - August 28, 2019 Category: Pathology Tags: Case Reviews Source Type: research

Transformed Mycosis Fungoides and Mimics: Exploring the Landscape of CD30: +: Cutaneous T-Cell Lymphomas
In this report, we present a case of CD30+ transformed MF and discuss the clues that allow us to make the challenging distinction between transformed MF and other CD30+ CTCLs. We review histologic and clinical features of these different disorders, with a focus on the revised World Health Organization classification of primary cutaneous lymphomas. (Source: Pathology Case Reviews)
Source: Pathology Case Reviews - August 28, 2019 Category: Pathology Tags: Case Reviews Source Type: research

B-Lymphoid Blast Phase of Chronic Myeloid Leukemia: A Case Report and Review of the Literature
Chronic myeloid leukemia (CML) is a clonal hematopoietic stem cell disorder characterized by a reciprocal translocation, t(9;22)(q34.1;q11.2). This leads to fusion of the BCR and ABL1 genes, encoding an active tyrosine kinase that causes unregulated proliferation of the myeloid lineage. The BCR/ABL1 fusion protein is found not only in CML, but also in a subset of de novo B-lymphoblastic leukemia (B-LL). However, the fusion protein in CML is characteristically the slightly longer p210 variant, whereas the p190 variant is more frequently found in B-LL. Without treatment, CML will progress to accelerated and/or blast phase (B...
Source: Pathology Case Reviews - August 28, 2019 Category: Pathology Tags: Case Reviews Source Type: research

Usual and Unusual Patterns of Transformation Among Lymphoid Neoplasms: Diagnostic Considerations for Pathologists
No abstract available (Source: Pathology Case Reviews)
Source: Pathology Case Reviews - August 28, 2019 Category: Pathology Tags: Editorial Source Type: research

High-Grade Serous Carcinoma Arising in Retroperitoneal Endosalpingiosis: A Case Report and Brief Review of Peritoneal Serous Metaplasia and Neoplasia
We report a case of high-grade serous carcinoma arising in retroperitoneal endosalpingiosis in a patient with a history of ovarian high-grade serous carcinoma, followed by a brief review of peritoneal serous metaplasia and neoplasia. (Source: Pathology Case Reviews)
Source: Pathology Case Reviews - April 26, 2019 Category: Pathology Tags: Case Reviews Source Type: research

Desmoplastic Small Round Cell Tumor: The Value of Cytology, Immunohistochemistry, and Molecular Features in the Diagnosis of This Challenging Diagnostic Entity
We present the case of a 16-year-old boy with unusually rapidly fatal desmoplastic small round cell tumor of the peritoneum with emphasis on the cytologic features. Recent advances in imaging techniques have resulted in increased number of cytologic preparations from unusual tumors or tumors in unusual locations. Morphologically, these tumors often display a divergent phenotype and immunophenotype. Immunohistochemistry plays a pivotal role in the characterization of these tumors and discrimination from other mimickers, particularly other “blue cell tumors.” Once suspected, identification of the characteristic transloca...
Source: Pathology Case Reviews - April 26, 2019 Category: Pathology Tags: Case Reviews Source Type: research

Pseudomyxoma Peritonei Arising From a Low-Grade Mucinous Neoplasm of the Urachus
We report a patient who presented with abdominal distension and had raised tumor markers. A computed tomography scan revealed a calcified cystic lesion of the bladder dome together with features of PMP. These findings were confirmed at laparotomy, and cytoreductive surgery with hyperthermic intraperitoneal chemotherapy was performed. Histologically, the urachal lesion was a low-grade urachal mucinous tumor (mucinous cystic tumor of low malignant potential). The peritoneal disease was associated with ovarian involvement and omental cake and was designated low-grade mucinous carcinoma peritonei on histology. The immunoprofil...
Source: Pathology Case Reviews - April 26, 2019 Category: Pathology Tags: Case Reviews Source Type: research

Low Grade Mesothelial Tumors of the Peritoneum: Multicystic Mesothelioma, Well-Differentiated Papillary Mesothelioma, and Adenomatoid Tumor
Low-grade neoplasms of the peritoneum are rare but include 2 entities that are more often encountered in the peritoneum than the pleura, namely, multicystic mesothelioma and well-differentiated papillary mesothelioma. Adenomatoid tumor is most common in the genital tract, but can occur in the peritoneum where it is sometimes found as a component of either multicystic mesothelioma or well-differentiated papillary mesothelioma. Indeed, all 3 tumors can occur in combination. The histological differential diagnosis of these neoplasms can include reactive mesothelial proliferations, malignant mesothelioma, and metastatic carcin...
Source: Pathology Case Reviews - April 26, 2019 Category: Pathology Tags: Case Reviews Source Type: research

Nonneoplastic Mimics of Appendiceal Mucinous Neoplasms
With the widespread acceptance of the term low-grade appendiceal mucinous neoplasm (LAMN) for relatively bland mucinous tumors of the appendix with pushing invasion and a propensity to disseminate to the peritoneal cavity, there has been increasing concern among pathologists of overlooking one of these tumors when an appendix shows extrusion of mucin into the wall or onto the serosa. The increased awareness of LAMN and its histologic features has caused some benign entities to be misinterpreted as LAMN based largely on the presence of mucin extrusion and hyperplastic and reactive appendiceal mucosa. The misinterpretation o...
Source: Pathology Case Reviews - April 26, 2019 Category: Pathology Tags: Reviews Source Type: research

The Enigma of Goblet Cell Tumors of the Appendix
The term goblet cell carcinoid was first proposed in 1974 by Subbuswamy et al for a rare tumor of the appendix. Since then, it has been debated whether these tumors should be regarded as adenocarcinomas or as neuroendocrine tumors. Research on these neoplasms has been hampered by their rarity and the low density of tumor cells. However, their particular morphology legitimates their classification as an entity specific for the appendix. In this review the morphology, immunohistochemical profile and differential diagnosis are discussed. Recent literature on grading and molecular profiling is reviewed. These data show that gr...
Source: Pathology Case Reviews - April 26, 2019 Category: Pathology Tags: Reviews Source Type: research

The Molecular Pathology of Appendiceal Neoplasms
We review the molecular pathological changes of appendiceal neoplasms, in part using the consensus terminology agreed by the Peritoneal Surface Oncology Group International (Am J Surg Pathol 2016;40(1):14–26; Histopathology 2017;71(6):847–858), with appendiceal neoplasms subcategorized as hyperplastic polyps and serrated lesions (with and without dysplasia), appendiceal mucinous neoplasms (low and high grade), adenocarcinoma, goblet cell tumors, and neuroendocrine neoplasms. (Source: Pathology Case Reviews)
Source: Pathology Case Reviews - April 26, 2019 Category: Pathology Tags: Reviews Source Type: research

Pseudomyxoma Peritonei: Pathology, a Historical Overview, and Proposal for Unified Nomenclature and Updated Grading
Pseudomyxoma peritonei (PMP) is a clinically distinctive form of malignancy that typically arises from the appendix and is characterized by bulky accumulation of mucinous tumor deposits in the peritoneal cavity. It has defied stable pathologic categorization ever since first being described in the late 19th century. Since the early 20th century, many accounts have emphasized the bland histology of those cases arising from appendiceal precursors that had been considered benign, in contrast to higher-grade cases with more conventionally malignant histology. Beginning in the late 20th century, this was exemplified by divided ...
Source: Pathology Case Reviews - April 26, 2019 Category: Pathology Tags: Reviews Source Type: research