Adenomatoid tumor of the adrenal gland: unexpected guest.

Adenomatoid tumor (AT) is a benign growth that originates from mesothelial cells. Typically, it encompasses the uterus, fallopian tubes, and paratesticular area. One-third of all AT is in paratesticular area, and it accounts for 60% of all benign paratesticular tumors. ATs have been seldom reported as a case report in adrenal gland, liver, pleura, and mediastinal cavity. To date, English literature has only documented 46 instances of adrenal AT. We present the occurrence of a new clinically unexpected case of adrenal AT in a 37-year-old male patient. Computed tomography (CT scan) incidentally revealed the presence of a tumor. To the best of our knowledge, this is the first reported instance of primary adrenal gland AT reported in Saudi Arabia and the 47th instance worldwide in the English-based published literature. It is crucial to thoroughly investigate these tumors, utilizing techniques such as histopathological analysis and immunohistochemical staining to correctly diagnose AT.

A Pathology Experience of Posttransplant Lymphoproliferative Disorder From One Tertiary Hospital: Pathology Concepts and Diagnostic Approach.

BACKGROUND: Solid organ transplantation and bone marrow/hematologic stem cell transplantation recipients face a heightened risk of developing malignancies or cancer as a result of immunosuppression. Posttransplant lymphoproliferative disorders (PTLD) are a range of disorders from benign lymphoid growth to lymphoma found post-transplant. Risk factors for PTLD include high immunosuppressive use and oncogenic effects of Epstein-Barr virus (EBV). There is a lack of comprehensive clinical and pathological documentation of PTLD cases among Saudi patients, and the available data are limited to a few case reports. As a result, a deeper understanding of this disease requires more clinicopathological information. MATERIAL AND METHOD: In this review, we share our insights on cases diagnosed with PTLD at King Faisal Specialist Hospital and Research Center, a prominent tertiary center in the western region of Saudi Arabia, from 2005-2023. RESULT: We have diagnosed a total of 14 cases of PTLD in our department, with an age range spanning from 3 to 62 years. These diagnoses were made based on biopsies or tumor resection procedures. The survival rate of patients is believed to be influenced by multiple factors, including histology, tumorigenesis, disease biology, and clinical stage. Additionally, Kaplan-Meier curve analysis indicates that female patients tend to have a higher estimated survival rate compared to males. CONCLUSION: PTLD diagnosis and therapy have greatly improved in the past 20 years. PTLD is treated with reduced immunosuppression, rituximab, chemotherapy, adoptive therapy, surgery, antiviral therapy, and radiotherapy. In this study, we present our experience from a large tertiary center in the western region of Saudi Arabia. Moreover, we will go through etiology, clinical features, and pathologic morphology along with the corresponding genetics, prevention, and valid treatment options.

Survivin expression is associated with lymph node metastasis and short survival in patients with colorectal adenocarcinoma.

BACKGROUND: Survivin, a protein belonging to the Inhibitor of apoptosis (IAP) family, is the smallest member in terms of size. It works by preventing programmed cell death and regulating the advancement of the cell cycle. Being a part of the group of inhibitors associated with apoptosis, survivin is connected to increased aggression and negative prognosis in different malignancies, including colorectal cancer (CRC). MATERIALS AND METHODS: Pathology tissue blocks of 209 primary tumors, and 44 adenomas, were used in this study, as well as an anti-Survivin antibody. A semiquantitative method was used to score the Survivin expression based on an evaluation of the percentage and intensity of nuclear expression. RESULT: Survivin expression was identified in 127 (60.8%) CRC samples and in 14 adenomas (31.8%). There was an association between positive Survivin immunostaining and lymph node metastasis (P: 0.001), lymphovascular invasion (P: 0.020), and short overall survival (Log-rank 4.012, P=0.045) and disease-free survival probabilities (Log Rank 4.921, P=0.027). There was no association between Survivin expression and age, gender, tumor location, size, stage, margin status, and tumor recurrence. CONCLUSION: Survivin immune expression is associated with worse prognoses in CRC patients. Survivin can be a potential disease biomarker and could be used in management plans for CRC patients.

Composite Lymphoma's Ability to Adapt and Change Through Plasticity: A Case Report and Literature Review.

Composite/synchronous lymphoma is an uncommon condition that presents a challenge in histopathological diagnosis due to the simultaneous appearance of two or more lymphomas, including instances where they are intermixed within the same location. Performing a core needle tissue biopsy adds a challenge for pathologists when trying to diagnose a condition that requires excisional tissue for a thorough evaluation. This report highlights a distinctive instance of composite lymphoma in which classical Hodgkin lymphoma (cHL) is combined with diffuse large B-cell lymphoma (DLBCL). All pertinent information including clinical, histopathological, and immunohistochemical data for each of these composite lymphomas is provided. In addition, we conducted a literature review of the published data. The findings from these data further support the theory of a shared clonal origin and transdifferentiation occurrence in the process of lymphoma development.

Diagnosing Lymphoma on Core Needle Biopsy and the Challenging Role of Immunohistochemistry.

Publications and primary literature both describe the latest advancements in immunohistochemical diagnosis of lymphomas. Along with the updated categorizations, the growing reliance on small biopsy samples to assess lymphoma poses a constant difficulty in hematopathology diagnosis and heightens the demand for immunohistochemistry (IHC). This study aimed to provide practicing lymphoma and hematopathologists with an overview of novel immunohistochemical markers and new applications of already established immunohistochemistry markers to be helpful in lymphoma diagnosis, especially in core needle biopsies. The information was sourced from a review of relevant literature and personal experience gained through professional practice. To effectively diagnose and treat hematolymphoid neoplasms, we need to have a comprehensive understanding of the constantly evolving range of immunohistochemistry studies. This article introduced new markers that contribute to our overall knowledge of the disease, diagnosis, and treatment. The addition of these markers might be helpful in supporting the tumor diagnosis on limited sample material obtained from needle biopsies.

Embryonal tumor with multilayered rosettes; rare pediatric CNS tumor. A case report and review of literature.

Embryonal tumor with multilayered rosettes (ETMR), C19MC-altered is a newly designated entity of the embryonal tumors of the central nervous system (CNS) according to the 2016 WHO classification system of CNS. Characteristically, these tumors are newly defined based on their specific molecular genetic amplification in chromosome 19q13.42 found at locus C19MC. To the best of our knowledge, we present the first reported case of ETMR in Saudi Arabian pediatric population. A 2-year-old boy presented to the hospital with generalized tonic-colonic seizure, vomiting, irritability, and inability to walk. Computed tomography (CT) scan showed a large left thalamic supratentorial brain tumor. The tumor measured 6.1 × 5.6 × 5.6 cm and was characterized by cystic changes, prominent vasculature, and calcifications. Histopathology, immunohistochemistry examination, and fluorescence in situ hybridization (FISH) analysis confirmed the diagnosis of ETMR. In addition to reporting this rare case, we provide a brief literature review, treatment options, patient outcome, and disease prognosis.

Future of Education or Present Reality?

CONTEXT.­: The main focus of education in most pathology residency and subspecialty pathology fellowships is the light microscopic examination of pathology specimens. Classes with multiheaded scopes are the most popular among pathology trainees. Until recently, it was difficult to imagine that this educational approach could change. In the beginning of March 2020, our country faced a serious challenge, which all of us now know as the coronavirus disease 2019 (COVID-19) pandemic. The rules of social distancing and work from home were applied. These types of restrictions were implemented in almost all parts of our life, including work and pathology education. OBJECTIVE.­: To share our experience in the Department of Hematopathology at the University of Texas MD Anderson Cancer Center during the COVID-19 pandemic. We describe our experience in modifying our approaches to education. We show how we overcame many obstacles to learning by building one of the largest virtual hematopathology educational platforms via Cisco WebEx and using social media, in particular Twitter. These tools facilitated the learning of hematopathology by medical students, pathology trainees, and practicing pathologists from all over the world. DATA SOURCES.­: During the first 3 months of the pandemic (April, May, and June, 2020), we evaluated the visitor attendance to the MD Anderson Cancer Center Hematopathology Virtual Educational Platform using data collected by the Cisco WebEx Web site. To determine the impact that the platform had on medical education for the hematopathology community on Twitter, the analytic metrics obtained from Symplur LLC (www.symplur.com, April 27, 2020) were used via its Symplur Signals program. CONCLUSIONS.­: Our experience using the MD Anderson Hematopathology Virtual Platform showed that there is substantial global interest and desire for virtual hematopathology education, especially during the COVID-19 pandemic.

The prognostic significance of immunostaining of Wnt signalling pathway molecules, E-cadherin and ß-catenin in colorectal carcinomacolorectal carcinoma.

BACKGROUND AND STUDY AIMS: Colorectal carcinoma (CRC) is associated with high morbidity and mortality. The E-cadherin-catenin complex is crucial in the development and progression of carcinomas. This study was conducted to evaluate the relation between E-cadherin and ß-catenin immunostaining and CRC outcome. PATIENTS AND METHODS: Tissue microarrays were constructed from CRC, nodal metastases, adenomas, and normal mucosa. E-cadherin and ß-catenin immunostaining was performed, and results were analyzed. RESULTS: For E-cadherin, the membranous fraction (MF) was higher in normal mucosa, adenoma, CRC, and nodal metastasis than the cytoplasmic fraction (CF), but no difference in nodal metastasis was observed. A low MF in CRC was associated with disease relapse. For ß-catenin, high MF and CF in normal mucosa, adenoma, CRC, and nodal metastasis were observed, whereas the nuclear fraction (NF) was high only in CRC. In CRC, a high CF was associated with nodal metastasis and the incidence of relapse and predicted nodal metastasis. A high NF could predict distance metastasis. A high CF in CRC was associated with favorable disease-free survival and overall survival. CONCLUSION: Reduced E-cadherin and ß-catenin immunostaining in CRC is related to prognostic factors. The Wnt/ß-catenin pathway may play a crucial role in CRC progression and help identify the high risk of adverse outcomes and indicate close follow-up.

Primary Gastrointestinal Stromal Tumor of the Prostate: Unexpected Guest.

Primary mesenchymal lesions of the prostate are exceptionally rare. They comprise 1% of all prostatic neoplasms. Despite its rare location, the diagnosis of primary gastrointestinal stromal tumors (GISTs) of the prostate gland should never be missed. Such a diagnosis can be made after the rolling out of direct extension from adjacent organs, especially the rectum. GIST diagnosis has a clinical impact on patient treatment and clinical outcomes. They harbor a certain KIT activation mutation that responds to pharmacologic therapy inhibitors. The objective of the current study was to provide a thorough review of GIST arising primarily in the prostate gland along with a comprehensive study of GIST pathogenesis, histologic morphology, immunohistochemistry, and molecular studies' findings, and their importance in differentiating GIST from other prostate mesenchymal tumors. This will emphasize the role of careful spindle cell lesion diagnosis in the prostate gland that can influence the prognostic stratification of clinical management, future follow-up, and disease outcome. Thirteen cases were collected after an extensive and detailed review of the English literature through PubMed, Medknow, Google Scholar, as well as personal experience. The anatomic location of this lesion plays a significant role in the differential diagnosis. It is difficult to establish the accurate primary origin of GIST on core needle tissue biopsy. Thus, clinical, and radiological examinations play a crucial role in rolling out the possibility of rectal GIST secondarily invading and involving the prostate gland. To conclude, primary prostatic GIST is a rare diagnosis. Extraintestinal, particularly rectal, GIST can clinically and radiologically mimic the impression of the prostatic lesion. Before diagnosing primary prostatic spindle cell lesions, such as solitary fibrous tumor (SFT), inflammatory myofibroblastic tumor (IMT), leiomyoma, leiomyosarcoma, or prostatic stromal tumors, one should include CD117/c-Kit in the workup of a prostatic spindle cell lesion. GIST has distinct pathogenesis, and its diagnosis can have a clinical impact on the patient's management plan and clinical outcome.

Giant Ileocecal Inflammatory Fibroid Polyp: Unique Clinical Presentation.

Inflammatory fibroid polyps (IFPs) are infrequent gastrointestinal tract benign neoplasms. They mostly occur in the stomach especially the antrum. Signs and symptoms depend on their anatomic location and lesion size. Lesion biopsies are very challenging for accurate diagnosis in these lesions. Histopathological examination of resected tissue along with immunohistochemical studies is the perfect way to confirm the final diagnosis. In this paper, the authors present an unusual case of IFP in a 23-year-old female patient presented to the emergency room (ER) with a picture of intestinal obstruction and severe abdominal pain. Her investigations revealed a huge mass located at the ileocecal valve, with overall features mimic gastrointestinal stromal tumor (GIST) clinical presentation. Surgical resection is considered the most commonly used treatment method. The overall prognosis is good with a low risk of recurrence.

Diagnostic Challenges in Chondroblastic Maxillary Osteosarcoma: A Case Report.

The diagnosis of craniofacial osteosarcoma can be quite challenging, and the condition often goes unrecognized for a considerable period of time. In this report, we discuss the case of a 21-year old woman who presented with a one-year history of a small swelling over the left maxillary alveolar ridge. Upon further investigation, the histopathological examination showed high-grade chondroblastic osteosarcoma. The option of four cycles of neoadjuvant chemotherapy regimen preoperatively was chosen, and left inferior maxillectomy was performed along with reconstruction with obturator prosthesis. This case highlights the difficulties encountered in such rare cases of craniofacial osteosarcomas both in terms of the delay in the establishment of the diagnosis as well as management protocol. A high index of suspicion is required in cases of craniofacial osteosarcoma and early surgical resection with adequate safety margins is warranted.

Desmoplastic Small Round Cell Tumor of Pancreatic Origin in a Young Child: A Case Report and Review of Literature.

BACKGROUND Desmoplastic small round cell tumor (DSRCT) is a rare lethal malignant tumor with young male predominance. The majority of cases arise in the abdominopelvic region and are hypothesized to have a mesothelial origin. However, extra-abdominal and extraperitoneal DSRCT have been reported. It is extremely uncommon for the pancreas to be a primary site for DSRCT, and only 5 cases have previously been reported in the English literature. Clinically, DSRCT has a wide range of presentations from asymptomatic to life-threatening comorbidity, and it responds poorly to treatment despite aggressive therapy. CASE REPORT We report a previously healthy 9-year-old boy with an incidentally discovered abdominal mass of pancreatic origin. All necessary laboratory investigations were within normal limits. Computed tomographic imaging showed a huge left-side retroperitoneal mass measuring 15 cm in the greatest dimension that was accompanied by vascular encasement. The mass was resected successfully. Histopathological examination along with ancillary tests favored a diagnosis of DSRCT over other small round blue cell tumors. Detection of translocation t(11;22)(p13;q12) with EWSR1-WT1 gene fusion, based on reverse transcription-polymerase chain reaction analysis, confirmed the diagnosis. Approximately 7 months later, the tumor recurred with mesenteric lymph nodes metastasis and the child was placed on palliative therapy. CONCLUSIONS It is worthwhile to consider DSRCT in the differential diagnosis of small round blue cell tumors, even in unusual sites, in a pediatric age group. Due to the poor prognosis, owing to chemotherapy resistance and a high rate of recurrence with significant tumor burden, reaching a precise diagnosis of DSRCT is essential. Almost all cases harbor the hallmark molecular alteration of t(11;22)(p13;q12) with EWSR1-WT1 gene fusion. Debulking surgery paired with a chemotherapy regimen comprising vincristine, doxorubicin, and cyclophosphamide and ifosfamide + etoposide has been shown to improve overall survival rate compared with other chemotherapeutic agents. However, no targeted therapeutic modality has been developed.

Condyloma accuminatum of the male urethra: A case report.

Condyloma acuminatum is an anogenital lesion caused by the human papillomavirus infection. It is a common, sexually transmitted disease. It usually affects the external genitalia, while urethral and bladder involvement is uncommon. Human papillomavirus types are classified into three categories depending on their oncogenic potential: low risk (type 6, 11, 42, 43, 44, 59, 66, 68, and 70), intermediate-risk (type 30, 31, 33, 34, 35, 39, 40, 49, 51, 52, 53, 57, 58, 63, and 64) and high risk (type 16, 18, 45, and 56). High-risk and intermediate-risk human papillomavirus DNA types, together with other co-factors still to be defined, account for over 90% of anogenital pre-malignant and malignant tumours. Herein, we report a unique case of condyloma acuminatum positive for human papillomavirus -6 involving the penile urethra, presents as the primary and only site of the disease in a diabetic type-2 patient.

Granular Cell Tumor of Thyroid: Challenging Pitfalls and Mimickers in Diagnosis.

Mesenchymal tumors of the thyroid are extremely rare. Only few isolated cases of primary thyroid granular cell tumor (GrCT) have been reported. The anatomic location of this lesion plays an important role in the differential diagnosis. It is well-known that GrCT commonly involves the head-and-neck region, lower extremity, nuchal region, chest wall, and internal viscera such as the gastrointestinal tract. However, primary GrCT of the thyroid are unexpected and might lead to misdiagnosis, especially with pathological diagnosis limitations such as frozen section and fine-needle aspiration. We believe that it is important to establish a good differential diagnosis because of its ability to simulate the appearance of invasive carcinoma, especially in cases lacking tissue block examination. In this paper, we try to focus on clinical, radiological potential characteristics, and the differential diagnosis of the tumor.

Increased osteopontin expression in endometrial carcinoma is associated with better survival outcome.

OBJECTIVES: Osteopontin (OPN) is a key extracellular matrix protein that is involved in cancer progression. The aim of the current study is to investigate the relation of OPN immunostaining in endometrial carcinoma with clinicopathological parameters. MATERIAL AND METHODS: Archival 71 endometrial carcinomas and 30 non-neoplastic endometrial tissues were obtained from the Department of Pathology at King Abdulaziz University Jeddah, Saudi Arabia. Tissue microarrays were constructed. Tissue sections were stained using anti-human OPN monoclonal antibody. Immunostaining results were recorded and analysed. RESULTS: In non-neoplastic endometrial tissues, high (increased) OPN immunostaining was observed in 100%. In endometrial carcinoma, high (increased) OPN immunostaining was seen in 64.8% of cases. High (increased) OPN immunostaining was more frequent in non-neoplastic tissues than in endometrial carcinoma (p < 0.001). OPN immunostaining showed no association with histological type, FIGO tumour grade, tumour size, myometrial invasion, lymphovascular invasion, surgical resection margin or lymph node metastasis. On the other hand, high (increased) OPN immunostaining was associated with better overall survival [Log Rank (Mantel-Cox) = 4.385, p = 0.003]. CONCLUSIONS: In endometrial carcinoma, immunohistochemical staining of OPN could be a helpful tool in the prediction survival pattern. OPN immunostaining showed no association with most clinicopathological features. Further investigations both clinical and molecular are needed to explore the downstream of OPN in endometrial carcinoma.

Collision Glial Neoplasms Arising in an Ovarian Mature Cystic Teratoma: A Rare Event.

Germ cell neoplasms represent around 20% of all ovarian tumors. They most frequently affect children and young adults. Mature cystic teratoma is a common benign ovarian neoplasm comprising about 95% and is made up of all three germ cell embryonic layers. By definition, mature cystic teratoma may be derived from any of the three germ cell lines. On the other hand, immature teratomas contain primitive neuroepithelial elements. However, it is quite uncommon in the English literature to have a neuroepithelial glial neoplasm arising in a mature cystic teratoma of an adolescent. Interestingly enough, all published cases described a single type of glial neoplasm arising in mature ovarian teratoma. Herein, the authors discuss a unique case of concomitant occurrence of two different glial neoplasms, namely pilocytic astrocytoma and subependymoma arising in an ovarian mature cystic teratoma. To the best of our knowledge, this is the first reported case with such a distinctive histopathologic finding.

Rosai-Dorfman disease with pulmonary involvement mimicking bronchogenic carcinoma.

BACKGROUND: Rosai-Dorfman disease is a histiocytic lesion that affects lung rarely. CASE PRESENTATION: We present a 52-year-old female diagnosed with right intrabronchial mass invading the bronchial wall and the extrabronchial tissues with lymphadenopathy. Multiple bronchoscopic biopsies were not diagnostic. Pneumonectomy was performed and postoperative histology revealed marked mucin impaction and bronchial dilatation. The pulmonary tissue showed areas of hemorrhage and chronic inflammation. The mass exhibited an excessive number of lymphocytes, plasma cells, and numerous histiocytes engulfing them (lymphocytophagocytosis). These histiocytes were S100 protein and CD68 positive. These features are consistent with Rosai-Dorfman disease. CONCLUSION: Rosai-Dorfman Disease with pulmonary affection can be misdiagnosed as malignancy. Careful histological examination of the specimen for emperipolesis or lymphocytophagocytosis together with S100 protein and CD68 positivity are the clue for proper diagnosis.

Pulmonary alveolar microlithiasis: A report of two unique cases.

Pulmonary alveolar microlithiasis (PAM) is an inherited autosomal recessive disease. PAM is classically characterized by calcium phosphate deposition within alveolar airspaces due to SLC34A2 (solute carrier family 34 member 2) gene mutation located on chromosome 4p15.2. Such cellular genetic mutation would lead to a defect in the sodium-phosphate transporter channel located in alveolar epithelial cells type-II. Ultimately, it would result in a malfunction of alveolar epithelial cells and the failure of these cells to clear-up the released phosphorous particles in the cellular surfactant recycling. PAM is usually diagnosed in adulthood, frequently notable in the third and fourth decades of life, occasionally can be associated with more severe clinical presentation and radiological findings. Nevertheless, the disease could manifest itself in the pediatric age group, which either shows non-specific signs and symptoms or be exclusively asymptomatic. Histopathological examination is the gold standard for the PAM diagnosis. Genetic counseling and testing might benefit the patient's family members. Herein, we present 2 cases of PAM in the pediatric age group, along with their clinical history, presentation, radiological studies, and histopathology findings, as well as a brief literature review.

Apocrine carcinoma of the scalp with neck lymph node metastasis: A case report and review of the literature.

Primary cutaneous apocrine carcinoma (PCAC) is an extremely rare malignancy. Distinguishing apocrine carcinoma from breast carcinoma metastasis is difficult. Only a few cases reported as PCAC of the scalp and primarily treated by wide local excision. The usual presentation is a skin lesion that rapidly progresses over the duration of a few weeks to few months. We reported a 56-year-old man with a right scalp ulcerative lesion diagnosed as a CAC. The patient underwent wide local excision. 1.5 years later, the patient developed neck lymph node metastasis that treated with neck dissection and adjuvant radiotherapy. No chemotherapy was given due to limited literature, suggesting substantial benefits of adjuvant chemotherapy for such cases. Review literature was performed to assess the clinical presentation, treatment, and prognosis of such malignancies. PCAC of the scalp is a challenging malignancy in the diagnosis and management.

Long-Term Remission of a Spinal Atypical Teratoid Rhabdoid Tumor in Response to Intensive Multimodal Therapy.

Atypical teratoid rhabdoid tumors (ATRTs) are rare and aggressive central nervous system tumors that infrequently arise in spinal locations in young children. Provided clinical and diagnostic suspicion is high, the histopathological diagnosis is relatively straightforward to secure by testing for the characteristic loss of the tumor suppressor protein SMARCB1/INI1. Here, we describe a case of thoracic spinal ATRT in a three-year-old boy that showed characteristic aggressive progression until managed with intensive multimodal therapy to achieve durable long-term remission. In doing so, we review the histopathological features, management, and current advances in molecular biology that hold promise for personalized ATRT therapy.