Sporadic Renal Hemangioblastoma: A Case Report of a Rare Entity.

Hemangioblastoma, also known as capillary hemangioblastoma, is a rare benign mesenchymal tumor commonly found in the central nervous system (CNS). It can also manifest in various organs, including the kidney. Renal hemangioblastoma (RH) is often associated with Von Hippel-Lindau (VHL) disease, but sporadic occurrences are observed infrequently. While RH is usually asymptomatic, it can also cause abdominal pain and hematuria. In this study, we present a case of an elderly patient without history of VHL but complaining of abdominal pain for three days. Serological evaluations were unremarkable, and a CT scan identified a 2.4 cm mixed solid-cystic mass lesion on the left kidney's superior aspect. The patient subsequently underwent a biopsy followed by lesion ablation. Microscopic analysis revealed sheets of eosinophilic cells with ovoid nuclei, showing focal rhabdoid and spindle cell features, with an intricate capillary network. Focal nuclear atypia without necrosis or mitosis was noted. Immunohistochemistry (IHC) demonstrated positive staining for inhibin, S100, PAX8, and vimentin, along with patchy positivity for CD10 and RCC. Negative staining was observed for cytokeratin AE1/AE3, CK7, EMA, CK8/18, desmin, and HMB-45. The overall morphological characteristics and distinct IHC markers were consistent with RH. Although its pathogenesis remains unclear because of its rarity, distinguishing RH from renal cell carcinoma is crucial. IHC markers facilitate differentiation among lesions. The preferred treatment involves ablation or partial nephrectomy. Further assessment for possible VHL syndrome is essential, considering the distinct management approaches for sporadic and VHL-linked RH.

Sclerosing Angiomatoid Nodular Transformation of the Spleen: A Report of Rare Case and Literature Review.

Sclerosing angiomatoid nodular transformation (SANT) is a benign vascular lesion of the spleen with uncertain etiology. It predominantly affects women between the ages of 30 and 60 years. Clinically, it is asymptomatic or can cause abdominal pain, but usually discovered incidentally on imaging, which can identify a mass but may not provide a definitive diagnosis. In uncertain vascular lesions, there is always a risk of spontaneous rupture of large vessels and the potential for spreading malignancy. Hence, the final diagnosis is rendered on microscopy after splenectomy. A middle-aged female came to the clinic complaining of abdominal pain. Radiology showed a solid splenic mass and the patient underwent splenectomy. Gross examination showed a 3 cm white firm mass with focal hemorrhage. Microscopy revealed multiple nodules of variable sizes surrounded by fibrosclerotic stroma. The nodules showed round to slit-like vascular spaces with numerous red blood cells. The internodular stroma consisted of dense fibrous tissue with scattered plump myofibroblasts and lymphoplasmacytic inflammatory cells. These distinctive features lead to the diagnosis of SANT. SANT possesses characteristic histologic features with distinctive immunohistochemistry (IHC). IHC reveals three different types of vessels within the nodules as follows: (1) small veins (CD34-, CD31+, CD8-), (2) sinusoids (CD34-, CD31+, CD8+), and (3) capillaries (CD34+, CD31+, CD8-). All three types of vessels are negative for CD21/CD35 and CD68. Hemangioma and littoral cell angioma are two frequent vascular tumors in the spleen that should be considered differential diagnoses. Both lesions lack the microscopic features of SANT and have only a single type of vessel. The vessels in hemangioma are (CD31+, CD34+, CD8-), while in littoral cell angioma they are (CD31+, CD34-, CD8-, CD21+, CD68+). There are no specific clinical or radiologic findings for SANT. It is important to recognize these characteristic features and to differentiate them from other benign and malignant lesions, such as angiosarcoma. A thorough histopathologic examination and IHC are helpful in making the correct diagnosis.

Colorectal Adenocarcinoma Derived From Mature Cystic Teratomas: A Case Report With Review of the Literature.

Mature cystic teratomas (MCTs) are the most common benign ovarian germ cell neoplasms in women of reproductive age. Rarely, somatic malignancies arise from MCTs, the most common being squamous cell carcinoma. Adenocarcinomas are less common and colorectal adenocarcinomas are extremely rare. We present a case of somatic adenocarcinoma of colorectal type which may pose challenges in diagnosis and treatment. A middle-aged female presented to the Emergency Department with lower abdominal pain. CT scan revealed an 11 cm sharply demarcated left pelvic mass. Laparoscopy showed a left ovarian mass with torsion, a smooth external surface, and thick brownish contents. An intraoperative evaluation was consistent with an adenocarcinoma. Permanent histopathology revealed adenocarcinoma of colorectal phenotype with necrosis. Additional evaluation of the cyst showed benign colonic epithelial lining. The immunohistochemistry (IHC) profile of positive CDX2 and CK20 and negative PAX8, CK7, ER, and PR suggested a colorectal-type somatic adenocarcinoma arising from the MCT and was staged as IA, after negative endoscopic findings. Due to their rarity and atypical symptoms, distinguishing metastatic tumors from MCT-derived somatic malignancies is a challenging process. CT scan and serum tumor markers can be helpful but are not definite. Thorough clinical evaluation and proper staging are necessary after pathologic evaluation. Extensive sampling and IHC can further characterize the origin of the tumor. Diligent sampling and a high index of suspicion in this case clinched the correct diagnosis and clinical management. The patient is being treated for stage IA ovarian cancer as opposed to stage IV metastatic colorectal cancer.

A 62-Year-Old Woman With Nodules, Cysts, and Activated Bone Marrow.

CASE PRESENTATION: A 62-year-old woman with a long-term smoking history was evaluated at our lung cancer clinic for a new 2.5-cm lung nodule. She had a history of well-controlled COPD and hypertension. She was in overall good health until 3 weeks before her evaluation in an ED for new-onset exertional dyspnea. Her physical examination was unremarkable, except for diffuse hyperpigmented scaly scalp lesions that coalesced into plaques. Her subjective symptoms were nonproductive cough, exertional dyspnea, unintentional weight loss of 10 lb, and fatigue that had started 2 months earlier. She did not have fever or night sweats.

Human Papillomavirus-Related Multiphenotypic Sinonasal Carcinoma: A Recent Discovery. A Case Report and Literature Review.

Human papillomavirus (HPV)-related multiphenotypic sinonasal carcinoma is a distinct, recently described neoplasm of salivary gland nature that has an unusual microscopic appearance exhibiting multidirectional differentiation. Originally described by Bishop et al. in 2012, this distinct form of head and neck cancer is a very rare entity that few pathologists have encountered in practice, and only 50 cases have been reported in the literature. It usually presents as a large, destructive mass confined to the nasal cavity or paranasal sinuses, and is always associated with high-risk HPV infection. Although histologically it often resembles adenoid cystic carcinoma, this neoplasm also consistently exhibits features of myoepithelial, ductal and squamous differentiation. Newly recognized characteristics have recently been described that include bizarre pleomorphism, sarcomatoid transformation, and heterologous cartilaginous differentiation. These unique features have continued to expand the morphologic spectrum of this neoplasm and justify the recent change in its nomenclature from "HPV-related carcinoma with adenoid cystic-like features" to "HPV-related multiphenotypic sinonasal carcinoma (HMSC)". In 2017, "HPV-related carcinoma with adenoid cystic like features" was included as a provisional tumor type by the World Health Organization Classification of Head and Neck Tumors. Despite the presence of high-grade histologic characteristics such as necrosis and brisk mitotic activity, and a tendency for recurrence, HMSC demonstrates indolent clinical behavior and carries a good prognosis.

A Case of Double Expresser Diffuse Large B Cell Lymphoma Treated with R-CODOX-M/R-IVAC.

Diffuse Large B Cell Lymphoma (DLBCL) is a heterogeneous disease with a variety of chromosomal abnormalities contributing to differences in management. While it is known that Double Hit Lymphomas (DHL) warrant more aggressive chemotherapy regimens, debate remains on how to treat Double Expresser Lymphomas (DEL). We present a case of a DEL treated with an aggressive regimen of 2 alternating cycles of R-CODOX-M (rituximab, cyclophosphamide, doxorubicin, vincristine and methotrexate) and R-IVAC (rituximab, ifosfamide, etoposide and high dose cytarabine). The regimen resulted in a significant response to treatment with marked reduction in tumor size and avidity, and an acceptable side effect profile. There was, however, residual metabolic activity on repeat PET CT scan. After consolidation with 36 Grey radiotherapy, a PET CT demonstrated a complete metabolic response. Debate remains regarding treatment approaches in DEL. Our case supports the categorization of DEL alongside DHL as resistant lymphomas requiring a more aggressive regimen than standard therapy.

Large cardiac fibroma and teratoma in children- case reports.

Primary cardiac tumours in paediatric population are an unusual occurrence. Although, majority of such tumours are benign (90%), the frequency and type of cardiac tumours in this age group is different from the adult population. There are several consecutive series published in the last decade on cardiac neoplasms. Therefore, this is not only an effort to contribute to the existing literature for better understanding and management of similar patients but also to highlight the importance of early detection either by prenatal imaging or careful evaluation of differential diagnosis of common symptoms. We herein, describe two infants with large cardiac tumours (fibroma and teratoma) both arising from the interventricular septum and underwent surgical excision. A possible role of cardiac remodeling in myocardial tissue healing after extensive tissue resection in such patients is hypothesised through available experimental or limited clinical information.