Renal cell carcinoma (RCC) is the predominant solid lesion found in the kidney. Extra-renal RCC is a rare entity. We present the case of a 75-year-old male with an incidentally discovered mass in the right iliac fossa. The patient underwent active surveillance because a percutaneous biopsy revealed a mesenchymal neoplastic lesion of benign biological behavior. As the mass had high growth rates, a decision for open surgical exploration and excision was made. The pathology results indicated clear cell renal carcinoma, and negative results on 18F-FDG whole-body positron emission tomography-computed tomography (PET/CT) established the diagnosis of extra-renal clear cell RCC. Similar types of neoplasms are extremely rare and are estimated to have developed primarily in mesodermal embryonic remnants. Clinicians should be aware of this rare entity as its diagnosis is challenging and is based on pathology.

Primary clear cell carcinoma of liver (PCCCL) is a special and relatively rare subtype of hepatocellular carcinoma (HCC), which is more common in people over 50 years of age, with a preference for men and a history of hepatitis B or C and/or cirrhosis. Herein, we present a case of a 60-year-old woman who came to our hospital for medical help with right upper abdominal pain. The imaging examination showed a low-density mass in the right lobe of his liver. In contrast enhanced computed tomography (CT) or T1-weighted imaging, significant enhancement can appear around the tumor during the arterial phase, and over time, the degree of enhancement of the tumor gradually decreases. The lession showed obviously increased fluorine-18 fluorodeoxyglucose (18F-FDG) uptake on positron emission tomography/CT. These imaging findings contribute to the diagnosis of PCCCL and differentiate it from other types of liver tumors.

•Primary peritoneal clear cell carcinoma can arise from endometriotic implants within the abdomen and pelvis.•Immunohistochemistry can be used to confirm primary disease site. Endometriotic origin can be inferred based on clinical history and intraoperative findings suggestive of endometriosis.•While no standardized treatment exists, consideration should be given to cytoreductive surgery with adjuvant chemotherapy. Adjuvant radiation can also be considered for local control.

Endometriosis is a benign disease, which is also regarded as a precursor to ovarian malignancy. Dienogest is a progestin treatment for endometriosis with efficacy and tolerability. A 35-year-old Taiwanese lady with ovarian endometrioma had taken dienogest for the last 5 years. During sonographic follow-up, surgery was suggested owing to suspicious of malignant transformation of ovarian endometrioma. While she hesitated and turned to receive two cycles of oocyte retrieval because of nulliparity. Meanwhile, more papillary growth in the ovarian endometrioma with intratumor flow was found during follow-up. Laparoscopic enucleation was performed later, and pathology revealed clear cell carcinoma with peritoneal involvement, at least FIGO stage IIB. She then underwent debulking surgery to grossly no residual tumor and received adjuvant chemotherapy with no tumor recurrence in post-operative 17-months follow-up. Considering fertility preservation, conservative treatment of ovarian endometrioma is typically indicated for those women who have not yet completed childbearing. However, malignant transformation may still occur despite long-term progestin treatment. Therefore, careful image follow-up is still indispensable.

Uterine adenosarcoma (AS) is a rare biphasic neoplasm composed of a malignant, usually low-grade stromal component and benign epithelial component, usually endometrioid. Pathogenesis is unknown; some cases are undoubtably associated with tamoxifen use. Endometrial clear cell carcinoma (CCC) is an aggressive subtype of endometrial cancer, accounting for less than 10% of all uterine carcinomas. The etiology is unknown but can rarely be associated with Lynch syndrome and tamoxifen administration. The development of a composite neoplasm consisting of adenocarcinoma in AS is extremely rare. Endometrioid carcinoma typically represents the epithelial component of the composite tumor. Here we present the very first case of composite tumor, namely, AS with CCC in which next-generation sequencing was performed. Patient was an 85-year-old woman treated with tamoxifen for 5 years. To better understand the pathobiology of two tumors, a targeted genomic analysis of both components was performed. We found seven identical somatic variants in the samples of both tumors, indicating that the tumors have a high probability of having the same origin. Dual amplification of CDK4 and MDM2 was the most likely primary cause of tumor formation, but also one driver variant in the DHX15 gene that was present in both tumor components, suggesting that DHX15 may play an important role in the initiation and development of sarcoma and carcinoma. The patient is followed by regular clinical controls and is alive without signs of disease recurrence 18 months after surgery.

Primary clear cell adenocarcinoma of the vagina represents a rare form of cancer historically correlated with in-utero diethylstibestrol (DES) exposure. Mainstay treatment modalities include surgery, radiation, and chemotherapy. There has been a growing interest in immunotherapy in the field of oncology. KEYNOTE 826 demonstrated that patients with persistent, recurrent, or metastatic cervical cancer including patients who had adenocarcinoma showed improved progression and overall survival by the addition Pembrolizumab to chemotherapy plus or minus bevacizumab. To date, there are no documented cases using pembrolizumab as adjuvant treatment for active or recurrent vaginal clear cell adenocarcinoma. We present a case of a young patient with recurrent vaginal clear cell carcinoma who showed a complete and durable response to Pembrolizumab.

We present the case of a 39-year-old woman with platinum-resistant ovarian cancer who was treated with pembrolizumab. After five cycles of pembrolizumab treatment, she suddenly developed cardiac tamponade with a pleural effusion. The malignant pericardial and pleural effusion had increased, while the other malignant lesions had diminished in size. After pericardial and pleural drainage, no re-accumulation occurred. Pembrolizumab was continued and the patient did not have tumor progression for > 20 months. In some patients with pembrolizumab-induced cardiac tamponade, continuation of pembrolizumab treatment may be possible if other lesions decrease in size and the pericardial effusion can be controlled after drainage.

UNASSIGNED: Endometrial cancer with high-risk histology is associated with a majority of recurrences and death. However, unlike other cancers, such as ovarian, there is a paucity of research demonstrating the benefits of secondary cytoreduction. In this case report we aim to aid in identifying individuals who may be ideal candidates for secondary cytoreduction surgery after minimally invasive hysterectomy and staging by a gynecologic oncologist at an academic institution and diagnosed with clear cell endometrial cancer.
METHODS: A 72 year-old female patient presented with postmenopausal bleeding and was subsequently diagnosed with Stage IIIC2 clear cell carcinoma of the endometrium. She represented 20 months after receiving initial staging and adjuvant chemotherapy with increasing CA-125 levels and radiographic evidence of left para-aortic lymph node oligo metastasis. She underwent secondary cytoreductive surgery via robotic-assisted laparoscopic para-aortic lymph node dissection and salvage chemotherapy. After 45 months of follow-up physical exam, CA-125 levels and CT of the abdomen and pelvis have remained without evidence of disease.
UNASSIGNED: We review the literature on secondary cytoreductive surgery (SCS) in endometrial cancer (EC) to identify factors associated with improved survival.
CONCLUSIONS: Secondary cytoreduction in endometrial cancer may lead to prolonged progression-free survival in well-selected patients.

Nephrogenic adenoma is a benign lesion of the urothelial tract characterized by tubules surrounded by thick, hyalinized basement membranes. There is a great variety of architectural patterns within nephrogenic adenomas, including patterns that mimic malignancy, such as focal clear or hobnail cells, areas of significant nuclear atypia, mitosis, and isolated cystic changes. This represents a diagnostic pitfall, where a malignant lesion can be mistaken for a nephrogenic adenoma, leading to a delay in diagnosis and treatment that adversely affects the outcome. In this case report, we describe a nephrogenic adenoma arising in a female urethral diverticulum and discuss the differential diagnosis, which includes clear cell carcinomas, microcystic variant urothelial carcinomas, and Skene\'s gland cysts.

UNASSIGNED: Clear cell ovarian carcinoma is rare and accounts for 1%-12% of ovarian epithelial carcinomas, depending on ethnicity. The prevalence of clear cell ovarian carcinoma in Asian, White, and Black women is 11.1%%, 4.8%, and 3.1%, respectively. Magnetic resonance imaging (MRI) shows that clear cell ovarian carcinomas are typically unilocular cyst-solid (34.9%) or multilocular-solid (41.4%); only 23.7% are solid with papillary projections. MRI can detect clear cell ovarian carcinoma with a sensitivity and specificity of 90% and 87%, respectively. Notably, sometimes ovarian masses have a solid feature and should be differentiated from uterine masses. Clear cell ovarian carcinoma has a better prognosis compared to serous carcinoma when diagnosed at an early stage, but it has a poorer prognosis at an advanced stage. The absence of a residual tumor is a favorable prognostic factor in patients with advanced-stage clear cell ovarian carcinoma. Herein, we present a case in which clear cell ovarian carcinoma was misdiagnosed as uterine sarcoma because imaging showed a mass with a solid uterine-like and necrotic area. In the present case, cytoreductive surgery was performed to remove the entire tumor and its infiltration to the sigmoid colon and left ureter. Hence, the patient had a better prognosis.
UNASSIGNED: A 57-year-old Indonesian woman presented to our hospital (Dr. Soetomo General Hospital) with post-menopausal bleeding, a large solid pelvic mass, and abdominal discomfort. The patient was diagnosed with uterine sarcoma due to the solid feature observed during ultrasonography and MRI. During the surgery, the mass was observed to originate from the left ovary, and primary debulking surgery with a multidisciplinary team was performed with zero residual tumor tissue. The tumor was histopathologically confirmed as clear cell carcinoma.
UNASSIGNED: MRI of clear cell ovarian carcinoma can be misdiagnosed as uterine sarcoma due to its solid feature. Additionally, the enlarged mass distorts the anatomical landmarks. Surgery with no residual tumor improves the prognosis for advanced-stage clear cell ovarian carcinoma.