Complete Pathologic Response With Pembrolizumab and Enfortumab Vedotin in Urothelial Carcinoma of the Upper Urinary Tract.

Urothelial carcinoma of the upper urinary tract (UTUC) presents a significant clinical challenge, often requiring aggressive surgical intervention for optimal management. We present a case of an 84-year-old woman with recurrent high-grade papillary UTUC of the left renal pelvis, refractory to prior endourologic interventions, who underwent neoadjuvant treatment with pembrolizumab and enfortumab vedotin (Pembro/EV) due to contraindications to cisplatin therapy. Following a favorable response to neoadjuvant therapy, the patient underwent laparoscopic left radical nephroureterectomy, achieving a pathologic complete response. We discuss the utility of Pembro/EV in the perioperative management of patients with UTUC, particularly in those ineligible for cisplatin-based therapy. In addition, we highlight the potential role of somatic mutation testing and the integration of novel therapeutic agents such as olaparib in personalized treatment strategies for UTUC. This case underscores the importance of exploring innovative treatment approaches and optimizing patient selection for kidney preservation strategies in the management of UTUC. Further research and clinical trials are warranted to elucidate the full therapeutic potential of Pembro/EV and other emerging therapies in this setting.

Association between Incidental Pelvic Inflammation and Aggressive Prostate Cancer.

The impact of pelvic inflammation on prostate cancer (PCa) biology and aggressive phenotype has never been studied. Our study objective was to evaluate the role of pelvic inflammation on PCa aggressiveness and its association with clinical outcomes in patients following radical prostatectomy (RP). This study has been conducted on a retrospective single-institutional consecutive cohort of 2278 patients who underwent robot-assisted laparoscopic prostatectomy (RALP) between 01/2013 and 10/2019. Data from 2085 patients were analyzed to study the association between pelvic inflammation and adverse pathology (AP), defined as Gleason Grade Group (GGG) > 2 and ≥ pT3 stage, at resection. In a subset of 1997 patients, the association between pelvic inflammation and biochemical recurrence (BCR) was studied. Alteration in tumor transcriptome and inflammatory markers in patients with and without pelvic inflammation were studied using microarray analysis, immunohistochemistry, and culture supernatants derived from inflamed sites used in functional assays. Changes in blood inflammatory markers in the study cohort were analyzed by O-link. In univariate analyses, pelvic inflammation emerged as a significant predictor of AP. Multivariate cox proportional-hazards regression analyses showed that high pelvic inflammation with pT3 stage and positive surgical margins significantly affected the time to BCR (p ≤ 0.05). PCa patients with high inflammation had elevated levels of pro-inflammatory cytokines in their tissues and in blood. Genes involved in epithelial-to-mesenchymal transition (EMT) and DNA damage response were upregulated in patients with pelvic inflammation. Attenuation of STAT and IL-6 signaling decreased tumor driving properties of conditioned medium from inflamed sites. Pelvic inflammation exacerbates the progression of prostate cancer and drives an aggressive phenotype.

Primary cutaneous Aspergillus fumigatus infection in immunocompetent host.

Primary cutaneous aspergillosis (PCA) occurs through inoculation of fungal spores directly into the skin from the environment through disrupted skin such as in burns, surgery or penetrating trauma patients. Most cases reported in literature were in the immunocompromised, rarely in immunocompetent patients. The characteristic lesion of cutaneous aspergillosis is a black eschar on a red plaque, or nodule at the site of skin injury. The diagnosis of PCA can be made by identifying hyphal forms on routine H&E staining or special stains such as periodic acid-Schiff or Gomori methenamine-silver stains on skin biopsy and by fungal cultures. We report a case of an 80-year-old farmer who developed cutaneous aspergillosis after a surgical procedure without any systemic spread. The diagnosis was made by histopathology and tissue fungal cultures. He was treated with incision and drainage followed by oral voriconazole for 4 weeks; which led to clinical recovery.

Plasmablastic Lymphoma, a Rare Entity in Bone Marrow with Unusual Immunophenotype and Challenging Differential Diagnosis.

Plasmablastic lymphoma (PBL) is an aggressive malignancy that usually occurs in the setting of immunosuppression. The immunohistochemical profile of PBL is that of terminally differentiated B lymphocytes. CD138, CD38, and MUM1 are usually immunopositive. However, pan B-cell markers such as CD20 and PAX-5 are usually negative. MYC rearrangement is the most commonly encountered genetic alteration, with immunoglobulin (IG), especially immunoglobulin heavy (IGH) chain, being the most frequent partner. We report a case of PBL in a 48-year-old human immunodeficiency virus- (HIV-) positive male who was admitted to the hospital with signs and symptoms suspicious for tumor lysis syndrome. Bone marrow examination revealed hypercellular marrow with trilineage hypoplasia and sheets of intermediate to large neoplastic cells with basophilic vacuolated cytoplasm comprising the majority of cellular elements of the bone marrow. The neoplastic cells were negative for conventional B-cell, T-cell, plasma cell, and myeloid markers, while flow cytometric analysis revealed an abnormal CD45-dim population that was partially weakly positive for CD71 and CD79b. The diagnosis was initially thought to be a high-grade primitive hematopoietic neoplasm, possibly an acute undifferentiated leukemia. BOB-1, however, was immunopositive in the neoplastic cells, confirming its B-cell origin. MYC was positive by immunohistochemistry and break-apart FISH, as were CD45, MUM-1, and EMA immunostains. There was immunoglobulin kappa (IGK) light chain gene rearrangement by polymerase chain reaction (PCR). Additionally, Epstein-Barr virus- (EBV-) encoded small RNAs (EBER) were positive by in situ hybridization (ISH). The tumor proliferation index by Ki-67 immunostaining approached 95%. Although the tumor cells were negative for CD38 and CD138, the diagnosis of PBL was still rendered. We recommend using a broad spectrum of B-cell markers, including BOB-1 and OCT-2, in such challenging cases of B-cell lymphomas with no expression of conventional B-cell markers. We also emphasize that the negative CD38 and CD138 should not exclude PBL from the differential diagnosis.

Anomalous origin of left coronary arteries from the pulmonary artery in an asymptomatic adult: An autopsy case report.

RATIONALE: Anomalous Origin of Left Coronary Arteries from the Pulmonary Artery (ALCAPA), also known as Bland-White-Garland (BWG), is a rare form of coronary artery anomaly that is usually discovered in the first few months of life. Only rarely can patients with this anomaly reach adulthood without symptoms. PATIENT CONCERNS: A 28-year-old female was witnessed suddenly collapse with a seizure-like episode by her colleagues at work. DIAGNOSIS AND INTERVENTION: Routine cardiopulmonary resuscitation was performed by emergency medical service technologists. The patient was unable to be revived. Postmortem examination revealed the patient had ALCAPA with a focal chronic ischemic injury of the left ventricle. Moreover, a high take-off of the right coronary artery was also discovered. OUTCOMES: The patient passed away due to ALCAPA. The mechanism of death was cardiac arrhythmia being triggered by myocardial ischemic changes. LESSONS: In the rare cases where ALCAPA manifests in an asymptomatic adult, the mortality rate is very high. This case demonstrates the importance of awareness of such patients living under the tremendous risk of sudden cardiac death.

Trigeminal hypertrophic interstitial neuropathy presenting as unilateral proptosis, ptosis, tearing, and facial neuralgia.

PURPOSE: To show the utility of MRI and histology in diagnosing rare cases of trigeminal hypertrophic interstitial neuropathy (HIN). OBSERVATIONS: A 57-year-old African-American woman presented with a 4-year history of right eye proptosis with tearing, headaches, and worsening right-sided trigeminal neuralgia symptoms and jaw pain. HIV and diabetes tests were negative and thyroid function was normal. MRI identified abnormal thickening of all trigeminal nerve divisions and proptosis secondary to right trigeminal nerve V1 division enlargement. The excised tissue contained S-100 positive Schwann cells in an onion-bulb pattern. Headaches resolved, but proptosis and mild trigeminal neuralgia remained 1 year post-surgery. CONCLUSIONS AND IMPORTANCE: Trigeminal HIN is very rare, but presents as chronic progressive ocular symptoms with trigeminal neuralgia. Trigeminal nerve hypertrophy is identified by MRI and confirmed histopathologically by detection of Schwann cells in an onion bulb formation.

Ovarian Adenomatoid Tumor Coexisting with Mature Cystic Teratoma: A Rare Case Report.

Adenomatoid tumor of the ovary is rare, and so are collision tumors in this location. The most common histological combination of ovarian collision tumors is the coexistence of mature cystic teratoma with ovarian cystadenoma or cystadenocarcinoma. Presented herein is a rare case of ovarian adenomatoid tumor found incidentally and coexisting with mature cystic teratoma. A 44-year-old woman presented with a one-year history of intermittent right-sided pelvic pain. Ultrasound evaluation revealed a heterogeneous cystic mass in the right ovary, and a clinical diagnosis of teratoma was made. The patient subsequently underwent a right salpingo-oophorectomy. Pathological examination revealed a mature cystic teratoma and coexistent adenomatoid tumor. The two tumors were separate and no transitional features were recognized histologically. To our knowledge, no previous report of coexistence of these two tumors has been reported. Both tumors are benign and completely excised; therefore no adverse consequences are expected.

Follicular dendritic sarcoma masquerading as fibrosing mediastinitis.

Fibrosing mediastinitis (FM) is a rare disorder resulting from abnormal immunological-mediated fibro-proliferative reaction in the mediastinum. Here, we describe a case of a 46-year-old female with an incidentally found 11×9 cm posterior mediastinal mass. Multiple biopsies of this unresectable, 18-fluorodeoxyglucose avid mass revealed marked fibrosis without any evidence of malignancy, suggesting idiopathic fibrosing mediastinitis as our initial diagnosis. Multiple interventions including a trial of steroids, fluconazole, and azathioprine to target fibrosing mediastinitis were not successful. Repeat biopsy was consistent with primary mediastinal follicular dendritic cell sarcoma. The manuscript highlights the heightened need for suspecting occult malignancies in cases of FM presenting with an indeterminate cause.

Oral foregut duplication cysts: A rare and fascinating congenital lesion. Case report and review of the literature.

Oral foregut duplication cysts are extremely rare lesions with approximately 57 cases reported. They are congenital cysts, located in the anterior or ventral tongue, and occur predominantly in males. They are lined by one or more types of epithelia which is limited to gastric, intestinal or respiratory epithelium. The differential diagnosis includes lymphangioma, hemangioma, ranula, epidermoid cyst, teratoma and less likely a malignant process.