Sarcomatoid urothelial carcinoma accompanied by elevated serum beta-HCG: Two case reports

Sarcomatoid urothelial carcinoma is a rare and aggressive variant. Serum beta-hCG levels are used as a tumor marker in gestational trophoblastic diseases and germ cell tumors, but may also be elevated in high-grade bladder cancers. Here, we report two urothelial carcinoma cases with sarcomatoid differentiation that relapsed early after surgery with elevated serum beta-hCG levels. The first case was a 65-year-old female and the second case was a 67-year-old man with sarcomatoid urothelial carcinoma located in the ureter and renal pelvicalyceal system, both of them relapsed with elevated beta-hCG serum level to 146.8 mIU/ mL and 242 mIU/mL, respectively. They died a few months after initial diagnosis;4.9 and 2.5 months respectively. Both sarcomatoid variant and beta-hCG expression were associated with poor prognosis and advanced stage. However, beta-hCG is not used as a tumor marker in urinary tract cancers yet, and its relationship with variant pathologies has not been clarified. We need multi-centered studies to reveal this relationship.Copyright © 2023, Derman Medical Publishing. All rights reserved.

A Case of Autosarcophagy in a Pediatric Patient With Depression

BACKGROUND: Self-mutilating behavior in the pediatric population is associated with psychiatric and psychosocial factors. Autosarcophagy, or self-cannibalism, is an extremely rare form of self-mutilation and is predominantly seen with psychosis or substance use.1 We report a case of oral autosarcophagy in a pediatric patient in the absence of substance use or psychosis. OBJECTIVE: To learn about autosarcophagy and its treatment in the pediatric population and to explore other neuropsychiatric disorders in which it is a predominant manifestation. METHODS: Review of a case using electronic medical records and relevant literature. Key terms: 'autosarcophagy,' 'body focused repetitive behavior,' 'oral self injury,' 'pediatric self-mutilation' using Medscape and Google Scholar. RESULTS: We present a 14-year-old female with history of seizure disorder in full remission, depression, self-cutting behavior, and suicidal ideation with 2 psychiatric hospitalizations, who presented to the pediatric emergency department with oral bleeding after eating one-third of her tongue over the course of a month. Evaluation was notable for poverty of speech and constricted affect. Patient stated she was 'trying to remove an infection' and alleviate discomfort. She denied that this behavior was an attempt to end her life but endorsed past suicidal ideations and cutting behavior. History revealed emergency room evaluation for aggressive behavior and episodes of volitional enuresis. We diagnosed major depressive disorder, recurrent episode in remission without psychosis. Drug screen, complete blood count, complete metabolic panel, COVID-19, urinalysis, thyroid-stimulating hormone, head computed tomography, and beta-human chorionic gonadotropin were negative. Patient continued home oral medications aripiprazole 10 mg daily, fluoxetine 30 mg daily, and levetiracetam 500 mg twice daily and was discharged the next day. CONCLUSIONS: Self-harm is observed in 17.2% of adolescents, 13.4% of young adults, and 5.5% of older adults.2 Cases of self-mutilation in pediatric patients typically present as cutting, burning, or head banging.3 Our differential diagnoses include borderline personality disorder due to repeated impulsivity and self-harm, and body focused repetitive behavior disorder (obsessive-compulsive disorder-related disorder), which presents with repetitive strain injuries and dental malocclusions. Treatment of self-mutilation involves treating the underlying psychiatric condition with psychotropic medications.4,5 In pediatric patients, dialectical behavioral therapy has been shown to reduce parasuicidal behaviors after 1 year of therapy.6 Our patient, under constant 24-hour observation, was cleared by medical, psychiatric, and dental teams. The patient followed up with outpatient psychotherapy and psychiatry. We are presenting this rare case for clinicians to identify and manage pediatric patients presenting with unique forms of self-harm tendencies.

Socio-economic Barriers to Care and Medical Complexity Leading to a 5-year Diagnostic Delay of Untreated Chronic Diabetes Insipidus and Rapidly Progressive Puberty in a 10-year-old Venezuelan Immigrant

Introduction: Germ cell tumors, including germinomas, account for 10% of pediatric chronic Diabetes Insipidus (DI) cases. Delays in diagnosis of germinomas are generally longer than six months, however, no reported cases of suprasellar germinomas causing chronic DI and precocious puberty have been known to exceed a 5-year delay in both treatment of DI symptoms and a definitive diagnosis. Case Description: A 10-year-old Hispanic male presented with a 5-year history of polydipsia and polyuria. He underwent evaluation in Venezuela, where DI was reportedly 'ruled out';however, no head MRI was performed. After two years in the US struggling to acquire insurance, he presented to his pediatrician with worsening symptoms. A head MRI, ordered to evaluate dilute high-volume urine output, revealed a suprasellar mass. He was admitted for diagnostic evaluation and met the criteria for DI. Notably, he had an elevated Beta-Human Chorionic Gonadotropin (B-HCG) level. Biopsy confirmed the diagnosis of a Central Nervous System (CNS) germinoma. He was treated with DDAVP and proton therapy with subsequent remission of his tumor. Discussion: Throughout the patient's disease course, there were multiple delays in seeking and receiving care. These include a 5-year delay in seeking care despite worsening symptoms, a one-month delay in completing a 24-hour urine collection, a one-month delay in consulting pediatric nephrology, and another month delay before completing a retroperitoneal ultrasound. Multiple medical and socio-economic factors led to these delays. The patient did not present with symptoms more typical of CNS Germinomas like headaches, nausea, and vomiting. He had no visual disturbances despite mass effect on his optic chiasm. His increased stretched penis length and Tanner staging, which were identified later in his disease course, were contradicted by his pre-pubertal testicular volume and bone age. The patient is from a Spanishspeaking/Limited English Proficiency (SSLEP) household. While Spanish interpreters were present at each appointment, the language barrier proved to be a consistent issue. Initially, the child's mother indicated that the diagnosis of DI was 'ruled out' in Venezuela. In reality, the recommended imaging was never performed. Mychart messages left by his father further highlighted communication difficulties. Without access to an interpreter, he was forced to use broken English to relay his concerns. These frantic messages indicated misunderstandings regarding scheduling with various services and completing vital labs. Care only proceeded after significant physician intervention. Poverty in Venezuela, lack of insurance, and anxiety regarding COVID-19 also contributed to these delays. Conclusion: To our knowledge, this is the first case report of a pediatric patient presenting with a 5-year history of untreated polyuria and polydipsia due to undiagnosed DI with a B-HCG secreting CNS germinoma, without spinal metastasis. This study also illustrates the importance of supporting SSLEP families as they grapple with the complicated process of navigating our healthcare system. Sagittal T1 post gadolinium contrast image (A) and axial T2 FLAIR image (B) show an enigmatic, homogeneous, briskly enhancing mass in the suprasellar cistern (red arrow) with mass effect on the optic chiasm which is displaced upward and anteriorly (green arrow).