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Role of Prophylactic Cranial Irradiation in Small Cell Carcinoma of Urinary Bladder: Case Report and Literature Review
INTRODUCTION
Urinary bladder is an extremely rare site of extrapulmonary small cell cancer (EPSCC). Unlike small cell lung cancer (SCLC), there is no clear guideline for prophylactic cranial irradiation (PCI) for EPSCC. In this case report and literature review, we discuss small cell cancer of urinary bladder (SCCUB) and the role of PCI in SCCUB.
CASE PRESENTATION
A 74-year-old male presented with gross hematuria and an unremarkable physical examination. CT showed 1.7 cm right anterolateral bladder wall thickening. Cystoscopy revealed a 2-3 cm high-grade bladder lesion. Pathology from transurethral resection of the tumor was consistent with T1N0M0 small cell carcinoma. MRI brain and FDG-PET showed no extravesical disease. Patient received four cycles of neoadjuvant carboplatin/etoposide per his preference as he wanted to protect his hearing due to his profession followed by radical cystoprostatectomy. Post-op pathology showed clear margins. We decided to forego PCI in favor of interval surveillance with MRI and follow- up images remain negative for distant metastases.
DISCUSSION
EPSCC accounts for 2.5-5% of all SCC, very rare in male genitourinary tract. Treatment approach is derived from SCLC, guided by extent of disease and patient’s functional status. Role of PCI in EPSCC has not been clearly described, and even less evidence is available for SCCUB. From a review of eleven studies in PubMed for the role of PCI in SCCUB or EPSCC, we found that SCCUB has lower incidence of brain metastases than SCLC. One study suggested that SCCUB arises from totipotent cells in the submucosa, unlike Kulchitsky cell origin of SCLC. This difference might explain the difference in their metastatic behavior. With this background, PCI is not routinely recommended for limited- stage SCCUB. There might still be a role for PCI in extensive SCCUB with high metastatic burden. More studies are needed to update the guidelines for the role of PCI for these tumors.
CONCLUSIONS
Per this literature review, PCI is not routinely recommended for SCCUB, likely due to different cells of origin compared to SCLC. Future studies should focus on characterizing differences in their metastatic behavior and updating guidelines for PCI for SCCUB.
INTRODUCTION
Urinary bladder is an extremely rare site of extrapulmonary small cell cancer (EPSCC). Unlike small cell lung cancer (SCLC), there is no clear guideline for prophylactic cranial irradiation (PCI) for EPSCC. In this case report and literature review, we discuss small cell cancer of urinary bladder (SCCUB) and the role of PCI in SCCUB.
CASE PRESENTATION
A 74-year-old male presented with gross hematuria and an unremarkable physical examination. CT showed 1.7 cm right anterolateral bladder wall thickening. Cystoscopy revealed a 2-3 cm high-grade bladder lesion. Pathology from transurethral resection of the tumor was consistent with T1N0M0 small cell carcinoma. MRI brain and FDG-PET showed no extravesical disease. Patient received four cycles of neoadjuvant carboplatin/etoposide per his preference as he wanted to protect his hearing due to his profession followed by radical cystoprostatectomy. Post-op pathology showed clear margins. We decided to forego PCI in favor of interval surveillance with MRI and follow- up images remain negative for distant metastases.
DISCUSSION
EPSCC accounts for 2.5-5% of all SCC, very rare in male genitourinary tract. Treatment approach is derived from SCLC, guided by extent of disease and patient’s functional status. Role of PCI in EPSCC has not been clearly described, and even less evidence is available for SCCUB. From a review of eleven studies in PubMed for the role of PCI in SCCUB or EPSCC, we found that SCCUB has lower incidence of brain metastases than SCLC. One study suggested that SCCUB arises from totipotent cells in the submucosa, unlike Kulchitsky cell origin of SCLC. This difference might explain the difference in their metastatic behavior. With this background, PCI is not routinely recommended for limited- stage SCCUB. There might still be a role for PCI in extensive SCCUB with high metastatic burden. More studies are needed to update the guidelines for the role of PCI for these tumors.
CONCLUSIONS
Per this literature review, PCI is not routinely recommended for SCCUB, likely due to different cells of origin compared to SCLC. Future studies should focus on characterizing differences in their metastatic behavior and updating guidelines for PCI for SCCUB.
INTRODUCTION
Urinary bladder is an extremely rare site of extrapulmonary small cell cancer (EPSCC). Unlike small cell lung cancer (SCLC), there is no clear guideline for prophylactic cranial irradiation (PCI) for EPSCC. In this case report and literature review, we discuss small cell cancer of urinary bladder (SCCUB) and the role of PCI in SCCUB.
CASE PRESENTATION
A 74-year-old male presented with gross hematuria and an unremarkable physical examination. CT showed 1.7 cm right anterolateral bladder wall thickening. Cystoscopy revealed a 2-3 cm high-grade bladder lesion. Pathology from transurethral resection of the tumor was consistent with T1N0M0 small cell carcinoma. MRI brain and FDG-PET showed no extravesical disease. Patient received four cycles of neoadjuvant carboplatin/etoposide per his preference as he wanted to protect his hearing due to his profession followed by radical cystoprostatectomy. Post-op pathology showed clear margins. We decided to forego PCI in favor of interval surveillance with MRI and follow- up images remain negative for distant metastases.
DISCUSSION
EPSCC accounts for 2.5-5% of all SCC, very rare in male genitourinary tract. Treatment approach is derived from SCLC, guided by extent of disease and patient’s functional status. Role of PCI in EPSCC has not been clearly described, and even less evidence is available for SCCUB. From a review of eleven studies in PubMed for the role of PCI in SCCUB or EPSCC, we found that SCCUB has lower incidence of brain metastases than SCLC. One study suggested that SCCUB arises from totipotent cells in the submucosa, unlike Kulchitsky cell origin of SCLC. This difference might explain the difference in their metastatic behavior. With this background, PCI is not routinely recommended for limited- stage SCCUB. There might still be a role for PCI in extensive SCCUB with high metastatic burden. More studies are needed to update the guidelines for the role of PCI for these tumors.
CONCLUSIONS
Per this literature review, PCI is not routinely recommended for SCCUB, likely due to different cells of origin compared to SCLC. Future studies should focus on characterizing differences in their metastatic behavior and updating guidelines for PCI for SCCUB.
Reversal of Ptosis in Metastatic Prostatic Adenocarcinoma Presenting as Cavernous Sinus Syndrome
INTRODUCTION
Prostate cancer rarely metastasizes to the pituitary gland and the close relationship of these sellar masses to cavernous sinuses and major vessels makes management challenging. We describe a unique case of complete reversal of ptosis in metastatic prostate cancer presenting as cavernous sinus syndrome
CASE REPORT
A 76-year-old male presented with left diplopia, ptosis, and facial numbness. Examination showed left oculomotor palsy and numbness in the V1 and V2 distribution of trigeminal nerve. MRI revealed an 11 × 26 × 17 mm posterior sellar mass extending into the left cavernous sinus. Prolactin was slightly elevated, but rest of the pituitary hormones were normal. Resection of the sellar mass showed metastatic prostatic adenocarcinoma positive for NKX-3.1 and prostate-specific antigen (PSA), and Gleason score 4. PSA was elevated at 32 ng/ mL. Positron emission tomography (PET) showed lesions in the left prostatic lobe, pelvic lymph nodes, L5 spine, and right femoral head. FoundationOne testing found no actionable mutations. He was started on leuprorelin-docetaxel and received radiation for the brain and bony lesions. He is currently being maintained on leuprorelin-abiraterone and prednisone, which he is tolerating well.
DISCUSSION
Pituitary metastases (PMs) from prostate cancer are rare and are usually confined to the posterior pituitary. The close relationship of pituitary masses to the cavernous sinuses and internal carotid artery can lead to catastrophic neurovascular consequences. Imaging has limited sensitivity for differentiating non-invasive metastases from adenomas. Older age, new-onset diabetes insipidus, invasive masses, and rapidly growing lesions should raise suspicion for PMs. Intracranial prostatic metastases indicate poor prognosis with a reported median survival of 6-10 months. Timely diagnosis and management can prevent permanent neurologic damage as illustrated by our case in which ptosis and extraocular symptoms were completely resolved by surgery and radiation. Such unique cases underline the significance of suspecting metastatic disease in appropriate demographic groups and the crucial role of multidisciplinary care for oncologic patients.
CONCLUSIONS
A high index of suspicion for PMs in elderly people with new-onset sellar symptoms and early involvement of multidisciplinary teams can lead to prevention and even reversal of serious neurologic symptoms.
INTRODUCTION
Prostate cancer rarely metastasizes to the pituitary gland and the close relationship of these sellar masses to cavernous sinuses and major vessels makes management challenging. We describe a unique case of complete reversal of ptosis in metastatic prostate cancer presenting as cavernous sinus syndrome
CASE REPORT
A 76-year-old male presented with left diplopia, ptosis, and facial numbness. Examination showed left oculomotor palsy and numbness in the V1 and V2 distribution of trigeminal nerve. MRI revealed an 11 × 26 × 17 mm posterior sellar mass extending into the left cavernous sinus. Prolactin was slightly elevated, but rest of the pituitary hormones were normal. Resection of the sellar mass showed metastatic prostatic adenocarcinoma positive for NKX-3.1 and prostate-specific antigen (PSA), and Gleason score 4. PSA was elevated at 32 ng/ mL. Positron emission tomography (PET) showed lesions in the left prostatic lobe, pelvic lymph nodes, L5 spine, and right femoral head. FoundationOne testing found no actionable mutations. He was started on leuprorelin-docetaxel and received radiation for the brain and bony lesions. He is currently being maintained on leuprorelin-abiraterone and prednisone, which he is tolerating well.
DISCUSSION
Pituitary metastases (PMs) from prostate cancer are rare and are usually confined to the posterior pituitary. The close relationship of pituitary masses to the cavernous sinuses and internal carotid artery can lead to catastrophic neurovascular consequences. Imaging has limited sensitivity for differentiating non-invasive metastases from adenomas. Older age, new-onset diabetes insipidus, invasive masses, and rapidly growing lesions should raise suspicion for PMs. Intracranial prostatic metastases indicate poor prognosis with a reported median survival of 6-10 months. Timely diagnosis and management can prevent permanent neurologic damage as illustrated by our case in which ptosis and extraocular symptoms were completely resolved by surgery and radiation. Such unique cases underline the significance of suspecting metastatic disease in appropriate demographic groups and the crucial role of multidisciplinary care for oncologic patients.
CONCLUSIONS
A high index of suspicion for PMs in elderly people with new-onset sellar symptoms and early involvement of multidisciplinary teams can lead to prevention and even reversal of serious neurologic symptoms.
INTRODUCTION
Prostate cancer rarely metastasizes to the pituitary gland and the close relationship of these sellar masses to cavernous sinuses and major vessels makes management challenging. We describe a unique case of complete reversal of ptosis in metastatic prostate cancer presenting as cavernous sinus syndrome
CASE REPORT
A 76-year-old male presented with left diplopia, ptosis, and facial numbness. Examination showed left oculomotor palsy and numbness in the V1 and V2 distribution of trigeminal nerve. MRI revealed an 11 × 26 × 17 mm posterior sellar mass extending into the left cavernous sinus. Prolactin was slightly elevated, but rest of the pituitary hormones were normal. Resection of the sellar mass showed metastatic prostatic adenocarcinoma positive for NKX-3.1 and prostate-specific antigen (PSA), and Gleason score 4. PSA was elevated at 32 ng/ mL. Positron emission tomography (PET) showed lesions in the left prostatic lobe, pelvic lymph nodes, L5 spine, and right femoral head. FoundationOne testing found no actionable mutations. He was started on leuprorelin-docetaxel and received radiation for the brain and bony lesions. He is currently being maintained on leuprorelin-abiraterone and prednisone, which he is tolerating well.
DISCUSSION
Pituitary metastases (PMs) from prostate cancer are rare and are usually confined to the posterior pituitary. The close relationship of pituitary masses to the cavernous sinuses and internal carotid artery can lead to catastrophic neurovascular consequences. Imaging has limited sensitivity for differentiating non-invasive metastases from adenomas. Older age, new-onset diabetes insipidus, invasive masses, and rapidly growing lesions should raise suspicion for PMs. Intracranial prostatic metastases indicate poor prognosis with a reported median survival of 6-10 months. Timely diagnosis and management can prevent permanent neurologic damage as illustrated by our case in which ptosis and extraocular symptoms were completely resolved by surgery and radiation. Such unique cases underline the significance of suspecting metastatic disease in appropriate demographic groups and the crucial role of multidisciplinary care for oncologic patients.
CONCLUSIONS
A high index of suspicion for PMs in elderly people with new-onset sellar symptoms and early involvement of multidisciplinary teams can lead to prevention and even reversal of serious neurologic symptoms.
A Rare Case of HHV8+ Multicentric Castleman Disease Presenting as Dermatitis
Introduction
Castleman disease (CD) is a rare non-neoplastic disorder presenting as lymphadenopathy. Skin involvement and progression to lymphomas are uncommon, and such presentation can pose a diagnostic challenge. We describe an interesting case of multicentric CD presenting as a rash.
Case Description
A 79-year-old male presented with a 1-year history of blanchable maculopapular rash and new onset dyspnea in the absence of fever, fatigue or weight loss. Examination revealed axillary, cervical and inguinal lymphadenopathy, and firm splenomegaly. Initial labs were notable for leukocytosis, occasional lymphoplasmacytic cells, anemia, thrombocytopenia, negative HIV screen, and elevated ESR and LDH. Further testing identified polyclonal hypergammaglobulinemia. CT scans revealed generalized lymphadenopathy, splenomegaly with infarcts and unilateral pleural effusion. An inguinal lymph node needle biopsy, skin biopsy and pleural fluid cytology were concerning for lymphoplasmacytic, so he was started on rituximab and bendamustine. However, B cell clonality could not be demonstrated, making these findings concerning for Castleman disease.
Results
Human herpesvirus 8 (HHV-8) testing performed on the inguinal lymph node sample came out positive, and he was diagnosed with HHV-8 positive multicentric Castleman disease and continued on weekly rituximab. He demonstrated an excellent response with complete resolution of rash, palpable lymphadenopathy and anemia after 4 cycles of treatment.
Discussion
Castleman disease (CD) is a rare disorder of polyclonal B cell proliferation classically presenting as lymphadenopathy with constitutional symptoms. Cutaneous presentations include eruptive angiomas or petechial rash but can be variable. Intrinsic or viral IL-6 play a key role in the pathogenesis of the disease. CD can be localised or multicentric (related to HHV-8 +/- HIV or idiopathic), and these subtypes differ in prognosis and management, with HIV and HHV-8 co-positivity indicating worse outcomes. While human IL-6 in unicentric and idiopathic multicentric disease respond well to IL-6 receptor antagonists, viral IL-6 in HHV-8 associated cases has a limited response. This is the rationale for preferring anti-CD20 therapy with rituximab in these patients.
Conculsions
Correct biopsy specimen, keen analysis of distinct pathologic features, and HHV-8 testing on tissue sample guide the diagnosis as HHV-8 serology can be falsely negative.
Introduction
Castleman disease (CD) is a rare non-neoplastic disorder presenting as lymphadenopathy. Skin involvement and progression to lymphomas are uncommon, and such presentation can pose a diagnostic challenge. We describe an interesting case of multicentric CD presenting as a rash.
Case Description
A 79-year-old male presented with a 1-year history of blanchable maculopapular rash and new onset dyspnea in the absence of fever, fatigue or weight loss. Examination revealed axillary, cervical and inguinal lymphadenopathy, and firm splenomegaly. Initial labs were notable for leukocytosis, occasional lymphoplasmacytic cells, anemia, thrombocytopenia, negative HIV screen, and elevated ESR and LDH. Further testing identified polyclonal hypergammaglobulinemia. CT scans revealed generalized lymphadenopathy, splenomegaly with infarcts and unilateral pleural effusion. An inguinal lymph node needle biopsy, skin biopsy and pleural fluid cytology were concerning for lymphoplasmacytic, so he was started on rituximab and bendamustine. However, B cell clonality could not be demonstrated, making these findings concerning for Castleman disease.
Results
Human herpesvirus 8 (HHV-8) testing performed on the inguinal lymph node sample came out positive, and he was diagnosed with HHV-8 positive multicentric Castleman disease and continued on weekly rituximab. He demonstrated an excellent response with complete resolution of rash, palpable lymphadenopathy and anemia after 4 cycles of treatment.
Discussion
Castleman disease (CD) is a rare disorder of polyclonal B cell proliferation classically presenting as lymphadenopathy with constitutional symptoms. Cutaneous presentations include eruptive angiomas or petechial rash but can be variable. Intrinsic or viral IL-6 play a key role in the pathogenesis of the disease. CD can be localised or multicentric (related to HHV-8 +/- HIV or idiopathic), and these subtypes differ in prognosis and management, with HIV and HHV-8 co-positivity indicating worse outcomes. While human IL-6 in unicentric and idiopathic multicentric disease respond well to IL-6 receptor antagonists, viral IL-6 in HHV-8 associated cases has a limited response. This is the rationale for preferring anti-CD20 therapy with rituximab in these patients.
Conculsions
Correct biopsy specimen, keen analysis of distinct pathologic features, and HHV-8 testing on tissue sample guide the diagnosis as HHV-8 serology can be falsely negative.
Introduction
Castleman disease (CD) is a rare non-neoplastic disorder presenting as lymphadenopathy. Skin involvement and progression to lymphomas are uncommon, and such presentation can pose a diagnostic challenge. We describe an interesting case of multicentric CD presenting as a rash.
Case Description
A 79-year-old male presented with a 1-year history of blanchable maculopapular rash and new onset dyspnea in the absence of fever, fatigue or weight loss. Examination revealed axillary, cervical and inguinal lymphadenopathy, and firm splenomegaly. Initial labs were notable for leukocytosis, occasional lymphoplasmacytic cells, anemia, thrombocytopenia, negative HIV screen, and elevated ESR and LDH. Further testing identified polyclonal hypergammaglobulinemia. CT scans revealed generalized lymphadenopathy, splenomegaly with infarcts and unilateral pleural effusion. An inguinal lymph node needle biopsy, skin biopsy and pleural fluid cytology were concerning for lymphoplasmacytic, so he was started on rituximab and bendamustine. However, B cell clonality could not be demonstrated, making these findings concerning for Castleman disease.
Results
Human herpesvirus 8 (HHV-8) testing performed on the inguinal lymph node sample came out positive, and he was diagnosed with HHV-8 positive multicentric Castleman disease and continued on weekly rituximab. He demonstrated an excellent response with complete resolution of rash, palpable lymphadenopathy and anemia after 4 cycles of treatment.
Discussion
Castleman disease (CD) is a rare disorder of polyclonal B cell proliferation classically presenting as lymphadenopathy with constitutional symptoms. Cutaneous presentations include eruptive angiomas or petechial rash but can be variable. Intrinsic or viral IL-6 play a key role in the pathogenesis of the disease. CD can be localised or multicentric (related to HHV-8 +/- HIV or idiopathic), and these subtypes differ in prognosis and management, with HIV and HHV-8 co-positivity indicating worse outcomes. While human IL-6 in unicentric and idiopathic multicentric disease respond well to IL-6 receptor antagonists, viral IL-6 in HHV-8 associated cases has a limited response. This is the rationale for preferring anti-CD20 therapy with rituximab in these patients.
Conculsions
Correct biopsy specimen, keen analysis of distinct pathologic features, and HHV-8 testing on tissue sample guide the diagnosis as HHV-8 serology can be falsely negative.