User login
Metastatic Urothelial Carcinoma Presenting as Mediastinal Lymphadenopathy Without Appreciable Bladder Mass in a Patient With Chronic Lymphocytic Leukemia
INTRODUCTION
Lymphadenopathy in Chronic Lymphocytic Leukemia (CLL) is a very common feature. However, sudden increase in lymphadenopathy or other symptoms like weight loss should be evaluated for possible metastatic malignancy. We describe a CLL patient with diffuse mediastinal lymphadenopathy who was diagnosed with metastatic bladder cancer without a primary bladder tumor mass on imaging.
CASE DESCRIPTION
A 60-year-old man with a 60 pack-year smoking history, alcoholic cirrhosis, and a 5-year history of stage 1 CLL presented with 3 months of progressive shortness of breath; persistent cough; chills; hemoptysis; and a steady weight loss of 35 lbs. Notably, he had no bladder symptoms. Initial labs showed leukocytosis of 35.8k with a lymphocytic predominance. Screening low-dose chest CT was positive for diffuse mediastinal lymphadenopathy. Subsequent PET/CT revealed numerous hypermetabolic lymph nodes in the neck, mediastinum, left hilum, and right periaortic abdominal region. CT Chest, Abdomen, Pelvis revealed progressive lymphadenopathy as seen in prior imaging, stable pulmonary nodules up to 4 mm in size, and splenomegaly. No distant primary sites, including of the bladder, were identified. Mediastinal lymph node biopsy confirmed metastatic poorly differentiated carcinoma with immunohistochemical staining negative for p40, p63, CK20, TTF-1, Napsin A, CDX2, CA19- 9, Calretinin, and D2-40 and positive for CK7, GATA3, Ber-EP4, and Uroplakin, supporting bladder as primary origin. Urology deferred a cystoscopy given his lack of urinary symptoms and positive biopsy and was started on Carboplatin/Gemcitabine for his metastatic disease. He was ineligible for Cisplatin given his cirrhosis and hearing impairment.
DISCUSSION
In patients with CLL, new onset mediastinal lymphadenopathy is concerning for disease progression and possible transformation to a diffuse b-cell lymphoma. However, this symptom has a broad differential, including primary lung carcinomas, sarcomas, and metastatic disease. While our patient’s PET/CT and pan-CT failed to identify a distant primary site, maintaining a low clinical suspicion for metastatic disease and doing a thorough work-up was paramount. Only through immunohistochemical staining were we able to diagnosis this patient with urothelial carcinoma.
CONCLUSIONS
Biopsy with immunohistochemical staining and maintaining a low suspicion for worsening lymphadenopathy can identify unusually presenting urothelial carcinomas in CLL patients.
INTRODUCTION
Lymphadenopathy in Chronic Lymphocytic Leukemia (CLL) is a very common feature. However, sudden increase in lymphadenopathy or other symptoms like weight loss should be evaluated for possible metastatic malignancy. We describe a CLL patient with diffuse mediastinal lymphadenopathy who was diagnosed with metastatic bladder cancer without a primary bladder tumor mass on imaging.
CASE DESCRIPTION
A 60-year-old man with a 60 pack-year smoking history, alcoholic cirrhosis, and a 5-year history of stage 1 CLL presented with 3 months of progressive shortness of breath; persistent cough; chills; hemoptysis; and a steady weight loss of 35 lbs. Notably, he had no bladder symptoms. Initial labs showed leukocytosis of 35.8k with a lymphocytic predominance. Screening low-dose chest CT was positive for diffuse mediastinal lymphadenopathy. Subsequent PET/CT revealed numerous hypermetabolic lymph nodes in the neck, mediastinum, left hilum, and right periaortic abdominal region. CT Chest, Abdomen, Pelvis revealed progressive lymphadenopathy as seen in prior imaging, stable pulmonary nodules up to 4 mm in size, and splenomegaly. No distant primary sites, including of the bladder, were identified. Mediastinal lymph node biopsy confirmed metastatic poorly differentiated carcinoma with immunohistochemical staining negative for p40, p63, CK20, TTF-1, Napsin A, CDX2, CA19- 9, Calretinin, and D2-40 and positive for CK7, GATA3, Ber-EP4, and Uroplakin, supporting bladder as primary origin. Urology deferred a cystoscopy given his lack of urinary symptoms and positive biopsy and was started on Carboplatin/Gemcitabine for his metastatic disease. He was ineligible for Cisplatin given his cirrhosis and hearing impairment.
DISCUSSION
In patients with CLL, new onset mediastinal lymphadenopathy is concerning for disease progression and possible transformation to a diffuse b-cell lymphoma. However, this symptom has a broad differential, including primary lung carcinomas, sarcomas, and metastatic disease. While our patient’s PET/CT and pan-CT failed to identify a distant primary site, maintaining a low clinical suspicion for metastatic disease and doing a thorough work-up was paramount. Only through immunohistochemical staining were we able to diagnosis this patient with urothelial carcinoma.
CONCLUSIONS
Biopsy with immunohistochemical staining and maintaining a low suspicion for worsening lymphadenopathy can identify unusually presenting urothelial carcinomas in CLL patients.
INTRODUCTION
Lymphadenopathy in Chronic Lymphocytic Leukemia (CLL) is a very common feature. However, sudden increase in lymphadenopathy or other symptoms like weight loss should be evaluated for possible metastatic malignancy. We describe a CLL patient with diffuse mediastinal lymphadenopathy who was diagnosed with metastatic bladder cancer without a primary bladder tumor mass on imaging.
CASE DESCRIPTION
A 60-year-old man with a 60 pack-year smoking history, alcoholic cirrhosis, and a 5-year history of stage 1 CLL presented with 3 months of progressive shortness of breath; persistent cough; chills; hemoptysis; and a steady weight loss of 35 lbs. Notably, he had no bladder symptoms. Initial labs showed leukocytosis of 35.8k with a lymphocytic predominance. Screening low-dose chest CT was positive for diffuse mediastinal lymphadenopathy. Subsequent PET/CT revealed numerous hypermetabolic lymph nodes in the neck, mediastinum, left hilum, and right periaortic abdominal region. CT Chest, Abdomen, Pelvis revealed progressive lymphadenopathy as seen in prior imaging, stable pulmonary nodules up to 4 mm in size, and splenomegaly. No distant primary sites, including of the bladder, were identified. Mediastinal lymph node biopsy confirmed metastatic poorly differentiated carcinoma with immunohistochemical staining negative for p40, p63, CK20, TTF-1, Napsin A, CDX2, CA19- 9, Calretinin, and D2-40 and positive for CK7, GATA3, Ber-EP4, and Uroplakin, supporting bladder as primary origin. Urology deferred a cystoscopy given his lack of urinary symptoms and positive biopsy and was started on Carboplatin/Gemcitabine for his metastatic disease. He was ineligible for Cisplatin given his cirrhosis and hearing impairment.
DISCUSSION
In patients with CLL, new onset mediastinal lymphadenopathy is concerning for disease progression and possible transformation to a diffuse b-cell lymphoma. However, this symptom has a broad differential, including primary lung carcinomas, sarcomas, and metastatic disease. While our patient’s PET/CT and pan-CT failed to identify a distant primary site, maintaining a low clinical suspicion for metastatic disease and doing a thorough work-up was paramount. Only through immunohistochemical staining were we able to diagnosis this patient with urothelial carcinoma.
CONCLUSIONS
Biopsy with immunohistochemical staining and maintaining a low suspicion for worsening lymphadenopathy can identify unusually presenting urothelial carcinomas in CLL patients.
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.