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Renal medullary cancer in a patient with sickle cell trait.

Alappan N, Marak CP, Chopra A, Joy PS, Dorokhova O, Guddati AK - Case Rep Oncol Med (2013)

Bottom Line: These patients often present with the cardinal symptoms of hematuria, flank pain, and an abdominal mass, and this malignancy has been associated with patients carrying sickle cell trait.Therefore, a high index of suspicion in a patient of African descent presenting with a right sided abdominal mass and hematuria may assist in an early diagnosis.It may also provide a bigger time frame for the initiation of novel chemotherapy regimens in patients who fail current chemotherapy regimens.

View Article: PubMed Central - PubMed

Affiliation: Division of Pulmonary and Critical Care Medicine, Montefiore Hospital, Albert Einstein College of Medicine, Yeshiva University, New York, NY, USA.

ABSTRACT
Renal medullary cancer is a rare malignancy almost exclusively seen in young patients of African ethnicity. These patients often present with the cardinal symptoms of hematuria, flank pain, and an abdominal mass, and this malignancy has been associated with patients carrying sickle cell trait. It is estimated that 300 million people worldwide carry sickle cell trait, and the presence of hematuria in these patients should be treated as a harbinger of a possible malignancy. Notably, this tumor mostly develops on the right side of the body. Patients often present with it at an advanced stage and the prognosis is poor. Therefore, a high index of suspicion in a patient of African descent presenting with a right sided abdominal mass and hematuria may assist in an early diagnosis. Current chemotherapy options are very limited, and early detection may provide a chance for surgical resection. It may also provide a bigger time frame for the initiation of novel chemotherapy regimens in patients who fail current chemotherapy regimens.

No MeSH data available.


Related in: MedlinePlus

(a) CT scan of the chest in lung windows shows large right pleural effusion, small left pleural effusion, and multiple pulmonary nodules and masses. (b) CT scan of the abdomen shows irregular left lower pole low attenuation cystic renal mass with adjacent lymphadenopathy.
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fig1: (a) CT scan of the chest in lung windows shows large right pleural effusion, small left pleural effusion, and multiple pulmonary nodules and masses. (b) CT scan of the abdomen shows irregular left lower pole low attenuation cystic renal mass with adjacent lymphadenopathy.

Mentions: A 33-year-old African American gentleman, who immigrated to the United States from Cameroon 8 years ago presented with complaints of exertional shortness of breath, orthopnea, and nonproductive cough for duration of 1 week. He had a history of hypertension well controlled with amlodipine. He had been a life-long nonsmoker and denied use of any illicit drugs. He also denied any fever or chills or upper respiratory symptoms, oliguria or pedal edema. Review of other systems was unremarkable. On examination, he was noted to be in mild respiratory distress but was able to converse in full sentences. He was afebrile; blood pressure was 133/83 mmHg and heart rate was 91/minute, breathing at a rate of 24/minute with an oxygen saturation of 96% on ambient air. He did not have palpable cervical, axillary, or epitrochlear lymph nodes. His chest auscultation was significant for decreased breath sounds in the right base with dullness to percussion, and the left lung had clear vesicular breath sounds. His abdomen was nondistended, without any organomegaly. Initial routine laboratory investigation was significant for a normal white count with differentials, normal hemoglobin, and platelets, blood urea nitrogen of 11 mg/dL, and serum creatinine of 1.2 mg/dL. The urine analysis was remarkable for occult blood but had no protein or glucose. Chest X-ray (CXR) revealed a large right pleural effusion, a small left pleural effusion, and multifocal bilateral pulmonary opacities. This prompted a computed tomography (CT) scan of the chest and abdomen, which confirmed a large loculated right pleural effusion, small layering left pleural effusion, and multiple pleural based nodular enhancements, the largest of which measured 9.5 × 1.6 cm. There were innumerable pulmonary nodules throughout the left lung and visualized portion of the right lung. The left hilar lymph nodes were significantly enlarged. There was also a 5.8 × 5.2 × 5 cm irregular left lower pole low attenuation cystic renal mass, with adjacent 2.6 × 2.1 cm left para-aortic lymphadenopathy and 1 × 1 cm exophytic cyst from the lower pole of the right kidney (Figures 1(a) and 1(b)). The ultrasound of the scrotum revealed a 3 × 2.2 × 2.9 cm fluid collection superior to the right testis suggestive of spermatocele. Serum tumor markers like Alpha Feto-Protein and serum human Chorionic Gonadotropin were unremarkable. A therapeutic thoracentesis on the right chest was done, and 1.8 L of serosanguinous fluid was removed. Pleural fluid analysis revealed a white blood cell count of 1175/mm3, predominantly polymorphic neutrophils (70%), with lactate dehydrogenase of 271 U/L, glucose of 63 mg/dL and total protein of 5 g/dL, suggestive of an exudate. Cultures for bacterial, fungal, and acid fast bacilli were negative. Cytology was reported to be negative for malignant cells. Video-assisted thoracoscopic surgery (VATS) was requested to obtain definitive diagnosis. Inspection of the pleural cavity after evacuation of 1 L of bloody fluid during VATS revealed multiple deposits in both the parietal and visceral pleura, which were biopsied. Histopathological examination of the pleural biopsies revealed infiltrating tumor growth demonstrating reticular, solid, microcystic, adenoid cystic patterns and desmoplastic stroma. The cells were pleomorphic with large vesicular nuclei with variable cytoplasm (Figures 2(a) and 2(b)). Immunohistochemistry was positive for cytokeratins: CK7, CK19, CK20 (focal staining), CK903, epithelial glycoprotein-2 (MOC31), epithelial cell adhesion molecule (BerEp), epithelial membrane antigen (EMA), mucin 1 (MUC1), vimentin, and calretinin (Figures 3(a), 3(b), 3(c), and 3(d)). They were negative for thyroid transcription factor-1 (TTF1), napsin; caudal type homeobox transcription factor 2 (CDX2), carcinoembryonic antigen (CEA); and human epidermal growth factor receptor 2 (Her2/neu), O-linked sialoglycoprotein (MW 40 kDa) [D2-40], transformation-related protein 63 (p63), cytokeratins 5/6 (CK5/6), placental alkaline phosphatase (PLAP), AFP, CD30, and alpha-methylacyl CoA racemase (p504S). These features were indicative of poorly differentiated adenocarcinoma of an unknown primary. Given the African American ethnicity of the patient, renal mass and metastatic poorly differentiated adenocarcinoma, a diagnosis of metastatic renal medullary cancer was considered. This diagnosis was further supported when hemoglobin electrophoresis results were found to have a sickle cell trait (HbS-40.4% and HbA-55.6%). His post-operative period was otherwise uneventful. Unfortunately, he was lost to further follow up.


Renal medullary cancer in a patient with sickle cell trait.

Alappan N, Marak CP, Chopra A, Joy PS, Dorokhova O, Guddati AK - Case Rep Oncol Med (2013)

(a) CT scan of the chest in lung windows shows large right pleural effusion, small left pleural effusion, and multiple pulmonary nodules and masses. (b) CT scan of the abdomen shows irregular left lower pole low attenuation cystic renal mass with adjacent lymphadenopathy.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3814076&req=5

fig1: (a) CT scan of the chest in lung windows shows large right pleural effusion, small left pleural effusion, and multiple pulmonary nodules and masses. (b) CT scan of the abdomen shows irregular left lower pole low attenuation cystic renal mass with adjacent lymphadenopathy.
Mentions: A 33-year-old African American gentleman, who immigrated to the United States from Cameroon 8 years ago presented with complaints of exertional shortness of breath, orthopnea, and nonproductive cough for duration of 1 week. He had a history of hypertension well controlled with amlodipine. He had been a life-long nonsmoker and denied use of any illicit drugs. He also denied any fever or chills or upper respiratory symptoms, oliguria or pedal edema. Review of other systems was unremarkable. On examination, he was noted to be in mild respiratory distress but was able to converse in full sentences. He was afebrile; blood pressure was 133/83 mmHg and heart rate was 91/minute, breathing at a rate of 24/minute with an oxygen saturation of 96% on ambient air. He did not have palpable cervical, axillary, or epitrochlear lymph nodes. His chest auscultation was significant for decreased breath sounds in the right base with dullness to percussion, and the left lung had clear vesicular breath sounds. His abdomen was nondistended, without any organomegaly. Initial routine laboratory investigation was significant for a normal white count with differentials, normal hemoglobin, and platelets, blood urea nitrogen of 11 mg/dL, and serum creatinine of 1.2 mg/dL. The urine analysis was remarkable for occult blood but had no protein or glucose. Chest X-ray (CXR) revealed a large right pleural effusion, a small left pleural effusion, and multifocal bilateral pulmonary opacities. This prompted a computed tomography (CT) scan of the chest and abdomen, which confirmed a large loculated right pleural effusion, small layering left pleural effusion, and multiple pleural based nodular enhancements, the largest of which measured 9.5 × 1.6 cm. There were innumerable pulmonary nodules throughout the left lung and visualized portion of the right lung. The left hilar lymph nodes were significantly enlarged. There was also a 5.8 × 5.2 × 5 cm irregular left lower pole low attenuation cystic renal mass, with adjacent 2.6 × 2.1 cm left para-aortic lymphadenopathy and 1 × 1 cm exophytic cyst from the lower pole of the right kidney (Figures 1(a) and 1(b)). The ultrasound of the scrotum revealed a 3 × 2.2 × 2.9 cm fluid collection superior to the right testis suggestive of spermatocele. Serum tumor markers like Alpha Feto-Protein and serum human Chorionic Gonadotropin were unremarkable. A therapeutic thoracentesis on the right chest was done, and 1.8 L of serosanguinous fluid was removed. Pleural fluid analysis revealed a white blood cell count of 1175/mm3, predominantly polymorphic neutrophils (70%), with lactate dehydrogenase of 271 U/L, glucose of 63 mg/dL and total protein of 5 g/dL, suggestive of an exudate. Cultures for bacterial, fungal, and acid fast bacilli were negative. Cytology was reported to be negative for malignant cells. Video-assisted thoracoscopic surgery (VATS) was requested to obtain definitive diagnosis. Inspection of the pleural cavity after evacuation of 1 L of bloody fluid during VATS revealed multiple deposits in both the parietal and visceral pleura, which were biopsied. Histopathological examination of the pleural biopsies revealed infiltrating tumor growth demonstrating reticular, solid, microcystic, adenoid cystic patterns and desmoplastic stroma. The cells were pleomorphic with large vesicular nuclei with variable cytoplasm (Figures 2(a) and 2(b)). Immunohistochemistry was positive for cytokeratins: CK7, CK19, CK20 (focal staining), CK903, epithelial glycoprotein-2 (MOC31), epithelial cell adhesion molecule (BerEp), epithelial membrane antigen (EMA), mucin 1 (MUC1), vimentin, and calretinin (Figures 3(a), 3(b), 3(c), and 3(d)). They were negative for thyroid transcription factor-1 (TTF1), napsin; caudal type homeobox transcription factor 2 (CDX2), carcinoembryonic antigen (CEA); and human epidermal growth factor receptor 2 (Her2/neu), O-linked sialoglycoprotein (MW 40 kDa) [D2-40], transformation-related protein 63 (p63), cytokeratins 5/6 (CK5/6), placental alkaline phosphatase (PLAP), AFP, CD30, and alpha-methylacyl CoA racemase (p504S). These features were indicative of poorly differentiated adenocarcinoma of an unknown primary. Given the African American ethnicity of the patient, renal mass and metastatic poorly differentiated adenocarcinoma, a diagnosis of metastatic renal medullary cancer was considered. This diagnosis was further supported when hemoglobin electrophoresis results were found to have a sickle cell trait (HbS-40.4% and HbA-55.6%). His post-operative period was otherwise uneventful. Unfortunately, he was lost to further follow up.

Bottom Line: These patients often present with the cardinal symptoms of hematuria, flank pain, and an abdominal mass, and this malignancy has been associated with patients carrying sickle cell trait.Therefore, a high index of suspicion in a patient of African descent presenting with a right sided abdominal mass and hematuria may assist in an early diagnosis.It may also provide a bigger time frame for the initiation of novel chemotherapy regimens in patients who fail current chemotherapy regimens.

View Article: PubMed Central - PubMed

Affiliation: Division of Pulmonary and Critical Care Medicine, Montefiore Hospital, Albert Einstein College of Medicine, Yeshiva University, New York, NY, USA.

ABSTRACT
Renal medullary cancer is a rare malignancy almost exclusively seen in young patients of African ethnicity. These patients often present with the cardinal symptoms of hematuria, flank pain, and an abdominal mass, and this malignancy has been associated with patients carrying sickle cell trait. It is estimated that 300 million people worldwide carry sickle cell trait, and the presence of hematuria in these patients should be treated as a harbinger of a possible malignancy. Notably, this tumor mostly develops on the right side of the body. Patients often present with it at an advanced stage and the prognosis is poor. Therefore, a high index of suspicion in a patient of African descent presenting with a right sided abdominal mass and hematuria may assist in an early diagnosis. Current chemotherapy options are very limited, and early detection may provide a chance for surgical resection. It may also provide a bigger time frame for the initiation of novel chemotherapy regimens in patients who fail current chemotherapy regimens.

No MeSH data available.


Related in: MedlinePlus