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Paraganglioma Presenting as Postpartum Fever of Unknown Origin.

Narechania S, Bath A, Ghassemi L, Lokhande C, Haddad A, Yousuf AM, Marquard J, Gopalakrishna KV - Case Rep Endocrinol (2015)

Bottom Line: A CT of the abdomen showed a retroperitoneal mass with two small pulmonary nodules and a bony metastatic lesion.She was found to have stage 4 extra-adrenal paraganglioma with metastases to the lungs and spine.Subsequent genetic testing identified a likely pathogenic variant in SDHB, confirming a diagnosis of Hereditary Paraganglioma-Pheochromocytoma syndrome.

View Article: PubMed Central - PubMed

Affiliation: Internal Medicine Residency, Fairview Hospital, Cleveland, OH 44111, USA.

ABSTRACT
A young healthy postpartum mother presented with intermittent high fevers and tachycardia. Appropriate testing was done to rule out infectious causes including pan cultures but no identifiable infectious source was found. A CT of the abdomen showed a retroperitoneal mass with two small pulmonary nodules and a bony metastatic lesion. She was found to have stage 4 extra-adrenal paraganglioma with metastases to the lungs and spine. She underwent resection of the mass and is currently undergoing palliative radiation to the spine for pain control. Subsequent genetic testing identified a likely pathogenic variant in SDHB, confirming a diagnosis of Hereditary Paraganglioma-Pheochromocytoma syndrome.

No MeSH data available.


Related in: MedlinePlus

CT scan abdomen/pelvis with contrast showing large retroperitoneal mass.
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fig1: CT scan abdomen/pelvis with contrast showing large retroperitoneal mass.

Mentions: A 21-year-old primigravida at 37-week gestation presented to the outpatient clinic for a routine obstetrical examination. Her pregnancy course had been uneventful. Her blood pressure at this visit was 184/108. She was asymptomatic except for occasional mild headaches. A urine dipstick showed 3+ proteinuria. Patient was diagnosed with preeclampsia and was admitted to the hospital for emergency induction of labor. She was started on IV magnesium for preeclampsia and labor was induced with oxytocin. She underwent an emergency cesarean section due to fetal decelerations during induction. Subsequently, she delivered a healthy male infant by low transverse C section. Four days after partum, she started spiking high fevers up to 39.5 degrees Celsius and developed tachycardia with heart rate of 140 to 150/min. She was otherwise completely asymptomatic and clinical exam was negative for any localizing signs. Her cesarean section incision looked clean; she had no breast tenderness. Infectious work-up including blood and urine cultures was negative. Her chest X-ray was normal; EKG showed sinus tachycardia. She was empirically started on broad spectrum antibiotics. After 48 hours of IV antibiotics, patient continued to spike high fevers and was still tachycardic. The differential diagnosis of endometritis and septic thrombophlebitis was high on the list even though her abdominal exam was benign. Computerized tomography (CT) scan of the abdomen and pelvis with contrast (Figure 1) was ordered to rule out septic thrombophlebitis, which showed a large right retroperitoneal 9.2 cm anteroposterior × 14 cm transverse × 12.5 cm craniocaudal mass with severe right renal hydronephrosis. Due to absence of any fat layer between the kidney and the mass, it was felt to be coming from the lower pole of the right kidney. The mass extended superiorly to the inferior vena cava (IVC) with a possible tumor thrombus in the IVC. The liver was mildly enlarged measuring 19.6 cm with fatty infiltration. The first segment of the sacrum showed a 3 cm × 2.1 cm × 2.6 cm lytic lesion. CT scan of the chest showed 2 pulmonary nodules measuring 7 mm and 11 mm in the left and right lower lobes of the lung, respectively. Due to the above imaging findings, suspicion was high for a stage 4 metastatic cancer arising from the kidney. Renal ultrasound confirmed a large retroperitoneal mass with hydronephrosis. MRI lumbar spine reported an abnormal marrow signal in the S1–S3 segments and an exophytic bony metastatic lesion in the dorsal aspect of S1 causing severe narrowing of the thecal sac. MRI abdomen/pelvis confirmed extension of the retroperitoneal mass into the inferior vena cava (IVC). MRI brain and CT brain were obtained showing calvarial lesions consistent with hemangiomas. A percutaneous CT guided right renal mass biopsy (Figure 2) revealed a cellular epithelioid proliferation arranged in nests and composed of round to ovoid cells with hyperchromatic nuclei, inconspicuous nucleoli, and granular amphophilic cytoplasm. Immunohistochemical staining was negative for HMB-45, S100, PAX-8, cytokeratin AE1/AE3, CD117, and DOG-1 but was positive for synaptophysin and chromogranin (Figure 3). The overall morphological and immunohistochemical findings were consistent with a diagnosis of paraganglioma. 24-hour urine testing revealed high levels of urinary nor epinephrine which was 862 mcg/24 hours (normal range 15 to 100 mcg/24 hours) and dopamine which was 902 mcg/24 hours (normal range 52 to 480 mcg/24 hours). Plasma-free metanephrine levels were also elevated at 1690 pg/mL (normal range 18 to 101 pg/mL). The patient continued to remain severely hypertensive after delivery and was started on combined alpha and beta blockade with doxazosin 8 mg daily and propranolol 40 mg twice daily. The patient underwent right sided nephrectomy, removal of the retroperitoneal mass, and resection of the perirenal IVC. Her postoperative course was complicated by severe hypotension and extensive bilateral lower extremity deep venous thromboses in the external iliac and common femoral veins. She required vasopressors for two days and was started on therapeutic anticoagulation with enoxaparin for the DVTs. She was discharged home 2 weeks after her surgery. A metaiodobenzylguanidine (MIBG) whole body scan done later as an outpatient did not demonstrate any focal abnormal uptake. Whole body PET scan showed increased FDG uptake in 3 pulmonary nodules, a large lytic sacral lesion, and a T9 vertebral body lesion all consistent with metastases. The patient underwent gamma knife palliative radiation of the S1 lesion and was started on Zometa for her bony metastases. The tyrosine kinase receptor inhibitor Sunitinib was started to treat her systemic disease.


Paraganglioma Presenting as Postpartum Fever of Unknown Origin.

Narechania S, Bath A, Ghassemi L, Lokhande C, Haddad A, Yousuf AM, Marquard J, Gopalakrishna KV - Case Rep Endocrinol (2015)

CT scan abdomen/pelvis with contrast showing large retroperitoneal mass.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4508462&req=5

fig1: CT scan abdomen/pelvis with contrast showing large retroperitoneal mass.
Mentions: A 21-year-old primigravida at 37-week gestation presented to the outpatient clinic for a routine obstetrical examination. Her pregnancy course had been uneventful. Her blood pressure at this visit was 184/108. She was asymptomatic except for occasional mild headaches. A urine dipstick showed 3+ proteinuria. Patient was diagnosed with preeclampsia and was admitted to the hospital for emergency induction of labor. She was started on IV magnesium for preeclampsia and labor was induced with oxytocin. She underwent an emergency cesarean section due to fetal decelerations during induction. Subsequently, she delivered a healthy male infant by low transverse C section. Four days after partum, she started spiking high fevers up to 39.5 degrees Celsius and developed tachycardia with heart rate of 140 to 150/min. She was otherwise completely asymptomatic and clinical exam was negative for any localizing signs. Her cesarean section incision looked clean; she had no breast tenderness. Infectious work-up including blood and urine cultures was negative. Her chest X-ray was normal; EKG showed sinus tachycardia. She was empirically started on broad spectrum antibiotics. After 48 hours of IV antibiotics, patient continued to spike high fevers and was still tachycardic. The differential diagnosis of endometritis and septic thrombophlebitis was high on the list even though her abdominal exam was benign. Computerized tomography (CT) scan of the abdomen and pelvis with contrast (Figure 1) was ordered to rule out septic thrombophlebitis, which showed a large right retroperitoneal 9.2 cm anteroposterior × 14 cm transverse × 12.5 cm craniocaudal mass with severe right renal hydronephrosis. Due to absence of any fat layer between the kidney and the mass, it was felt to be coming from the lower pole of the right kidney. The mass extended superiorly to the inferior vena cava (IVC) with a possible tumor thrombus in the IVC. The liver was mildly enlarged measuring 19.6 cm with fatty infiltration. The first segment of the sacrum showed a 3 cm × 2.1 cm × 2.6 cm lytic lesion. CT scan of the chest showed 2 pulmonary nodules measuring 7 mm and 11 mm in the left and right lower lobes of the lung, respectively. Due to the above imaging findings, suspicion was high for a stage 4 metastatic cancer arising from the kidney. Renal ultrasound confirmed a large retroperitoneal mass with hydronephrosis. MRI lumbar spine reported an abnormal marrow signal in the S1–S3 segments and an exophytic bony metastatic lesion in the dorsal aspect of S1 causing severe narrowing of the thecal sac. MRI abdomen/pelvis confirmed extension of the retroperitoneal mass into the inferior vena cava (IVC). MRI brain and CT brain were obtained showing calvarial lesions consistent with hemangiomas. A percutaneous CT guided right renal mass biopsy (Figure 2) revealed a cellular epithelioid proliferation arranged in nests and composed of round to ovoid cells with hyperchromatic nuclei, inconspicuous nucleoli, and granular amphophilic cytoplasm. Immunohistochemical staining was negative for HMB-45, S100, PAX-8, cytokeratin AE1/AE3, CD117, and DOG-1 but was positive for synaptophysin and chromogranin (Figure 3). The overall morphological and immunohistochemical findings were consistent with a diagnosis of paraganglioma. 24-hour urine testing revealed high levels of urinary nor epinephrine which was 862 mcg/24 hours (normal range 15 to 100 mcg/24 hours) and dopamine which was 902 mcg/24 hours (normal range 52 to 480 mcg/24 hours). Plasma-free metanephrine levels were also elevated at 1690 pg/mL (normal range 18 to 101 pg/mL). The patient continued to remain severely hypertensive after delivery and was started on combined alpha and beta blockade with doxazosin 8 mg daily and propranolol 40 mg twice daily. The patient underwent right sided nephrectomy, removal of the retroperitoneal mass, and resection of the perirenal IVC. Her postoperative course was complicated by severe hypotension and extensive bilateral lower extremity deep venous thromboses in the external iliac and common femoral veins. She required vasopressors for two days and was started on therapeutic anticoagulation with enoxaparin for the DVTs. She was discharged home 2 weeks after her surgery. A metaiodobenzylguanidine (MIBG) whole body scan done later as an outpatient did not demonstrate any focal abnormal uptake. Whole body PET scan showed increased FDG uptake in 3 pulmonary nodules, a large lytic sacral lesion, and a T9 vertebral body lesion all consistent with metastases. The patient underwent gamma knife palliative radiation of the S1 lesion and was started on Zometa for her bony metastases. The tyrosine kinase receptor inhibitor Sunitinib was started to treat her systemic disease.

Bottom Line: A CT of the abdomen showed a retroperitoneal mass with two small pulmonary nodules and a bony metastatic lesion.She was found to have stage 4 extra-adrenal paraganglioma with metastases to the lungs and spine.Subsequent genetic testing identified a likely pathogenic variant in SDHB, confirming a diagnosis of Hereditary Paraganglioma-Pheochromocytoma syndrome.

View Article: PubMed Central - PubMed

Affiliation: Internal Medicine Residency, Fairview Hospital, Cleveland, OH 44111, USA.

ABSTRACT
A young healthy postpartum mother presented with intermittent high fevers and tachycardia. Appropriate testing was done to rule out infectious causes including pan cultures but no identifiable infectious source was found. A CT of the abdomen showed a retroperitoneal mass with two small pulmonary nodules and a bony metastatic lesion. She was found to have stage 4 extra-adrenal paraganglioma with metastases to the lungs and spine. She underwent resection of the mass and is currently undergoing palliative radiation to the spine for pain control. Subsequent genetic testing identified a likely pathogenic variant in SDHB, confirming a diagnosis of Hereditary Paraganglioma-Pheochromocytoma syndrome.

No MeSH data available.


Related in: MedlinePlus