Adrenal hemorrhage can be associated with trauma, sepsis, hematologic disorders, anticoagulation, pregnancy and adrenal masses (1-4). Masses causing adrenal hemorrhage can be metastases, pheochromocytoma, adrenocortical cancers or hematomas masquerading as neoplasm (2,5-9). Clinical presentation depends upon the rate and degree of hemorrhage. Focal hemorrhage usually presents sub clinically and can be managed conservatively but massive hemorrhage can lead to cardiovascular collapse and ultimately death if not managed early and meticulously. English literatures describing spontaneous adrenal hemorrhage associated with primary adrenal lymphoma is not there to the best of our knowledge. Here we describe a case where we managed a case of spontaneous adrenal hemorrhage surgically which turned out to be primary adrenal lymphoma.
Presentation of Case
67-year-old male patient known case of diabetes, hypertension and ischemic heart disease presents with complain of left abdominal pain since few hours associated with 2 episodes of vomiting. Pain was sudden in onset, severe in intensity located in left flank, non-radiating. No h/o fever, nausea and vomiting. No h/o LUTS, hematuria, lithuria. On examining he was vitally stable with per abdomen distension and tenderness over left flank. Bowel sounds were present with no obvious organomegaly. External genitalia and per rectal examinations were normal. Rest systems were normal. On investigating, laboratory data showed mild anemia (hemoglobin 10.9) with leukocytosis (13,700) and deranged blood sugar level (random blood sugar 350). There was no hepatic, renal or adrenal dysfunction except of low cortisol level from the blood and urine examination he underwent. Also, his coagulation profile was within normal range. Contrast enhanced CT scan showed large ill defined non enhancing collection is seen in left abdomen extending from sub-splenic region to left iliac fossa measuring 22*10*8cm. left adrenal showed 3*4cm lesion and is not visualized separately from the collection. Right adrenal showed an enhancing 2*2cm lesion. No lymph nodes, rest were all normal.In view of above finding, patient was given supportive management in the form of IV fluid and cross match was sent. Endocrinologist opinion was taken for deranged blood sugar with low cortisol level with the background of adrenal bleed. Injection hydrocortisone 100mg tds was started. Patient cardiologist was also informed about his condition. Patient suddenly deteriorated vitally and was taken up for surgery. Left retro peritoneum was entered through flank approach, 11th rib incision- hematoma was evacuated, the adrenal was found to be ruptured, left adrenalectomy was done. Intra operatively 3 packed cell volume and 4 FFP were transfused. Patient was shifted to Surgical ICU and was closely monitored.
Immediately post operatively, serum cortisol was done and it came out to be 20 microgram per deciliter, which was below normal in stressful conditions like surgery, hence the patient was continued with hydrocortisone and his Blood Pressure came normal. Blood sugar and BP were kept under control with astute management of insulin drips and IV fluids. Once patient was stable, he was shifted to urology ward.
Histopathology report described the adrenal lesion as High Grade Diffuse Large B cell lymphoma with hematoma described as blood clots and fibrinoid debris. Oncologist advised a PET-CT whole body which showed uptake in right adrenal., with no lymph nodes. Injection endoxan was started and at present patient is on follow up with oncologist for his chemotherapy.
Adrenal hemorrhage is a rare life-threatening condition which can be secondary to traumatic or non-traumatic etiology. Also, it can be unilateral or bilateral and it has been an autopsy finding in most of the reported cases. 10. Vella et al. have summarized 141 cases of AH in a 25 years’ experience period at the Mayo Clinic . They classified AH into the following seven categories: incidentaloma (28 cases), spontaneous AH (16 cases), AH associated with antiphospholipid- and heparin-associated thrombocytopenia (20 cases), postoperative AH (14 cases), AH associated with anticoagulation therapy (3 cases), AH associated with trauma (4 cases), and AH associated with sepsis (especially meningococcemia) or severe stress (56 cases). Our case is an example of spontaneous adrenal hemorrhage associated with a mass. Adrenal gland being highly vascular is particularly susceptible to hemorrhage and it usually arises from medulla.
Clinical manifestation of adrenal hemorrhage has a wide spectrum depending upon the degree and rate of hemorrhage as well as the amount of gland compromised by the hemorrhage. It can have a subclinical presentation in case of isolated focal unilateral bleed or can present as rapid cardiovascular collapse secondary to massive bilateral adrenal hemorrhage. Depending on the underlying cause, clinical presentation
varies and symptoms may include abdominal or flank pain, nausea, vomiting, weakness, weight loss, or mental confusion (adrenal insufficiency). Those are often accompanied with physical findings of fever, hypotension, abdominal tenderness or distension, and a palpable abdominal mass.
The laboratory findings also vary and may include a falling hematocrit level, leukocytosis, or electrolytes abnormalities. Some authors concluded that diagnosis of adrenal hemorrhage is often complicated by its nonspecific presentation and tendency to occur in the setting of acute ill- ness and other complicating medical conditions. It must include biochemical tests (serum cortisol, adrenocorticotropic hormones, catecholamines, aldosterone and dehydroepiandrosterone) to rule out hyper or hypocortisolemia, hyperaldosternomeia and Conn’s syndrome, pheochromocytoma or virializing tumors. Nonfunctioning tumors vary significantly in appearance and presentation, and, due to that, the preoperative diagnosis is often missed, as others support.
Imaging assists and complements the clinical and endocrine evaluation.
Ultrasonography is usually the first investigation in the emergency room, but its reliability is restricted to infants and kids. Computer tomography (with or without iv contrast) is the cornerstone of imaging studies. Adrenal hemorrhage is usually first diagnosed via CT or MRI. Hematoma appears heterogenous and of high density on CT (50-70 Hounsfield units). Magnetic resonance imaging has been reported to be more accurate than other imaging modalities for diagnosing adrenal hematoma, with high signal intensity on T1-weighted images. Magnetic resonance imaging also may differentiate subacute hemorrhage from chronic hemorrhage but still remains an expensive and not always available imaging modality. In case of a suspected tumor, adrenal scintigraphy has been added by few authors. It can determine whether the tumor is functioning or if there is extra-adrenal disease. Sensitivity ranges from77%to 89%and specificity from88% to 100%, but radiation exposure, high cost, and limited availability restrict its use.