Cavernous adrenal hemangioma

Cavernous adrenal hemangioma

CASE REPORT CAVERNOUS ADRENAL HEMANGIOMA EDMUND SABANEGH, JR ., M .D . MICHAEL J . HARRIS, M .D . DOUGLAS GRIDER, M .D . From the Departments of Urol...

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CASE REPORT

CAVERNOUS ADRENAL HEMANGIOMA EDMUND SABANEGH, JR ., M .D . MICHAEL J . HARRIS, M .D . DOUGLAS GRIDER, M .D . From the Departments of Urology and Pathology, Wilford Hall USAF Medical Center, San Antonio, Texas

ABSTRACT-Cavernous hemangiomas are unusual tumors with a propensity for skin and liver involvement and rarely involving genitourinary organs . We report an unusual case of adrenal hemangioma which was successfully treated surgically . In addition, we review the salient clinical, radiographic, and pathologic features of this disease .

Adrenal hemangiomas are rare, nonfunctioning tumors, usually discovered at autopsy However, over the past fifteen years, increasing numbers of cases have come to clinical attention, often as incidental findings noted on radiographic imaging for nonurologic complaints . Certain features of this unique tumor, although not pathognomonic, should suggest the diagnosis to the clinician . We present a case of a cavernous hemangioma of the adrenal gland which was successfully treated surgically. In addition, we review the salient clinical, radiographic, and pathologic features of this disease . CASE REPORT A sixty-year-old Hispanic woman was referred to the urology service for evaluation of left upper quadrant abdominal calcifications noted on spine radiographs for workup of low-back pain . She reported that the onset of her pain coincided with a fall onto her left side which had occurred one year prior to her presentation . Past medical history was only notable for a long history of adult onset diabetes mellitus which was well controlled on a single oral agent. Physical examination revealed an obese female with a 20 x 20 cm non-tender mass in the left upper abdominal quadrant . She had no clinical manifestations of adrenal medullary or cortical hyperfunction . All admitting laboratory values including complete blood count, urinalysis, and The opinions expressed herein are those of the authors and do not necessarily reflect those of the Department of the Air Force or the Department of Defense . Submitted : March 26, 1993, accepted : April 2, 1993

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serum metabolic profile (SMA-17) were within normal range . Endocrine evaluation including serum and urine catecholamines and steroid levels was also normal . Abdominal ultrasound revealed a large heterogeneous left suprarenal mass with multiple hypoechoic areas (Fig . 1) . Computerized tomography scan confirmed these findings as well as noting a lack of contrast enhancement and the presence of multiple speckled calcifications (Fig . 2) . Magnetic resonance imaging (MRI) was performed in the sagittal, coronal, and axial planes with both T i and Ta weighted images . T t weighted images provided excellent anatomic definition of the mass showing it to be positioned immediately superior to the left renal hilum (Fig . 3) . Areas of high-signal intensity on T t weighted images were consistent with focal hemorrhage . The patient was explored through a left thoracoabdominal incision . The mass was well-encapsulated with an easy plane of dissection between the mass and the renal hilum . Following the left adrenalectomy, her abdomen was explored, and no other pathology was noted . Her convalescence was uneventful, and she was discharged from the hospital on postoperative day 7 . PATHOLOGIC FINDINGS The surgical specimen consisted of a well-circumscribed, encapsulated, (15 cm) spheroidal mass, weighing 1,076 g . Serial sectioning revealed a fibrous capsule enclosing a thin rim of yellow soft tissue . Beneath this yellow rim were variably sized fluid-filled cystic spaces (Fig . 4) . Microscopically, the periphery of the mass consisted of a thick fibrous capsule enclosing a thin rim of normal-appearing adrenal cortex . Beneath the adrenal 327

FIGURE 1 .

Ultrasound reveals large heterogeneous left suprarenal mass with multiple calcifications .

Contrast-enhanced CT scan shows large heterogeneous left adrenal mass with internal calcification .

Cross specimen contains well-circumscribed 15-cm soft tissue mass with areas of organizing hematoma, hemorrhage, and thin rim of normal tissue at periphery . FIGURE 4 .

FIGURE 2 .

Below capsule and thin rim of adrenal cortical tissue are endothelial lined vascular spaces and organizing hemorrhage (hematoxylin and eosin ; original magnification x 200) . FIGURE 5 .

cortical cells were massively dilated lacunae lined by endothelial cells among a deposition of fibrin (Fig. 5) . The lining cells of all the vascular spaces were Factor VIII immunohistochemical reaction positive (Dako Monoclonal Mouse Anti-Human Von Willebrand Factor) demonstrating their endothelial nature . The remainder of the specimen showed organizing hematoma with focal areas of hemosiderin deposition and calcification . COMMENT

FIGURE 3 . FI weighted MRI sagittal image shows large left adrenal mass (A) in close proximity to left renal hilum, displacing kidney (K) inferiorly-

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Cavernous hemangiomas are unusual tumors, showing a propensity for skin and liver involvement and rarely involving genitourinary organs . While there have been several autopsy reports of adrenal hemangiomas dating back as far as 1869, the first clinical presentation was not reported UROLOGY

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NUMBER 3

TABLE] .

Series

Cavernous adrenal hemangioma

Age (Yrs .)

Gender

Incidental

Johnson and Jeppesen' Weiss and Schulteb Ruebel' Rothbergetal. 4

46 70 74 72 74

F

Y

Vargas' Orringer et al .' Derchi etaL&

67 51 69 60 73

F M F F F M F M M

Y Y Y Y Y N N N Y

74 60

F F

Y Y

Honigetal .s Salupetal . 9 Sabanegh et

al.

until 1955 by Johnson and Jeppesen .' Since that time, there have been 13 clinical cases reported in the literature (Table I) . 2.10 Patient age ranged between forty-seven and seventy-four years with the vast majority in the sixth or seventh decade of life . Reported cases show a clear predilection for women with a 2 :1 female :male ratio. Bilateral adrenal involvement has been described twice .2 Cavernous adrenal hemangiomas show no characteristic symptomatology unless they reach a sufficient size to produce mechanical pressure on adjacent organs . In fact, 75 percent of the reported cases were discovered incidentally during workup of unrelated complaints . Symptoms from adrenal medullary or cortical hyperfunction have not been noted with adrenal hemangiomas, in contrast with adrenal cortical carcinoma, pheochromocytoma, and other hormonally active adrenal tumors . There has been one report of significant hemorrhage induced by vigorous palpation of an adrenal hemangioma during an initial physical examination .' This raises questions about the safety of diagnostic fine-needle aspiration and needle biopsy of adrenal masses . Since the differential diagnosis between benign and malignant adrenal lesions rarely can be made by aspiration cytology alone, we believe that these diagnostic modalities are not clinically helpful and may cause troublesome bleeding in a highly vascular lesion . Because these tumors may remain asymptomatic until they reach large sizes, the diagnosis is often suggested by incidental findings on radiographic studies for unrelated complaints . Plain abdominal radiographs revealed a calcified adrenal mass in 7 of 11 cases (64%) . Calcifications characteristically appeared speckled throughout the entire mass rather than the curvilinear calcification suggestive UROLOGY /

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Presentation

Calcification

Hypertension Retention Prostatism Hypertension Hypertension Routine examination Pain (RUQ) Left flank mass Pain (RUQ) Prostatism Syncope Low-back pain

Not reported Yes (IVP) Yes (IVP) Yes (IVP) No

Yes

(BE)

No

Yes (CT) No Yes (IVP) No Yes (spine series)

of an adrenal pseudocyst . It has been suggested that this pattern of calcification is secondary to multiple phleboliths which form within the dilated vascular spaces of the hemangioma .' Ultrasound and abdominal CT reveal a heterogeneous suprarenal mass with a thick capsule . Cystic spaces as well as diffuse areas of calcification also may be evident, Enhanced CT may exhibit patchy enhancement of the periphery of the tumor where residual areas of normal compressed adrenal remain . In addition, the early phases of a dynamic CT may reveal contrast material pooling at the periphery of the lesion consistent with the large venous sinuses seen histologically. To our knowledge, magnetic resonance image (MRI) findings have been described only in one report prior to our case .' As with our case, hemangiomas have an isointense T r weighted image with respect to the liver . However, multiple highintensity foci became apparent on T i images after administering Gd-diethylenetriamine-pentaacetic acid (Gd-DTPA), suggesting the presence of venous sinuses . Multiple areas of necrosis and hemorrhage were also noted on T 1 images with the suggestion of calcified areas on T i and T 2 images. These findings are analogous to those seen with hemangiomas of the liver. Angiography, while not required as part of the routine workup of adrenal masses, may show pathognomonic features allowing the preoperative diagnosis of cavernous hemangioma . 4 Angiomas characteristically have marked neovascularity with small vascular channels, often arranged in a ring-like manner, which retain contrast material in delayed films . Despite the benign nature of adrenal hemangiomas, surgical excision is recommended since 329

there have been 2 cases of malignant adrenal hemangioblastomas described .' In addition, while the natural history of these lesions is not known, a significant number of patients appear to develop local complaints due to the large size of these lesions. Finally, because of a propensity for spontaneous hemorrhage or bleeding induced from minimal trauma, it is recommended that all suspected lesions be excised . Edmond Sabanegh, Jr., M .D . The Cleveland Clinic Foundation Department of Urology 9500 Euclid Avenue, Desk A-100 Cleveland, Ohio 44195-5041 REFERENCES 1 . Johnson CC, and Jeppesen FB : Hemangioma of the adrenal . j Urol 74 :573-575, 1955 . 2 . Vargas AO : Adrenal hemangioma . Urology 16 ; 389-390,1980 .

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3 . Orringer RD, Lynch JA, and McDermott WV : Cavernous hemangioma of the adrenal gland . J Surg Oncol 22 : 106-108,1983 . 4 . Rothberg M, Bastidas J, Mattey WE, and Bernas E : Adrenal hemangiomas : angiographic appearance of a rare tumor. Radiology 126 : 341-344, 1978 . 5 . Honig SC, Klavans MS, Hyde C, and Siroky MB : Adrenal hemangioma : an unusual adrenal mass delineated with magnetic resonance imaging . J Urol 146 : 400-402, 1991 . 6 . Weiss J, and Schulte J : Adrenal hemangioma : a case report . J Urol 95 : 604-606, 1966 . 7 . Ruebel AA: Adrenal hemangioma . Urology 2 : 289-291, 1973 . 8 . Derchi LE, Rapaccini GL, Banderali A, Danza FM, and Grillo F : Ultrasound and CT findings in two cases of hemangioma of the adrenal gland . J Comput Assist Tomogr 13 : 659-661,19899 . Salup R, Finegold R, Borochovitz D, Boehnke M, and Posner M: Cavernous hemangioma of the adrenal gland . J Urol147 :110-112,1992 . 10 . Glazer GM : MR imaging of the liver, kidneys and adrenal glands . Radiology 166 : 303-312, 1988 .

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