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Adrenal lymphangioma: a case report
Journal of Clinical Imaging 24 (2000) 104 ± 106
Adrenal lymphangioma: a case report J.M. Longo, S.Z. Jafri*, K.B. Bis Department of Diagnostic Radiology, William Beaumont Hospital, 3601 West 13 Mile Road, Royal Oak, MI 48073, USA Received 1 December 1999; accepted 14 June 2000
Abstract We present a case of a 30-year-old female who was evaluated for right flank pain. Clinical and diagnostic work up revealed a urinary tract infection with focal pyelonephritis of the right kidney. Ultrasound and computed tomography of the abdomen were included in the evaluation and revealed incidental finding of cystic structure at right suprarenal space. Follow-up evaluation for further characterization of cyst was performed with MRI and displayed a 4.8 4.5-cm right adrenal cyst containing dystrophic calcification, septations, and minimal nodularity. Surgical resection and histologic findings were compatible with cystic lymphangioma of the adrenal gland. Adrenal lymphangioma is a rare and benign lesion that is most often identified incidentally during radiological investigation or at autopsy. Diagnostic features of adrenal cysts including lymphangiomas are discussed. D 2000 Elsevier Science Inc. All rights reserved. Keywords: Cystic lymphangioma; Adrenal lymphangioma
1. Case report A 30-year-old female presented to the emergency center with right flank discomfort. On physical examination, she was febrile with tenderness to palpation at the right flank. Initial laboratory evaluation revealed leukocytosis with elevated neutrophil count and a normal hemoglobin level. Urinalysis with culture was performed and displayed white and red blood cells with a positive culture ( > 100,000 cfu/ml) for Escherichia coli. An intravenous urogram was performed displaying curvilinear calcification in region of upper pole of right kidney. This was followed up with an abdominal ultrasound, which displayed a 2-cm focal hypoechoic lesion with scattered peripheral echoes demonstrating posterior acoustic shadowing at the upper pole of right kidney and a 4-cm cyst believed to be related to the liver or right adrenal gland (Fig. 1). Abdominal CT displayed a complex cystic mass at the upper pole of right kidney laterally measuring around 3 cm containing small punctate calcifications and internal low density. The surrounding renal parenchyma was abnormal in appearance with low attenuation. In addition, a 5-cm cystic lesion was identified at the right adrenal gland containing dystrophic calcification (Fig. 2). The patient was
* Corresponding author. Tel.: +1-248-551-6069; fax: +1-248-5513521.
admitted to the hospital to receive intravenous antibiotics and hydration for treatment of right pyelonephritis.
Fig. 1. Ultrasound. Oblique sonographic image shows a complex cystic mass, suprarenal in location with peripheral foci of calcifications (arrows).
0899-7071/00/$ ± see front matter D 2000 Elsevier Science Inc. All rights reserved. PII: S 0 8 9 9 - 7 0 7 1 ( 0 0 ) 0 0 1 9 2 - 3
S.Z. Jafri et al. / Journal of Clinical Imaging 24 (2000) 104±106
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Fig. 2. Computed tomogram. Post-contrast axial computed tomogram shows a nonenhancing cystic mass at right suprarenal location with internal calcifications.
After a short hospital stay, the patient was discharged and a follow-up CT of the abdomen displayed interval decrease in the size of right upper pole kidney lesion consistent with resolving focal pyelonephritis given the patient history. Plans were made to further evaluate right adrenal cyst. A laboratory evaluation was performed revealing unremarkable values for 24 h metanephrines (71 mg/col, normal 399) and 17-ketosteroids (8.4 mg/col, normal range 6.0 ± 15.0), as well as urine-free cortisol (46.2 mg/24 h, normal range 20 ± 90). MRI of the adrenal glands was performed displaying right adrenal cyst with dystrophic calcifications, enhancing septations, and mini-
Fig. 4. MRI. Post-contrast coronal breath hold T1-weighted gradient echo (FLASH) image demonstrates internal septations at the cephalad aspect of right suprarenal mass (arrows). On the T2-weighted image (not shown) the mass demonstrated homogeneous high signal intensity.
mal nodularity (Figs. 3 and 4). Further characterization could not be made. Right adrenalectomy was performed for definitive diagnosis. Grossly, the adrenal gland measured around 6.5 3.5 3.0 cm with a 4.0 3.5 3.0 cm cystic component. Cut section revealed a serous filled cystic cavity. A soft myxoid area was identified adjacent to cyst measuring around 1.5 1.3 0.7 cm. On frozen sections, the cyst contained no epithelial lining and myxoid component contained numerous fluid-filled lymphatics. Histologic sections revealed multiple cystic spaces lined by flat endothelial lining. The surrounding adrenal tissue appeared normal. The cellular lining of cyst displayed no evidence of atypia. Overall, the findings were consistent with benign cystic lymphangioma of right adrenal gland. A follow-up abdominal ultrasound examination nearly 2 years later displayed interval removal of right adrenal cyst with no evidence of recurrence. 2. Discussion
Fig. 3. MRI. Pre-contrast coronal breath hold T1-weighted gradient echo (FLASH) image reveals a low-intensity right suprarenal mass (arrow). The low signal suggests the presence of cystic mass with simple fluid.
Cysts of the adrenal glands are rare and most often identified incidentally during radiological investigation or at autopsy. Their incidence in autopsy studies has been reported in the literature to range from 0.064% to 0.18% [1,3]. In a series of 14,000 autopsies, adrenal cysts were found in 0.06% [5]. The incidence may be higher based on the frequent finding of incidental cysts with imaging modalities(US, CT, MRI) [2]. Although adrenal cysts occur at all ages, there is a peak in incidence between the third to sixth decades [2]. They are typically unilateral with distribution
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S.Z. Jafri et al. / Journal of Clinical Imaging 24 (2000) 104±106
equal on each side [2]. Bilateral cysts have been reported in up to 10% of cases, mostly occurring in children [2]. The female to male ratio is 2:1 [2]. Adrenal cysts are usually asymptomatic without a characteristic symptom. If symptoms do occur, they are usually related to size and position of cyst and can include pain, gastrointestinal disturbance, or palpable mass. Acute symptoms can also occur with cystic hemorrhage, rupture, or infection. Laboratory findings are non-specific and usually not helpful as a diagnostic tool. Low hemoglobin/hematocrit with hemorrhage, leukocytosis with infection, or eosinophilia related to parasite infection may occasionally aid in diagnosis. Rarely, small adrenal cysts may be associated with Cushing syndrome, virilization, or pheochromocytoma in which laboratory evaluation plays a role. Adrenal cysts have been classified histologically into four main groups [2]: (1) Endothelial cysts (45%) include angiomatous, lymphangiomatous, and hamartomatous cysts. Lymphangiomatous cysts are characterized by multiloculated cystic and endothelial lined cavities. (2) Pseudocysts (39%) occur with hemorrhage into a normal adrenal gland or adrenal tumor. Hemorrhage can occur secondary to trauma, bleeding disorder, burns, shock, or toxemia. (3) Epithelial cysts (9%) are comprised of cystic adenomas, glandular or retention cysts, and cystic transformation of embryonal remnants. (4) Parasitic cysts (7%) are most commonly due to echinoccocal infection with associated disseminated systemic hydatidosis. Calcification is identified radiographically in 15% of the cases and is typically peripheral and curvilinear [3,5]. In the past, in addition to plain radiograph, evaluation was often based on intravenous urography and angiography. Presently, imaging with US, CT, and MRI has become the major modality for cyst evaluation. Ultrasound will demonstrate a well-marginated lesion with good through transmission. If calcification or internal debris is present, ultrasound may show more complicated appearance with acoustic shadowing and internal echoes. On CT, uncomplicated adrenal cysts are characterized by lack of enhancement with intravenous contrast. The fluid in cyst will measure water density or higher if hemorrhagic or protein component is present. A
small adrenal cyst with near water attenuation and a thin wall (3 mm) is likely to be benign [3]. On MRI, uncomplicated adrenal cysts are low in signal intensity on T1weighted images and high on T2-weighted images. Complicated adrenal cysts will be high in signal intensity on both T1- and T2-weighted images. MRI in the coronal and sagital plane is particularly helpful for determining the location of a large cystic mass in region of adrenal gland. Clinical management of an adrenal cyst can be aided by the imaging findings. If a small lesion is identified in adrenal gland that meets criteria for a cyst with benign features, it can usually be followed with serial imaging [2]. If the appearance remains unchanged after 18 months without evidence of a clinically or biologically active tumor, then conservative management is justified [2]. Management of larger lesions or lesions causing symptoms may require surgical resection to determine diagnosis or relieve symptoms. Surgery is indicated for large and complicated cysts, parasitic cysts, and functioning and malignant cysts. Percutaneous aspiration of cysts by US or CT guidance has been proposed as initial management by several authors [1,4]. Aspiration has been recommended for uncomplicated cysts with smooth lining [4]. Although percutaneous aspiration is limited in its ability to determine histology of cyst and by the high incidence of reaccumulation of cyst fluid, 50% of cases in one series [1], it may be the best and only option in a patient who is at high surgical risk.
References [1] Tagge DU, Baron PL. Giant adrenal cyst: management and review of the literature. Am Surg 1997;63:744 ± 6. [2] Khoda J, Hertzanu Y, Sebbag G, Lantsberg L, Barky Y. Adrenal cysts: diagnosis and therapeutic approach. Int Surg 1993;78:239 ± 42. [3] Rozenblit A, Morehouse HT, Amis ES. Cystic adrenal lesions: CT features. Radiology 1996;201:541 ± 8. [4] Gleeson MJ, McMullin JP. Cystic lymphangiomata of the adrenal gland. Br J Urol 1988;62:93 ± 4. [5] Pollack HM. Miscellaneous conditions of the adrenals and adrenal pseudotumors. Clin Urogr 1990;3:2403 ± 5.
Adrenal lymphangioma: a case report J.M. Longo, S.Z. Jafri*, K.B. Bis Department of Diagnostic Radiology, William Beaumont Hospital, 3601 West 13 Mile Road, Royal Oak, MI 48073, USA Received 1 December 1999; accepted 14 June 2000
Abstract We present a case of a 30-year-old female who was evaluated for right flank pain. Clinical and diagnostic work up revealed a urinary tract infection with focal pyelonephritis of the right kidney. Ultrasound and computed tomography of the abdomen were included in the evaluation and revealed incidental finding of cystic structure at right suprarenal space. Follow-up evaluation for further characterization of cyst was performed with MRI and displayed a 4.8 4.5-cm right adrenal cyst containing dystrophic calcification, septations, and minimal nodularity. Surgical resection and histologic findings were compatible with cystic lymphangioma of the adrenal gland. Adrenal lymphangioma is a rare and benign lesion that is most often identified incidentally during radiological investigation or at autopsy. Diagnostic features of adrenal cysts including lymphangiomas are discussed. D 2000 Elsevier Science Inc. All rights reserved. Keywords: Cystic lymphangioma; Adrenal lymphangioma
1. Case report A 30-year-old female presented to the emergency center with right flank discomfort. On physical examination, she was febrile with tenderness to palpation at the right flank. Initial laboratory evaluation revealed leukocytosis with elevated neutrophil count and a normal hemoglobin level. Urinalysis with culture was performed and displayed white and red blood cells with a positive culture ( > 100,000 cfu/ml) for Escherichia coli. An intravenous urogram was performed displaying curvilinear calcification in region of upper pole of right kidney. This was followed up with an abdominal ultrasound, which displayed a 2-cm focal hypoechoic lesion with scattered peripheral echoes demonstrating posterior acoustic shadowing at the upper pole of right kidney and a 4-cm cyst believed to be related to the liver or right adrenal gland (Fig. 1). Abdominal CT displayed a complex cystic mass at the upper pole of right kidney laterally measuring around 3 cm containing small punctate calcifications and internal low density. The surrounding renal parenchyma was abnormal in appearance with low attenuation. In addition, a 5-cm cystic lesion was identified at the right adrenal gland containing dystrophic calcification (Fig. 2). The patient was
* Corresponding author. Tel.: +1-248-551-6069; fax: +1-248-5513521.
admitted to the hospital to receive intravenous antibiotics and hydration for treatment of right pyelonephritis.
Fig. 1. Ultrasound. Oblique sonographic image shows a complex cystic mass, suprarenal in location with peripheral foci of calcifications (arrows).
0899-7071/00/$ ± see front matter D 2000 Elsevier Science Inc. All rights reserved. PII: S 0 8 9 9 - 7 0 7 1 ( 0 0 ) 0 0 1 9 2 - 3
S.Z. Jafri et al. / Journal of Clinical Imaging 24 (2000) 104±106
105
Fig. 2. Computed tomogram. Post-contrast axial computed tomogram shows a nonenhancing cystic mass at right suprarenal location with internal calcifications.
After a short hospital stay, the patient was discharged and a follow-up CT of the abdomen displayed interval decrease in the size of right upper pole kidney lesion consistent with resolving focal pyelonephritis given the patient history. Plans were made to further evaluate right adrenal cyst. A laboratory evaluation was performed revealing unremarkable values for 24 h metanephrines (71 mg/col, normal 399) and 17-ketosteroids (8.4 mg/col, normal range 6.0 ± 15.0), as well as urine-free cortisol (46.2 mg/24 h, normal range 20 ± 90). MRI of the adrenal glands was performed displaying right adrenal cyst with dystrophic calcifications, enhancing septations, and mini-
Fig. 4. MRI. Post-contrast coronal breath hold T1-weighted gradient echo (FLASH) image demonstrates internal septations at the cephalad aspect of right suprarenal mass (arrows). On the T2-weighted image (not shown) the mass demonstrated homogeneous high signal intensity.
mal nodularity (Figs. 3 and 4). Further characterization could not be made. Right adrenalectomy was performed for definitive diagnosis. Grossly, the adrenal gland measured around 6.5 3.5 3.0 cm with a 4.0 3.5 3.0 cm cystic component. Cut section revealed a serous filled cystic cavity. A soft myxoid area was identified adjacent to cyst measuring around 1.5 1.3 0.7 cm. On frozen sections, the cyst contained no epithelial lining and myxoid component contained numerous fluid-filled lymphatics. Histologic sections revealed multiple cystic spaces lined by flat endothelial lining. The surrounding adrenal tissue appeared normal. The cellular lining of cyst displayed no evidence of atypia. Overall, the findings were consistent with benign cystic lymphangioma of right adrenal gland. A follow-up abdominal ultrasound examination nearly 2 years later displayed interval removal of right adrenal cyst with no evidence of recurrence. 2. Discussion
Fig. 3. MRI. Pre-contrast coronal breath hold T1-weighted gradient echo (FLASH) image reveals a low-intensity right suprarenal mass (arrow). The low signal suggests the presence of cystic mass with simple fluid.
Cysts of the adrenal glands are rare and most often identified incidentally during radiological investigation or at autopsy. Their incidence in autopsy studies has been reported in the literature to range from 0.064% to 0.18% [1,3]. In a series of 14,000 autopsies, adrenal cysts were found in 0.06% [5]. The incidence may be higher based on the frequent finding of incidental cysts with imaging modalities(US, CT, MRI) [2]. Although adrenal cysts occur at all ages, there is a peak in incidence between the third to sixth decades [2]. They are typically unilateral with distribution
106
S.Z. Jafri et al. / Journal of Clinical Imaging 24 (2000) 104±106
equal on each side [2]. Bilateral cysts have been reported in up to 10% of cases, mostly occurring in children [2]. The female to male ratio is 2:1 [2]. Adrenal cysts are usually asymptomatic without a characteristic symptom. If symptoms do occur, they are usually related to size and position of cyst and can include pain, gastrointestinal disturbance, or palpable mass. Acute symptoms can also occur with cystic hemorrhage, rupture, or infection. Laboratory findings are non-specific and usually not helpful as a diagnostic tool. Low hemoglobin/hematocrit with hemorrhage, leukocytosis with infection, or eosinophilia related to parasite infection may occasionally aid in diagnosis. Rarely, small adrenal cysts may be associated with Cushing syndrome, virilization, or pheochromocytoma in which laboratory evaluation plays a role. Adrenal cysts have been classified histologically into four main groups [2]: (1) Endothelial cysts (45%) include angiomatous, lymphangiomatous, and hamartomatous cysts. Lymphangiomatous cysts are characterized by multiloculated cystic and endothelial lined cavities. (2) Pseudocysts (39%) occur with hemorrhage into a normal adrenal gland or adrenal tumor. Hemorrhage can occur secondary to trauma, bleeding disorder, burns, shock, or toxemia. (3) Epithelial cysts (9%) are comprised of cystic adenomas, glandular or retention cysts, and cystic transformation of embryonal remnants. (4) Parasitic cysts (7%) are most commonly due to echinoccocal infection with associated disseminated systemic hydatidosis. Calcification is identified radiographically in 15% of the cases and is typically peripheral and curvilinear [3,5]. In the past, in addition to plain radiograph, evaluation was often based on intravenous urography and angiography. Presently, imaging with US, CT, and MRI has become the major modality for cyst evaluation. Ultrasound will demonstrate a well-marginated lesion with good through transmission. If calcification or internal debris is present, ultrasound may show more complicated appearance with acoustic shadowing and internal echoes. On CT, uncomplicated adrenal cysts are characterized by lack of enhancement with intravenous contrast. The fluid in cyst will measure water density or higher if hemorrhagic or protein component is present. A
small adrenal cyst with near water attenuation and a thin wall (3 mm) is likely to be benign [3]. On MRI, uncomplicated adrenal cysts are low in signal intensity on T1weighted images and high on T2-weighted images. Complicated adrenal cysts will be high in signal intensity on both T1- and T2-weighted images. MRI in the coronal and sagital plane is particularly helpful for determining the location of a large cystic mass in region of adrenal gland. Clinical management of an adrenal cyst can be aided by the imaging findings. If a small lesion is identified in adrenal gland that meets criteria for a cyst with benign features, it can usually be followed with serial imaging [2]. If the appearance remains unchanged after 18 months without evidence of a clinically or biologically active tumor, then conservative management is justified [2]. Management of larger lesions or lesions causing symptoms may require surgical resection to determine diagnosis or relieve symptoms. Surgery is indicated for large and complicated cysts, parasitic cysts, and functioning and malignant cysts. Percutaneous aspiration of cysts by US or CT guidance has been proposed as initial management by several authors [1,4]. Aspiration has been recommended for uncomplicated cysts with smooth lining [4]. Although percutaneous aspiration is limited in its ability to determine histology of cyst and by the high incidence of reaccumulation of cyst fluid, 50% of cases in one series [1], it may be the best and only option in a patient who is at high surgical risk.
References [1] Tagge DU, Baron PL. Giant adrenal cyst: management and review of the literature. Am Surg 1997;63:744 ± 6. [2] Khoda J, Hertzanu Y, Sebbag G, Lantsberg L, Barky Y. Adrenal cysts: diagnosis and therapeutic approach. Int Surg 1993;78:239 ± 42. [3] Rozenblit A, Morehouse HT, Amis ES. Cystic adrenal lesions: CT features. Radiology 1996;201:541 ± 8. [4] Gleeson MJ, McMullin JP. Cystic lymphangiomata of the adrenal gland. Br J Urol 1988;62:93 ± 4. [5] Pollack HM. Miscellaneous conditions of the adrenals and adrenal pseudotumors. Clin Urogr 1990;3:2403 ± 5.