Gigantic adrenal pseudocyst: A case report

INTRODUCTION

The incidence rate fluctuates between 0.06% and 0.18%.^[1] They are rare, non-functional, and asymptomatic; around 500 cases have been reported in Western literature.^[2] Endothelial (45%), epithelial (9%), parasitic (7%), and pseudocyst (39%) are the four types of adrenal cysts.^[2] The adrenal pseudocyst is a gland-fibrous cyst without any endothelium or epithelial lining. There is a diagnostic dilemma regarding the origin of an enormous adrenal cyst. It usually occurs in the 3rd and 4th decade of life with a female preponderance 3:1.^[3] In our case study, we report a case of a gigantic left-sided adrenal pseudocyst measuring about 25 cm in largest diameter, one of the largest adrenal hemorrhagic pseudocysts.

CASE REPORT

A 50 years old hypertensive and obese woman presented with a 6-months history of abdominal pain and distension. The pain was dull in nature, originating from the left upper abdomen. On examination, a smooth surface mass was palpated in the left-side abdomen with an unreachable lower limit. The mass was dull to percussion and mildly tender to palpation. There was no history of trauma or malignancy.

Management

The biochemical profile showed normal complete blood count (CBC), renal function tests (RFTs) and liver function tests (LFTs). Serum amylase (N:25–125U/L) and lipase (N 0–60 U/L) were 49 and 38 (unremarkable), respectively.

Cancer embryonic antigen (CEA) and Carbohydrate antigen (CA 19-9) levels were normal at 1.53 (N:0–2.5 ng/ml) and 10.9 (N:0–35 U/ml). Hormonal status was also normal; showed serum cortisol 4.8 (N:2.9–17.3 ug/dl), urine metanephrine 90 (<350 ug/day), plasma-free metanephrines 16.98 (N:0–90 pg/ml), urine normetanephrine 117.2 (N:<600 ug/dl), plasma free normetanephrine 20.44 (N:0–190 pg/ml) and urine dopamine level was 60 (N:<600 ug/dl).

Computed Tomography (CT) scan abdomen and pelvis with IV-contrast showed a huge cyst measuring (22 × 22 × 25) involving the left hypochondrium and epigastric region and appears to be arising from the left adrenal gland showing no internal solid component or septations. However, small focal wall calcifications are noted. This cyst displaces the pancreas anterosuperiorly. The left kidney was replaced inferiorly, with its axis rotated laterally [Figure 1].

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Figure 1:

CT-Scan Abdomen with IV-Contrast. Panel (a) Axial view of CT-Abdomen IV contrast showing huge left-sided cyst 25*20 cm with no solid component and septations with few areas of calcifications. Panel (b) Sagittal View of CT-Abdomen IV contrast showing huge cyst arising from left adrenal gland displacing pancreas superiorly and kidneys inferiorly. Panel (c) Axial view of CT-Abdomen showing cyst compressing the left kidney.

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In a multidisciplinary team (MDT) meeting, the patient was discussed, and a plan for midline excision was formed due to the risk of malignancy associated with the massive size of the cyst. This operation was conducted under general anesthesia with the patient lying supine. A midline laparotomy incision was made, and intraoperative findings revealed a huge cystic mass in the left side of the abdomen from the epigastric area to the pelvic region, which crosses the midline [Figure 2]. The cyst was arising from the left adrenal gland. Around 3 liters of brownish-gray fluid was aspirated from the cyst, and excision of the cyst with complete left-sided adrenalectomy was done. Fluid and cyst were sent for cytology and histopathology, respectively.

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Figure 2:

Shinny cyst extending from epigastrium to pelvis and also crossing the midline.

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Cystic fluid was found negative for malignant cells. Histological examinations of sections revealed adrenal gland parenchyma showing a cystic cavity containing hemorrhagic material and hemosiderin-laden macrophages in the lumen. No definitive cyst lining epithelium was identified, and a diagnosis of an adrenal pseudocyst was confirmed [Figure 3].

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Figure 3:

Histopathology: (a) Histological examination of sections reveals adrenal gland parenchyma showing a cystic cavity containing hemorrhagic material and hemosiderin-laden macrophages in the lumen. (b) No definitive cyst lining epithelium is identified in the section examined. No evidence of any granulomatous process of malignancy is seen.

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DISCUSSION

Although benign and may develop at any age, adrenal cysts are more common from the third to the fifth decade of life. In 1966, adrenal cysts classification was modified into four categories: endothelial (45%), pseudocyst (39%), epithelial (9%), and parasitic (7%). Pseudocysts are fibrous-walled cyst that lacks an epithelium. These bleeds are secondary to vascular malformation, trauma, cystic degeneration of the adrenal gland, or vascular neoplasm.^[4] Generally, adrenal cysts are asymptomatic, but larger cysts may present with lumbar pain, vomiting or gastrointestinal discomfort, or a palpable mass. Acute abdomen may occasionally appear, mainly due to rupture, intra cystic bleeding, or infection.^[5] The true origin of adrenal pseudocyst is still controversial. According to one theory, intrarenal hemorrhage is caused by trauma or sepsis. This injury causes a cavity to form with a fibrous, scarred lining that gradually gets bigger over time. Another idea contends that due to swelling and bleeding inside the cyst, adrenal cysts are real cysts that have lost their cellular lining.^[6] Malignant adrenal cysts can be seen in up to 7% of cases, and the risk rises with the pseudocyst size.^[7] Therefore, a careful preoperative workup is mandatory to rule out functioning adrenal carcinoma or pheochromocytoma, especially serum vanillylmandelic acid, serum cortisol, and adrenocorticotropic hormone levels.^[3,7] CT scan is the gold standard for studying adrenal masses with a sensitivity of 85% to 100% and specificity of 95% to 100%.^[8] The appearance of adrenal pseudocyst may range from cystic, solid, or mixed masses on a CT scan. An adrenal pseudocyst with a solid component on a CT scan could be an adrenal tumor. However, no contrast enhancement of solid components on CT confirms a diagnosis of adrenal pseudocyst and helps differentiate it from adrenal carcinoma.^[9] On magnetic resonance imaging (MRI), the cyst has a characteristic homogeneous low T1 or high T2 signal, with no soft tissue component and internal enhancement.^[8] Voudoukis et al. recently demonstrated that endoscopic ultrasound (EUS) is superior to all other imaging modalities for preoperative diagnosis of adrenal cystic lesions, particularly when the pancreatic origin is difficult to rule out by conventional diagnostic methods.^[10] Malignant adrenal tumors, cystic pathologies of the spleen, liver, and kidney, cystic neoplasm of the pancreatic tail, retroperitoneal mucinous cystic neoplasms, and mesenteric and urachal cysts are all included in the differential diagnosis.^[11] Because adrenal pseudocysts are often uncommon lesions, there is disagreement regarding how to treat them. When symptoms are present, a malignancy suspicion exists, and the cyst’s size and functioning have increased, surgical excision—either open or laparoscopic—is necessary.^[12] Recent research on laparoscopic adrenalectomy recommends performing the procedure regardless of the size of the adrenal pseudocyst.^[13] They contend that laparoscopic enlarged vision makes it possible to see tiny arteries, nerves, and fascial laminations hidden from view during traditional open surgery.^[14] The laparoscopic approach has several benefits smaller incisions, short hospital stays, and early recovery. The majority of authors, however, choose open adrenalectomy when treating gigantic adrenal pseudocysts because it is challenging to control such sizable masses when there is internal bleeding. Because of the possibility of malignant potential. It is advised to treat cysts larger than 6 cm using open surgery.^[3]

CONCLUSION

The adrenal pseudocyst is a rare lesion. It is usually asymptomatic and diagnosed incidentally on imaging. Symptoms are related to size and local pressure effect. Regarding diagnosis, imaging might be deceiving. In particular, the adrenal, kidney, and pancreas should be considered when diagnosing benign and malignant masses of nearby organs. After surgical excision, a pathological study is necessary for a conclusive diagnosis.