A 20.1 kg adrenal cavernous haemangioma presenting as a giant retroperitoneal mass: diagnostic difficulties and operative strategy - a case report

Background

Adrenal haemangiomas are exceptionally rare benign vascular tumours, usually discovered incidentally and measuring only a few centimetres. But in rare cases, lesions exceeding 20 kg are distinctly uncommon and pose major diagnostic and operative challenges.

Case presentation

A 67-year-old woman presented with six months of progressive abdominal distension, early satiety, and exertional dyspnea on a four years background of intermittent left loin pain. Her medical history includes HTN, T2DM, AF and heart failure. No surgical history was reported. Imaging revealed a giant left retroperitoneal mass displacing adjacent viscera. Hormonal evaluation showed hypothyroidism, consistent with a non-functional adrenal lesion. Tumour markers were negative. After multidisciplinary discussion, open trans-abdominal excision was performed. Operative time was 4 h 45 min with an estimated blood loss of 500 mL. Two units of packed red blood cells and two units of plasma were transfused pre-operatively and the same post-operatively. She underwent midline laparotomy. The tumour, densely adherent to aorta, inferior vena cava and ureter, was removed en‑bloc using meticulous dissection and a radiofrequency scissor. Concomitant umbilical hernia was repaired with a 30 × 30 cm pre‑peritoneal polypropylene underlay mesh (5 cm overlap). The excised specimen weighed 20.1 kg, verified intra-operatively. Histopathology confirmed a cavernous haemangioma without atypia or malignancy. Post-operative recovery was uneventful. Follow-up at two months with ultrasound showed no recurrence.

Conclusions

Although giant adrenal cavernous haemangioma is benign, it can mimic other retroperitoneal tumours and demands meticulous pre-operative optimisation and multidisciplinary planning. Surgical excision remains the treatment of choice, and structured long-term surveillance is advised.

Supplementary Information

The online version contains supplementary material available at 10.1186/s12893-026-03553-4.

Highlights

Giant (20.1 kg) adrenal cavernous haemangioma successfully excised via open trans-abdominal approach.

Multidisciplinary optimisation enabled safe resection with limited blood loss despite tumour size.

Non-functional lesion confirmed by normal hormonal profile.

Classic cavernous morphology eliminated need for immunohistochemistry.

Early follow-up shows no recurrence; structured surveillance planned.

Supplementary Information

The online version contains supplementary material available at 10.1186/s12893-026-03553-4.

Background

Adrenal haemangiomas are benign vascular malformations representing < 0.1% of adrenal tumours [1, 2]. They are rare, generally non-functioning, usually 2–25 cm in size, and discovered incidentally [2–4]. Their aetiology remains uncertain, possibly related to developmental vascular malformations or acquired venous obstruction. When large in size, they can mimic malignant retroperitoneal tumours and displace nearby organs, and make it tough to pinpoint their origin or decide on surgical approach [1, 3, 5]. Most of what we know comes from isolated case reports and a few scattered reviews, which repeatedly highlight the same themes of how rare they are, how non-specific the imaging is, and how often the diagnosis is only made after surgery [2, 6]. This case report has been reported in line with the SCARE 2020 criteria [24].

Case presentation

A 67-year-old woman presented with a six-month history of progressive abdominal enlargement and early satiety. Past medical history included HTN, T2DM, AF, heart failure and chronic anaemia. She had no prior surgeries. Medications included amiodarone 100 mg OD, furosemide 20 mg OD, rivaroxaban 20 mg OD, and an SGLT2 inhibitor (gliflozin) 10 mg OD. Examination revealed a tense abdomen with a large, firm, non-tender mass occupying the left flank and epigastrium. Laboratory investigations showed Hb 9 g/dL (increased to 11 g/dL after blood transfusion), HbA1c 49 mmol/mol, TSH initially 8 mIU/L (reduced to 4.5 mIU/L after 100 µg levothyroxine), free T4 6.4 pmol/L, and 24-h urine protein 80 mg/day, tumour markers (AFP, CEA, CA19-9) were negative. Biochemical tests showed that there was no problem with the endocrine system (morning cortisol 11 µg/dL, plasma aldosterone 7,4 ng/dl, plasma renin 20,1 µU/ml, urinary adrenaline 4.49 µg /24 h; urinary noradrenaline 43.65 pg/24 h, urinary metanephrine 121.20 µg/24 h, urine normetanephrine 252.15 µg/24 h); supporting a non-functional lesion [2]. Contrast-enhanced CT revealed a 22.3 × 20.9 × 24.0 cm heterogeneous, well-circumscribed, encapsulated mass occupying the left retroperitoneum, displacing the left kidney antero-medially and compressing the aorta and spleen without invasion; a small volume of perisplenic fluid was reported, multiple coarse calcifications (2–10 mm) were present, and the left adrenal gland was not separately visualised (Figs. 1 and 2-3). Pre-operative optimisation comprised transfusion of 2 units packed red blood cells (PRBC) plus 2 units fresh frozen plasma (FFP) one day before surgery. Endocrine work-up and pheochromocytoma exclusion per ESE/ENSAT; haemodynamic precautions if pheochromocytoma suspected [7]. Following optimisation, open trans-abdominal excision was undertaken via a midline incision; dense adhesions to adjacent structures were carefully lysed, stepwise vascular control was secured, careful avascular-plane dissection with a radiofrequency scissor was performed with protection of the ureter and great vessels, and en-bloc tumour resection was achieved [7, 8]. Operative time was 4 h 45 min with an EBL of approximately 500 mL; the patient received 2 units PRBC plus 2 units FFP post-operatively (Figs. 4 and 5). The mass was excised intact (Figs. 6 and 7), and a concomitant giant umbilical hernia (11 cm) was repaired using a 30 × 30 cm polypropylene underlay (pre-peritoneal) mesh with 5 cm overlap; the abdominal cavity was irrigated with normal saline and a closed-suction (Redon) drain placed. The specimen weighed 20.1 kg on the operating-room scale, consistent with the radiologist’s pre-operative estimate (> 15 kg owing to calcifications) and with a postoperative drop in patient weight from 85 kg pre-operatively to 62 kg ten days after surgery. Histopathology showed a well-demarcated lesion composed of numerous large, dilated vascular spaces lined by flattened endothelial cells without atypia or mitotic activity; the intervening fibrous stroma demonstrated thrombosis and areas of both recent and old haemorrhage, with residual normal adrenal tissue at the periphery (Figs. 8 and 9-10). Immunohistochemistry was not required in this case, as the classical morphology was diagnostic of cavernous haemangioma; IHC (CD34, CD31, ERG) can be used to support the diagnosis [1, 2]. Post-operative recovery was uneventful; the regimen included intravenous fluids (saline/dextrose), levofloxacin 500 mg, metronidazole 500 mg, hydrocortisone, omeprazole 40 mg, furosemide, paracetamol, calcium, plasma as indicated, pethidine as needed, and enoxaparin 40 mg for venous thromboembolism prophylaxis. Follow-up at 10 days, 1 month, and 2 months (including repeat biochemistry and abdominal ultrasound at 2 months) showed no residual or recurrent lesion; planned surveillance comprises contrast-enhanced CT at 6 and 12 months.

Fig. 1.

Axial contrast-enhanced CT (arterial phase) demonstrating a heterogeneous retroperitoneal mass (orange arrows) with an aberrant feeding vessel (green arrows). The pancreas (red arrows), abdominal aorta (yellow arrows), spleen, and right kidney (RK) are labeled for orientation

Fig. 2.

Axial contrast-enhanced CT (venous phase) showing an encapsulated retroperitoneal mass (orange arrows), an umbilical hernia (green arrows), and rightward displacement of bowel loops (red arrows)

Fig. 3.

Coronal contrast-enhanced CT (arterial phase) depicting the retroperitoneal mass (orange arrows) in relation to the left kidney (green arrows) and right kidney (red arrows)

Fig. 4.

Pre-operative theatre photograph: marked abdominal distension

Fig. 5.

Intra-operative view: encapsulated mass occupying left retroperitoneum prior to mobilisation

Fig. 6.

Gross appearance of resected specimen: 20.1 kg tumour with intact capsule

Fig. 7.

Cut surface: spongy, blood-filled spaces consistent with cavernous haemangioma

Fig. 8.

Histology: Microscopic study showed lesion that composed of proliferation of many large blood vessels, that lined by flattened endothelial cells without cytologic atypia & were filled of blood

Fig. 9.

Histopathological images of an adrenal cavernous hemangioma cross section, showing presence of dilated blood vessels contained blood with intervening fibrous stroma & foci of residual adrenal gland tissue

Fig. 10.

Histopathological image showed presence of fibrous tissue with marked recent hemorrhage

Timeline

• Four years before admission: intermittent left loin pain.

• Six months before admission: progressive abdominal distension, early satiety, and exertional dyspnoea.

• Pre-operative day: optimisation (endocrine work-up; transfusion of PRBC/FFP).

• Day 0: open trans-abdominal excision with en-bloc resection and concurrent umbilical hernia mesh repair.

• Post-operative day 10: clinical review; weight reduction from 85 kg pre-operatively to 62 kg.

• Two-month follow-up: abdominal ultrasound showed no recurrence; surveillance CT planned at 6 and 12 months.

Discussion

Adrenal cavernous haemangioma is an uncommon benign vascular tumour with less than 100 surgically confirmed cases reported since its first description in the 1950s, and most series emphasise incidental discovery and non-functionality [1, 2]. Contemporary reviews and case syntheses estimate lesion sizes typically between 2 and 25 cm, with a female predominance and middle-to-older-age peak incidence. Calcification is frequent, reflecting chronic thrombosis and involutional change [1, 2, 5]. Selected surgically treated adrenal cavernous haemangioma cases from the literature are summarised in Table 1.

Table 1.

Literature review

Author (year)	Side	Size (cm)	Weight (g)	Presentation	Operation	Histology	Outcome/Follow-up	citation
Present case (2025)	L	22.3 × 20.9 × 24.0	20 100	Loin pain → progressive distension	Open trans-abdominal excision; en-bloc; hernia mesh repair	Cavernous haemangioma	Well at > 2 months, no recurrence.
Degheili et al., 2019 [1]	R	8 × 7 × 3	NR	Vague abdominal & flank pain	Adrenalectomy	Cavernous haemangioma	Good postoperative course.	1
Feo et al., 2018 [3]	L	9.0 × 6.5 × 7.0	NR	Incidental adrenal mass	Open adrenalectomy	Cavernous haemangioma	Disease-free at 53 months.	3
Alhajri et al. (2011) [4]	L	15 × 14 × 18	2230	Flank pain	Open adrenalectomy	Infarcted cavernous haemangioma	uneventful	4
Toklu et al., 2022 [6]	L	4.5	54	unexplained weight loss	adrenalectomy	adrenal hemangioma	uneventful	6
Tarchouli et al., 2015 [10]	R	42 × 38 × 17	NR	5 yrs intermittent pain, distension	Open conventional	Cavernous haemangioma	Well at 2 yrs.	10
Wang et al., 2014) [11]	R	6 × 5 × 4.5	150	Incidental mass with HTN/headache	Adrenalectomy (approach NR)	Cavernous haemangioma	Uneventful; outcome reported as favourable.	11
Noh et al., 2014 [18]	R	7.8 × 7.8	NR	Epigastric discomfort/vomiting in pregnancy	Laparoscopic adrenalectomy	Cavernous haemangioma (CD31/34+)	Uneventful; discharged POD 4.	18
Quildrián et al., 2013 [19]	L	12.5 × 11.5 × 8.0	707	Incidental mass	Open resection	Cavernous haemangioma	Uneventful; no recurrence reported.	19
Hashimoto et al., 2018 [20]	L	27 × 17 × 5.5	2000	Loss of appetite; large incidental mass; PET negative	Total resection	Cavernous haemangioma	Good recovery; no recurrence stated.	20
Tan et al., 2024 [21]	R	10.3 × 8.5 × 6.9	319	Diagnostic imaging conundrum	Adrenalectomy	Cavernous haemangioma	Noted as benign course.	21
Antar et al., 2023 [22]	L	7.5 × 6.5 × 3.7	55	Spontaneous retroperitoneal haemorrhage	Surgical management	Cavernous haemangioma	Recovery reported.	22
Testini et al., 2021 [23]	R	18 × 18 × 15	1200	Incidental; imaging-led	laparoscopic right adrenalectomy	Cavernous haemangioma	4-year imaging follow-up: stable/no recurrence.	23

Comparative summary of selected adrenal cavernous haemangioma cases. Sizes/clinical details are as reported in the cited articles

NR  Not reported

The present 20.1 kg specimen represents an extreme outlier compared with previously reported adrenal haemangiomas. In the literature, so-called “giant” adrenal haemangiomas typically reach diameters of up to approximately 42 cm and weigh from a few hundred grams to at most 1–5 kg; ongoing growth is thought to be driven by progressive intralesional thrombosis, haemorrhage and dystrophic calcification (phleboliths) [4, 10, 21, 24]. This discrepancy between overall tumour volume and the relatively limited amount of viable endothelium helps explain why mass effect and weight can far exceed what would usually be expected for a benign adrenal lesion. Histologically, the combination of a well-organised network of cavernous vascular spaces and residual adrenal cortex at the periphery confirms an adrenal cavernous haemangioma with secondary haemorrhagic change, rather than a purely organised haematoma, despite long-term rivaroxaban therapy.

Cross-sectional imaging is central to pre-operative characterisation. On CT, adrenal haemangiomas are usually well-circumscribed, heterogeneous lesions that may contain calcifications and demonstrate peripheral, nodular or patchy enhancement with delayed centripetal fill-in; features that parallel hepatic haemangiomas and favour a benign vascular aetiology. MRI typically shows marked T2 hyperintensity, variable T1 signal (depending on haemorrhage), and the same progressive peripheral-to-central enhancement; diffusion restriction is generally absent. In combination, these features can suggest a benign vascular mass that displaces rather than invades adjacent organs, as in the current case [5, 12, 13].

Guideline-based imaging pathways support this approach. The 2023 European Society of Endocrinology (ESE)/ENSAT guideline recommends non-contrast CT as first-line to identify unequivocally benign lipid-rich lesions (homogeneous, ≤ 10 HU), with additional imaging when indeterminate. MRI is an appropriate second-line modality when CT is non-diagnostic or to refine characterisation without radiation. 18 F-FDG PET/CT is not a routine test for adrenal incidentalomas but is useful in patients with a history of extra-adrenal malignancy or when indeterminate morphology persists [7]. Significantly, benign adrenal vascular tumours may show low-to-moderate FDG uptake, so PET must be interpreted in clinical context [12, 13].

Peri-operative steroid supplementation was not administered in this case because preoperative endocrine evaluation did not demonstrate adrenal insufficiency, and the contralateral adrenal gland was intact and radiologically normal. Current evidence indicates that patients undergoing unilateral adrenalectomy for non-functioning adrenal masses maintain adequate hypothalamic-pituitary-adrenal (HPA) axis reserve, and routine peri-operative glucocorticoid administration is not required in this setting [14]. Excessive or unnecessary steroid use carries its own risks (including impaired wound healing, glucose dysregulation, and increased infection susceptibility) and should be reserved for patients with biochemical adrenal insufficiency or bilateral adrenalectomy [7, 14]. International endocrine guidelines similarly recommend avoiding peri-operative glucocorticoids for unilateral non-functional adrenal tumors when cortisol levels are within the normal physiologic range, as was seen in this patient [14].

Peri-operative strategy is dictated by size, vascularity and relations. While minimally invasive adrenalectomy is the standard for most benign tumours and is feasible for selected large masses in experienced units, current ESE/ENSAT guidance and multiple comparative series advise open adrenalectomy where there is very large size, suspicion of malignancy, local invasion, or need for secure vascular control and safe mobilisation of compressed viscera (conditions commonly present with giant retroperitoneal masses). In this context, open laparotomy allows early venous control, controlled dissection from major vessels and adjacent organs, and en-bloc resection when indicated. Multidisciplinary planning involving general and vascular surgery, anaesthesia, oncology, endocrinology, histopathology and radiology was central to the favourable outcome [7, 8, 15].

This differs from other large retroperitoneal tumours, such as retroperitoneal sarcoma (RPS). For primary RPS, including dedifferentiated liposarcoma, the surgical objective is oncologically complete en-bloc resection, often with contiguous organ removal (kidney, colon, pancreas, spleen, and occasionally major vascular reconstruction) to achieve negative margins; pre-operative core-needle biopsy is recommended for histological subtyping and planning. By contrast, benign adrenal haemangioma resection prioritises haemostasis and organ preservation, as invasion is unusual and margin-negative “wide” excisions are unnecessary [9, 16].

Compare to adrenal myelolipoma, myelolipoma is usually diagnosed non-invasively by macroscopic fat on CT/MRI and is often managed conservatively unless symptomatic, very large, or haemorrhagic; when resection is needed, minimally invasive adrenalectomy is common, given the soft, less vascular nature of these lesions. These contrasts in imaging signature (bulk fat) and operative risk profile help separate myelolipoma from vascular tumours such as haemangioma [17].

Limitations include single-case design and limited follow-up; long-term imaging surveillance is ongoing.

Conclusions

Giant adrenal cavernous haemangioma, while benign histologically, can present with mass effect and mimic malignant retroperitoneal tumours. Careful preoperative assessment, optimisation, and multidisciplinary planning allow safe resection. Long-term surveillance is recommended.

Abbreviations

HTN

Hypertension

T2DM

Type 2 diabetes mellitus

AF

Atrial fibrillation

OD

Once daily

SGLT2

Sodium-Glucose Co-transporter 2

Hb

Haemoglobin

HbA1c

Glycated haemoglobin

TSH

Thyroid-stimulating hormone

T4

Thyroxine

AFP

Alpha-fetoprotein

CEA

Carcinoembryonic antigen

CA19-9

Carbohydrate antigen 19 − 9

CT

Computed tomography

PRBC

Packed red blood cells

FFP

Fresh frozen plasma

ESE

The European Society of Endocrinology

ENSAT

European Network for the Study of Adrenal Tumors

EBL

Estimated blood loss

MRI

Magnetic Resonance Imaging

PET

Positron Emission Tomography

IHC

Immunohistochemistry

HPA

Hypothalamic-pituitary-adrenal

RPS

Retroperitoneal sarcoma

Authors’ contributions

Ali Taghi and Fares Issa led the conception of the case report, acquisition of clinical data, literature review, and drafting of the manuscript. Ahmad Alhamid, Aya Alayyoubi, Mohammad Atia and Baraa Basbous contributed to acquisition and interpretation of clinical data and critically revised the manuscript. Areen Glore was the operating surgeon and contributed to clinical management, operative planning and strategy, and critical revision of the manuscript. All authors read and approved the final manuscript.

Funding

This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sector.

Data availability

All relevant data are included within the article.

Declarations

Ethics approval and consent to participate

Written informed consent was obtained from the patient for publication of this case report. Ethics committee approval was not required for this single patient case report in accordance with institutional policy.

Competing interests

The authors declare no competing interests.