Keywords
cavernous hemangiomas - benign tumors - dilated vascular channels
Introduction
Hemangiomas are benign vascular tumors.[1] Cavernous hemangioma, a subtype, is still a rare entity in the adrenal gland and
was first published by Johnson and Jeppesen in 1955. These represent 0.01% of adrenal
tumors. Literature shows 67 cases of adrenal cavernous hemangioma published between
the year 1955 and 2018.[2] Such masses are usually nonfunctioning and asymptomatic. This is a rare case of
incidentally detected cavernous hemangioma in the adrenal gland post adrenalectomy.
Discussion
Adrenal cavernous hemangioma is commonly present in the sixth and seventh decades
of life with a female to male ratio of 2:1.[3] There is usually no preference for laterality. The mean size noted in the literature
is 11 cm.[2] In most cases, the patients have been asymptomatic with normal metabolic workup.[3]
[4] Some presented with nonspecific symptoms like vague abdominal pain, bloating, heaviness,
bilateral flank pain, and chronic abdominal distension.[2]
[5] These tumors gradually increase in size and may reach 10 to 35 cm in diameter[3] and cause mass effects. Literature shows other less common presenting features:
night sweats and generalized fatigue, hyperfunctioning adrenal mass, subclinical Cushing
syndrome, hypertension as presenting symptom with normal adrenal functions, and hyperaldosteronism
with hypokalemia, hypovolemic shock due to spontaneous rupture.[6]
[7]
[8] Our case presentation is unique, considering the patient's age, only 30 years, making
this already rare lesion further rare. Also, the size was 5.3 cm in greatest dimension.
Moreover, contrary to the majority of asymptomatic presentations, it presented with
nausea and vague abdominal pain.
Hemangiomas are benign vascular tumors characterized by increased blood vessels or
abnormal vessels filled with blood. These mainly involve head and neck but can seldom
be more extensive and can occur internally. Malignant change is rarely seen. Based
on histology and clinical variants, it has four subtypes: capillary, cavernous, juvenile
and pyogenic. Cavernous hemangiomas are infiltrative, frequently involve deep structures,
and do not spontaneously regress. Histology shows an unencapsulated mass with infiltrative
borders made of large, cavernous blood-filled vascular spaces separated by connective
tissue stroma.[1]
A CT scan usually diagnoses benign vascular tumors. The characteristic feature of
adrenal hemangioma in CECT is a peripheral patchy enhancement and highly dense outer
rim.[4] Specificity of CT and magnetic resonance imaging is low when diagnosing adrenal
malignancy, but benign masses are quickly picked up.[9] Final diagnosis in most cases is by histopathology in postsurgical resection.
Most hemangiomas are managed surgically. Small asymptomatic, benign-looking masses
on radiography can be managed conservatively with close follow-up. Symptomatic, large
masses, or with suspected malignant potential have to undergo resection.[2] Laparoscopic adrenalectomy is known to have lesser complications and better postoperative
results than open adrenalectomy.[10] But despite the advantages of laparoscopy, the open technique is preferred when
mimicking malignancy.
Conclusion
Adrenal cavernous hemangioma is a rare lesion in itself. Moreover, presenting at the
age of 30 years makes the case further unique. It is essential to consider this lesion
as a differential diagnosis for adrenal masses, even in young patients, to avoid spontaneous
hemorrhage or intraoperative chances of bleeding and hypovolemic shock during hemangioma
excision.
