‘Peliosis’ was first used by Wagner in 1861 to describe the gross appearance of lesions found on the cut surface of a liver affected by the condition.2
It is mostly seen in organs belonging to the mononuclear phagocytic system—for example, liver (most common), spleen, bone marrow and lymph nodes—but can also appear in the lungs, kidneys and parathyroid glands.1
Splenic peliosis may be idiopathic or associated with infections such as tuberculosis, haematological diseases such as myeloma and myelofibrosis, and with the use of androgenic steroids and erythropoietin.3
However, in our patient, no predisposing factor can be associated.
Pathogenesis of peliosis remains uncertain. Some suggest that there is more than one way of pathogenesis (eg, congenital malformation of vessels, acquired vascular disorder triggered by toxic noxae, angiogenesis on the benign end of spectrum of neoplastic vascular changes).1
Peliosis can usually be recognised macroscopically. The splenic surface may show nodular areas, but it is usually the cut surface that demonstrates the blood-filled cavities usually in the parafollicular area of the red pulp.1 4
Isolated peliosis of the spleen is extremely rare occurring in less than 1% of cases at autopsy.1
Our case is somewhat unusual as the patient was symptomatic for other diseases and it was not until an incidental finding on a CT scan that we picked up this lesion. Furthermore, due to the undistinguished nature of the lesion to malignancy, the patient had to undergo a collection of investigations and, certainly, posed a challenge for us in reaching a definitive diagnosis. In addition, more invasive diagnostic tools,5 6
such as imaging guided or laparoscopic biopsy, may have caused serious complications that we were attempting to prevent—for example, spontaneous haemorrhage.7
However, it is important to explore for other organ involvement.
Histologically, differential diagnoses considered for the lesion in our patient include angiomatous lesions (littoral cell angioma, lymphangioma, angiosarcoma, bacillary angiomatosis and sclerosing angiomatoid nodular transformation). However, all of these were excluded based on the fact that there was no vascular proliferation demonstrated within this nodule. There was no cytological atypia too. Amyloidosis was also considered but ruled out as the amorphous eosinophilic material (though reminiscent to amyloid) was negative with Congo red.
Currently, there are no guidelines describing the surgical management of patients with an incidental finding of splenic peliosis as well as the role of elective splenectomy in such patients. The diagnosis is of such a rare entity; therefore, clinicians should retain a high index of suspicion for the presence of this condition and be aware of the potentially fatal consequences.
- This is a rare condition, which requires a high index of suspicion when common causes of symptoms have been ruled out.
- Occasionally, one may come across a dilemma in reaching firm diagnosis despite history, clinical examination as well as radiological findings. This poses a challenge in general surgery when decision making becomes crucial as to whether to pursue an operative exploration and intervention.