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Year : 2015  |  Volume : 58  |  Issue : 3  |  Page : 359-361
Sclerosing angiomatoid nodular transformation of spleen masquerading as a splenic abscess

Department of Pathology, Armed Forces Medical College, Pune, Maharashtra, India

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Date of Web Publication14-Aug-2015


Here we report an incidental case of sclerosing angiomatoid nodular transformation (SANT) in an open splenectomy specimen from a young man who presented with insidious abdominal pain. The condition was mimicking as a splenic abscess. Histopathological and immunohistochemical analysis confirmed this to be SANT. SANT presents a diagnostic challenge to clinicians, radiologists, as well as pathologists because of its extreme rarity. Therefore, it must be included as a differential diagnosis in cases with nonspecific pain or radiological splenic mass. This case highlights that SANT can present with varied clinical features and at a wide range of ages. A high index of suspicion is required for its diagnosis.

Keywords: Sclerosing angiomatoid nodular transformation, splenectomy, splenic abscess, splenic tumor

How to cite this article:
Bagul KA, Sen A. Sclerosing angiomatoid nodular transformation of spleen masquerading as a splenic abscess. Indian J Pathol Microbiol 2015;58:359-61

How to cite this URL:
Bagul KA, Sen A. Sclerosing angiomatoid nodular transformation of spleen masquerading as a splenic abscess. Indian J Pathol Microbiol [serial online] 2015 [cited 2022 Dec 6];58:359-61. Available from:

   Introduction Top

Sclerosing angiomatoid nodular transformation of the spleen (SANT) was first described by Martel et al. in 2004 in a series of 25 cases and he coined the term SANT. [1] Since then <100 cases have been reported in the literature worldwide. [2] Etiopathogenetic mechanism for this condition is yet obscure. [3] Here we report a case of SANT, which was diagnosed as chronic splenic abscess and underwent open splenectomy. The nonspecific and insidious clinical presentation including various diagnostic difficulties and its resemblance to other surgical entities makes the case interesting and hence to be kept as a differential diagnosis.

   Case Report Top

A 24-year-old male presented with mild, pricking type of pain in left hypochondrium. The pain was insidious in onset, gradually progressive, intermittent, and lasting for few minutes with symptom free intervals of 15-20 days since last 4 years. Since 3 months both intensity and duration of episodes increased and since last 15 days he experienced continuous pain in both hypochondria radiating to the back. No aggravating or relieving factors noted. No altered bowel habits, weight loss or breathing difficulty was present. General examination was normal. Patient was afebrile with stable vitals. No pallor or icterus noted. External abdominal examination did not reveal any abnormality. On palpation, tenderness was present in left hypochondrium. No guarding, rigidity, organomegaly or lumps were detected. Another systemic examination was within normal limits. Ultrasonography (USG) abdomen and pelvis were done which showed a large mass superior to the upper pole of the left kidney measuring 6.4 cm × 5 cm and opined as ?Adrenal mass, No other sonological abnormality was detected. As USG was nonconfirmatory computed tomography (CT) scan of abdomen and pelvis [Figure 1] ordered which revealed a hypodense mass lesion, measuring 4.8 cm × 4.6 cm, showing homogeneous peripheral enhancement at superomedial pole of spleen. Diffuse perilesional parenchymal edema was noted and opined as developing splenic abscess/granulomatous lesion. Hematological investigations were unremarkable apart from neutrophilic leukocytosis. Total leukocyte count-24,200/cumm, Renal and liver function tests were within normal limits. Clinical diagnosis of a splenic abscess was made, and the patient underwent open splenectomy.
Figure 1: Computed tomography scan image of transabdominal section: Supero medial pole of the spleen showing a mass (arrow)

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   Pathologic Finding Top

Gross-A specimen of spleen measuring 12 cm × 10 cm × 8 cm weighing 290 g with an non-capsulated lesion measuring 5 cm in diameter, firm in consistency noted at one pole [[Figure 2], upper right inset]. Cut surface [Figure 2] showed well circumscribed tense lesion dark red in color with interspersed stellate white projections. No cyst or abscess found.
Figure 2: Cut surface of the spleen. Inset (upper right corner) shows external surface of the spleen

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Microscopy-All sections from the lesion revealed similar histomorphology [Figure 3]. At lower magnification alternate hypo and hypercellular areas were noted [Figure 3], upper left and right inset]. At higher magnification multiple nodules were found composed of concentric collagen fibers with slit-like capillaries [Figure 3]; lower left inset] admixed with chronic inflammatory infiltrates consisting of lymphocytes and plasma cells. The vascular spaces were lined by plump endothelial cells [Figure 3], lower right inset]. The internodular area showed congested red pulp area consisting of endothelial cells, plasma cells, lymphocytes, and hemosiderin laden macrophages. White pulp was almost absent in the lesion. Immunohistochemical analysis demonstrated positivity for CD34 [Figure 4]a and CD31 [Figure 4]b and negative for CD8 in the endothelial lining cells. The macrophages were positive for CD68 [Figure 4]c. The case was signed out as SANT.
Figure 3: At lower magnification (sclerosing angiomatoid nodular transformation, H and E, ×40) alternate hypo and hypercellular areas were noted (upper left and right inset). Lower left inset shows multiple nodules composed of concentric collagen fibers (×100). These nodules at higher magnification (×400) shows lymphocytes and plasma cells (lower right inset)

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Figure 4: Immunohistochemical photomicrographs showing positivity for (a) CD31, (b) CD34 and (c) CD68 (sclerosing angiomatoid nodular transformation)

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   Discussion Top

Sclerosing angiomatoid nodular transformation is a rare benign vascular lesion affecting the spleen with marked sclerosis and obscure etiopathogenesis. [1] The lesion has been reported synonymously as splenic hamartoma, cord capillary hemangioma, and multinodular hemangioma. [4] A slight predilection for the female gender has been reported in the literature, but it may be due to the scarcity of reported cases. [5] It usually affects middle-aged adults, and often discovered incidentally on radiographic imaging, or at the time of operation for some other condition. In patients presenting with symptoms, mild abdominal pain is the predominant one. Our case however presented with an episodic pricking type of abdominal pain. Other presentations include: A palpable left upper quadrant mass, cytopenias, flank pain, pelvic pain and long-standing fever. [2] Another interesting finding in our case was neutrophilic leukocytosis instead of cytopenia misleading to a clinical diagnosis of splenic abscess. Radiological findings in our case were consistent with those described previously. As SANT is a vascular lesion due to over-proliferation of blood vessels, its differential diagnosis includes other benign lesions such as hamartomas, hemangiomas, hemangioendotheliomas, littoral cell angiomas, or inflammatory myofibroblastic lesions. Abundance of plasma cells in sclerotic nodule may suggest a relationship of SANT to IgG4 sclerosing lesions. [6] The diagnosis of SANT should be kept in mind in patients with clinical and radiological features like in our case. Confirmation of diagnosis of SANT requires histological and immunohistochemical evaluation. Three variants are found based on the type of vasculature. First is the capillary rich, which have CD31 and 34 positive, and CD8 negative vessels like in our case. Second are the sinusoid rich, which are CD31, and 8 positive but CD34 negative. Lastly are the small vein rich, which are CD34 negative but positive for CD31 and CD8. [1] CD68 a marker for macrophages has florid expression in our case. A high index of suspicion is essential for diagnosis of the condition. Trucut biopsy can be attempted in suspected cases of SANT with valid accuracy. As the differential diagnosis for SANT includes malignant pathologies, and currently no reliable diagnostic radiological feature has been identified to differentiate between these conditions. Surgical histopathology will be a sine qua non for definitive diagnosis of SANT unless efficient, sensitive, and specific clinical or imaging techniques are developed.

   Acknowledgment Top

We thank Dr. Nikhil Deshpande for providing gross photographs and Dr. Vinay Mishra for helping in compiling this case report.

   References Top

Martel M, Cheuk W, Lombardi L, Lifschitz-Mercer B, Chan JK, Rosai J. Sclerosing angiomatoid nodular transformation (SANT): Report of 25 cases of a distinctive benign splenic lesion. Am J Surg Pathol 2004;28:1268-79.  Back to cited text no. 1
Falk GA, Nooli NP, Morris-Stiff G, Plesec TP, Rosenblatt S. Sclerosing Angiomatoid Nodular Transformation (SANT) of the spleen: Case report and review of the literature. Int J Surg Case Rep 2012;3:492-500.  Back to cited text no. 2
Pradhan D, Mohanty SK. Sclerosing angiomatoid nodular transformation of the spleen. Arch Pathol Lab Med 2013;137:1309-12.  Back to cited text no. 3
Awamleh AA, Perez-Ordoñez B. Sclerosing angiomatoid nodular transformation of the spleen. Arch Pathol Lab Med 2007;131:974-8.  Back to cited text no. 4
Bamboat ZM, Masiakos PT. Sclerosing angiomatoid nodular transformation of the spleen in an adolescent with chronic abdominal pain. J Pediatr Surg 2010;45:E13-6.  Back to cited text no. 5
Nagai Y, Hayama N, Kishimoto T, Furuya M, Takahashi Y, Otsuka M, et al. Predominance of IgG4+ plasma cells and CD68 positivity in sclerosing angiomatoid nodular transformation (SANT). Histopathology 2008;53:495-8.  Back to cited text no. 6

Correspondence Address:
Dr. Kunal Ashok Bagul
Department of Pathology, Diamond Jubilee Block, Armed Forces Medical College, Solapur Road, Pune - 411 040, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.162900

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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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