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Department of Radiology
Seth G.S. Medical College and K.E.M. Hospital, Mumbai , India
Case of the Month
Case of the Month
A Large Spleen Hides a Secret : A Case of Littoral Cell Angioma
A Large Spleen Hides a Secret : A Case of Littoral Cell Angioma
Contributed by : Yash Khandwe
Contributed by : Yash Khandwe
Introduction :
Introduction :
Littoral Cell Angioma (LCA) is a rare vascular tumor arising from the littoral cells lining the venous sinusoids of the splenic red pulp. It is an uncommon primary splenic neoplasm and can present as multiple splenic lesions with splenomegaly.
Littoral Cell Angioma (LCA) is a rare vascular tumor arising from the littoral cells lining the venous sinusoids of the splenic red pulp. It is an uncommon primary splenic neoplasm and can present as multiple splenic lesions with splenomegaly.
Radiologically, LCA may mimic several other splenic pathologies, particularly splenic lymphoma and other vascular tumors. As imaging features are not pathognomonic, histopathological examination following splenectomy remains the definitive method of diagnosis.
Radiologically, LCA may mimic several other splenic pathologies, particularly splenic lymphoma and other vascular tumors. As imaging features are not pathognomonic, histopathological examination following splenectomy remains the definitive method of diagnosis.
Case presentation :
Case presentation :
A 36-year-old woman presented with a four year history of dull aching pain in the left hypochondrium with gradual worsening over the preceding year. There was no history of fever, weight loss, night sweats, jaundice, or bowel complaints.
A 36-year-old woman presented with a four year history of dull aching pain in the left hypochondrium with gradual worsening over the preceding year. There was no history of fever, weight loss, night sweats, jaundice, or bowel complaints.
On examination, mild splenomegaly was palpable approximately 3 cm below the left costal margin. The liver was not enlarged and there was no peripheral lymphadenopathy.
On examination, mild splenomegaly was palpable approximately 3 cm below the left costal margin. The liver was not enlarged and there was no peripheral lymphadenopathy.
Laboratory investigations including complete blood count, liver function tests, renal function tests, and serum LDH were within normal limits.
Laboratory investigations including complete blood count, liver function tests, renal function tests, and serum LDH were within normal limits.
In view of persistent splenomegaly, imaging evaluation was performed.
In view of persistent splenomegaly, imaging evaluation was performed.
Radiological findings
Radiological findings
Ultrasonography of the abdomen
Ultrasonography of the abdomen
Gray-scale ultrasonography demonstrated moderate splenomegaly measuring 14.6 cm with multiple variable-sized focal lesions diffusely involving the splenic parenchyma (Fig. 1A). The lesions demonstrated heterogeneous echotexture with hypoechoic, hyperechoic, and cystic components.
Gray-scale ultrasonography demonstrated moderate splenomegaly measuring 14.6 cm with multiple variable-sized focal lesions diffusely involving the splenic parenchyma (Fig. 1A). The lesions demonstrated heterogeneous echotexture with hypoechoic, hyperechoic, and cystic components.
Color Doppler interrogation demonstrated minimal peripheral vascularity without internal high-flow signals or turbulent bidirectional flow (Fig. 1B). The splenic vessels appeared normal and no abdominal lymphadenopathy was detected.
Color Doppler interrogation demonstrated minimal peripheral vascularity without internal high-flow signals or turbulent bidirectional flow (Fig. 1B). The splenic vessels appeared normal and no abdominal lymphadenopathy was detected.
Fig 1
(A) Gray-scale ultrasonography demonstrates splenomegaly (14.6 cm) with multiple diffusely distributed focal lesions in the splenic parenchyma (white arrows).
(B) The colour Doppler image shows minimal peripheral vascularity (yellow arrows), with no internal high-flow signals.
Contrast-Enhanced CT of the abdomen and pelvis
Contrast-Enhanced CT of the abdomen and pelvis
Contrast-enhanced CT confirmed splenomegaly measuring 14.6 cm with multiple well-defined hypodense lesions scattered throughout the splenic parenchyma(Fig. 2A).
Contrast-enhanced CT confirmed splenomegaly measuring 14.6 cm with multiple well-defined hypodense lesions scattered throughout the splenic parenchyma(Fig. 2A).
On portal venous phase imaging, the lesions demonstrated progressive heterogeneous enhancement, with the largest lesion at the inferior pole measuring 2.6 × 4.3 cm (Fig. 2B).
On portal venous phase imaging, the lesions demonstrated progressive heterogeneous enhancement, with the largest lesion at the inferior pole measuring 2.6 × 4.3 cm (Fig. 2B).
Fig 2
Contrast-Enhanced CT of the Abdomen (A) Axial pre-contrast CT image demonstrates multiple well-defined hypodense splenic lesions (white arrows). (B) Portal venous phase image shows progressive heterogeneous enhancement of the lesions (white arrows). The largest lesion is seen at the inferior pole (black arrow).
Coronal reformatted images demonstrated the multifocal distribution of enhancing splenic lesions (Fig. 3). No splenic hilar, mesenteric, or retroperitoneal lymphadenopathy were identified.
Coronal reformatted images demonstrated the multifocal distribution of enhancing splenic lesions (Fig. 3). No splenic hilar, mesenteric, or retroperitoneal lymphadenopathy were identified.
An incidental fat-density lesion in the left lateral abdominal wall consistent with a subcutaneous lipoma was also noted (Fig. 3).
An incidental fat-density lesion in the left lateral abdominal wall consistent with a subcutaneous lipoma was also noted (Fig. 3).
Figure 3
Contrast-Enhanced CT of the Abdomen : Coronal image confirms splenomegaly with multiple enhancing splenic lesions (white arrows). An incidental subcutaneous lipoma is noted (yellow arrow)
Timeline
Timeline
Discussion
Discussion
Littoral cell angioma is a rare primary vascular tumor arising from the littoral cells that line the splenic sinusoids. These cells demonstrate a unique dual immunophenotype with expression of both endothelial and histiocytic markers, which forms the basis for histological diagnosis [1].
Littoral cell angioma is a rare primary vascular tumor arising from the littoral cells that line the splenic sinusoids. These cells demonstrate a unique dual immunophenotype with expression of both endothelial and histiocytic markers, which forms the basis for histological diagnosis [1].
Patients most commonly present with splenomegaly and vague left upper quadrant pain, although the condition may remain asymptomatic for years. LCA has also been reported in association with visceral malignancies including colorectal carcinoma, renal cell carcinoma, melanoma, and lymphoma [2].
Patients most commonly present with splenomegaly and vague left upper quadrant pain, although the condition may remain asymptomatic for years. LCA has also been reported in association with visceral malignancies including colorectal carcinoma, renal cell carcinoma, melanoma, and lymphoma [2].
On ultrasonography, LCA typically appears as multiple splenic nodules with variable echogenicity, reflecting the heterogeneous composition of vascular channels and fibrotic stroma [3]. On contrast-enhanced CT, the lesions are usually hypodense and demonstrate progressive enhancement in portal venous and delayed phases, corresponding to slow contrast filling within dilated sinusoidal vascular channels [3,4].
On ultrasonography, LCA typically appears as multiple splenic nodules with variable echogenicity, reflecting the heterogeneous composition of vascular channels and fibrotic stroma [3]. On contrast-enhanced CT, the lesions are usually hypodense and demonstrate progressive enhancement in portal venous and delayed phases, corresponding to slow contrast filling within dilated sinusoidal vascular channels [3,4].
The differential diagnosis of multiple splenic lesions includes splenic lymphoma, hemangiomatosis, metastases, sarcoidosis, and fungal microabscesses. Splenic lymphoma remains the most important differential and often presents with associated lymphadenopathy or diffuse splenic infiltration [4].
The differential diagnosis of multiple splenic lesions includes splenic lymphoma, hemangiomatosis, metastases, sarcoidosis, and fungal microabscesses. Splenic lymphoma remains the most important differential and often presents with associated lymphadenopathy or diffuse splenic infiltration [4].
Since many imaging findings zare common to several splenic pathologies, histopathological confirmation remains essential for definitive diagnosis.
Since many imaging findings zare common to several splenic pathologies, histopathological confirmation remains essential for definitive diagnosis.
Conclusion
Conclusion
Littoral Cell Angioma is a rare vascular tumor that should be considered in the differential diagnosis of multiple splenic lesions with splenomegaly, particularly when imaging demonstrates progressive enhancement without lymphadenopathy.
Littoral Cell Angioma is a rare vascular tumor that should be considered in the differential diagnosis of multiple splenic lesions with splenomegaly, particularly when imaging demonstrates progressive enhancement without lymphadenopathy.
Although imaging findings may strongly suggest the diagnosis, histopathological examination remains necessary, and splenectomy serves both diagnostic and therapeutic roles.
Although imaging findings may strongly suggest the diagnosis, histopathological examination remains necessary, and splenectomy serves both diagnostic and therapeutic roles.
References:
References:
1. Falk S, Stutte HJ, Frizzera G. Littoral cell angioma: A novel splenic vascular lesion
1. Falk S, Stutte HJ, Frizzera G. Littoral cell angioma: A novel splenic vascular lesion
demonstrating histiocytic differentiation. Am J Surg Pathol. 1991;15(11):1023–1033.
demonstrating histiocytic differentiation. Am J Surg Pathol. 1991;15(11):1023–1033.
2. Levy AD, Abbott RM, Abbondanzo SL. Littoral cell angioma of the spleen: CT features
2. Levy AD, Abbott RM, Abbondanzo SL. Littoral cell angioma of the spleen: CT features
with clinicopathologic correlation. Radiology. 2004;230(2):485–490.
with clinicopathologic correlation. Radiology. 2004;230(2):485–490.
3. Ferrozzi F, Bova D, Draghi F, Garlaschi G. Littoral cell angioma of the spleen: CT and
3. Ferrozzi F, Bova D, Draghi F, Garlaschi G. Littoral cell angioma of the spleen: CT and
MR imaging findings. Eur Radiol. 2000;10(2):386–389.
MR imaging findings. Eur Radiol. 2000;10(2):386–389.
4. Abbott RM, Levy AD, Aguilera NSI, Gorospe L, Thompson WM. Primary vascular
4. Abbott RM, Levy AD, Aguilera NSI, Gorospe L, Thompson WM. Primary vascular
neoplasms of the spleen: Radiologic–pathologic correlation. Radiographics.
neoplasms of the spleen: Radiologic–pathologic correlation. Radiographics.
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