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Advances in Anatomic Pathology:
doi: 10.1097/PAP.0000000000000031
AMR Series

Selected Case From the Arkadi M. Rywlin International Pathology Slide Series: Lymphangiomatosis of the Spleen Associated With Ipsilateral Abdominopelvic and Lower Extremity Venolymphatic Malformations: A Case Report and Review of the Literature

Bisceglia, Michele MD*; Chiaramonte, Antonio MD; Lauriola, Walter MD; Galliani, Carlos A. MD§; Musto, Pellegrino MD*; Colacchio, Giovanni MD

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Author Information

*IRCCS, Referral Cancer Center of Basilicata, Rionero in Vulture (Pz), Italy

Department of Surgery

Division of Vascular and Interventional Radiology, IRCCS Casa Sollievo della Sofferenza Hospital, San Giovanni Rotondo (Fg), Italy

Division of Vascular Surgery, IRCCS F. Miulli Hospital, Acquaviva delle Fonti (Ba), Italy

§Department of Pathology, Cook’s Children Medical Center, Fort Worth, TX

The authors have no funding or conflicts of interest to disclose.

Reprints: Michele Bisceglia, MD, Via Santa Chiara, 9, Manfredonia 71043, Foggia, Italy (e-mail:

All figures can be viewed online in color at

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Herein, we report a 26-year-old patient with lymphangiomatosis of the spleen associated with multiple lymphatic and venous malformations. This patient underwent excision of a large lymphatic malformation of the left abdominal wall during childhood. A venous malformation of her left lower limb was excised during adolescence. Additional lymphatic malformations were found in the soft tissue of her left thigh at the age of 20. During hospitalization for a huge vulvar hemangioma at the age of 26, she was incidentally found to have asymptomatic splenomegaly, for which she underwent splenectomy. Examination of the spleen revealed diffuse involvement by a lymphatic anomaly predominantly forming small cystic spaces. Lymphangiomatosis of the spleen is rare and is classically separated into an isolated or pure form and a generalized form when it is associated with involvement of other viscera and/or multiple soft-tissue planes. This patient was affected by a borderline form of splenic lymphangiomatosis with limited somatic involvement of the superficial soft tissues and blood vessels. Notably, all the additional vascular malformations in this patient were left sided, and at this time there was no additional involvement of internal organ. No hereditary or known syndrome was identified.
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Diagnosis: Lymphangiomatosis of the spleen with peripheral venolymphatic malformations.

Referral sources: The Fifth International AMR Symposium in Anatomic Pathology, June 14 to 16, 2012, held at Stockholm, Sweden—case no. 39, contributed by M. Bisceglia, MD.

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A 26-year-old woman underwent splenectomy because of splenomegaly. No additional clinical information was provided. The splenectomy specimen was sent to the anatomic pathology laboratory for histologic examination.

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A >2400 g (expected=130 g), 28×22×18 cm (expected=11×4.4×7.8 cm) massively and uniformly enlarged, bosselated, easily compressible spleen covered by an intact, smooth, blue-purple-ochre capsule was received. The cut surface revealed homogeneously distributed, closely placed, variably sized cysts, from being barely visible to ∼1.0 cm in diameter, with a thin, smooth, translucent lining, imparting a real spongy appearance. Only occasional cavernous spaces were 2 to 4 cm in diameter. The cystic replacement of the spleen was such that almost no residual pulp was visible. The cysts contained clear citrine to dark brown, watery to serous fluid, and some cysts were filled with fibrinous clots. At the periphery of some cysts a proliferation of small ectatic lymphatic vessels was also noticed (Figs. 1A–C).

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Histologically, the cystic spaces corresponded to thin-walled lymphatic spaces filled with eosinophilic proteinaceous fluid and were lined by flat, bland-appearing endothelium. The splenic pulp was reduced to a vanishingly compressed intercavitary compartment (Figs. 2A–F). The endothelial lining was immunoreactive for CD34, CD31, FVIII-Rag, and D2-40/podoplanin (Figs. 3A–D).

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Cystic lymphangiomatosis of the spleen.

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Clinical Antecedents

The patient’s medical history was remarkable for multiple previous excisions of separate vascular malformations, including a 7.0 cm suprapubic subcutaneous lymphatic malformation and a 25 cm left abdominal wall hemangioma at the ages of 4 and 7 years, respectively. A complex venous malformation involving the left femoral-iliac axis, requiring angioplastic reconstruction of the common femoral vein, was excised at the age of 16 (Figs. 4A–B). Furthermore, 2 additional subcutaneous masses, about 5 and 10 cm, were disclosed at the age of 20 on the medial aspect of her left thigh, consistent with lymphatic malformations, which were not biopsied.

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At the age of 26, a 10-cm angiomatous mass from the right vulvar labium maius was noticed and the patient came to the hospital for treatment with sclerotherapy injections of 95% alcohol applied directly into the venous lakes (Figs. 5A–C). Physical examination, performed at the time of vulvar sclerotherapy, disclosed massive splenomegaly, that surprisingly did not cause any significant discomfort to the patient.

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Abdominal ultrasound (US) revealed a large, heterogeneous left flank mass with multiple hypoechoic foci, causing extrinsic compression of the left lobe of the liver and left kidney. Computed tomography (CT) scan, performed on an emergency basis soon after the splenomegaly was discovered, demonstrated innumerable low-attenuation splenic cystic cavities. After contrast injection the splenic cystic lesions were nonenhancing, except for 2 large pools that contrast enhanced during the late venous phases (Fig. 6).

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Angiomatosis of the spleen was suspected, but other infiltrative lesions could not be excluded. The liver, pancreas, and kidneys were normal. No retroperitoneal mass lesion was discerned. The left psoas muscle was hypotrophic. No family history of vascular malformations was elicited.

Peripheral blood count performed before the splenectomy revealed mild anemia (hemoglobin: 11.1 g/dL), mild thrombocytopenia (platelet count: 105,000/mm3), and a normal white blood cell count. Presplenectomy antibiotic prophylaxis was administered according to the standard protocol.

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Splenic lymphangiomatosis associated with left abdominopelvic and lower limb venolymphatic malformations.

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Three and a half years after the splenectomy, the patient is doing well. She received additional sclerotherapy that resulted in significant regression of the vulvar angioma.

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Benign lymphatic vessel proliferations may involve the spleen either as single or as multiple discrete multiloculated cystic lesions of various sizes (solitary or multiple lymphangiomas), or involve the entire organ as innumerable intraparenchymal lesions in a diffusely infiltrative form (lymphangiomatosis).1 On the basis of the calibre of the lymphatic ectasia, 3 histologic types of lymphangioma and lymphangiomatosis of the spleen are traditionally recognized: capillary, cystic, cavernous, and mixed.

Patients with solitary and multiple lymphangiomas as well as lymphangiomatosis of the spleen may present with involvement of other viscera as well as of soft tissues of multiple tissue planes. Lymphangiomatosis restricted to the spleen is the isolated (or pure) form of splenic lymphangiomatosis. Splenic lymphangiomatosis occurring in association with simultaneous or metachronous involvement of other viscera and tissues is considered to be the systemic or generalized or disseminated form.1,2 Occasionally, splenic lymphangiomatosis occurs in the setting of Gorham disease3–6 and Klippel-Trenaunay-Weber syndrome (splenic lymphangiomatosis in this latter syndrome was reported as lymphangioma).7–9

Lymphangiomatosis of the spleen is rare, and may occur in both children and adults. Pyatt et al10 recounted about 90 cases reported up to 1981. On the basis of a computerized literature search (PubMed) using lymphangiomatosis AND spleen and lymphangioma AND spleen, 55 additional cases of lymphangiomatosis with and without extrasplenic involvement were found after 1981. In sum, a total of <150 cases of both systemic and isolated lymphangiomatosis of the spleen have been reported by the end of 2013. Lymphangiomatosis in children is often found in the setting of multivisceral or multisystemic involvement,11–13 but may occur without extrasplenic involvement.2

Lymphangiomatosis restricted to the spleen is more frequently seen in adults, including young adults, with around 20 cases reported so far10,14–29 of which three ones have been reported as splenic lymphangiomas.20,24,29 The generalized form of splenic lymphangiomatosis only occasionally affects this age group.30 Concurrent involvement of accessory spleens has been documented in 2 cases.16,31 The clinical symptoms are usually proportional to the dimensions of the spleen. Left upper quadrant pain, sense of fullness, and abdominal distension are mentioned. Occasionally, despite splenomegaly, affected individuals may be asymptomatic,24,32 as our patient was. Lymphangiomatosis may even occur in a normal-sized spleen.28 The usual complications include consumptive coagulopathy, hypersplenism, bleeding or splenic rupture, and portal hypertension. Intrabdominal (portal or mesenteric) phlebothrombosis may also complicate lymphangiomatosis.33

Lymphangioma and lymphangiomatosis are considered localized and diffuse maldevelopments of the lymphatic vessels, due to an incompletely elucidated disarray of lymphangiogenesis.34,35 Cystic lymphatic malformations, often affecting the head, neck, and axillary regions are seen in genetic abnormalities such as Turner and Noonan syndromes and trisomies of chromosomes 13, 18, and 21. Lymphatic development and maintenance is likely regulated by endocrine, paracrine, or autocrine factors, including VEGF-C and its receptors VEGFR-3 and VEGFR-2.35–39 Because the lymphatic system arises from embryonic veins and retains stable anatomic connections with drainage into the central venous circulation after development is completed, it is not surprising to find lymphatic malformations associated with other complex vascular lesions, such as hemangiomas (Maffucci syndrome) or arteriovenous fistulas.40 As a major lymphoid organ, the spleen is in the transport pathway of lymphatic vessels and may become a target of developmental disturbances.

Despite their indubitable clinical significance, a simple yet comprehensive, meaningful, and reproducible classification of lymphatic vessel disorders remains to be devised.40–42 Our case does not fit isolated splenic lymphangiomatosis because it was associated with other venolymphatic abnormalities, and cannot be considered generalized lymphangiomatosis, because it was restricted in its extent of involvement just to a single tissue plane (ie, somatic superficial soft tissue). Limited forms of lymphangiomatosis, with involvement of another organ or tissue plane, mainly the liver,43–47 stomach,27 thymus (case termed splenic hemolymphangiomatosis),48 adrenal,49 somatic soft tissues,31 or soft tissue of large body cavities50,51 (one of these cases labeled as lymphangioma51) have been rarely reported. And 1 case of isolated splenic lymphangiomatosis has been observed in a patient diagnosed with Proteus syndrome.17

To the best of our knowledge, this patient had no family history of lymphatic malformations, or had recognizable syndromic features.40 Conceivably, this was a unique occurrence within the broad spectrum of developmental lymphatic and venous vascular diseases. Our patient manifested diffuse lymphangiomatosis of the spleen, regional lymphangiomatosis of soft tissue of the thigh and pubis, a complex venous malformation affecting the femoral-iliac axis, and asymmetric hypotrophy of the left psoas muscle. There may be pathogenetic implications in that the left side was predominantly affected. All of our patient’s lesions, except for the vulvar angioma, affected the left side of her body. In the usual body habitus, situs solitus, the caval veins are right-sided, thus the left-sided veins need to cross the midline, and are therefore longer. Because resistance to blood flow is directly related to the length of a vessel, the left-sided veins are more prone to developing sluggish flow and the consequences of venous ectasia, such as varicocele, complications of varicous veins, thrombosis, etc. The spleen is the only organ in humans that its anlage is strictly left sided, unless the determination of right and left is altered, as it happens in the heterotaxia syndromes associated with asplenia or polysplenia, and in conditions of situs inversus and situs ambiguous.

There is just another single record of left hemilateral hemangiomas involving the upper limb and chest wall which was associated with lymphangiomatosis of the spleen, but this occurred in a patient with Klippel-Trenaunay-Weber syndrome,9 a condition with occasional familiar aggregation characterized by vascular malformation of the skin, abnormalities of the venous and lymphatic systems, and limb enlargement due to hypertrophy of the soft tissue and bone.40,52,53 Two additional patients affected by Klippel-Trenaunay-Weber syndrome were also observed in whom multiple hepatic hemangiomas54 or mixed form of splenic hemangio-lymphangiomatosis55 have also been reported.

A preoperative diagnosis of lymphangiomatosis can be suspected with all imaging modalities: US, CT, magnetic resonance imaging, and angiography. By US, lymphangiomatosis appear as hypoechoic masses with internal septations. CT scan demonstrates thin-walled low-attenuation masses with sharp margins. Magnetic resonance imaging shows cystic lesions that appear hypointense on T1-weighted images and hyperintense on T2-weighted images. Celiac angiography reveals multiple avascular defects in the parenchymal phase, exhibiting the so-called Swiss-cheese appearance.9,23

The differential diagnosis by imaging includes parasitic cysts (mainly hydatid disease), nonparasitic cysts, and other benign and malignant splenic vascular proliferations.23,56–59 In certain clinical contexts, it may mimic metastatic disease.31 Even polycystic disease may be considered when splenic lymphangiomatosis is associated with liver and/or kidney involvement.30,60

A definitive diagnosis of lymphangiomatosis can be established histologically. The eosinophilic proteinaceous fluid-filled ectatic spaces are characteristically, lined by flat endothelium. When in doubt, and other types of nonparasitic cystic lesions need to be excluded, such as mesothelial cysts,61,62 the endothelial lineage can be conclusively demonstrated by immunohistochemistry using vascular markers, such as CD34, CD31, von Willebrand factor (FVIII-Rag), and ERG. The chief difficulty in the differential diagnosis is in those cases of lymphangiomatosis with intralesional bleeding when hemangiomas or hemangiomatosis63–65 cannot be excluded. If a higher level of proof is required, one might consider using immunohistochemical markers for lymphatic endothelium, such as Prox-1, LYVE-1, and podoplanin (D2-40), with the caveat that the latter may not be expressed in the large cystic component of lymphangiomatosis, whereas it is positive in the aforementioned mesothelial cysts.

Littoral cell angioma and angiosarcoma are easily excluded due to their characteristic morphologic and immunophenotypical features (tall endothelium coexpressing CD8 as well as histiocityic and endothelial markers). Splenic angiosarcoma is unlikely to be considered in the differential diagnosis because of its unmistakable complex architecture with papillary and solid areas and overt cytologic atypia with or without necrosis.66 A case with features of both lymphangioma and lymphangiosarcoma66 and another unusual case of benign lymphangioma with papillary features are also on record.67 Most recently a new form of clinically aggressive lymphatic proliferation, the so-called Kaposiform lymphangiomatosis has been described, which consistently affects the lung, but can also simultaneously involve other viscera, including the spleen.68 This new form of lymphangiomatosis, which mostly affects the children, has also been seen in an adult woman.69 Kaposiform lymphangiomatosis may enter the differential diagnosis. However, we believe there are sufficient clinical and histologic features to discern between the 2 entities. Histologically, Kaposiform lymphangiomatosis has been reported to be composed of endothelial spindle cells forming slit-like vascular spaces, morphologically different from capillary or cystic or cavernous lymphatic proliferations seen in the lymphangiomatosis entity which is the subject of this case report.

Treatment of lymphangiomatosis largely depends on the extent of the disease. In isolated splenomegaly, especially in adults, splenectomy is definitive. Splenectomy is also indicated in cases of hypersplenism or consumption coagulopathy, with mandatory preoperative antibacterial prophylaxis and immunization to prevent overwhelming postsplenectomy infections. Interferon-α70–73 and radiation treatment have been given when surgery was not feasible. Recently, sildenafil was found to cause marked regression of large lymphatic malformations, a therapeutic modality that may prove to be just as promising as propranolol for the management of infantile hemangiomas.74

In isolated lymphangiomatosis of the spleen, the prognosis is generally favorable. In multisystemic lymphangiomatosis, the morbidity and outcome depend on the extent of involvement and functional impairment of other viscera, with lung involvement generally having an unfavorable prognosis.12

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lymphangiomatosis; splenomegaly; venous malformation; lymphatic malformation; multiple cysts of spleen

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