Adrenal lymphangioma masquerading as a pancreatic tail cyst.

Cystic lymphangiomas of the adrenal gland are rare. A 79-year-old female presented in the emergency room with epigastric discomfort, and an immovable mass was palpated in her abdomen upon physical examination. Imaging studies revealed a large cystic lesion in the pancreatic tail. The radiologic impression ruled out the possibility of a mucinous cystic neoplasm, or a pseudocyst in the pancreas. The operative findings demonstrated that the cystic mass originated in the left adrenal gland. A laparoscopic excision of the cystic mass was performed, and immunohistochemistry confirmed that this mass was a lymphangioma of the adrenal gland. Several prior reports have suggested that lymphangioma can mimic renal or splenic cysts. However, lymphangioma cases mimicking pancreatic cysts are very rare.

Diagnostic utility of WT-1 cytoplasmic stain in variety of vascular lesions.

Vascular lesions are commonly encountered in routine pathologic practice and often pose diagnostic challenges owing to their morphologic diversity. Although WT-1 expression was reported in some vascular tumors, little is known about its staining patterns in a spectrum of vascular lesions from various locations. We examined WT-1 immunostain in 95 cases of vascular lesions including angiosarcomas (AS, 19 cases), hemangioendotheliomas (HE, 5), Kaposi's sarcomas (KS, 4), cavernous hemangiomas (CVH, 12), capillary hemangiomas (CPH, 7), pyogenic granulomas (PG, 4), lymphangiomas (LA, 4), hemangiopericytomas (HP, 5), glomus tumors (GT, 8), vascular malformation (VM, 13) and granulation tissue (GRT, 14). Strong WT-1 cytoplasmic stain was invariably observed in all cases of malignant and borderline vascular tumors including AS (19/19), KS (4/4) and HE (5/5). WT-1 was also consistently expressed in CPH (7/7), PG (4/4), and GRT (14/14), while it became weaker in VM (10/13) and often negative in CVH (2/12) and LA (0/4). WT1 stain was not demonstrated in HP (0/5) and rarely in GT (2/8). We conclude that consistent and diffuse WT-1 cytoplasmic stain in AS, HE and KS can be useful in distinguishing these tumors from poorly differentiated tumors with mimicking features. On the other hand, reliable WT-1 stain in CPH, PG and GRT may help in differential diagnosis with non-endothelial vascular tumors such as GT and HP. Recognizing the WT-1 cytoplasmic stain in a broad spectrum of benign and neoplastic tissues is critical in formulating appropriate immunohistochemical panels and avoiding misinterpretation of results.

Unusual multifocal intraosseous papillary intralymphatic angioendothelioma (Dabska tumor) of facial bones: a case report and review of literature.

Papillary intralymphatic angioendothelioma (PILA) or Dabska tumor is extremely rare, and often affects the skin and subcutaneous tissues of children. Since its first description by Dabska, only a few intraosseous cases have been described in the literature and none of them presents with multifocal osteolytic lesion of bones. We present a case of unusual multifocal intraosseous PILA in facial bones occurring in a 1 year 3 month old male child. Computed tomography (CT) scan revealed multifocal osteolytic lesions were located at the left zygoma, left orbital bone and right maxillary. Histologically, the lesions were ill-defined and composed of multiple delicate interconnecting vascular channels with papillae formation which projected into the lumen lined by atypical plumped endothelial cells. The vascular channels were also lined by plump cuboidal endothelial cells with focal hobnailed or "match-head" appearance. In some areas, endothelial cells formed solid-appearing aggregates with vessel lumens. By immunohistochemistry, the tumor cells were positive for CD31, CD34 and D2-40 at varying intensity. A final diagnosis of intraosseous PILA was made. To the best of our knowledge, this case is the first case of primary multifocal osseous PILA. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1919488629100787.

Solitary hepatic lymphangioma: report of a case.

A 52-year-old woman presented with upper abdominal pain. Abdominal ultrasonography showed a 4-cm well-defined mass containing solid and cystic components in segment IV of the liver, and contrast-enhanced T1-weighted magnetic resonance imaging revealed heterogeneous enhancement within the tumor, indicating a solid or fibrous component. There were no cystic lesions in any other organs. A partial hepatectomy was performed, based on a preoperative diagnosis of sclerosing hemangioma and biliary cystadenoma or cystadenocarcinoma. Pathologically, the tumor appeared to be a multilocular and cystic lesion lined by attenuated endothelial- like cells with no atypia. Immunohistochemistry demonstrated the endothelial-like cells to be positive for the lymphatic-specific markers D2-40, LYVE-1, and Prox-1, which proved helpful for confirming the diagnosis as solitary hepatic lymphangioma. This case is presented with details of the pathologic and radiologic findings, because solitary hepatic lymphangioma is an extremely rare tumor and no previous reports have provided details of the immunohistochemical characteristics.

Blue rubber bleb nevus syndrome: novel lymphangiomatosis-like growth pattern within the uterus and immunohistochemical analysis.

Blue Rubber Bleb Nevus Syndrome is a rare, primarily sporadic condition characterized by vascular lesions principally involving the skin and gastrointestinal tract. Although considered a venous malformation, telangiectatic capillaries, arteriovenous malformations, and lymphangiomas have been reported, but a lymphangiomatosis-like growth pattern has not been described. This case of Blue Rubber Bleb Nevus Syndrome demonstrated a labyrinth of variably sized vascular spaces lined by an attenuated layer of bland endothelial cells, dissecting uterine tissues and sequestering remaining myometrium. Immunohistochemical profile of lesional endothelial cells from the myometrium included strong, diffuse CD31; variable CD34; strong, patchy D2-40; weak, patchy factor VIII-related antigen; focal linear subendothelial collagen type IV; Ki-67 in 1% of cells; and no GLUT-1 or WT1 expression. This report expands the morphological spectrum of vascular lesions in Blue Rubber Bleb Nevus Syndrome to include a lymphangiomatosis-like growth pattern and the immunohistochemistry suggests dual vascular and lymphatic differentiation, supporting the current belief that these lesions are malformations.

Mediastinal lymphangiomatosis coexisting with occult thymic carcinoma.

Mediastinal lymphangiomatosis in a 70-year-old woman was diagnosed on a medical checkup. The tumor was resistant to sclerotherapy with OK432 or bleomycin. The patient continued on a downhill course and died approximately 3 years after the initial diagnosis. Autopsy revealed a large tumor mass occupying the anterior mediastinum and firmly adhered to the pericardium and the pleura. The tumor consisted of two intermingled lesions: dilated vessels lined with D2-40-positive lymphatic endothelium and CD5-positive atypical cell nests with focal keratinization. The former was diagnosed as lymphangiomatosis and the latter as thymic squamous cell carcinoma. Vascular endothelial growth factor (VEGF)-C, a growth factor for lymphatic endothelial cells, was expressed by the carcinoma, and VEGF-C receptor was expressed by the endothelium of lymphangiomatosis. These findings suggested that VEGF-C derived from the thymic carcinoma induced the lymphangiomatosis lesion in a paracrine manner.

Lymphangioma-like Kaposi sarcoma.

BACKGROUND: Lymphangioma-like Kaposi's sarcoma (LLKS) is a rare morphologic expression of Kaposi's sarcoma (KS) that occurs in virtually all of the well-recognized clinical subtypes of the disease and has the potential to mimic other pathologic processes. In this study, we present the clinical and pathological features of four patients with LLKS. METHODS: Four cases of LLKS were retrieved from the dermatopathology files of our institution. All four tumours were tested immunohistochemically with anti-human herpesvirus-8 (HHV-8) latent nuclear antigen-1 (LNA-1) and anti-CD34 antibodies. RESULTS: Clinically, each patient presented with violaceous patches, papules or plaques; one patient presented with bullous lesions. All of the LLKS biopsy specimens revealed areas with characteristic light microscopic features of KS. Lymphangioma-like foci consisted of ectatic, irregularly shaped vascular spaces lined by mildly atypical endothelial cells. All tumour cells, including those associated with LLKS foci, showed a strong and diffuse reactivity for anti-HHV-8 LNA-1 and anti-CD34. KS progressed slowly in two patients with adequate follow-up. CONCLUSIONS: As LLKS can mimic other disease processes, the correct diagnosis relies heavily on the recognition of salient clinical and histological features of conventional KS, including a strong immunohistochemical expression of HHV-8-associated LNA-1 in lesional cells.

Case of adrenal lymphangioma with atypical lymphocytes in aspirate cytology.

Cytological diagnosis of adrenal cysts is becoming more important, with the increase in cases identified due to the widespread use of imaging modalities. Adrenal cysts have been classified into four main groups: endothelial, epithelial, parasitic, and pseudocyst, with endothelial cysts further classified as lymphangiomatous or angiomatous. We report on a case of adrenal lymphangioma with atypical lymphocytes found in the intraoperative aspirate, suggesting an inconclusive diagnosis, although immunohistochemical study led us to suppose their B-cell origin. This case provided important cytological findings that may assist in the prevention of cytological overdiagnosis.

Splenic lymphangioma with papillary endothelial proliferation: a case report and review of the literature.

A 76-year-old man complained of difficulty breathing. A solitary mass was found in the spleen by ultrasonography and the tumor was excised. Grossly, the tumor was 3.9 x 2.9 cm in size, solid and brownish in color. A stellate scar-like fibrosis was observed in the center of the tumor. Histologically, the tumor consisted of the proliferation of irregular and small lymph vessel-like spaces, with sclerotic change in the center. The lymph vessel-like spaces showed papillary projections of the lining cells. The lumen contained amorphous proteinaceous fluid. Immunohistochemically, the lining cells of lymph vessel-like spaces were positive for endothelial markers (CD31, CD34, factor VIII-related antigen), and bound Ulex europaeus agglutinin-1. The tumor was diagnosed as splenic lymphangioma, but its appearance was rather unusual for a typical splenic lymphangioma because of the presence of papillary endothelial proliferation and scar-like fibrosis. Splenic lymphangioma with papillary endothelial proliferation is uncommon, and there have been only four cases reported.

The transcription factor Prox1 is a marker for lymphatic endothelial cells in normal and diseased human tissues.

Detection of lymphatic endothelal cells (LECs) has been problematic because of the lack of specific markers. The homeobox transcription factor Prox1 is expressed in LECs of murine and avian embryos. We have studied expression of Prox1 in human tissues with immunofluorescence. In 19-wk-old human fetuses, Prox1 and vascular endothelial growth factor receptor-3 (VEGFR-3) are coexpressed in LECs of lymphatic trunks and lymphatic capillaries. Prox1 is located in the nucleus, and its expression is mutually exclusive with that of the blood vascular marker PAL-E. Prox1 is a constitutive marker of LECs and is found in tissues of healthy adults and lymphedema patients. Blood vascular endothelial cells (BECs) of hemangiomas express CD31 and CD34, but not Prox1. A subset of these cells is positive for VEGFR-3. Lymphatics in the periphery of hemangiomas express Prox1 and CD31, but not CD34. In lymphangiomas, LECs express Prox1, CD31, and VEGFR-3, but rarely CD34. In the stroma, spindle-shaped CD34-positive cells are present. We show that Prox1 is a reliable marker for LECs in normal and pathologic human tissues, coexpressed with VEGFR-3 and CD31. VEGFR-3 and CD34 are less reliable markers for LECs and BECs, respectively, because exceptions from their normal expression patterns are found in pathologic tissues.

Orbital lymphangioma with positive immunohistochemistry of lymphatic endothelial markers (vascular endothelial growth factor receptor 3 and podoplanin).

BACKGROUND: Existence of true orbital lymphangiomas has been questioned in recent years. Therefore an orbital lymphangioma was analyzed with two new specific markers of lymphatic endothelium. METHODS: Case-report with clinicopathological, immunohistochemical, and ultrastructural findings. A 25-year-old man presented with recurrent lower lid "hematomas" and a pea-sized tumor palpable in the left lower lid. Magnetic resonance imaging showed an inferonasally located orbital tumor which extended to the posterior pole of the eye. The highly vascularized tumor was excised by medial orbitotomy. RESULTS: Histopathologically, the mass consisted of large, erythrocyte-filled cavernous vessels without evidence of smooth muscle cells or pericytes surrounding them. Numerous lymph follicles and small arterioles were scattered between them. Immunohistochemically, endothelial cells lining the lumina of the cavernous vessels were partly positive for podoplanin and vascular endothelial growth factor receptor 3 (flt-4), two markers of lymphatic endothelium. These markers did not react with endothelial cells lining the arterioles. Ultrastructurally, cavernous vessels displayed features characteristic of lymphatic vessels, and the smaller vessels demonstrated signs of arterioles. CONCLUSION: Ultrastructural analysis and immunohistochemistry using two new markers of lymphatic endothelium suggest a lymphatic nature of large vessels in an orbital lymphangioma. A greater series of vascular orbital tumors must be studied with these new lymph endothelial markers to confirm the existence of true orbital lymphangiomas and to analyze different profiles of lymph endothelial marker expression.

Splenic angiosarcoma: a clinicopathologic and immunophenotypic study of 28 cases.

Primary angiosarcoma of the spleen is a rare neoplasm that has not been well characterized. We describe the clinical, morphologic, and immunophenotypic findings of 28 cases of primary splenic angiosarcoma, including one case that shares features of lymphangioma/lymphangiosarcoma. The patients included 16 men and 12 women, aged 29 to 85 years, with a mean of 59 years and median of 63 years. The majority of patients (75%) complained of abdominal pain, and 25% presented with splenic rupture. The most common physical finding was splenomegaly (71%). Seventeen of 21 patients were reported to have anemia. Macroscopic examination showed splenomegaly in 85% cases. Sectioning revealed discrete lesions in 88% of cases, ranging from well-circumscribed firm nodules to poorly delineated foci of necrosis and hemorrhage associated with cystic spaces. Microscopically, the tumors were heterogenous; however, all cases demonstrated at least a focal vasoformative component lined by atypical endothelial cells. Solid sarcomatous, papillary, and epithelioid growth patterns were observed. The solid sarcomatous component resembled fibrosarcoma in two cases and malignant fibroushistiocytoma in one case. Hemorrhage, necrosis, hemosiderin, extramedullary hematopoiesis, and intracytoplasmic hyaline globules were frequently identified. A panel of immunohistochemical studies revealed that the majority of tumors were immunoreactive for at least two markers of vascular differentiation (CD34, FVIIIRAg, VEGFR3, and CD31) and at least one marker of histiocytic differentiation (CD68 and/or lysozyme). Metastases developed in 100% of patients during the course of their disease. Twenty-six patients died of disease despite aggressive therapy, whereas only two patients are alive at last follow-up, one with disease at 8 years and the other without disease at 10 years. In conclusion, primary splenic angiosarcoma is an extremely aggressive neoplasm that is almost universally fatal. The majority of splenic angiosarcomas coexpress histiocytic and endothelial markers by immunohistochemical analysis, which suggest that some tumors may originate from splenic lining cells.

Benign lymphangioendothelioma of the thigh simulating a low-grade angiosarcoma.

Benign lymphangioendothelioma (BL) is a rare vascular neoplasm that can histopathologically mimic a low-grade angiosarcoma or the patch stage of Kaposi sarcoma. We report on the case of a 49-year-old man with a benign lymphangioendothelioma on the right thigh that evolved on a vascular birthmark after a trauma. Because of constant pain and the slow but progressive growth of the lesion, we decided to excise the tumor. Three stages of surgery were needed to obtain negative margins. We review the reports of BL to date, with special attention to those that developed after trauma and those that had a preexistent vascular lesion, and expound on the histopathologic differential diagnosis with low-grade angiosarcoma.

Bilateral lymphangiomas of the ovary: an immunohistochemical characterization and review of the literature.

Lymphangiomas of the ovary are rare tumors, with only 13 cases reported. The diagnoses of these tumors have been based on histologic findings without immunohistochemical confirmation of endothelial cell origin. It is uncertain if these tumors are true neoplasms or if some represent reactive lesions. In this report, the literature is reviewed, and a 53-year-old woman with bilateral ovarian lymphangiomas is described. The ovarian masses were composed of numerous, thin-walled, cystic spaces containing a proteinaceous fluid, mature lymphocytes, and occasional erythrocytes. The cyst walls were lined by flat, benign-appearing cells that were immunoreactive for factor VIII-related antigen, CD34, and CD31. Further examination of the specimen showed absent fallopian tube fimbriae, tuboovarian adhesions, and chronic follicular salpingitis, suggesting that the lymphatic proliferation in the ovaries was a reactive change secondary to impaired regional lymphatic drainage.

A clinicopathologic and immunohistochemical study of ten pancreatic lymphangiomas and a review of the literature.

BACKGROUND: Pancreatic lymphangiomas are rare benign tumors, of which only a few cases have been reported in the literature. In this study, the authors present a series of primary pancreatic lymphangiomas. METHODS: Cases of nonepithelial pancreatic cystic tumors (lymphangiomas) diagnosed between 1966 and 1994 were retrieved from the Endocrine Pathology Registry of the Armed Forces Institute of Pathology. Histologic features (in 10 cases) as well as histochemical and immunohistochemical studies (in 6 cases) were reviewed. Long term patient follow-up data were obtained in 9 cases. RESULTS: The patients included 8 females and 2 males ages 2-61 years (mean age, 28.9 years) at initial presentation. The tumors were circumscribed and occurred predominantly (in 6 of 10 cases) in the tail of the pancreas. The multicystic, serous, or chylous fluid-filled cystic tumors ranged from 3 to 20 cm (average, 12.7 cm) in greatest dimension. Histologically, the tumors consisted of multilocular cystic spaces of various sizes, lined by endothelial cells. The stroma contained smooth muscle and mature lymphocytes. Immunohistochemistry determined the endothelial lining cells to be factor VIII-R antigen and CD31 positive (in all cases tested) but usually CD34 negative. All patients for whom follow-up data were obtained (n=9) were alive without evidence of disease an average of 7.2 years after initial diagnosis. CONCLUSIONS: Pancreatic lymphangiomas occur predominantly in females within a wide age range. Multilocular, fluid-filled cysts, with endothelial immunoreactivity for factor VIII-R antigen and CD31, are characteristic of these tumors. Complete surgical excision of these benign tumors resulted in excellent long term prognoses for all patients studied.

Multilocular cystic epithelioid leiomyoma of the uterus with focal lymphangioma-like pattern: report of a case with immunohistochemical study.

We report a rare case of epithelioid leiomyoma of the uterus presenting as a multilocular cystic mass containing clear fluid. The histologic basis of cysts was the diffuse intratumoral cystic degeneration. Histologically, the presence of numerous cystic cavities of variable size raised the possibility that vascular abnormalities, such as hemangioma, lymphangioma, and diffuse cavernous angiomatosis, could be associated with epithelioid leiomyoma. An immunohistochemical study using endothelial cell and basement membrane markers, was helpful in excluding the vascular nature of the cystic cavities and revealed an unexpected focal lymphangioma-like pattern as a peculiar component of tumor.

Intra-abdominal lymphangiomas in children and adults. Assessment of proliferative activity.

OBJECTIVE: Intra-abdominal lymphangiomas are rare in children and even more exceptional in adults. Because these lesions occasionally progressively enlarge, we analyzed seven adult and four pediatric cases for evidence of proliferative activity. DESIGN: Immunohistochemical analysis was performed retrospectively on representative tissue sections using antibodies to the following antigens: Ki-67, proliferating cell nuclear antigen, and p53 gene product (eight cases). DNA ploidy was examined in five cases. PATIENTS: The study group consisted of seven adult women (aged 24 to 73 years), a 3.5-year-old girl, and two boys, aged 3.5 and 9 years, the last with a recurrence at age 15. The lymphangiomas ranged from 1.7 to 23 cm in maximum size. RESULTS: Ranges of percentages of cells staining for proliferating cell nuclear antigen, Ki-67, and p53 were similar between the pediatric and adult cases. Antibody to Ki-67 stained from 0.5% to 17% of the stromal and endothelial components of the lymphangiomas. Proliferating cell nuclear antigen activity was noted in 16% to 52% of lesional cells. Reactivity was noted almost exclusively in areas of inflammation and fibroplasia. For comparison, 10% to 50% of intermixed lymphocytes stained for Ki-67 and proliferating cell nuclear antigen. There was no labeling with p53. DNA content was uniformly diploid. CONCLUSIONS: The scant staining for Ki-67 in the majority of the lesions, combined with proliferative rates that were only focally elevated, suggests that lymphangiomas in children and adults are quiescent lesions whose enlargement is due to engorgement by chyle and localized secondary inflammation rather than primary tumoral growth.

Splenic mesothelial cysts mimicking lymphagiomas.

Lymphangiomas of the spleen may occur as part of lymphangiomatosis or may represent solitary lesions. Solitary splenic lymphangiomas are described traditionally as subcapsular, multicystic proliferations that are often incidental findings. Six cases of splenic tumors with morphologic features similar to those described for solitary lymphangioma were studied using an immunohistochemical panel that included epithelial and vascular markers. None of the patients had evidence of lymphangiomatosis, and all tumors were incidental findings in splenectomy specimens. All cases demonstrated lining cells that were positive for keratin and the mesothelial cell-associated antibody HBME-1 but were negative for the vascular markers Factor VIII-related antigens, CD31, and CD34. The immunohistochemical findings are suggestive of a mesothelial derivation of these multicystic proliferations rather than representing true lymphangiomas.