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Year : 2020  |  Volume : 63  |  Issue : 2  |  Page : 292-294
A case of giant anastomosing hemangioma of the kidney with extramedullary hematopoiesis: A great mimicker

1 Department of Pathology, SRL Diagnostics, Bengaluru, Karnataka, India
2 Department of Surgical Oncology, Kerudi Cancer Hospital, Bagalkot, Karnataka, India

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Date of Web Publication18-Apr-2020


Anastomosing hemangioma (AH) are rare mesenchymal neoplasms affecting kidney with about 50 cases reported in English literature. In general, they are accidentally detected during examination or imaging done for nonspecific symptoms or wellness check-up. There are no key diagnostic features on the imaging modalities. The most common preoperative diagnosis has been a malignant primary renal carcinoma. Surgical resection remains the mainstay of treatment and of choice in symptomatic lesions. We are presenting a rare case of giant AH of the kidney mimicking a renal cell carcinoma on imaging. The lesions are characterized by anastomosing sinusoidal-like vascular spaces lined by banal endothelial cells with occasional hobnail morphology and associated with extramedullary hematopoiesis. The treatment of choice could be a conservative approach in small and asymptomatic lesions and patients with pre-existing renal dysfunction; hence, this entity should be in the differential of vascular renal neoplasms considering its proclivity to the urogenital tract. This is the first case in Indian literature to the best of our knowledge.

Keywords: Anastomosing, benign, hemangioma, renal

How to cite this article:
Manohar V, Krishnamurthy S, Ranganathan J, Pai VD. A case of giant anastomosing hemangioma of the kidney with extramedullary hematopoiesis: A great mimicker. Indian J Pathol Microbiol 2020;63:292-4

How to cite this URL:
Manohar V, Krishnamurthy S, Ranganathan J, Pai VD. A case of giant anastomosing hemangioma of the kidney with extramedullary hematopoiesis: A great mimicker. Indian J Pathol Microbiol [serial online] 2020 [cited 2021 Jul 26];63:292-4. Available from: https://www.ijpmonline.org/text.asp?2020/63/2/292/279833

   Introduction Top

Hemangiomas are common mesenchymal tumors which typically arise in skin and subcutaneous soft tissue. Occasionally, they involve viscera with liver being the most common affected organ. Hemangioma arising from kidney is rare with <300 cases reported in literature.[1],[2],[3] Among the reported cases, most are classified as capillary or cavernous subtype. Anastomosing hemangioma (AH) is a unique subtype characterized by an unusual sinusoidal pattern which is reminiscent of splenic parenchyma.[4]

Extramedullary hematopoiesis (EMH) commonly occurs in liver, spleen, and lymph nodes. EMH occurs either actively or passively in response to diverse changes in the hematopoietic environment. We are reporting a case of a large AH with associated EMH in a middle-aged woman with a preoperative diagnosis of renal cell carcinoma treated with radical nephrectomy.

   Case Report Top

A 40-year-old, otherwise healthy woman presented with history of vague upper abdominal pain of 6-month duration. Per abdominal examination revealed a well-defined mass arising from left kidney occupying left hypochondrium, left lumbar, epigastric, and umbilical area. Contrast enhanced computed tomography (CECT) of the abdomen revealed 12 cm × 12 cm × 11 cm, well-defined, heterogeneously enhancing, predominantly exophytic mass with chunky coarse calcification and surrounding fat stranding arising from the lower pole of left kidney [Figure 1]a. It was seen to compress the left ureter with resultant hydronephrosis of the upper calyces [Figure 1]b, suggesting a malignant neoplasm renal. Right kidney was normal. Rest of the laboratory investigations, including hematology, were within normal limits. Left radical nephrectomy with left para aortic lymph node dissection was performed with a provisional diagnosis of renal cell carcinoma in view of large size and imaging findings [Figure 1]c. Intra- and postoperative course was uneventful and she was discharged on fifth postoperative day.
Figure 1: (a) Contrast enhanced computed tomography (CECT) abdomen showing a well-defined, heterogeneously enhancing, predominantly exophytic mass measuring about 12 cm × 12 cm × 11 cm arising from the lower pole of left kidney. (b) CECT abdomen showing hydronephrosis of the upper calyces as a result of compression of the ureter by the tumor. (c) Left radical nephrectomy gross specimen

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Grossly, the resected specimen showed a well-circumscribed, large solid-cystic tumor in the lower pole of the kidney measuring 14 cm × 9 cm × 9 cm. On cut section, the tumor was tan brown with a spongy texture, abutting the renal capsule without invasion. Histopathological examination revealed a well-circumscribed, partially encapsulated tumor, with compressed renal tubules at the periphery. The tumor was composed of loose edematous stroma with ectatic, anastomosing vessels, lined by flattened to plump endothelial cells [Figure 2]a and [Figure 2]b. The cells were banal, with no evidence of mitosis. The stroma showed hemorrhage, extravasated red blood cells (RBC), and foci of hyalinization and calcification [Figure 2]c. Immunohistochemical study revealed positivity for CD31 and CD34 in the endothelial cell and negativitiy for Pan CK [Figure 2]e. The areas of EMH showed MPO positivity confirming the myeloid cell nature [Figure 2]d. The diagnosis of AH with EMH was rendered. At 2 years of follow-up, she is alive and in good health.
Figure 2: (a) Photomicrograph showing variably sized blood vessels surrounded by a cellular stroma (H and E, ×100). (b) Photomicrograph showing clusters of myeloid and megakaryocytic series and intervening anastomosing blood vessels (H and E, ×400). (c) Photomicrograph shows a central megakaryocyte and surrounding anastomosing variably sized vessels with maturing myeloid series (H and E, ×400). (d) Immunohistochemical stain, MPO shows positivity in myeloid series of EMH (×400). (e) Negative staining of the endothelium with immunostain Pan CK (×100)

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   Discussion Top

AH is a variant of capillary hemangioma first reported by Montogomery and Epstein.[1] The term is derived from the unusual sinusoidal pattern, which is reminiscent of splenic parenchyma. Although it is believed that AH is unique to the genitourinary system, cases involving other sites of the body, such as liver, adrenal gland, thigh, and abdominal wall, have also been reported.[5],[6],[7] Less than 50 cases of renal AH have been reported in the English literature.[8]

The median age of presentation is 50 years and males are affected more frequently than females. Majority of the patients remain asymptomatic and are detected incidentally during clinical examination or on imaging done for some other reason. When symptomatic, the varied presentations include hematuria, abdominal pain, and urinary tract infection.[8] Our case presented with a vague, nonspecific upper abdominal pain.

Imaging studies, CECT of the abdomen typically shows solid, well-defined, heterogeneous mass with intense enhancement in the arterial phase in majority of these cases.[9] The majority of reported cases in literature have been in the region of the hilum. Although enhancement pattern of these tumors differs from renal cell carcinoma, it is difficult to diagnose this entity with certainty on imaging because of its rarity.[5],[8] The present case was also suspected to be a renal cell carcinoma based on the imaging study as the lesion was large with a lower pole location. This potential pitfall is acknowledged and one has to be aware of this entity, although rare. The radiologists should consider AH in the differential diagnosis of any vascular renal mass, which shows enhancement with contrast administration. The other consideration during imaging and clinical work-up includes an adrenal myelolipoma; however; the location in the lower pole in our case and the absence of adipose tissue on microscopy enabled an easy exclusion.

AH is a pathological diagnosis. Macroscopic examination of these tumors shows red or mahogany brown lesions, which are typically spongy in consistency. Microscopic examination reveals histology, which is typical as described. Intravascular papillary areas, mild cytological atypia, sclerosis, hyalinization, hyaline globules, and fatty change are some of the variations of the classical histological type described in the literature. The association of EMH has been extensive.[2],[3],[4],[5],[6],[7],[8],[9],[10] Differential diagnosis includes angiosarcoma (AS), intravascular papillary endothelial hyperplasia (IPEH), angiomyolipoma (AML), and Kaposi sarcoma. Although AS and AH have overlapping clinical and pathological features, such as with hyaline globules and endothelial marker positivity on IHC, AS are invariable large, necrotic with infiltrative margins, and a highly cellular and mitotically active.[8] AS is the most important lesion to be successfully discriminated. The common association of EMH in AH is a clue. IPEH differes from AH in that IPEH shows papillary histomorphology lined by hyperplastic endothelium. Kaposi sarcoma characteristically shows positivity for staining of HHV-8, which is absent in AH. AML is characterized by diffuse positivity for smooth muscle cells and melanocytic markers.

Principles of management are not well defined as diagnosis of AH is established on histopathological examination of the resected specimen. However, all but one reported case in literature has been treated with nephrectomy.[5],[6],[9] There has not been any evidence of recurrence or metastasis in any of the reported cases.[9]

   Conclusion Top

The purpose of reporting this case is to stress the fact that the pathological diagnosis is not difficult if one is aware of this entity and its proclivity for the genitourinary. The accurate diagnosis lies on the onus of tissue histology. Preoperative diagnosis is elusive and is often mistaken for malignancy. Large lesions can pose a diagnostic pitfall. Despite the increasing numbers reported in literature, this lesion is usually approached as a definitive surgical management. Treatment can be controversial in cases with pre-existing morbidities, including end-stage renal disease and a more conservative approach is practical and the need of the hour. The treatment options include partial nephrectomy, embolization, and radical surgery. This is a benign lesion in all the existing literature analysis and it is best to consider an interventional option with least complications and comorbidities for the patient.

To summarize, this is the first case report in Indian literature with a unique lower pole presentation mimicking a renal cell carcinoma with a large size warranting a nephrectomy. This distinctive lesion is a growing knowledge among clinicians and requires consideration and better understanding to offer an optimal treatment.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Katabathina VS, Vikram R, Nagar AM, Tamboli P, Menias CO, Prasad SR. Mesenchymal neoplasms of the kidney in adults: Imaging spectrum with radiologic-pathologic correlation. Radiographics 2010;30:1525-40.  Back to cited text no. 1
Brown JG, Folpe AL, Rao P, Lazar AJ, Paner GP, Gupta R, et al. Primary vascular tumors and tumor-like lesions of the kidney: A clinicopathologic analysis of 25 cases. Am J Surg Pathol 2010;34:942-9.  Back to cited text no. 2
Mehta V, Ananthanarayanan V, Antic T, Krausz T, Milner J, Venkataraman G, et al. Primary benign vascular tumors and tumorlike lesions of the kidney: A clinicopathologic analysis of 15 cases. Virchows Arch 2012;461:669-76.  Back to cited text no. 3
Montgomery E, Epstein JI. Anastomosing hemangioma of the genitourinary tract: A lesion mimicking angiosarcoma. Am J Surg Pathol 2009;33:1364-9.  Back to cited text no. 4
Wetherell DR, Skene A, Manya K, Manecksha RP, Chan Y, Bolton DM. Anastomosing haemangioma of the kidney: A rare morphological variant of haemangioma characteristic of genitourinary tract location. Pathology 2013;45:193-6.  Back to cited text no. 5
Kryvenko ON, Gupta NS, Meier FA, Lee MW, Epstein JI. Anastomosing hemangioma of the genitourinary system: Eight cases in the kidney and ovary with immunohistochemical and ultrastructural analysis. Am J Clin Pathol 2011;136:450-7.  Back to cited text no. 6
Lin J, Bigge J, Ulbright TM, Montgomery E. Anastomosing hemangioma of the liver and gastrointestinal tract: An unusual variant histologically mimicking angiosarcoma. Am J Surg Pathol 2013; 37:1761-5.  Back to cited text no. 7
Kryvenko ON, Haley SL, Smith SC, Shen SS, Paluru S, Gupta NS, et al. Haemangiomas in kidneys with end-stage renal disease: A novel clinicopathological association. Histopathology 2014;65:309-18.  Back to cited text no. 8
Omiyale AO. Anastomosing hemangioma of the kidney: A literature review of a rare morphological variant of hemangioma. Ann Transl Med 2015;3:151.  Back to cited text no. 9
Perdiki M, Datseri G, Liapis G, Chondros N, Anastasiou I, Tzardi M, et al. Anastomosing hemangioma: Report of two renal cases and analysis of the literature. Diagnostic Pathology 2017;12:14.  Back to cited text no. 10

Correspondence Address:
Vidhya Manohar
Department of Pathology, SRL Diagnostics, Bengaluru, Karnataka - 560 076
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_434_18

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