Childhood splenic hemangiopericytoma: A previously unreported entity

Childhood Splenic Hemangiopericytoma: A Previously Unreported Entity By Arbay

0. Ciftci, Gijkhan

Gedikoilu,

Plnar A. Ftrat, Mehmet Ankara,

E. Senocak,

and Nebil

Biiytikpamukqu

Turkey

The first childhood case of splenic hemangiopericytoma in a IO-year-old boy is presented. The clinicopathologic features of this unique entity are discussed with special emphasis on differential diagnosis and treatment by comparing with the previously reported adult cases. There are no specific presumptive clinical and laboratory findings, including tumor markers and imaging techniques that distinguish hemangiopericytoma from other splenic masses. The most important diagnostic aid is to bear this entity in mind when a child

presents with an unexplained splenic mass. Splenectomy associated with chemotherapy or radiotherapy in the presence of systemic or local recurrences is mandatory for the appropriate treatment. J Pediatr Surg 34:1884-1886. Copyright o 1999 by W.B. Saunders Company.

B

peripheral smear, liver and renal function tests, and urinalysis were within normal limits. An abdominal ultrasound scan showed a 4- X 4- X 4-cm solitary, hetereogeneous, hypoechoic nodule in the spleen. Computerized tomography with contrast enhancement confirmed the splenic mass (Fig 1). No other abnormality was detected in the abdomen either by ultrasound scan or computerized tomography. Results of imaging studies were considered to be suggestive of hamartoma. At surgery, a 4- X 4- X 3-cm tumor of spleen was found. It was regular, yellowish brown, and strongly adherent to the surrounding splenic tissue (Fig 2). No evidence of any other intraabdominal pathology was found. Splenectomy was performed. Microscopically, the mass was composed of fairly uniform spindle cells and rich in vascular spaces (Fig 3). These anastomosing vascular channels showed highly dense reticular network among vascular spaces and neoplastic cells (Fig 4). Cytologically, imprint and scrape preparations showed uniform, spindle cells without pleomorphism and mitotic activity. There were foci of cartilaginous metaplasia with toluidin blue staining. Immunohistochemically, tumoral cells were negatively stained with factor-VIII-related antigen, desmin, smooth muscle actin, myoglobin, cytokeratin, epithelial membrane antigen, and S-100 protein, except vimentin. Ultrastructurally, the spindle cells had ovoid nuclei with few organelles, which were mainly composed of the rough endoplasmic reticulum and there were numerous vascular spaces. Postoperative course was uneventful. He was free of symptoms and had no complaints at his last outpatient appointment 6 months after surgery.

ENIGN AND MALIGNANT tumors of the spleen are quite unusual and mainly include cysts, hamartomas, hemangiomas, lymphangiomas, and lymphoma.’ Hemangiopericytoma is an uncommon benign vascular tumor, but has a relatively high malignant potential. It commonly arises in soft tissues of the lower extremities and retroperitoneum. Primary visceral lesions, especially of parenchymatous origin, are extremely rare.2 Limited numbers of splenic hemangiopericytoma have been reported in adults.3-5 Herein, we report on a lo-year-old child presenting with splenic mass that was found to be an hemangiopericytoma after splenectomy. To the best of our knowledge, this is the first case ever reported of a splenic hemangiopericytoma in a child. The clinicopathologic features of this unique case are discussed with special emphasis on differential diagnosis and treatment by comparing with the previously reported adult cases. CASE

REPORT

A lo-year-old boy was referred to our department with a presumptive diagnosis of splenic cyst based on an ultrasound scan finding and persistent left upper quadrant pain. On admission, his height and weight were at the 50th percentile, axillary temperature was 37.2”C, pulse was 84/min, and blood pressure was 110/65 mm Hg. There was no significant past medical history. His appetite, bowel, and urinary habits were normal. Physical examination showed normal findings apart from a slight tenderness on the left upper quadrant. Laboratory investigation findings including complete blood count,

From the Departments of Pediatric Surgery and Pathology, Hacettepe University Medical Faculty, Ankara, Turkey. Address reprint requests to Arbay 0. Cijtci, MD, Associate Professor of Pediatric Surgery, Hacettepe University Medical Faculty, 06100, Ankara, Turkey. Copyright o 1999 by WI. Saunders Company 0022-3468/99/3412-0036$03.00/O

1884

INDEX

WORDS:

Spleen,

hemangiopericytoma.

DISCUSSION

Splenic tumors have been classified into 4 categories as lymphoid tumors, nonlymphoid tumors, metastatic tumors, and tumorlike lesions, such as cysts and hamartomas.6 The most common nonlymphoid tumors are reported to be vascular tumors, which include benign and malignant forms of hemangiomas, hymphangiomas, hemangioendotheliomas, and hemangiopericytomas. However, the first case of splenic hemangiopericytoma was reported 4 years after this classification.3 Splenic hemangiopericytoma was presented previously in 3 adult cases consisting of 2 boys and 1 girl with a mean age of 43.3

Journa/ofPediatricSurgery,Vol34,

No 12 (December),

1999: pp 1884-1886

SPLENIC

HEMANGIOPERICYTOMA

1885

Fig 3. arranged Fig 1.

CT scan shows

mass

lesion

Macroscopic

appearance

richly vascular pattern and neoplastic (H&E, original magnification x230.)

cells

in the spleen.

years (range, 38 to 58 years). The tumor was palpable only in 1 case. Splenomegaly was not determined in any of the cases. Multiple hemangiopericytomas were noted in 1 case, whereas the others were isolated forms. The clinical characteristics of these patients are outlined in Table 1. Hemangiopericytoma is most common in the lower extremities, pelvic fossa, and retroperitoneum followed by head and neck regions and tnmk.2~5 The clinical features are neither striking nor characteristic. Slowly growing, painless (rarely painful) mass is the most common clinical finding. Other symptoms and signs usually develop because of the site of the tumor (ie, hydronephrosis in pelvic tumor). Hemangiopericytoma may produce secretions causing systemic signs such as renin resulting in hypertension.7 Hypoglycemia was also reported in some adult patients of abdominal hemangioperyctoma including one of the splenic involvement.2,3

Fig 2.

Tumor showed in haphazardly.

of the splenic

mass.

The preoperative diagnosis of hemangiopericytoma, particularly those originating from the spleen, is difficult: Hemangiopericytomas are generally reported as hypoechoic with a heterogeneous echo pattern as noted in our case.5 However, splenic metastasis may show a variety of echogenicities unrelated to primary hemangiopericytoma such as mixed echogenic structure or target sign.s Computerized tomography and magnetic resonance imaging have also limited value in differential diagnosis. There are no specific presumptive clinical and laboratory findings, including tumor markers and imaging techniques that distinguish hemangiopericytoma from other splenic tumors. The most important diagnostic aid is to bear this entity in mind when a child presents with an unexplained splenic mass. Histopathological and ultrastructural evaluation of the hemangiopericytoma is very important for the outcome

Fig 4. Dense reticulum fibers were seen vessels and neoplastic cells. (Silver staining, x115.)

among original

the branching magnification

CIFTCI

1886

Table 1. Clinical Age Study

Jurado

et al3 1989

Neil1 and Park4

(Yd

SW

Symptoms and Signs

38

Male

44

Female

58

Male

-

10

Male

Abdominal

1991 Hosotani 1992

et aI5

Characteristics Associated Diseases

-

Weight loss, epigastric mass, hypoglycemia -

Pancreatic docyst,

Ultrasonographic Findings

Heterogeneous, solid mass -

pseuchronic

renal failure -

Multiple, hypoechoic, solid

Ciftci (current case)

-

pain

and long-term follow-up. The tumor can be classified as benign, intermediate, and malignant according to the mitotic figure and cellular anaplasia. Various series showed an incidence of local or disseminated recurrence as great as 50% in intermediate and malignant forms.9 The lung is the most frequent site of metastases, which also may occur in the chest wall, brain, small bowel, and orbit. Because of the lack of data, the prognosis of splenic hemangioperyctoma currently is uncertain. But the treat-

ET AL

Treatment

outcome

Splenectomy

Generalized metastases

Splenectomy, drainage of

Recovery

pseudocyst Splenectomy

Recovery

Splenectomy

Recovery

masses

Heterogeneous, hypoechoic nodule

ment of choice for splenic involvement should be splenectomy because extensive excision is mandatory for the other locations according to the literature review.2,9J0 We would like to emphasize that hemangiopericytoma should be considered in the differential diagnosis of splenic masses in children. Splenectomy associated with chemotherapy or radiotherapy in the presence of systemic or local recurrences is mandatory for the appropriate treatment.

REFERENCES 1. Ein SH: Splenic lesions, in Ashcraft KW, Holder TM (eds): Pediatric Surgery. Philadelphia, PA. Saunders, 1993, pp 535-545 (chap 43) 2. Enzinger FM, Smith BH: Hemangiopericytoma-An analysis of 106 cases. Hum Path01 7:61-82, 1976 3. Jurado JG, Fuentes FT, Menendez CG, et al: Hemangiopericytoma of the spleen. Surgery 100:575-577, 1989 4. Neil1 JSA, Park HK: Hemangiopericytoma of the spleen. Anat Path01 95:680-683, 1991 5. Hosotani Ryo, Momoi H, Uchida H, et al: Multiple hemangiopericytomas of the spleen. Am J Gastroenterol87:1863-1865, 1992

6. Morgenstern L, Rosenberg J, Galler SA: Tumors of the spleen. World J Surg 9:468-476, 1985 7. Robertson PW, Klidjian A, Harding LK, et al: Hypertension due to a renin-secreting renal tumor. Am J Med 43:963-976, 1967 8. Siniluoto T, Paivansalo M, Lahde S: Ultrasonography of splenic metastases. Acta Radio1 30:463-466, 1989 9. McMaster MJ, Soule EH, Ivins JC: Hemangiopericytoma. A clinicopathologic study and long term follow up of 60 patients. Cancer 3612232.2244,1975 10. Nappi 0, Ritter JH, Pettinato G, et al: Hemangiopericytoma: Histopathological pattern or clinicopathologic entity. Semin Diagn Path01 12:221-232, 1995