- Email: [email protected]
Hemangioendothelioma of liver and spleen: Trauma-induced consumptive coagulopathy
Hemangioendothelioma of Liver and Spleen: Trauma-Induced Consumptive Coagulopathy By Anju Goyal, S. Nagesh Babu, Vaiphei Kim, S. Katariya, and K.L.N. Rao Chandigarh, India
The case of a 9-year-old boy with hemangioendothelioma of the liver and spleen who presented with consumptive coagulopathy one month after sustaining a blunt trauma to his abdomen is reported. A contrast enhanced computed tomography scan of the abdomen showed a ruptured spleen with multiple lesions in the liver that were enhancing with contrast. On exploration, the child was found to have splenic rupture with multiple vascular lesions of the liver. A splenectomy with liver biopsy was done. The histopathologic exam-
ination found that both the liver and spleen had a similar tumor morphology characteristic of an epithelioid and spindle cell hemangioendothelioma. The child ultimately died of relentless consumptive coagulopathy. J Pediatr Surg 37:E29. Copyright 2002, Elsevier Science (USA). All rights reserved.
V
was diffuse ooze from all peritoneal surfaces including liver surface. As a desperate measure, hepatic artery ligation with abdominal packing was done. The patient was treated for KMS with methyl prednisolone, 30 mg/kg/d intravenously, for 3 days. His condition improved dramatically with clotting parameters returning to normal. Platelet count increased to 1.8 ⫻ 105/mm3. However, 2 days later he again had disseminated coagulopathy. His platelet count dropped to 11,000/mm3. He died of massive gastrointestinal hemorrhage caused by bleeding diathesis. Outer surface of spleen showed multiple nodular areas, the largest measuring 3 cm in diameter. The histopathologic examination of the spleen and liver biopsy showed involvement of the spleen by multiple nodular lesions similar in morphology to that involving the liver. The tumor nodules were demarcated sharply from the surrounding parenchyma by thin fibrous septae. There was a biphasic growth pattern characterized by discrete, nodular areas composed of round epithelioid cells, admixed with areas containing a meshwork of vascular channels lined by elongated oval to spindle cells. The epithelioid cells within the nodular areas showed abundant eosinophilic cytoplasm with centrally placed occasionally vesicular nucleus with prominent nucleoli. Many mitotic figures were seen (Fig 2). The intervening areas showed meshwork of vascular channels lined by oval to spindle-shaped cells with large nuclei and prominent nucleoli (Fig 3). The vascular spaces contained many red blood cells. The spleen, in addition, showed large areas of hemorrhage and necrosis probably related to trauma. The tumor cells in both the epithelioid and spindle cell areas were strongly immunoreactive with antibodies against vimentin and CD-31.
ASCULAR PROLIFERATIONS ACCOUNT for the majority of benign neoplasms of spleen and liver.1 Kasabach Merritt syndrome (KMS) or consumptive coagulopathy usually manifests during early infancy. The case reported here is unique in that the KMS presented later in life and was precipitated by trauma, hitherto unreported. Also, the entity, epithelioid and spindle cell hemangioendothelioma involving both liver and spleen simultaneously, is being reported for the first time. CASE REPORT
A 9-year-old boy presented with a history of progressive pallor, weight loss, and fever. He had sustained a cycle handle bar injury to his abdomen one month before. On examination, the child was sick looking with severe pallor. He was febrile with a pulse rate of 120 beats per minute and blood pressure of 110/60 mm Hg. The abdomen was distended. There was hepatosplenomegaly. The laboratory results showed hemoglobin level of 5.2 g/dL, total leukocyte count of 19,300/ mm3, and a platelet count of 19,000/mm3. The coagulation findings showed an increased prothrombin time and increased activated partial thromboplastin time with fibrinogen levels less than 120 mg/dL (normal, 200 to 400 mg/dL). On computed tomography (CT) of the abdomen there was mild hepatomegaly. There were multiple hypodense areas of varying sizes scattered in both the lobes of liver. The splenic shadow was enlarged and had a heterogenous density. Both hypodense and hyperdense areas were seen with in it. On giving contrast (Fig 1), there was peripheral enhancement around the hypodense lesions in the liver. Also, there was vascular enhancement seen in the solid areas of spleen. There was evidence of blood in the peritoneal cavity. The findings were consistent with multiple hemangioma of the liver with evidence of hemangiomatous pathology of spleen with a large hematoma. On exploration, there was a ruptured spleen with 1.5 L of hemoperitoneum, and the liver was found to be studded with multiple vascular lesions. A splenectomy and liver biopsy was done. This stabilized the patient temporarily. However, continued intraabdominal bleeding leading to gross abdominal distension and consequent respiratory compromise led us to reexplore the patient 4 days later. On laparotomy, there Journal of Pediatric Surgery, Vol 37, No 10 (October), 2002: E29
INDEX WORDS: Consumptive coagulopathy, hemangioendothelioma liver and spleen, Kasabach Merritt syndrome.
From the Department of Pediatric Surgery, Pathology, and Radiodiagnosis, Post Graduate Institute of Medical Education and Research, Chandigarh, India. Address reprint requests to Professor K.L.N. Rao, Head, Department of Pediatric Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India 160012. Copyright 2002, Elsevier Science (USA). All rights reserved. 1531-5037/02/3710-0036$35.00/0 doi:10.1053/jpsu.2002.35440 1
2
GOYAL ET AL
Fig 1. Contrast enhanced CT scan shows multiple hypodense lesions scattered in both lobes of the liver and spleen with peripheral enhancement (arrow).
DISCUSSION
Hemangioma is a common vascular lesion involving liver and spleen. Association of hemangioma with thrombocytopenia and consumptive coagulopathy, known as Kasabach Merritt syndrome, is well known.2 KMS also has been known to be associated with hemangioendotheliomas.3 It usually presents in infancy, although it may present at a later age.4 The exact mechanism that initiates the coagulopathy is not clearly understood. KMS triggered by trauma has not been reported before. The patients could have a severe bleeding diathesis only after partial excision of a cavernous hemangioma was attempted, suggesting that tissue damage and release of thromboplastin material into the circulation may have initiated the consumptive coagulopathy. In our patient also, probably trauma leading to splenic rupture and subsequent thromboplastin release caused by tissue damage initiated the coagulopathy.
Fig 3. Photomicrograph from an area that shows many vascular channels lined by plump epithelial cells with intervening innocuous appearing spindle cells (H&E, original magnification ⴛ 275).
From a histopathologic point of view, this case is unique in that it displayed features of both epithelioid and spindle cell hemangioendothelioma, a combination of features that, to the best of our knowledge, has been described in only one previous case report in the Englishlanguage literature.4 Weiss and Enzinger5 introduced the term epithelioid hemangioendothelioma in 1982 to designate a distinctive vascular tumor of soft tissue that pursued a clinical course intermediate between hemangioma and angiosarcoma. Another closely related lesion also described by Weiss and Enzinger6 in 1986 is spindle cell hemangioendothelioma, a low-grade malignant vascular neoplasm that combines features of both a cavernous hemangioma and Kaposi’s sarcoma. Suster4 described a splenic tumor displaying a biphasic growth pattern characterized by discrete, nodular areas composed of a monotonous proliferation of round epithelioid cells, surrounded by areas containing a meshwork of vascular channels lined by elongated oval to spindle cells. Our case had histologic picture similar to the above described epithelioid and spindle-cell hemangioendothelioma. There are various modalities of treatment of KMS. Isolated splenic involvement may respond to splenectomy.2 Multivisceral involvement may respond to splenectomy or may necessitate a trial of steroids. Other treatment options include antiangiogenic factors like interferon alpha and laser therapy.3 REFERENCES
Fig 2. Medium-power photomicrograph of the tumor shows solid areas comprising epithelioid cells. Individual cells have abundant pale eosinophilic cytoplasm and centrally placed pleomorphic vesicular nucleus with prominent nucleolus. Two mitotic figures (arrows) are also seen (H&E, original magnification ⴛ 275).
1. Luks FI, Yazbeck S, Brandt ML, et al: Benign liver tumors in children. A 25-year experience. J Pediatr Surg 26:1326-1330 1991 2. Dufau JP, le Tourneau A, Audouin J, et al: Isolated diffuse hemangiomatosis of the spleen with Kasabach Merritt like syndrome. Histopathology 35: 337-344, 1999 3. Deb G, Jenker A, De Sio L, et al: Spindle cell (Kaposiform)
HEMANGIOENDOTHELIOMA OF LIVER AND SPLEEN
hemangioendothelioma with Kasabach-Merritt syndrome in an infant: Successful treatment with alpha-2A interferon. Med Pediatr Oncol 28:358-361, 1997 4. Suster S: Epithelioid and spindle-cell hemangioendothelioma of the spleen—Report of a distinctive splenic vascular neoplasm of childhood. Am J Surg Pathol 16:785-792, 1992
3
5. Weiss SW, Enzinger FM: Epithelioid hemangioendothelioma: A vascular tumor often mistaken for a carcinoma. Cancer 50:970-981, 1982 6. Weiss SW, Enzinger FM: Spindle cell hemangioendothelioma: A low grade angiosarcoma resembling a cavernous hemangioma and Kaposi’s sarcoma. Am J Surg Pathol 10:521-530, 1986
The case of a 9-year-old boy with hemangioendothelioma of the liver and spleen who presented with consumptive coagulopathy one month after sustaining a blunt trauma to his abdomen is reported. A contrast enhanced computed tomography scan of the abdomen showed a ruptured spleen with multiple lesions in the liver that were enhancing with contrast. On exploration, the child was found to have splenic rupture with multiple vascular lesions of the liver. A splenectomy with liver biopsy was done. The histopathologic exam-
ination found that both the liver and spleen had a similar tumor morphology characteristic of an epithelioid and spindle cell hemangioendothelioma. The child ultimately died of relentless consumptive coagulopathy. J Pediatr Surg 37:E29. Copyright 2002, Elsevier Science (USA). All rights reserved.
V
was diffuse ooze from all peritoneal surfaces including liver surface. As a desperate measure, hepatic artery ligation with abdominal packing was done. The patient was treated for KMS with methyl prednisolone, 30 mg/kg/d intravenously, for 3 days. His condition improved dramatically with clotting parameters returning to normal. Platelet count increased to 1.8 ⫻ 105/mm3. However, 2 days later he again had disseminated coagulopathy. His platelet count dropped to 11,000/mm3. He died of massive gastrointestinal hemorrhage caused by bleeding diathesis. Outer surface of spleen showed multiple nodular areas, the largest measuring 3 cm in diameter. The histopathologic examination of the spleen and liver biopsy showed involvement of the spleen by multiple nodular lesions similar in morphology to that involving the liver. The tumor nodules were demarcated sharply from the surrounding parenchyma by thin fibrous septae. There was a biphasic growth pattern characterized by discrete, nodular areas composed of round epithelioid cells, admixed with areas containing a meshwork of vascular channels lined by elongated oval to spindle cells. The epithelioid cells within the nodular areas showed abundant eosinophilic cytoplasm with centrally placed occasionally vesicular nucleus with prominent nucleoli. Many mitotic figures were seen (Fig 2). The intervening areas showed meshwork of vascular channels lined by oval to spindle-shaped cells with large nuclei and prominent nucleoli (Fig 3). The vascular spaces contained many red blood cells. The spleen, in addition, showed large areas of hemorrhage and necrosis probably related to trauma. The tumor cells in both the epithelioid and spindle cell areas were strongly immunoreactive with antibodies against vimentin and CD-31.
ASCULAR PROLIFERATIONS ACCOUNT for the majority of benign neoplasms of spleen and liver.1 Kasabach Merritt syndrome (KMS) or consumptive coagulopathy usually manifests during early infancy. The case reported here is unique in that the KMS presented later in life and was precipitated by trauma, hitherto unreported. Also, the entity, epithelioid and spindle cell hemangioendothelioma involving both liver and spleen simultaneously, is being reported for the first time. CASE REPORT
A 9-year-old boy presented with a history of progressive pallor, weight loss, and fever. He had sustained a cycle handle bar injury to his abdomen one month before. On examination, the child was sick looking with severe pallor. He was febrile with a pulse rate of 120 beats per minute and blood pressure of 110/60 mm Hg. The abdomen was distended. There was hepatosplenomegaly. The laboratory results showed hemoglobin level of 5.2 g/dL, total leukocyte count of 19,300/ mm3, and a platelet count of 19,000/mm3. The coagulation findings showed an increased prothrombin time and increased activated partial thromboplastin time with fibrinogen levels less than 120 mg/dL (normal, 200 to 400 mg/dL). On computed tomography (CT) of the abdomen there was mild hepatomegaly. There were multiple hypodense areas of varying sizes scattered in both the lobes of liver. The splenic shadow was enlarged and had a heterogenous density. Both hypodense and hyperdense areas were seen with in it. On giving contrast (Fig 1), there was peripheral enhancement around the hypodense lesions in the liver. Also, there was vascular enhancement seen in the solid areas of spleen. There was evidence of blood in the peritoneal cavity. The findings were consistent with multiple hemangioma of the liver with evidence of hemangiomatous pathology of spleen with a large hematoma. On exploration, there was a ruptured spleen with 1.5 L of hemoperitoneum, and the liver was found to be studded with multiple vascular lesions. A splenectomy and liver biopsy was done. This stabilized the patient temporarily. However, continued intraabdominal bleeding leading to gross abdominal distension and consequent respiratory compromise led us to reexplore the patient 4 days later. On laparotomy, there Journal of Pediatric Surgery, Vol 37, No 10 (October), 2002: E29
INDEX WORDS: Consumptive coagulopathy, hemangioendothelioma liver and spleen, Kasabach Merritt syndrome.
From the Department of Pediatric Surgery, Pathology, and Radiodiagnosis, Post Graduate Institute of Medical Education and Research, Chandigarh, India. Address reprint requests to Professor K.L.N. Rao, Head, Department of Pediatric Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India 160012. Copyright 2002, Elsevier Science (USA). All rights reserved. 1531-5037/02/3710-0036$35.00/0 doi:10.1053/jpsu.2002.35440 1
2
GOYAL ET AL
Fig 1. Contrast enhanced CT scan shows multiple hypodense lesions scattered in both lobes of the liver and spleen with peripheral enhancement (arrow).
DISCUSSION
Hemangioma is a common vascular lesion involving liver and spleen. Association of hemangioma with thrombocytopenia and consumptive coagulopathy, known as Kasabach Merritt syndrome, is well known.2 KMS also has been known to be associated with hemangioendotheliomas.3 It usually presents in infancy, although it may present at a later age.4 The exact mechanism that initiates the coagulopathy is not clearly understood. KMS triggered by trauma has not been reported before. The patients could have a severe bleeding diathesis only after partial excision of a cavernous hemangioma was attempted, suggesting that tissue damage and release of thromboplastin material into the circulation may have initiated the consumptive coagulopathy. In our patient also, probably trauma leading to splenic rupture and subsequent thromboplastin release caused by tissue damage initiated the coagulopathy.
Fig 3. Photomicrograph from an area that shows many vascular channels lined by plump epithelial cells with intervening innocuous appearing spindle cells (H&E, original magnification ⴛ 275).
From a histopathologic point of view, this case is unique in that it displayed features of both epithelioid and spindle cell hemangioendothelioma, a combination of features that, to the best of our knowledge, has been described in only one previous case report in the Englishlanguage literature.4 Weiss and Enzinger5 introduced the term epithelioid hemangioendothelioma in 1982 to designate a distinctive vascular tumor of soft tissue that pursued a clinical course intermediate between hemangioma and angiosarcoma. Another closely related lesion also described by Weiss and Enzinger6 in 1986 is spindle cell hemangioendothelioma, a low-grade malignant vascular neoplasm that combines features of both a cavernous hemangioma and Kaposi’s sarcoma. Suster4 described a splenic tumor displaying a biphasic growth pattern characterized by discrete, nodular areas composed of a monotonous proliferation of round epithelioid cells, surrounded by areas containing a meshwork of vascular channels lined by elongated oval to spindle cells. Our case had histologic picture similar to the above described epithelioid and spindle-cell hemangioendothelioma. There are various modalities of treatment of KMS. Isolated splenic involvement may respond to splenectomy.2 Multivisceral involvement may respond to splenectomy or may necessitate a trial of steroids. Other treatment options include antiangiogenic factors like interferon alpha and laser therapy.3 REFERENCES
Fig 2. Medium-power photomicrograph of the tumor shows solid areas comprising epithelioid cells. Individual cells have abundant pale eosinophilic cytoplasm and centrally placed pleomorphic vesicular nucleus with prominent nucleolus. Two mitotic figures (arrows) are also seen (H&E, original magnification ⴛ 275).
1. Luks FI, Yazbeck S, Brandt ML, et al: Benign liver tumors in children. A 25-year experience. J Pediatr Surg 26:1326-1330 1991 2. Dufau JP, le Tourneau A, Audouin J, et al: Isolated diffuse hemangiomatosis of the spleen with Kasabach Merritt like syndrome. Histopathology 35: 337-344, 1999 3. Deb G, Jenker A, De Sio L, et al: Spindle cell (Kaposiform)
HEMANGIOENDOTHELIOMA OF LIVER AND SPLEEN
hemangioendothelioma with Kasabach-Merritt syndrome in an infant: Successful treatment with alpha-2A interferon. Med Pediatr Oncol 28:358-361, 1997 4. Suster S: Epithelioid and spindle-cell hemangioendothelioma of the spleen—Report of a distinctive splenic vascular neoplasm of childhood. Am J Surg Pathol 16:785-792, 1992
3
5. Weiss SW, Enzinger FM: Epithelioid hemangioendothelioma: A vascular tumor often mistaken for a carcinoma. Cancer 50:970-981, 1982 6. Weiss SW, Enzinger FM: Spindle cell hemangioendothelioma: A low grade angiosarcoma resembling a cavernous hemangioma and Kaposi’s sarcoma. Am J Surg Pathol 10:521-530, 1986