- Email: [email protected]
Intimal sarcoma of the abdominal aorta presenting as a retroperitoneal mass
CORRESPONDENCE
metaplasia usually arising in the background of the spindle cell ovarian-like stroma. Such stromal features were not seen in our case, and both the epithelial and stromal tumour cells were negative for ER and PR. CCRCC with smooth muscle cell stroma should also be excluded in the differential diagnosis of PRCC with smooth muscle rich stroma. The epithelial component of recently described CCRCC with smooth muscle cell stroma is composed of tubules, glands, nests, cords and sheets of malignant clear cells.3,4 No region composed of clear cell epithelial structures was seen in our case. In the vast majority of CCRCCs, alterations of the VHL gene are seen.1 VHL gene sequencing analysis revealed no mutation in our case. Furthermore, FISH analysis of chromosome 3, performed in the study of Shannon et al.,5 showed loss of the entire chromosome 3 in two cases and loss of 3p in the third case, consistent with CCRCC;12 whereas FISH analysis in our case revealed cytogenetic alterations characteristic for PRCC,12 gain of chromosomes 3, 7 and 17 in 32, 33 and 37% of epithelial tumour cells, respectively, and loss of chromosome Y in 62% of epithelial tumour cells. Moreover, our case showed no shark’s smile features and there were no tubulocystic elements which excludes from the differential diagnoses RAT6 and smooth muscle rich renal tumours reported by Merino et al.7 In conclusion, smooth muscle rich stroma can be seen in an otherwise classic case of PRCC. This unique case of PRCC with smooth muscle rich stroma expands the already heterogeneous group of renal tumours in which this phenotypic characteristic may be observed. To our knowledge this is the first PRCC with smooth muscle rich stroma reported in the literature. Acknowledgements: The authors wish to thank Sanja Davidovic´-Mrsic´, MD, PhD, Department of Laboratory Diagnostics, Clinical Hospital Center Zagreb, for performing FISH analysis. Conflicts of interest and sources of funding: This study was supported in part by the Ministry of Science, Education and Sports, Croatia, project number 108-1081870-1884 to K.B. The authors state that there are no conflicts of interest to disclose. Rine¨ Limani* Lumturije Gashi Luci* Zlatko Marusˇ ic´{ Zoran Gatalica§ Bozˇo Krusˇ lin{{ *Institute of Anatomical Pathology, Faculty of Medicine and University Clinical Center of Kosova, Prishtina, Kosovo, {‘Ljudevit Jurak’ University Department of Pathology, Sestre Milosrdnice University Hospital, Zagreb, zDepartment of Pathology, School of Medicine, University of Zagreb, Croatia; §Creighton University School of Medicine, Omaha, Nebraska, USA Contact Dr R. Limani. E-mail: [email protected] 1. Gatalica Z, Lilleberg SL, Vranic S, Eyzaguirre E, Orihuela E, Velagaleti G. Novel intronic germline FLCN gene mutation in a patient with multiple ipsilateral renal neoplasms. Hum Pathol 2008; 40: 1813–9. 2. Qian J, Bostwick DG, Takahashi S, et al. Comparison of fluorescence in situ hybridization analysis of isolated nuclei and routine histological sections
3.
4. 5.
6.
7.
8. 9.
10.
11. 12.
485
from paraffin-embedded prostatic adenocarcinoma specimens. Am J Pathol 1996; 149: 1193–9. Kuhn E, Anda JDe, Manoni S, Netto G, Rosai J. Renal cell carcinoma associated with prominent angioleiomyoma-like proliferation. Report of 5 cases and review of the literature. Am J Surg Pathol 2006; 30: 1372–81. Shannon BA, Cohen RG, Segal A, Baker EG, Murch AR. Clear cell renal cell carcinoma with smooth muscle stroma. Hum Pathol 2009; 40: 425–9. Brunelli M, Menestrina F, Segala D, et al. Renal cell carcinoma associated with prominent leiomyomatous proliferation appears not to be a variant of clear cell renal cell carcinoma. Mod Pathol 2010; 22: 160A–1A. Michal M, Hes O, Nemcova J, et al. Renal angiomyoadenomatous tumor: morphologic, immunohistochemical, and molecular genetic study of a distinct entity. Virchows Arch 2008; 454: 89–99. Merino MJ, Jinping L, Killian K, Linehan M. Smooth muscle rich, tubulocystic papillary renal cell carcinoma. A new renal tumor? Immunohistochemical and genetic characteristics. Mod Pathol 2010; 23: 206A. Srigley JR, Delahunt B. Uncommon and recently described renal carcinomas. Mod Pathol 2009; 22: S2–23. Zhou M, Roma A, Magi-Galluzi C. The usefulness of immunohistochemical markers in the differential diagnosis of renal neoplasms. Clin Lab Med 2005; 25: 247–57. Nikaido T, Nakano M, Kato M, Suzuki M, Ishikura H, Aizawa S. Characterization of smooth muscle components in renal angiomyolipomas: Histological and immunohistochemical comparison with renal capsular leiomyomas. Pathol Int 2004; 54: 1–9. Adsay NV, Eble JN, Srigley JR, Jones EC, Grignon DJ. Mixed epithelial and stromal tumor of the kidney. Am J Surg Pathol 2000; 24: 958–70. Sanjmyatav J, Schubert J, Junker K. Comparative study of renal cell carcinoma by CGH, multicolor-FISH and conventional cytogenic banding analysis. Oncol Rep 2005; 14: 1183–7.
DOI: 10.1097/PAT.0b013e3283559e90
Intimal sarcoma of the abdominal aorta presenting as a retroperitoneal mass Sir, Primary sarcoma of large vessels is uncommon and often does not have any classifiable histological pattern. The majority of cases are believed to arise in the intima as an intraluminal growth.1 Given this growth pattern, it is perhaps not surprising to find that the most common associated signs and symptoms are similar to other vaso-occlusive diseases of large vessels: rapid lower-extremity ischaemia, decreased peripheral pulses, embolic impairment of vital organs, pain/claudication and hypertension.1,2 With only approximately 100 cases of primary intimal sarcoma reported thus far in the literature,2 much remains to be learned about this rare neoplasm. We report a case presenting as an L3/4 radiculopathy with few vascular signs or symptoms. A 79-year-old male with type II diabetes mellitus and chronic kidney disease presented with 6 months of progressive ‘knife-like’ pain beginning in his right calf and radiating into his right groin. Pain worsened with walking and improved with rest; it was exacerbated by prolonged standing and relieved by sitting or lying down. He denied fever, chills, night sweats or weight loss, and had no history of malignancy. Physical examination was significant for 3/5 strength in the proximal right lower extremity (RLE), and 4/5 in the distal RLE, with a positive seated straight-leg raise test. Sensory testing, reflexes and peripheral pulses were normal. Ultrasound of the RLE vessels, plain films and nerve conduction studies were non-contributory. Figure 1 shows imaging and pathological findings. Computed tomography (CT) of the spine revealed a lobulated, irregular retroperitoneal mass destroying the L3 vertebral body, and partially occluding
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
486
CORRESPONDENCE
Pathology (2012), 44(5), August
Fig. 1 Imaging and pathological findings. (A) Pretreatment CT shows aortic wall (arrow) calcification and a periaortic mass with intraluminal extension. (B) Posttreatment MRI reveals a heterogeneous mass without significant change in size. (C) Gross morphology of the tumour with attached vertebral body (V). (D) Needle biopsy demonstrates a spindle cell proliferation involving fat. (E) Elastic stain of aortic wall (arrow), intraluminal (right lower) and periaortic (left upper) tumour. (F) Lower power H&E morphology of hypercellular and hypocellular areas. (G) Trichrome stain highlights tumour growth over blood clot. (H) High power H&E morphology of hypercellular and hypocellular junction. Arrow, aortic wall; V, vertebral body.
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
CORRESPONDENCE
and displacing the aorta anteriorly at the same level (Fig. 1A). The mass measured 9.1 6.3 cm in greatest dimension on sagital slice, and 6.4 5.8 cm in greatest dimension on axial slice. Magnetic resonance imaging (MRI) revealed similar findings. Serum LDH, CA19–9, and CEA levels were within normal limits, with PSA slightly elevated at 4.95. CBC was within normal limits save for a haematocrit of 35%. A needle core biopsy showed a spindle cell proliferation involving fat with a focal vague storiform pattern and low mitotic activity (Fig. 1D). These cells were weakly positive for smooth muscle actin and muscle specific actin, but negative for S-100, myogenin and c-kit. There was no significant pleomorphism or necrosis. These findings were consistent with a low grade spindle cell neoplasm that could not be further classified with limited biopsy material. As the patient had severe neurological pain, but was reluctant to have surgery, he underwent external beam radiation for palliation and minimally invasive tumour management. However, with progressively worsening symptoms and no reduction in tumour size on magnetic resonance imaging (MRI) (Fig. 1B) after 5 months of therapy, the patient agreed to surgical resection. Gross examination of formalin fixed tissue revealed a 6 cm aortic segment that included the iliac bifurcation distally. There was a 7 5 4.5 cm mass encircling the aorta with eccentric outgrowth behind it (Fig. 1C). The aortic lumen was occluded by intraluminal extension of the tumour that closely approximated the proximal and distal surgical resection margins. The cut surfaces were tan-white to tan-yellow with gelatinous appearance and focal necrosis and haemorrhage. Microscopically, the tumour showed morphology typical of intimal sarcoma (Fig. 1E–H) with hypercellular and hypocellular areas (Fig. 1F). Thin layers of atypical spindle cells were observed on the blood clot and the luminal surface (Fig. 1G). There was also lobulated fibroblastic proliferation with a focal vague storiform pattern, consistent with core biopsy findings (Fig. 1F). In addition, there was increased cellularity at the periphery of the lobule (Fig. 1F,H). Interestingly, tumour morphology was similar inside and outside the aorta, with the exception of an absence of blood clot outside. Retroperitoneal sarcomas are difficult to diagnose even following surgical resection. The most common sarcoma occurring at this site is liposarcoma. Although the current case simulated liposarcoma with a spindle cell proliferation involving fat, no lipoblasts were present. Nevertheless, a dedifferentiated liposarcoma could still explain these findings, especially on initial needle core biopsy, and should still be considered in the differential diagnosis, along with fibromatosis, inflammatory myofibroblastic tumour, leiomyoma, leiomyosarcoma, angiomyolipoma, and schwannoma. However, the imaging findings, overall morphology, and immunohistochemical staining pattern of this case were classic for aortic intimal sarcoma. Earlier case reports have showed that intimal sarcomas tend to grow intraluminally.1 Extravascular growth is often seen at local recurrence sites following surgical resection or involving distant metastasis.2 Unlike their intravascular counterparts, extravascular intimal sarcomas often differentiate toward a distinct cell lineage and have a classifiable histology.2 In this case, both components demonstrate similar spindle cell proliferation of cell types focally positive for either endothelial or myofibroblastic markers, but not any differentiated histological pattern. The main difference is that the intraluminal tumour is predominantly composed of thrombus.
487
Intimal sarcoma is known for its resistance to chemotherapy and radiation.2 This patient had 5 months of external beam radiation without radiological evidence of tumour shrinkage. Subjectively, the patient complained of worsening pain, which may be explained by either tumour progression or radiation associated fibrosis and neuritis. Pathological evaluation did reveal treatment effect with approximately 20% tumour necrosis. This suggests radiation therapy has certain effect on intimal sarcomas, or at least may retard their progression. The most common presentation of primary intimal sarcoma of the abdominal aorta is pain attributable to ischaemia.2 This patient’s presentation of pain had a significant neurological element, primarily due to or complicated by nerve root compression. This was supported by the fact that while his peripheral pulses and vascular examination were within normal limits, his neurological examination did elicit some deficits. Although his ‘cramping pain’ relieved by rest is typical for ischaemic pain, his associated weakness, pattern of pain (anteromedial thigh and groin, ‘shooting pain down the leg’) exacerbated by standing and improving with sitting, along with the positive straight leg tests made a stronger case for a neurogenic cause. Therefore, patients with intimal sarcoma may actually experience a mixed picture of vascular and neurogenic claudication, depending on tumour location and extent. Conflicts of interest and sources of funding: The authors state that there are no conflicts of interest to disclose. Andrea Klunder Kristina Subik Bo Ye Haodong Xu Faqian Li Department of Pathology and Laboratory Medicine, University of Rochester Medical Center, Rochester, NY, USA Contact Dr F. Li. E-mail: [email protected] 1. Burke AP, Virmani R. Sarcomas of the great vessels. A clinicopathologic study. Cancer 1993; 71: 1761–73. 2. Sebenik M, Ricci A Jr, DiPasquale B, et al. Undifferentiated intimal sarcoma of large systemic blood vessels: report of 14 cases with immunohistochemical profile and review of the literature. Am J Surg Pathol 2005; 29: 1184–93.
DOI: 10.1097/PAT.0b013e3283559ed1
Cystic tumour of the atrioventricular node: a case report Sir, The majority of sudden cardiac deaths are due to ischaemic heart disease. A very small number can be contributed to cardiac tumours (0.0025%).1 Metastatic disease, including melanoma, is far more common than primary tumours of the heart. Of 120 cases of primary tumours of the heart causing sudden cardiac death, cystic tumour of the atrioventricular node
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
metaplasia usually arising in the background of the spindle cell ovarian-like stroma. Such stromal features were not seen in our case, and both the epithelial and stromal tumour cells were negative for ER and PR. CCRCC with smooth muscle cell stroma should also be excluded in the differential diagnosis of PRCC with smooth muscle rich stroma. The epithelial component of recently described CCRCC with smooth muscle cell stroma is composed of tubules, glands, nests, cords and sheets of malignant clear cells.3,4 No region composed of clear cell epithelial structures was seen in our case. In the vast majority of CCRCCs, alterations of the VHL gene are seen.1 VHL gene sequencing analysis revealed no mutation in our case. Furthermore, FISH analysis of chromosome 3, performed in the study of Shannon et al.,5 showed loss of the entire chromosome 3 in two cases and loss of 3p in the third case, consistent with CCRCC;12 whereas FISH analysis in our case revealed cytogenetic alterations characteristic for PRCC,12 gain of chromosomes 3, 7 and 17 in 32, 33 and 37% of epithelial tumour cells, respectively, and loss of chromosome Y in 62% of epithelial tumour cells. Moreover, our case showed no shark’s smile features and there were no tubulocystic elements which excludes from the differential diagnoses RAT6 and smooth muscle rich renal tumours reported by Merino et al.7 In conclusion, smooth muscle rich stroma can be seen in an otherwise classic case of PRCC. This unique case of PRCC with smooth muscle rich stroma expands the already heterogeneous group of renal tumours in which this phenotypic characteristic may be observed. To our knowledge this is the first PRCC with smooth muscle rich stroma reported in the literature. Acknowledgements: The authors wish to thank Sanja Davidovic´-Mrsic´, MD, PhD, Department of Laboratory Diagnostics, Clinical Hospital Center Zagreb, for performing FISH analysis. Conflicts of interest and sources of funding: This study was supported in part by the Ministry of Science, Education and Sports, Croatia, project number 108-1081870-1884 to K.B. The authors state that there are no conflicts of interest to disclose. Rine¨ Limani* Lumturije Gashi Luci* Zlatko Marusˇ ic´{ Zoran Gatalica§ Bozˇo Krusˇ lin{{ *Institute of Anatomical Pathology, Faculty of Medicine and University Clinical Center of Kosova, Prishtina, Kosovo, {‘Ljudevit Jurak’ University Department of Pathology, Sestre Milosrdnice University Hospital, Zagreb, zDepartment of Pathology, School of Medicine, University of Zagreb, Croatia; §Creighton University School of Medicine, Omaha, Nebraska, USA Contact Dr R. Limani. E-mail: [email protected] 1. Gatalica Z, Lilleberg SL, Vranic S, Eyzaguirre E, Orihuela E, Velagaleti G. Novel intronic germline FLCN gene mutation in a patient with multiple ipsilateral renal neoplasms. Hum Pathol 2008; 40: 1813–9. 2. Qian J, Bostwick DG, Takahashi S, et al. Comparison of fluorescence in situ hybridization analysis of isolated nuclei and routine histological sections
3.
4. 5.
6.
7.
8. 9.
10.
11. 12.
485
from paraffin-embedded prostatic adenocarcinoma specimens. Am J Pathol 1996; 149: 1193–9. Kuhn E, Anda JDe, Manoni S, Netto G, Rosai J. Renal cell carcinoma associated with prominent angioleiomyoma-like proliferation. Report of 5 cases and review of the literature. Am J Surg Pathol 2006; 30: 1372–81. Shannon BA, Cohen RG, Segal A, Baker EG, Murch AR. Clear cell renal cell carcinoma with smooth muscle stroma. Hum Pathol 2009; 40: 425–9. Brunelli M, Menestrina F, Segala D, et al. Renal cell carcinoma associated with prominent leiomyomatous proliferation appears not to be a variant of clear cell renal cell carcinoma. Mod Pathol 2010; 22: 160A–1A. Michal M, Hes O, Nemcova J, et al. Renal angiomyoadenomatous tumor: morphologic, immunohistochemical, and molecular genetic study of a distinct entity. Virchows Arch 2008; 454: 89–99. Merino MJ, Jinping L, Killian K, Linehan M. Smooth muscle rich, tubulocystic papillary renal cell carcinoma. A new renal tumor? Immunohistochemical and genetic characteristics. Mod Pathol 2010; 23: 206A. Srigley JR, Delahunt B. Uncommon and recently described renal carcinomas. Mod Pathol 2009; 22: S2–23. Zhou M, Roma A, Magi-Galluzi C. The usefulness of immunohistochemical markers in the differential diagnosis of renal neoplasms. Clin Lab Med 2005; 25: 247–57. Nikaido T, Nakano M, Kato M, Suzuki M, Ishikura H, Aizawa S. Characterization of smooth muscle components in renal angiomyolipomas: Histological and immunohistochemical comparison with renal capsular leiomyomas. Pathol Int 2004; 54: 1–9. Adsay NV, Eble JN, Srigley JR, Jones EC, Grignon DJ. Mixed epithelial and stromal tumor of the kidney. Am J Surg Pathol 2000; 24: 958–70. Sanjmyatav J, Schubert J, Junker K. Comparative study of renal cell carcinoma by CGH, multicolor-FISH and conventional cytogenic banding analysis. Oncol Rep 2005; 14: 1183–7.
DOI: 10.1097/PAT.0b013e3283559e90
Intimal sarcoma of the abdominal aorta presenting as a retroperitoneal mass Sir, Primary sarcoma of large vessels is uncommon and often does not have any classifiable histological pattern. The majority of cases are believed to arise in the intima as an intraluminal growth.1 Given this growth pattern, it is perhaps not surprising to find that the most common associated signs and symptoms are similar to other vaso-occlusive diseases of large vessels: rapid lower-extremity ischaemia, decreased peripheral pulses, embolic impairment of vital organs, pain/claudication and hypertension.1,2 With only approximately 100 cases of primary intimal sarcoma reported thus far in the literature,2 much remains to be learned about this rare neoplasm. We report a case presenting as an L3/4 radiculopathy with few vascular signs or symptoms. A 79-year-old male with type II diabetes mellitus and chronic kidney disease presented with 6 months of progressive ‘knife-like’ pain beginning in his right calf and radiating into his right groin. Pain worsened with walking and improved with rest; it was exacerbated by prolonged standing and relieved by sitting or lying down. He denied fever, chills, night sweats or weight loss, and had no history of malignancy. Physical examination was significant for 3/5 strength in the proximal right lower extremity (RLE), and 4/5 in the distal RLE, with a positive seated straight-leg raise test. Sensory testing, reflexes and peripheral pulses were normal. Ultrasound of the RLE vessels, plain films and nerve conduction studies were non-contributory. Figure 1 shows imaging and pathological findings. Computed tomography (CT) of the spine revealed a lobulated, irregular retroperitoneal mass destroying the L3 vertebral body, and partially occluding
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
486
CORRESPONDENCE
Pathology (2012), 44(5), August
Fig. 1 Imaging and pathological findings. (A) Pretreatment CT shows aortic wall (arrow) calcification and a periaortic mass with intraluminal extension. (B) Posttreatment MRI reveals a heterogeneous mass without significant change in size. (C) Gross morphology of the tumour with attached vertebral body (V). (D) Needle biopsy demonstrates a spindle cell proliferation involving fat. (E) Elastic stain of aortic wall (arrow), intraluminal (right lower) and periaortic (left upper) tumour. (F) Lower power H&E morphology of hypercellular and hypocellular areas. (G) Trichrome stain highlights tumour growth over blood clot. (H) High power H&E morphology of hypercellular and hypocellular junction. Arrow, aortic wall; V, vertebral body.
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.
CORRESPONDENCE
and displacing the aorta anteriorly at the same level (Fig. 1A). The mass measured 9.1 6.3 cm in greatest dimension on sagital slice, and 6.4 5.8 cm in greatest dimension on axial slice. Magnetic resonance imaging (MRI) revealed similar findings. Serum LDH, CA19–9, and CEA levels were within normal limits, with PSA slightly elevated at 4.95. CBC was within normal limits save for a haematocrit of 35%. A needle core biopsy showed a spindle cell proliferation involving fat with a focal vague storiform pattern and low mitotic activity (Fig. 1D). These cells were weakly positive for smooth muscle actin and muscle specific actin, but negative for S-100, myogenin and c-kit. There was no significant pleomorphism or necrosis. These findings were consistent with a low grade spindle cell neoplasm that could not be further classified with limited biopsy material. As the patient had severe neurological pain, but was reluctant to have surgery, he underwent external beam radiation for palliation and minimally invasive tumour management. However, with progressively worsening symptoms and no reduction in tumour size on magnetic resonance imaging (MRI) (Fig. 1B) after 5 months of therapy, the patient agreed to surgical resection. Gross examination of formalin fixed tissue revealed a 6 cm aortic segment that included the iliac bifurcation distally. There was a 7 5 4.5 cm mass encircling the aorta with eccentric outgrowth behind it (Fig. 1C). The aortic lumen was occluded by intraluminal extension of the tumour that closely approximated the proximal and distal surgical resection margins. The cut surfaces were tan-white to tan-yellow with gelatinous appearance and focal necrosis and haemorrhage. Microscopically, the tumour showed morphology typical of intimal sarcoma (Fig. 1E–H) with hypercellular and hypocellular areas (Fig. 1F). Thin layers of atypical spindle cells were observed on the blood clot and the luminal surface (Fig. 1G). There was also lobulated fibroblastic proliferation with a focal vague storiform pattern, consistent with core biopsy findings (Fig. 1F). In addition, there was increased cellularity at the periphery of the lobule (Fig. 1F,H). Interestingly, tumour morphology was similar inside and outside the aorta, with the exception of an absence of blood clot outside. Retroperitoneal sarcomas are difficult to diagnose even following surgical resection. The most common sarcoma occurring at this site is liposarcoma. Although the current case simulated liposarcoma with a spindle cell proliferation involving fat, no lipoblasts were present. Nevertheless, a dedifferentiated liposarcoma could still explain these findings, especially on initial needle core biopsy, and should still be considered in the differential diagnosis, along with fibromatosis, inflammatory myofibroblastic tumour, leiomyoma, leiomyosarcoma, angiomyolipoma, and schwannoma. However, the imaging findings, overall morphology, and immunohistochemical staining pattern of this case were classic for aortic intimal sarcoma. Earlier case reports have showed that intimal sarcomas tend to grow intraluminally.1 Extravascular growth is often seen at local recurrence sites following surgical resection or involving distant metastasis.2 Unlike their intravascular counterparts, extravascular intimal sarcomas often differentiate toward a distinct cell lineage and have a classifiable histology.2 In this case, both components demonstrate similar spindle cell proliferation of cell types focally positive for either endothelial or myofibroblastic markers, but not any differentiated histological pattern. The main difference is that the intraluminal tumour is predominantly composed of thrombus.
487
Intimal sarcoma is known for its resistance to chemotherapy and radiation.2 This patient had 5 months of external beam radiation without radiological evidence of tumour shrinkage. Subjectively, the patient complained of worsening pain, which may be explained by either tumour progression or radiation associated fibrosis and neuritis. Pathological evaluation did reveal treatment effect with approximately 20% tumour necrosis. This suggests radiation therapy has certain effect on intimal sarcomas, or at least may retard their progression. The most common presentation of primary intimal sarcoma of the abdominal aorta is pain attributable to ischaemia.2 This patient’s presentation of pain had a significant neurological element, primarily due to or complicated by nerve root compression. This was supported by the fact that while his peripheral pulses and vascular examination were within normal limits, his neurological examination did elicit some deficits. Although his ‘cramping pain’ relieved by rest is typical for ischaemic pain, his associated weakness, pattern of pain (anteromedial thigh and groin, ‘shooting pain down the leg’) exacerbated by standing and improving with sitting, along with the positive straight leg tests made a stronger case for a neurogenic cause. Therefore, patients with intimal sarcoma may actually experience a mixed picture of vascular and neurogenic claudication, depending on tumour location and extent. Conflicts of interest and sources of funding: The authors state that there are no conflicts of interest to disclose. Andrea Klunder Kristina Subik Bo Ye Haodong Xu Faqian Li Department of Pathology and Laboratory Medicine, University of Rochester Medical Center, Rochester, NY, USA Contact Dr F. Li. E-mail: [email protected] 1. Burke AP, Virmani R. Sarcomas of the great vessels. A clinicopathologic study. Cancer 1993; 71: 1761–73. 2. Sebenik M, Ricci A Jr, DiPasquale B, et al. Undifferentiated intimal sarcoma of large systemic blood vessels: report of 14 cases with immunohistochemical profile and review of the literature. Am J Surg Pathol 2005; 29: 1184–93.
DOI: 10.1097/PAT.0b013e3283559ed1
Cystic tumour of the atrioventricular node: a case report Sir, The majority of sudden cardiac deaths are due to ischaemic heart disease. A very small number can be contributed to cardiac tumours (0.0025%).1 Metastatic disease, including melanoma, is far more common than primary tumours of the heart. Of 120 cases of primary tumours of the heart causing sudden cardiac death, cystic tumour of the atrioventricular node
Copyright © Royal College of pathologists of Australasia. Unauthorized reproduction of this article is prohibited.