- Email: [email protected]
Multifocal epithelioid angiosarcoma of the jejunum: a case report
CORRESPONDENCE
Conflict of interest and sources of funding: The authors state that there are no conflicts of interest to disclose. Ana Varallo-Nunez1 Man-Yuk Ho2 Winny Varikatt1 1
Pathology West, ICPMR, Westmead Hospital, and 2NSW Health, Nepean Hospital, Haematology Department, NSW, Australia Contact Dr Ana Varallo-Nunez. E-mail: [email protected]
1. Ackerman AB. Histologic Diagnosis of Inflammatory Skin Diseases. Philadelphia: Lea and Febinger, 1978;483e5. 2. LeBoit PE, Backstead JH, Bond B, Epstein WL, Friede IJ, Parslow TG. Granulomatous slack skin: clonal rearrangement of the proliferative nature of a cutaneous elastolytic disorder. J Invest Dermatol 1987; 89: 183e6. 3. Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005; 105: 3768e85. 4. Clarijs M, Poot F, Andoni L, Pirard C, Bourlond A. Granulomatous slack skin: treatment with extensive surgery and review of the literature. Dermatology 2003; 206: 393e7. 5. Balus L, Manente L, Remotti D, et al. Granulomatous slack skin: report of a case and review of the literature. Am J Dermatopathol 1996; 18: 199e206. 6. Kempf W, Ostheeren-Michaelis S, Paulli M, et al. Granulomatous mycosis fungoides and granulomatous slack skin: a multicenter study of the Cutaneous Lymphoma Histopathology Task Force Group of the European Organization for Research and Treatment of Cancer (EORTC). Arch Dermatol 2008; 144: 1609e17. 7. Ikonomou IM, Aamot HV, Heim S, Fosså A, Delabie J. Granulomatous slack skin with a translocation t(3;9)t (q12;p24). Am J Surg Pathol 2007; 31: 803e6. 8. Karakelides H, Geller JL, Schroeter AL, et al. Vitamin D-mediated hypercalcemia in slack skin disease: evidence for involvement of extrarenal 25-hydroxyvitamin D 1alpha-hydroxylase. J Bone Miner Res 2006; 21: 1496e9. 9. LeBoit PE, Burg G, Weedon D, Sarasin A. World Health Organization Classification of Tumors: Pathology and Genetics of Skin Tumours. Lyon: IARC Press, 2006;178. 10. Oberholzer PA, Cozzio A, Dummer R, French LE, Hofbauer GF. Granulomatous slack skin responds to UVA1 phototherapy. Dermatology 2009; 219: 268e71. 11. Kavusi S, Nazemi MJ, Ghiasi M, Sedaghat Y, Nasertork A. Granulomatous slack skin. Dermatol Online J 2006; 12: 20.
DOI: http://dx.doi.org/10.1016/j.pathol.2015.11.005
Multifocal epithelioid angiosarcoma of the jejunum: a case report Sir, Angiosarcoma is a malignant aggressive tumour of endothelial cell origin and comprises 1e2% of all sarcomas. Angiosarcoma occurs mainly in the skin, soft tissue, breast, liver, spleen, and lung.1 Angiosarcoma of the small intestine is extremely rare. Herein, we report one case of multifocal epithelioid angiosarcoma of the jejunum and analyse its clinicopathological features and differential diagnosis. A 62-year-old man with a medical history of hypertension for 20 years and aneurismal bone cyst curettage of the right distal femur 1 year prior presented with poor appetite, weakness, dizziness, melaena, and weight loss of 3 kg over 3 months, which increased in severity for 1 month prior to presentation. The laboratory investigation revealed haemoglobin
91
(Hb) level of 32 g/L (normal 135e170 g/L), with a normal mean corpuscular volume, and a positive faecal occult blood test. Endoscopic examination showed chronic gastritis and amounts of blood in the whole colon without active haemorrhagic lesions. Abdominal computed tomography (CT) scan showed multiple cysts of liver, cysts of both kidneys, and gallbladder stones. The patient was suspicious of haemorrhage of the digestive tract. Exploratory laparotomy was performed and multiple polypoid tumours and active haemorrhagic lesions of the small intestine were found 15e95 cm distal to the ligament of Treitz. Partial intestinal resection was performed. The patient did not receive adjuvant radiotherapy or chemotherapy because of poor health status. Positron emission tomography-CT (PET-CT) showed increased activity in the pelvic cavity consistent with tumour metastasis 1 month after surgery. Grossly, nine dark-red polypoid tumours were found in the mucosa of the small intestine. Some tumours were film with ulceration on the surface. The tumours ranged from 0.5 to 0.8 cm in greatest diameter (Fig. 1A). Microscopically, a number of tumours (at least 14 lesions) with clear boundary were located in the mucosa and submucosa layer of the jejunum and almost all tumours had ulceration on the surface. The tumour had a predominantly solid growth pattern that comprised sheets of plump spindled and epithelioid tumour cells with abundant lightly eosinophilic cytoplasm, large vesicular nuclei and prominent nucleoli. Some areas were composed of cords of epithelioid endothelial cells containing vacuoles in the cytoplasm. Vasoformative areas consisted of ramifying channels lined by atypical endothelial cells. Mitotic figures were about 9 per 10 high power fields. Atypical mitotic figures, coagulative necrosis, and haemorrhage were identified in most of the tumours (Fig. 1BeE). The tumour cells were positive for vimentin, CD31 and CD34, focally positive for pancytokeratin, but negative for cytokeratin 20, SMA, CD117, synaptophysin, and chromogranin A by immunohistochemical staining (Fig. 2). Based on these histopathological findings, we made the diagnosis of multifocal epithelioid angiosarcoma of the jejunum. Angiosarcoma is very rare in the small intestine. To date, there have been only 32 cases including our current case reported in the English literature.2e11 Twenty of these patients were male and 11 were female. The patients’ ages ranged from 25 to 87 years (mean 65.1 years, median 67 years). Of 32 cases, 28 were primary angiosarcoma in the small intestine and four cases were indeterminate. Angiosarcoma is classified as well-differentiated, poorly differentiated, and epithelioid angiosarcoma. Epithelioid angiosarcoma is characterised by epithelioid endothelial cells with abundant amphophilic to lightly eosinophilic cytoplasm, large vesicular nuclei and prominent nucleoli. There are only seven cases (including our current case) of epithelioid angiosarcoma of the small intestine reported in the literature.2,5,6,8 Six cases were primary and one case was indeterminate. All seven cases were male. The patients’ ages ranged from 45 to 87 years (mean 66.6 years, median 67 years). The majority of patients presented with intestinal bleeding, intestinal obstruction, melaena, anaemia, weight loss, and weakness. Ten patients had a history of irradiation expose or radiation therapy; the longest time since irradiation exposure was 30 years. Therefore, irradiation is closely related to tumourigenesis of angiosarcoma in the small intestine.
92
CORRESPONDENCE
Pathology (2016), 48(1), January
(A) Grossly, two dark-red polypoid tumours in the mucosa of the small intestine (arrows). (B) Histologically, the tumour was located in the mucosa and submucosa layer of the jejunum with ulceration on the surface. (C) The tumour had a predominantly solid growth pattern. (D) Cords of epithelioid endothelial cells containing vacuoles in the cytoplasm in some areas of tumour. (E) Epithelioid tumour cells with abundant lightly eosinophilic cytoplasm, large vesicular nuclei and prominent nucleoli, and atypical mitotic figure (H&E).
Fig. 1
Fig. 2
Tumour cells were immunoreactive for (A) CD31 and (B) CD34.
CORRESPONDENCE
Grossly, all epithelioid angiosarcomas of the small intestine including our current case reported were multiple, except one case.2,5,6,8 Microscopically, epithelioid angiosarcoma of the small intestine is characterised by solid and epithelioid growth of large polygonal cells. Tumour cells are positive for vimentin, CD31, CD34 and factor VIII, and focally positive for cytokeratin by immunohistochemical staining. Epithelioid angiosarcoma of the small intestine should be differentiated from carcinoma, neuroendocrine carcinoma, gastrointestinal stromal tumour (GIST), and malignant melanoma of the small intestine. Epithelioid angiosarcoma can be arranged in sheets, composed of abundant vessels and tumour cells are usually immunoreactive for cytokeratin, similar to carcinoma and neuroendocrine carcinoma of the small intestine. However, carcinoma and neuroendocrine carcinoma of the small intestine are not immunoreactive for endothelial markers such as CD31, CD34 or factor VIII, but are positive for CEA, cytokeratin 20. Furthermore, neuroendocrine carcinoma is also positive for neuroendocrine markers such as CD56, synaptophysin, and chromogranin A by immunohistochemical staining. GIST of small intestine is the most common mesenchymal tumour of the gastrointestinal tract, which is mainly composed of spindle cells. Some GISTs were predominated by epithelioid cells and immunoreactive for CD34, which is easily confused with epithelioid angiosarcoma. However, GIST is short of vasoformative areas and immunoreactive for CD117 and DOG-1. Malignant melanoma is composed of atypical tumour cells with vesicular nuclei and prominent nucleoli, which is similar to epithelioid angiosarcoma. However, malignant melanoma is usually immunoreactive for HMB45, melanin A, and S-100 protein, and negative for CD31 and CD34. The prognosis of angiosarcoma is very poor with a high rate of tumour-related death. As for the prognosis of epithelioid angiosarcoma in the small intestine, all six patients reported in the literature died 6 weeks to 1 year after initial diagnosis.2,5,6,8 Our current case had increased activity in the pelvic cavity by PET-CT which was consistent with tumour metastasis at 1 month after surgery. Because of the infiltrative and multifocal nature of this malignancy, complete surgical excision is often not possible. Azzariti et al. recently reported that the anti-angiogenetic agents are a reliable therapeutic opportunity for angiosarcoma patients.12 The role of adjuvant therapy has yet to be determined. Conflicts of interest and sources of funding: The authors state that there are no conflicts of interest to disclose. Huijuan Shi Tiantian Zhen Fenfen Zhang Yu Dong Anjia Han Department of Pathology, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, China Contact Dr Anjia Han. E-mail: [email protected] 1. Rouhani P, Fletcher CD, Devesa SS, Toro JR. Cutaneous soft tissue sarcoma incidence patterns in the US: an analysis of 12,114 cases. Cancer 2008; 113: 616e27.
93
2. Navarro-Chagoya D, Figueroa-Ruiz M, Lopez-Gomez J, et al. Obscure gastrointestinal bleeding due to multifocal intestinal angiosarcoma. Int J Surg Case Rep 2015; 10: 169e72. 3. Takahashi M, Ohara M, Kimura N, et al. Giant primary angiosarcoma of the small intestine showing severe sepsis. World J Gastroenterol 2014; 20: 16359e63. 4. Zacarias FL, Macher A, Braunstein S, Knoefel WT, Topp SA. Small intestine bleeding due to multifocal angiosarcoma. World J Gastroenterol 2012; 18: 6494e500. 5. Grewal JS, Daniel AR, Carson EJ, Catanzaro AT, Shehab TM, Tworek JA. Rapidly progressive metastatic multicentric epithelioid angiosarcoma of the small bowel: a case report and a review of literature. Int J Colorectal Dis 2008; 23: 745e56. 6. Al AJ, Ko HH, Owen D, Steinbrecher UP. Epithelioid angiosarcoma of the small bowel. Gastrointest Endosc 2006; 64: 1018e21. 7. Policarpio-Nicolas ML, Nicolas MM, Keh P, Laskin WB. Postradiation angiosarcoma of the small intestine: a case report and review of literature. Ann Diagn Pathol 2006; 10: 301e5. 8. Khalil MF, Thomas A, Aassad A, Rubin M, Taub RN. Epithelioid angiosarcoma of the small intestine after occupational exposure to radiation and polyvinyl chloride: a case report and review of literature. Sarcoma 2005; 9: 161e4. 9. Kelemen K, Yu QQ, Howard L. Small intestinal angiosarcoma leading to perforation and acute abdomen: a case report and review of the literature. Arch Pathol Lab Med 2004; 128: 95e8. 10. Delvaux V, Sciot R, Neuville B, et al. Multifocal epithelioid angiosarcoma of the small intestine. Virchows Arch 2000; 437: 90e4. 11. Watanabe K, Hoshi N, Suzuki T, Suzuki T. Epithelioid angiosarcoma of the intestinal tract with endothelin-1-like immunoreactivity. Virchows Arch A Pathol Anat Histopathol 1993; 423: 309e14. 12. Azzariti A, Porcelli L, Mangia A, et al. Irradiation-induced angiosarcoma and anti-angiogenic therapy: a therapeutic hope? Exp Cell Res 2014; 321: 240e7.
DOI: http://dx.doi.org/10.1016/j.pathol.2015.11.013
Sclerosing polycystic adenosis in a frozen section Sir, Sclerosing polycystic adenosis (SPA) is a rare, reactive, inflammatory process of the salivary glands first described in 1996 by Smith et al.1 at the Armed Forces Institute of Pathology. A recent literature review2 noted just over 50 cases with an age range of 9e84 years (median age 40) and the parotid was the most common gland affected (approximately 80% of cases). The process is usually unifocal, well circumscribed, composed of lobulated ductal and acinar hyperplasia in a dense background of sclerosis.3, 4 Characteristically the acinar cells display conspicuous eosinophilic granules in the cytoplasm similar to Paneth cells. It is usually a benign process, but foci of mild atypia are noted in approximately 50% of cases3 and severe dysplasia/carcinoma in situ is known to occur.5 Recurrence occurs in 19% of patients, usually due to incomplete primary excision, and is not known to behave aggressively.3 Immunohistochemically the ductal and acinar cells are pan-keratin positive. p63, smooth muscle actin and calponin are useful for detecting the myoepithelial component of SPA.2 Progesterone and oestrogen receptors are variably positive (approximately 80% and 20%, respectively).4 We present a case of 67-year-old male with an 8 mm solid/ cystic lesion in the right parotid gland. Fine needle aspiration had shown benign squamous cells and we were sent a superficial parotidectomy for frozen section, querying squamous cell carcinoma. Macroscopically, the specimen consisted of a 28 mm superficial parotidectomy with an 8 mm
Conflict of interest and sources of funding: The authors state that there are no conflicts of interest to disclose. Ana Varallo-Nunez1 Man-Yuk Ho2 Winny Varikatt1 1
Pathology West, ICPMR, Westmead Hospital, and 2NSW Health, Nepean Hospital, Haematology Department, NSW, Australia Contact Dr Ana Varallo-Nunez. E-mail: [email protected]
1. Ackerman AB. Histologic Diagnosis of Inflammatory Skin Diseases. Philadelphia: Lea and Febinger, 1978;483e5. 2. LeBoit PE, Backstead JH, Bond B, Epstein WL, Friede IJ, Parslow TG. Granulomatous slack skin: clonal rearrangement of the proliferative nature of a cutaneous elastolytic disorder. J Invest Dermatol 1987; 89: 183e6. 3. Willemze R, Jaffe ES, Burg G, et al. WHO-EORTC classification for cutaneous lymphomas. Blood 2005; 105: 3768e85. 4. Clarijs M, Poot F, Andoni L, Pirard C, Bourlond A. Granulomatous slack skin: treatment with extensive surgery and review of the literature. Dermatology 2003; 206: 393e7. 5. Balus L, Manente L, Remotti D, et al. Granulomatous slack skin: report of a case and review of the literature. Am J Dermatopathol 1996; 18: 199e206. 6. Kempf W, Ostheeren-Michaelis S, Paulli M, et al. Granulomatous mycosis fungoides and granulomatous slack skin: a multicenter study of the Cutaneous Lymphoma Histopathology Task Force Group of the European Organization for Research and Treatment of Cancer (EORTC). Arch Dermatol 2008; 144: 1609e17. 7. Ikonomou IM, Aamot HV, Heim S, Fosså A, Delabie J. Granulomatous slack skin with a translocation t(3;9)t (q12;p24). Am J Surg Pathol 2007; 31: 803e6. 8. Karakelides H, Geller JL, Schroeter AL, et al. Vitamin D-mediated hypercalcemia in slack skin disease: evidence for involvement of extrarenal 25-hydroxyvitamin D 1alpha-hydroxylase. J Bone Miner Res 2006; 21: 1496e9. 9. LeBoit PE, Burg G, Weedon D, Sarasin A. World Health Organization Classification of Tumors: Pathology and Genetics of Skin Tumours. Lyon: IARC Press, 2006;178. 10. Oberholzer PA, Cozzio A, Dummer R, French LE, Hofbauer GF. Granulomatous slack skin responds to UVA1 phototherapy. Dermatology 2009; 219: 268e71. 11. Kavusi S, Nazemi MJ, Ghiasi M, Sedaghat Y, Nasertork A. Granulomatous slack skin. Dermatol Online J 2006; 12: 20.
DOI: http://dx.doi.org/10.1016/j.pathol.2015.11.005
Multifocal epithelioid angiosarcoma of the jejunum: a case report Sir, Angiosarcoma is a malignant aggressive tumour of endothelial cell origin and comprises 1e2% of all sarcomas. Angiosarcoma occurs mainly in the skin, soft tissue, breast, liver, spleen, and lung.1 Angiosarcoma of the small intestine is extremely rare. Herein, we report one case of multifocal epithelioid angiosarcoma of the jejunum and analyse its clinicopathological features and differential diagnosis. A 62-year-old man with a medical history of hypertension for 20 years and aneurismal bone cyst curettage of the right distal femur 1 year prior presented with poor appetite, weakness, dizziness, melaena, and weight loss of 3 kg over 3 months, which increased in severity for 1 month prior to presentation. The laboratory investigation revealed haemoglobin
91
(Hb) level of 32 g/L (normal 135e170 g/L), with a normal mean corpuscular volume, and a positive faecal occult blood test. Endoscopic examination showed chronic gastritis and amounts of blood in the whole colon without active haemorrhagic lesions. Abdominal computed tomography (CT) scan showed multiple cysts of liver, cysts of both kidneys, and gallbladder stones. The patient was suspicious of haemorrhage of the digestive tract. Exploratory laparotomy was performed and multiple polypoid tumours and active haemorrhagic lesions of the small intestine were found 15e95 cm distal to the ligament of Treitz. Partial intestinal resection was performed. The patient did not receive adjuvant radiotherapy or chemotherapy because of poor health status. Positron emission tomography-CT (PET-CT) showed increased activity in the pelvic cavity consistent with tumour metastasis 1 month after surgery. Grossly, nine dark-red polypoid tumours were found in the mucosa of the small intestine. Some tumours were film with ulceration on the surface. The tumours ranged from 0.5 to 0.8 cm in greatest diameter (Fig. 1A). Microscopically, a number of tumours (at least 14 lesions) with clear boundary were located in the mucosa and submucosa layer of the jejunum and almost all tumours had ulceration on the surface. The tumour had a predominantly solid growth pattern that comprised sheets of plump spindled and epithelioid tumour cells with abundant lightly eosinophilic cytoplasm, large vesicular nuclei and prominent nucleoli. Some areas were composed of cords of epithelioid endothelial cells containing vacuoles in the cytoplasm. Vasoformative areas consisted of ramifying channels lined by atypical endothelial cells. Mitotic figures were about 9 per 10 high power fields. Atypical mitotic figures, coagulative necrosis, and haemorrhage were identified in most of the tumours (Fig. 1BeE). The tumour cells were positive for vimentin, CD31 and CD34, focally positive for pancytokeratin, but negative for cytokeratin 20, SMA, CD117, synaptophysin, and chromogranin A by immunohistochemical staining (Fig. 2). Based on these histopathological findings, we made the diagnosis of multifocal epithelioid angiosarcoma of the jejunum. Angiosarcoma is very rare in the small intestine. To date, there have been only 32 cases including our current case reported in the English literature.2e11 Twenty of these patients were male and 11 were female. The patients’ ages ranged from 25 to 87 years (mean 65.1 years, median 67 years). Of 32 cases, 28 were primary angiosarcoma in the small intestine and four cases were indeterminate. Angiosarcoma is classified as well-differentiated, poorly differentiated, and epithelioid angiosarcoma. Epithelioid angiosarcoma is characterised by epithelioid endothelial cells with abundant amphophilic to lightly eosinophilic cytoplasm, large vesicular nuclei and prominent nucleoli. There are only seven cases (including our current case) of epithelioid angiosarcoma of the small intestine reported in the literature.2,5,6,8 Six cases were primary and one case was indeterminate. All seven cases were male. The patients’ ages ranged from 45 to 87 years (mean 66.6 years, median 67 years). The majority of patients presented with intestinal bleeding, intestinal obstruction, melaena, anaemia, weight loss, and weakness. Ten patients had a history of irradiation expose or radiation therapy; the longest time since irradiation exposure was 30 years. Therefore, irradiation is closely related to tumourigenesis of angiosarcoma in the small intestine.
92
CORRESPONDENCE
Pathology (2016), 48(1), January
(A) Grossly, two dark-red polypoid tumours in the mucosa of the small intestine (arrows). (B) Histologically, the tumour was located in the mucosa and submucosa layer of the jejunum with ulceration on the surface. (C) The tumour had a predominantly solid growth pattern. (D) Cords of epithelioid endothelial cells containing vacuoles in the cytoplasm in some areas of tumour. (E) Epithelioid tumour cells with abundant lightly eosinophilic cytoplasm, large vesicular nuclei and prominent nucleoli, and atypical mitotic figure (H&E).
Fig. 1
Fig. 2
Tumour cells were immunoreactive for (A) CD31 and (B) CD34.
CORRESPONDENCE
Grossly, all epithelioid angiosarcomas of the small intestine including our current case reported were multiple, except one case.2,5,6,8 Microscopically, epithelioid angiosarcoma of the small intestine is characterised by solid and epithelioid growth of large polygonal cells. Tumour cells are positive for vimentin, CD31, CD34 and factor VIII, and focally positive for cytokeratin by immunohistochemical staining. Epithelioid angiosarcoma of the small intestine should be differentiated from carcinoma, neuroendocrine carcinoma, gastrointestinal stromal tumour (GIST), and malignant melanoma of the small intestine. Epithelioid angiosarcoma can be arranged in sheets, composed of abundant vessels and tumour cells are usually immunoreactive for cytokeratin, similar to carcinoma and neuroendocrine carcinoma of the small intestine. However, carcinoma and neuroendocrine carcinoma of the small intestine are not immunoreactive for endothelial markers such as CD31, CD34 or factor VIII, but are positive for CEA, cytokeratin 20. Furthermore, neuroendocrine carcinoma is also positive for neuroendocrine markers such as CD56, synaptophysin, and chromogranin A by immunohistochemical staining. GIST of small intestine is the most common mesenchymal tumour of the gastrointestinal tract, which is mainly composed of spindle cells. Some GISTs were predominated by epithelioid cells and immunoreactive for CD34, which is easily confused with epithelioid angiosarcoma. However, GIST is short of vasoformative areas and immunoreactive for CD117 and DOG-1. Malignant melanoma is composed of atypical tumour cells with vesicular nuclei and prominent nucleoli, which is similar to epithelioid angiosarcoma. However, malignant melanoma is usually immunoreactive for HMB45, melanin A, and S-100 protein, and negative for CD31 and CD34. The prognosis of angiosarcoma is very poor with a high rate of tumour-related death. As for the prognosis of epithelioid angiosarcoma in the small intestine, all six patients reported in the literature died 6 weeks to 1 year after initial diagnosis.2,5,6,8 Our current case had increased activity in the pelvic cavity by PET-CT which was consistent with tumour metastasis at 1 month after surgery. Because of the infiltrative and multifocal nature of this malignancy, complete surgical excision is often not possible. Azzariti et al. recently reported that the anti-angiogenetic agents are a reliable therapeutic opportunity for angiosarcoma patients.12 The role of adjuvant therapy has yet to be determined. Conflicts of interest and sources of funding: The authors state that there are no conflicts of interest to disclose. Huijuan Shi Tiantian Zhen Fenfen Zhang Yu Dong Anjia Han Department of Pathology, The First Affiliated Hospital, Sun Yat-Sen University, Guangzhou, China Contact Dr Anjia Han. E-mail: [email protected] 1. Rouhani P, Fletcher CD, Devesa SS, Toro JR. Cutaneous soft tissue sarcoma incidence patterns in the US: an analysis of 12,114 cases. Cancer 2008; 113: 616e27.
93
2. Navarro-Chagoya D, Figueroa-Ruiz M, Lopez-Gomez J, et al. Obscure gastrointestinal bleeding due to multifocal intestinal angiosarcoma. Int J Surg Case Rep 2015; 10: 169e72. 3. Takahashi M, Ohara M, Kimura N, et al. Giant primary angiosarcoma of the small intestine showing severe sepsis. World J Gastroenterol 2014; 20: 16359e63. 4. Zacarias FL, Macher A, Braunstein S, Knoefel WT, Topp SA. Small intestine bleeding due to multifocal angiosarcoma. World J Gastroenterol 2012; 18: 6494e500. 5. Grewal JS, Daniel AR, Carson EJ, Catanzaro AT, Shehab TM, Tworek JA. Rapidly progressive metastatic multicentric epithelioid angiosarcoma of the small bowel: a case report and a review of literature. Int J Colorectal Dis 2008; 23: 745e56. 6. Al AJ, Ko HH, Owen D, Steinbrecher UP. Epithelioid angiosarcoma of the small bowel. Gastrointest Endosc 2006; 64: 1018e21. 7. Policarpio-Nicolas ML, Nicolas MM, Keh P, Laskin WB. Postradiation angiosarcoma of the small intestine: a case report and review of literature. Ann Diagn Pathol 2006; 10: 301e5. 8. Khalil MF, Thomas A, Aassad A, Rubin M, Taub RN. Epithelioid angiosarcoma of the small intestine after occupational exposure to radiation and polyvinyl chloride: a case report and review of literature. Sarcoma 2005; 9: 161e4. 9. Kelemen K, Yu QQ, Howard L. Small intestinal angiosarcoma leading to perforation and acute abdomen: a case report and review of the literature. Arch Pathol Lab Med 2004; 128: 95e8. 10. Delvaux V, Sciot R, Neuville B, et al. Multifocal epithelioid angiosarcoma of the small intestine. Virchows Arch 2000; 437: 90e4. 11. Watanabe K, Hoshi N, Suzuki T, Suzuki T. Epithelioid angiosarcoma of the intestinal tract with endothelin-1-like immunoreactivity. Virchows Arch A Pathol Anat Histopathol 1993; 423: 309e14. 12. Azzariti A, Porcelli L, Mangia A, et al. Irradiation-induced angiosarcoma and anti-angiogenic therapy: a therapeutic hope? Exp Cell Res 2014; 321: 240e7.
DOI: http://dx.doi.org/10.1016/j.pathol.2015.11.013
Sclerosing polycystic adenosis in a frozen section Sir, Sclerosing polycystic adenosis (SPA) is a rare, reactive, inflammatory process of the salivary glands first described in 1996 by Smith et al.1 at the Armed Forces Institute of Pathology. A recent literature review2 noted just over 50 cases with an age range of 9e84 years (median age 40) and the parotid was the most common gland affected (approximately 80% of cases). The process is usually unifocal, well circumscribed, composed of lobulated ductal and acinar hyperplasia in a dense background of sclerosis.3, 4 Characteristically the acinar cells display conspicuous eosinophilic granules in the cytoplasm similar to Paneth cells. It is usually a benign process, but foci of mild atypia are noted in approximately 50% of cases3 and severe dysplasia/carcinoma in situ is known to occur.5 Recurrence occurs in 19% of patients, usually due to incomplete primary excision, and is not known to behave aggressively.3 Immunohistochemically the ductal and acinar cells are pan-keratin positive. p63, smooth muscle actin and calponin are useful for detecting the myoepithelial component of SPA.2 Progesterone and oestrogen receptors are variably positive (approximately 80% and 20%, respectively).4 We present a case of 67-year-old male with an 8 mm solid/ cystic lesion in the right parotid gland. Fine needle aspiration had shown benign squamous cells and we were sent a superficial parotidectomy for frozen section, querying squamous cell carcinoma. Macroscopically, the specimen consisted of a 28 mm superficial parotidectomy with an 8 mm