Brief reports
plasma cells. The abundant free eosinophilic material also stained for kappa light chain. It was concluded that the nodules represented a plasmacytoma, producing abundant extracellular immunoglobulin which was being ingested by macrophages in particular, but also by alveolar and bronchiolar epithelialcells. Nine years later the patient is well and has no evidence of recurrence or of myelomatosis: he has no abnormal serum or urine immunoglobulin and no skeletal abnormalities: total serum protein is 79.8 g/1 and there are no abnormal fractions.
Discussion Crystalline cytoplasmic inclusions have been described in both normal and neoplastic plasma cells, in macrophages in both myelomatosis and solitary plasmacytomas and in renal epithelium in myelomat~sis~-~. The uptake of immunoglobulin by airway epithelial cells, demonstrated in this case, is unusual but not surprising as some of these cells normally process immunoglobulin A, adding secretory piece as this immunoglobulin crosses the epitheliumb.What defeated the initial diagnostic attempts in this case was the sparsity of plasma cells and the marked local deposition of their secretory
287
product. Electronmicroscopy suggested the correct diagnosis which was confirmed by immunocytochemistry.
Acknowledgement The immunocytochemistrywas kindly performed in the laboratory of Professor P.Isaacson.
References 1. Addis BJ. Lymphoproliferative disease. In Corrin B ed. The Lungs. Edinburgh: Churchill Livingston. 1990: 328. 2. Addis BJ. Isaacson P. Billings JA. Plasmacytoma of lymph nodes. Cancer 1980: 46; 340-346. 3. Mullen B, Chalvardjian A. Crystalline tissue deposits in a case of multiple myeloma. Arch. Pathol. Lab. Med. 1981: 105; 94-97. 4. Chejfec G. Natarelli J, Gould VE. ‘Myeloma lung’--a previously unreported complicationof multiple myeloma. Hum. Pathol. 1983: 14; 558-561. 5. Yamamoto T, Hishida A. Honda N. Ito I, Shirasawa H. Nagase M. Crystal-storing histiocytosis and crystalline tissue deposition in multiple myeloma. Arch. Pathol. Lab. Med. 1991: 115: 351-354. 6. Goodman JR, Link DW. Brown WR. Nakane PK. Ultrastructural evidence of transport of secretory IgA across bronchial epithelium. Am. Rev. Resp. Dis. 1981: 123: 115-119.
BRIEF REPORT
Malignant peripheral nerve sheath tumour with rhabdomyoblastic and glandular differentiation: immunohistochemical features D.S.C.ROSE, M.J.WILKINS, R.BIRCH* & D.J.EVANS Departments of Histopathology, St. Mary’s Hospital School 01 Medicine and *Orthopaedic Surgery, St. Mary’s Hospital, London, and Royal National Orthopaedic Hospital, Stanmore, Middlesex, UK Date of submission 18 December 199 1 Accepted for publication 24 March 1992
Keywords: malignant peripheral nerve sheath tumour, rhabdomyoblast. triton tumour, glandular schwannoma. immunohistochemistry
Introduction
show foci of rhabdomyoblastic differentiation (triton tumours), and exceptionally feature a glandular component. We report a case with both rhabdomyoblasts and glands and describe the immunohistochemical findings.
Malignant peripheral nerve sheath tumours may rarely
Case report
Address for correspondence: Dr D.S.C.Rose. Department of Hlstopathology. University College and Middlesex School of Mediclne. Rockefeller Building, University Street. London WClE 6JJ,UK.
A 34-year-old man presented with a n 18-month history of progressive painful paraesthesiae and weakness of his right arm.A tumour was found arising from the medial
288
Brief reports
cord and lower trunk of his brachial plexus. He was also noted to have a fusiform swelling of his right median cutaneous nerve, Caf6-au-lait patches, and cutaneous neurofibromas. He gave no family history of neurofibromatosis and genetic studies were not done. The axillary mass, the cutaneous nerve lesion, and a smaller neuroma found at operation were excised. He subsequently underwent chemotherapy and radiotherapy, but 7 months later required a forequarter amputation for recurrent tumour, followed by a further recurrence at 14 months. He was treated conservatively and died at 18 months, with tuplour extension into the trachea and carotid vessels. Autopsy was not performed. PATHOLOGICAL FINDINGS
The primary axillary tumour was a partially encapsulated irregular mass 130 x 90 x 70 mm with a pale yellow-green myxoid cut surface showing areas of necrosis. The smaller lesions proved to be benign neurofibromas. After fixation the specimens were routinely processed and sections stained with H & E. Subsequently selected blocks were stained with Alcian blue/PASD. Immunohistochemistry was performed using the avidin-biotin-complex technique with commercially available monoclonal antibodies against cytokerati (CAM 5.2), vimentin, myoglobin, desmin, epithelial membrane antigen (EMA),chromogranin. and polyclonal sera against S-100 protein and carcinoembryonic antigen (CEA). Microscopically, the tumour was a typical malignant peripheral nerve sheath tumour with an additional population of large cells with ovoid nuclei and ample eosinophilic cytoplasm (Figure la). Recurrent tumour showed a similar pattern with the presence of mucinsecreting glands. The glandular epithelium showed a moderate degree of nuclear atypia and occasional mitoses. Many cells showed characteristics intermediate between the spindle and glandular forms. These cells had plump ovotd vesicular nuclei with vacuolated eosinophilic cytoplasm. Often the cells collected into aggregates, many of which showed varying degrees of central lumen development with, ultimately. a transition to wellformed glands (Figure l b , c). Immunohistochemistry revealed focal positive staining for S-100 protein in the stromal component, more marked in the more 'schwannian' areas, and weaker in the intermediate component. Focal vimentin positivity was noted. The intermediate cellular aggregates and gland epithelium stained with C A M 5.2 (Figure2a), and theluminalmembraneshowed CEA and weak EMA staining. Occasional epithelial cells contained chromogranin-positivecytoplasmic granules (Figure 2b). The rhabdomyoblasts stained strongly for
Figure 1. a A collection of rhabdomyoblasta. b Glands and welldefined cellular aggregates. c Illdehed 'intermediate' cellular aggregates.
desmin (Figure 3a) and myoglobin, and moderately strongly for S-100 protein (Figure 3b). The observation that individual cells were showing both S-100 and desmin positivity was confirmed with a double immunostaining alkaline phosphatase/peroxidase technique.
Discussion Malignant peripheral nerve sheath tumours are uncommon. Approximately 5% show an epithelioid growth
Brief reports
289
Figure 2. a cellular aggregates and glandular structures stained with C A M 5.2. b Gland with a chromogranin positive-cell (arrow). Figure 3. Rhabomyoblasts stained a for desmin and b for S-100 protein (no counterstain used in these sections).
pattern, and rare variants include those with chondroid, myoblastic,and glandular differentiation'. To our knowledge only two cases with both myoblastic and glandular differentiation have been r e p ~ r t e d ~When . ~ . glandular foci are present, the glands resemble intestinal type epithelium which is usually well-differentiated, but which may be, as in our case, atypical2.Like Christensen & W0odruffe4we found positive staining for cytokeratin, CEA, and chromogranin in the glandular component, and the stromal component showed the expected staining characteristic^^*^*^ of s-100 protein and vimentii positivity. In addition, however, there were two features of particular interest. First, many cells showed an appearance intermediatebetween Schwann cells and glandular cells. As the morphology became more epithelial, the degree of positivity for S-100 was less marked and the cells stained for cytokeratin, the latter becoming more pronounced as the cells formed more identifiablyglandular aggregates. The glands in glandular malignant peripheral nerve sheath tumours are said to 'invariably
arise abruptly from the stroma". However, the differential diagnosis of a synovial sarcoma is most unlikely in a tumour arising from a major nerve root in a patient with neurofibromatosis, showing intestinal type glands with neuroendocrine cells, and containing rhabdomyoblasts. Interestingly, the glands illustrated in the study by Daimaru et al. do not appear to be sharply demarcated from the stromal element. The second interesting observation was that, on co-staining for desmin and S-100 protein, individual rhabdomyoblasts frequently stained strongly for both antigens, giving a variegated pattern. Previous authors have referred to co-staining of tumours3, but no specific mention of co-staining of individual cells has been made. Occasional rhabdomyosarcomas may show S-100 protein positivity, but polyclonal serum against S-100 protein is not generally reported as staining striated. cardiac, or smooth muscle, although we accept that low level positivity may be seen. In conclusion, malignant peripheral nerve sheath tumours may show an unusual diversity of differentiation with neural, muscular and epithelial glandular
290
Brief reports
components. The dual staining of the rhabdomyoblasts and the demonstration of intermediate forms between the spindle cell and glandular elements, both morphologically and on staining with cytokeratin, may perhaps be regarded as further evidence of divergent differentiation of uncommitted malignant cells.
Acknowledgements We should like to thank Lynn Trute for the immunohistochemistry, and Miss S. Van Noorden for the confirmatory double staining.
Peripheral nerve tumors showing glandular differen2. Woodruff JM, tiation (glandularschwannomas). Cancer 1976; 37; 2399-241 3. 3. Daimaru Y, Hashimoto H, Enjoji M. Malignant 'Triton' tumors: A clinicopathologic and immunohistochemical study of nine cases. Hum. Pathol. 1984; 15; 768-778. 4. Christensen WN. WoodruffJM.Neuroendocrine(NE)differentiation in peripheral nerve sheath tumors (PNSTs).Lab. Invest. 1988: 58; 17A. 5. Daimaru Y. Hashimoto H, Enjoji M. Malignant peripheral nervesheath tumors (malignant schwannomas). An irnmunohistochemica1 study of 29 cases. Am. 1. Surg. Pathol. 1985; 9; 434-444. 6. Wick MR,Swanson PR, Scheithauer BW, Manivel JG. Malignant peripheral nerve sheath tumor. An immunohistochemicalstudy of 62 cases. Am. J. Clin. Pathol. 1987; 87; 425-433.
References 1 . Enanger PM,Weiss SW. Malignanttumors of peripheral nerves. In Soft Tissue Tumors. 2nd edn. St.Louis: CV Mosby. 1988: 781-815.
Histopathologll 1992, 21, 290-292
BRIEF REPORT
Recurrent malignant fibrous histiocytorna of the liver B.J.MCGRADY & M.M.MIRAKHUR Institute of Pathology, Royal Victoria Hospital, Bevast, UK Date of submission 20 January 1992 Accepted for publication 23 March 1992
Keywords: liver, malignant fibrous histiocytoma
Introduction Primary liver mesenchymal malignancies are uncommon: most are classifled as angiosarcoma, leiomyosarcoma or embryonal sarcoma. We report an example of a primary hepatic malignant fibrous histiocytoma with an unusual clinical presentation, subsequent recurrence and survival in good health 9 years after the initial diagnosis.
Address for correspondence:Dr B.J.McGrady,Institute of Pathology. Royal Victoria Hospital,Belfast. UK.
Case report The patient, a 53-year-old female, was admitted with acute severe left-sided chest pain. An ultrasound and CAT scan of abdomen suggested the presence of a splenic tumour. Laparotomy revealed a 14 cm diameter tumour that was confined to the left lobe of liver. The initial diagnosis was a malignant mesenchymal tumour, possibly leiomyosarcoma. The patient made a good recovery. Five years later she developed anorexia and weight loss. Ultrasound scanning revealed the presence of a tumour mass in the right lobe of the liver. At laparotomy a 6.5 cm diameter lesion was removed. The tumour had the histological, immunocytochemical and ultrastructural features of a malignant fibrous histiocytoma (MFH), The original tumour w a reassessed ~ and found to have identical features to the later recurrence. The patient is currently alive and well.
plasma cells. The abundant free eosinophilic material also stained for kappa light chain. It was concluded that the nodules represented a plasmacytoma, producing abundant extracellular immunoglobulin which was being ingested by macrophages in particular, but also by alveolar and bronchiolar epithelialcells. Nine years later the patient is well and has no evidence of recurrence or of myelomatosis: he has no abnormal serum or urine immunoglobulin and no skeletal abnormalities: total serum protein is 79.8 g/1 and there are no abnormal fractions.
Discussion Crystalline cytoplasmic inclusions have been described in both normal and neoplastic plasma cells, in macrophages in both myelomatosis and solitary plasmacytomas and in renal epithelium in myelomat~sis~-~. The uptake of immunoglobulin by airway epithelial cells, demonstrated in this case, is unusual but not surprising as some of these cells normally process immunoglobulin A, adding secretory piece as this immunoglobulin crosses the epitheliumb.What defeated the initial diagnostic attempts in this case was the sparsity of plasma cells and the marked local deposition of their secretory
287
product. Electronmicroscopy suggested the correct diagnosis which was confirmed by immunocytochemistry.
Acknowledgement The immunocytochemistrywas kindly performed in the laboratory of Professor P.Isaacson.
References 1. Addis BJ. Lymphoproliferative disease. In Corrin B ed. The Lungs. Edinburgh: Churchill Livingston. 1990: 328. 2. Addis BJ. Isaacson P. Billings JA. Plasmacytoma of lymph nodes. Cancer 1980: 46; 340-346. 3. Mullen B, Chalvardjian A. Crystalline tissue deposits in a case of multiple myeloma. Arch. Pathol. Lab. Med. 1981: 105; 94-97. 4. Chejfec G. Natarelli J, Gould VE. ‘Myeloma lung’--a previously unreported complicationof multiple myeloma. Hum. Pathol. 1983: 14; 558-561. 5. Yamamoto T, Hishida A. Honda N. Ito I, Shirasawa H. Nagase M. Crystal-storing histiocytosis and crystalline tissue deposition in multiple myeloma. Arch. Pathol. Lab. Med. 1991: 115: 351-354. 6. Goodman JR, Link DW. Brown WR. Nakane PK. Ultrastructural evidence of transport of secretory IgA across bronchial epithelium. Am. Rev. Resp. Dis. 1981: 123: 115-119.
BRIEF REPORT
Malignant peripheral nerve sheath tumour with rhabdomyoblastic and glandular differentiation: immunohistochemical features D.S.C.ROSE, M.J.WILKINS, R.BIRCH* & D.J.EVANS Departments of Histopathology, St. Mary’s Hospital School 01 Medicine and *Orthopaedic Surgery, St. Mary’s Hospital, London, and Royal National Orthopaedic Hospital, Stanmore, Middlesex, UK Date of submission 18 December 199 1 Accepted for publication 24 March 1992
Keywords: malignant peripheral nerve sheath tumour, rhabdomyoblast. triton tumour, glandular schwannoma. immunohistochemistry
Introduction
show foci of rhabdomyoblastic differentiation (triton tumours), and exceptionally feature a glandular component. We report a case with both rhabdomyoblasts and glands and describe the immunohistochemical findings.
Malignant peripheral nerve sheath tumours may rarely
Case report
Address for correspondence: Dr D.S.C.Rose. Department of Hlstopathology. University College and Middlesex School of Mediclne. Rockefeller Building, University Street. London WClE 6JJ,UK.
A 34-year-old man presented with a n 18-month history of progressive painful paraesthesiae and weakness of his right arm.A tumour was found arising from the medial
288
Brief reports
cord and lower trunk of his brachial plexus. He was also noted to have a fusiform swelling of his right median cutaneous nerve, Caf6-au-lait patches, and cutaneous neurofibromas. He gave no family history of neurofibromatosis and genetic studies were not done. The axillary mass, the cutaneous nerve lesion, and a smaller neuroma found at operation were excised. He subsequently underwent chemotherapy and radiotherapy, but 7 months later required a forequarter amputation for recurrent tumour, followed by a further recurrence at 14 months. He was treated conservatively and died at 18 months, with tuplour extension into the trachea and carotid vessels. Autopsy was not performed. PATHOLOGICAL FINDINGS
The primary axillary tumour was a partially encapsulated irregular mass 130 x 90 x 70 mm with a pale yellow-green myxoid cut surface showing areas of necrosis. The smaller lesions proved to be benign neurofibromas. After fixation the specimens were routinely processed and sections stained with H & E. Subsequently selected blocks were stained with Alcian blue/PASD. Immunohistochemistry was performed using the avidin-biotin-complex technique with commercially available monoclonal antibodies against cytokerati (CAM 5.2), vimentin, myoglobin, desmin, epithelial membrane antigen (EMA),chromogranin. and polyclonal sera against S-100 protein and carcinoembryonic antigen (CEA). Microscopically, the tumour was a typical malignant peripheral nerve sheath tumour with an additional population of large cells with ovoid nuclei and ample eosinophilic cytoplasm (Figure la). Recurrent tumour showed a similar pattern with the presence of mucinsecreting glands. The glandular epithelium showed a moderate degree of nuclear atypia and occasional mitoses. Many cells showed characteristics intermediate between the spindle and glandular forms. These cells had plump ovotd vesicular nuclei with vacuolated eosinophilic cytoplasm. Often the cells collected into aggregates, many of which showed varying degrees of central lumen development with, ultimately. a transition to wellformed glands (Figure l b , c). Immunohistochemistry revealed focal positive staining for S-100 protein in the stromal component, more marked in the more 'schwannian' areas, and weaker in the intermediate component. Focal vimentin positivity was noted. The intermediate cellular aggregates and gland epithelium stained with C A M 5.2 (Figure2a), and theluminalmembraneshowed CEA and weak EMA staining. Occasional epithelial cells contained chromogranin-positivecytoplasmic granules (Figure 2b). The rhabdomyoblasts stained strongly for
Figure 1. a A collection of rhabdomyoblasta. b Glands and welldefined cellular aggregates. c Illdehed 'intermediate' cellular aggregates.
desmin (Figure 3a) and myoglobin, and moderately strongly for S-100 protein (Figure 3b). The observation that individual cells were showing both S-100 and desmin positivity was confirmed with a double immunostaining alkaline phosphatase/peroxidase technique.
Discussion Malignant peripheral nerve sheath tumours are uncommon. Approximately 5% show an epithelioid growth
Brief reports
289
Figure 2. a cellular aggregates and glandular structures stained with C A M 5.2. b Gland with a chromogranin positive-cell (arrow). Figure 3. Rhabomyoblasts stained a for desmin and b for S-100 protein (no counterstain used in these sections).
pattern, and rare variants include those with chondroid, myoblastic,and glandular differentiation'. To our knowledge only two cases with both myoblastic and glandular differentiation have been r e p ~ r t e d ~When . ~ . glandular foci are present, the glands resemble intestinal type epithelium which is usually well-differentiated, but which may be, as in our case, atypical2.Like Christensen & W0odruffe4we found positive staining for cytokeratin, CEA, and chromogranin in the glandular component, and the stromal component showed the expected staining characteristic^^*^*^ of s-100 protein and vimentii positivity. In addition, however, there were two features of particular interest. First, many cells showed an appearance intermediatebetween Schwann cells and glandular cells. As the morphology became more epithelial, the degree of positivity for S-100 was less marked and the cells stained for cytokeratin, the latter becoming more pronounced as the cells formed more identifiablyglandular aggregates. The glands in glandular malignant peripheral nerve sheath tumours are said to 'invariably
arise abruptly from the stroma". However, the differential diagnosis of a synovial sarcoma is most unlikely in a tumour arising from a major nerve root in a patient with neurofibromatosis, showing intestinal type glands with neuroendocrine cells, and containing rhabdomyoblasts. Interestingly, the glands illustrated in the study by Daimaru et al. do not appear to be sharply demarcated from the stromal element. The second interesting observation was that, on co-staining for desmin and S-100 protein, individual rhabdomyoblasts frequently stained strongly for both antigens, giving a variegated pattern. Previous authors have referred to co-staining of tumours3, but no specific mention of co-staining of individual cells has been made. Occasional rhabdomyosarcomas may show S-100 protein positivity, but polyclonal serum against S-100 protein is not generally reported as staining striated. cardiac, or smooth muscle, although we accept that low level positivity may be seen. In conclusion, malignant peripheral nerve sheath tumours may show an unusual diversity of differentiation with neural, muscular and epithelial glandular
290
Brief reports
components. The dual staining of the rhabdomyoblasts and the demonstration of intermediate forms between the spindle cell and glandular elements, both morphologically and on staining with cytokeratin, may perhaps be regarded as further evidence of divergent differentiation of uncommitted malignant cells.
Acknowledgements We should like to thank Lynn Trute for the immunohistochemistry, and Miss S. Van Noorden for the confirmatory double staining.
Peripheral nerve tumors showing glandular differen2. Woodruff JM, tiation (glandularschwannomas). Cancer 1976; 37; 2399-241 3. 3. Daimaru Y, Hashimoto H, Enjoji M. Malignant 'Triton' tumors: A clinicopathologic and immunohistochemical study of nine cases. Hum. Pathol. 1984; 15; 768-778. 4. Christensen WN. WoodruffJM.Neuroendocrine(NE)differentiation in peripheral nerve sheath tumors (PNSTs).Lab. Invest. 1988: 58; 17A. 5. Daimaru Y. Hashimoto H, Enjoji M. Malignant peripheral nervesheath tumors (malignant schwannomas). An irnmunohistochemica1 study of 29 cases. Am. 1. Surg. Pathol. 1985; 9; 434-444. 6. Wick MR,Swanson PR, Scheithauer BW, Manivel JG. Malignant peripheral nerve sheath tumor. An immunohistochemicalstudy of 62 cases. Am. J. Clin. Pathol. 1987; 87; 425-433.
References 1 . Enanger PM,Weiss SW. Malignanttumors of peripheral nerves. In Soft Tissue Tumors. 2nd edn. St.Louis: CV Mosby. 1988: 781-815.
Histopathologll 1992, 21, 290-292
BRIEF REPORT
Recurrent malignant fibrous histiocytorna of the liver B.J.MCGRADY & M.M.MIRAKHUR Institute of Pathology, Royal Victoria Hospital, Bevast, UK Date of submission 20 January 1992 Accepted for publication 23 March 1992
Keywords: liver, malignant fibrous histiocytoma
Introduction Primary liver mesenchymal malignancies are uncommon: most are classifled as angiosarcoma, leiomyosarcoma or embryonal sarcoma. We report an example of a primary hepatic malignant fibrous histiocytoma with an unusual clinical presentation, subsequent recurrence and survival in good health 9 years after the initial diagnosis.
Address for correspondence:Dr B.J.McGrady,Institute of Pathology. Royal Victoria Hospital,Belfast. UK.
Case report The patient, a 53-year-old female, was admitted with acute severe left-sided chest pain. An ultrasound and CAT scan of abdomen suggested the presence of a splenic tumour. Laparotomy revealed a 14 cm diameter tumour that was confined to the left lobe of liver. The initial diagnosis was a malignant mesenchymal tumour, possibly leiomyosarcoma. The patient made a good recovery. Five years later she developed anorexia and weight loss. Ultrasound scanning revealed the presence of a tumour mass in the right lobe of the liver. At laparotomy a 6.5 cm diameter lesion was removed. The tumour had the histological, immunocytochemical and ultrastructural features of a malignant fibrous histiocytoma (MFH), The original tumour w a reassessed ~ and found to have identical features to the later recurrence. The patient is currently alive and well.
