Auris·Nasus·Larynx (Tokyo) 19,209-214 (1992)
MALIGNANT FIBROUS HISTIOCYTOMA OF THE AURICLE: AN IMMUNOHISTO CHEMICAL AND ELECTRONMIC ROSCOPIC STUDY Megumi MoRITA, M.D., Toshio YosHIHARA, M.D., Nanami NARITA, M.D., Tetsuo ISHII, M.D., and Motohiko AlBA, M.D.* Department of Otolaryngology and *Pathology, Tokyo Women's Medical College, Tokyo, Japan
Malignant fibrous histiocytoma (MFH) is a soft-tissue tumor of late adult life. This tumor is uncommon in the head and neck region. A case of MFH arising in the auricle is presented with immunohistochemical and electronmicroscopic findings. Histologically the tumor was characterized by numerous spindle cells with abundant eosinophilic cytoplasm and many small capillaries. They were positively stained with vimentin, a 1-antitrypsin, and a 1-antichymotrypsin. Electronmicroscopically, some tumor cells contained abundant lysosomes and others contained numerous filaments. Malignant fibrous histiocytoma (MFH) is a common soft-tissue neoplasm in late adult life. According to Blitzer et al, paranasal sinuses, mandible and temporal bone, salivary gland, and respiratory tract are common sites of MFH in head and neck regions. 1 Only a few cases arising in the temporal fossa have been reported. 1' 2 MFH arising in the auricle is extremely rare. In the present report, we described the immunohistochemical and ultrastructural feature of the MFH of an auricle. CASE REPORT A 20-year-old male developed a dark red tumor mass on his right auricle in January 1990 (Fig. 1). This tumor was incompletely removed at another hospital. One month later the tumor recurred and enlarged to 2 X 3 em in size. Initial pathological report at the previous hospital was non-epithelial tumor. On March 12, he was admitted to our hospital. We performed a total resection of the tumor and reconstructed the defective region by a rotation skin flap. The surgical margin Received for publication February 21, 1992 209
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Fig. I.
The tumor was localized at the right auricle.
of the resected tumor was negative. The tumor was diagnosed as MFH. Postoperatively three courses of systemic chemotherapy were administered including dacarbazine, vincristin, cyclophosphamide, and pirarubicin. To the present there has been no evidence of distant metastasis or local recurrence. Histopathologic examination Tissues obtained by auricular tumor excision were fixed in 10% formalin and embedded in paraffin. The sections were stained with hematoxylin and eosin (H-E). In addition, immunohistochemical studies were performed using the avidin-biotin-peroxidase complex (ABC) techniques. The following antibodies were employed: cytokeratin (KL-1 ), vimentin, desmin, muscle specific actin, S-100 protein, factor VIII related antigen (FVIIIRA), a 1-antitrypsin (a1AT), and a 1-antichymotrypsin (a1ACT). Tissues for electronmicroscopy were fixed in 2.5% glutaraldehyde and postfixed in 1.0% osmium tetroxide, dehydrated in graded ethanols, and embedded in Epok 812. Ultrathin sections were stained with uranyl acetate and lead citrate, and were examined with an H-7000 electronmicroscope. 1.
Lightmicroscopic findings The tumor consisted of round or spindle cells with granulated nucleoli and abundant eosinophilic cytoplasm. They showed a storiform-pleomorphic pattern. Many small capillaries were found in the cellular spindle bundles. There was considerable mitotic activity of the tumor cells (Fig. 2a). The tumor cells were immunoreactive for vim en tin (Fig. 2b) and
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Fig. 2. Light microscopic findings. a: The tumor consisted of numerous spindle cells showing a storiform-pleomorphic pattern. (hematoxylin and eosin staining) X 40. b: The tumor cells were positively stained with vimentin. X 160. c: a 1-Antitrypsin was positive in most tumor cells. >< 80.
a 1-antitrypsin (Fig. 2c). a 1-Antichymotrypsin was also pos1ttve in some tumor cells. All other antibodies, including cytokeratin, desmin, muscle specific actin, S100 protein, and factor VIII proved to be negative results. 2.
Electron microscopic findings The tumor cells were oval or spindle in shape and they had round to ovoid nuclei. Mitotic figures were often observed (Fig. 3a). The tumor cells could be classified into three types: cells containing numerous filaments arranged in bundles, cells containing abundant lysosomes (Fig. 3b ), and an intermediate type which has both numerous intermediate filaments and lysosomes. Desmosomes between the tumor cells were not found.
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Fig. 3. Electronmicroscopic findings. a: Spindle-shaped cells contained round or ovoid nuclei. Note a mitotic figure (arrow). X 3,000. b: The spindle cells contained numerous intermediate filaments in bundles in their cytoplasm. Another type of cells contained many lysosomes and several numbers of mitochondria. X 7,500.
DISCUSSION
MFH is the most common soft-tissue tumor in late adult life. Three to 10% of all MFHs occur in the head and neck region. 3 Weiss and Enzinger reported that the occurrence of MFH in late adult (from 41 to 70 years old) is 74% of all cases. On the other hand, patients from 11 to 20 years old accounted for only 3%. Male patients were predominant (male 64%, female 36%). The sinonasal tract is the most common location in the head and neck. On the other hand, auricular tumor of MFH is extremely rare.
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213
MFH is one of the most pleomorphic soft-tissue tumors. Histologically the tumor is characterized by both fibroblast-like spindle cells arranged in bundles and histiocyte-like round cells. Generally, MFHs are divided into five subtypes: storiform-pleomorphic followed by the myxoid, inflammatory, giant cell, and angiomatoid variants. The present case was considered to be a combined storiformpleomorphic and angiomatoid type. The angiomatoid type is common in the extremities of young patients. At the light microscopic level, MFH may be confused with rhabdomyosarcoma, leiomyosarcoma, malignant shwannoma, and malignant hemangiopericytoma. Our case was also difficult to differentiate from these non-epithelial tumors. Immunological studies have shown that the tumor cells were positively stained with several antibodies such as vimentin, a 1-antitrypsin, and a 1-antichymotrypsin. On the other hand, desmin, muscle specific actin, and S-100 protein were negative. These results showed that the tumor was not of muscle or neural origin. Electronmicroscopically, spindle-shaped cells contained abundant intermediate filaments and many lysosomes. Most of the cytoplasmic constituents in the cell were replaced by a feltwork of lOnm filaments. Morphologically, these filaments were different from actin filaments, and lacked dense bodies. These filaments correspond to the presence of immunoreactive vimentin. These findings are consistent with previous reports on the ultrastructural features of MFH. 4 •5 The treatment of MFH is not standardized but usually consists of wide local excision. Radiation therapy results have been difficult to assess because of limited data. Radio- and chemotherapy has been reserved for patients with a positive surgical margin of the resected tumor or proven metastasis. 3•6 According to Weiss and Enzinger the 2 year survival rate is 60% in postoperated patient from all areas of the body, the local recurrence rate is 26% and the metastasis rate 42%. There is a high incidence of metastases in lung, lymph node, liver, and bone. In the head and neck area, the local recurrence rate is 20 to 42% and systemic metastases occur in 25 to 35% of cases. 1' 7' 8 In our case there was no local recurrence or distant metastasis for about 3 years. We are grateful to Miss Naoko Abo for her technical assistance.
REFERENCES I.
2. 3. 4.
Blitzer A, Lawson W, Zak FG, et al: Clinical-pathological determinants in prognosis of fibrous histiocytomas of head and neck. Laryngoscope 91:2053-2069, 1981. Daou RA, Attia EL, Viloria JB: Malignant fibrous histiocytomas of the head and neck. J Otolaryngol 12:383-388, 1983. Weiss SW, Enzinger FM: Malignant fibrous histiocytoma. An analysis of 200 cases. Cancer 41: 2250-2266, 1978. ChadD, Colvin RB, Digirolami U, et al: Spindle cell neoplasms. Dikersin GR (ed): Diagnostic Electron Microscopy. pp 158-260, Igaku-shoin, New York/Tokyo, 1944.
M. MORITA eta!
214
5.
6.
7. 8.
Henderson DW, Papadimitriou JM, Coleman M: Fibrohistiocytic Tumors, Ultrastructural Appearances of Tumors. pp 221-230, Churchill Livingstone, Edinburgh/London/Melbourne/New York, 1986. Kearney MM, Soule EH, Ivins JC: Malignant fibrous histiocytoma. A retrospective study of 167 cases. Cancer 45:167-178, 1980. Barns, L: Tumor and tumorlike lesion of the soft tissue. Barns L (ed): Surgical Pathology of the Head and Neck. pp 725-880, Marcel Dekker Inc., New York, 1985. Barns L, Kanbour A: Malignant fibrous histiocytoma of the head and neck. Arch Otolaryngol Head Neck Surg 114:1149-1156, 1988.
Request reprints to:
Dr. M. Morita, Department of Otolaryngology, Tokyo Women's Medical College, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 161, Japan
MALIGNANT FIBROUS HISTIOCYTOMA OF THE AURICLE: AN IMMUNOHISTO CHEMICAL AND ELECTRONMIC ROSCOPIC STUDY Megumi MoRITA, M.D., Toshio YosHIHARA, M.D., Nanami NARITA, M.D., Tetsuo ISHII, M.D., and Motohiko AlBA, M.D.* Department of Otolaryngology and *Pathology, Tokyo Women's Medical College, Tokyo, Japan
Malignant fibrous histiocytoma (MFH) is a soft-tissue tumor of late adult life. This tumor is uncommon in the head and neck region. A case of MFH arising in the auricle is presented with immunohistochemical and electronmicroscopic findings. Histologically the tumor was characterized by numerous spindle cells with abundant eosinophilic cytoplasm and many small capillaries. They were positively stained with vimentin, a 1-antitrypsin, and a 1-antichymotrypsin. Electronmicroscopically, some tumor cells contained abundant lysosomes and others contained numerous filaments. Malignant fibrous histiocytoma (MFH) is a common soft-tissue neoplasm in late adult life. According to Blitzer et al, paranasal sinuses, mandible and temporal bone, salivary gland, and respiratory tract are common sites of MFH in head and neck regions. 1 Only a few cases arising in the temporal fossa have been reported. 1' 2 MFH arising in the auricle is extremely rare. In the present report, we described the immunohistochemical and ultrastructural feature of the MFH of an auricle. CASE REPORT A 20-year-old male developed a dark red tumor mass on his right auricle in January 1990 (Fig. 1). This tumor was incompletely removed at another hospital. One month later the tumor recurred and enlarged to 2 X 3 em in size. Initial pathological report at the previous hospital was non-epithelial tumor. On March 12, he was admitted to our hospital. We performed a total resection of the tumor and reconstructed the defective region by a rotation skin flap. The surgical margin Received for publication February 21, 1992 209
M. MORITA et al
210
Fig. I.
The tumor was localized at the right auricle.
of the resected tumor was negative. The tumor was diagnosed as MFH. Postoperatively three courses of systemic chemotherapy were administered including dacarbazine, vincristin, cyclophosphamide, and pirarubicin. To the present there has been no evidence of distant metastasis or local recurrence. Histopathologic examination Tissues obtained by auricular tumor excision were fixed in 10% formalin and embedded in paraffin. The sections were stained with hematoxylin and eosin (H-E). In addition, immunohistochemical studies were performed using the avidin-biotin-peroxidase complex (ABC) techniques. The following antibodies were employed: cytokeratin (KL-1 ), vimentin, desmin, muscle specific actin, S-100 protein, factor VIII related antigen (FVIIIRA), a 1-antitrypsin (a1AT), and a 1-antichymotrypsin (a1ACT). Tissues for electronmicroscopy were fixed in 2.5% glutaraldehyde and postfixed in 1.0% osmium tetroxide, dehydrated in graded ethanols, and embedded in Epok 812. Ultrathin sections were stained with uranyl acetate and lead citrate, and were examined with an H-7000 electronmicroscope. 1.
Lightmicroscopic findings The tumor consisted of round or spindle cells with granulated nucleoli and abundant eosinophilic cytoplasm. They showed a storiform-pleomorphic pattern. Many small capillaries were found in the cellular spindle bundles. There was considerable mitotic activity of the tumor cells (Fig. 2a). The tumor cells were immunoreactive for vim en tin (Fig. 2b) and
MFH OF THE AURICLE
211
Fig. 2. Light microscopic findings. a: The tumor consisted of numerous spindle cells showing a storiform-pleomorphic pattern. (hematoxylin and eosin staining) X 40. b: The tumor cells were positively stained with vimentin. X 160. c: a 1-Antitrypsin was positive in most tumor cells. >< 80.
a 1-antitrypsin (Fig. 2c). a 1-Antichymotrypsin was also pos1ttve in some tumor cells. All other antibodies, including cytokeratin, desmin, muscle specific actin, S100 protein, and factor VIII proved to be negative results. 2.
Electron microscopic findings The tumor cells were oval or spindle in shape and they had round to ovoid nuclei. Mitotic figures were often observed (Fig. 3a). The tumor cells could be classified into three types: cells containing numerous filaments arranged in bundles, cells containing abundant lysosomes (Fig. 3b ), and an intermediate type which has both numerous intermediate filaments and lysosomes. Desmosomes between the tumor cells were not found.
212
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Fig. 3. Electronmicroscopic findings. a: Spindle-shaped cells contained round or ovoid nuclei. Note a mitotic figure (arrow). X 3,000. b: The spindle cells contained numerous intermediate filaments in bundles in their cytoplasm. Another type of cells contained many lysosomes and several numbers of mitochondria. X 7,500.
DISCUSSION
MFH is the most common soft-tissue tumor in late adult life. Three to 10% of all MFHs occur in the head and neck region. 3 Weiss and Enzinger reported that the occurrence of MFH in late adult (from 41 to 70 years old) is 74% of all cases. On the other hand, patients from 11 to 20 years old accounted for only 3%. Male patients were predominant (male 64%, female 36%). The sinonasal tract is the most common location in the head and neck. On the other hand, auricular tumor of MFH is extremely rare.
MFH OF THE AURICLE
213
MFH is one of the most pleomorphic soft-tissue tumors. Histologically the tumor is characterized by both fibroblast-like spindle cells arranged in bundles and histiocyte-like round cells. Generally, MFHs are divided into five subtypes: storiform-pleomorphic followed by the myxoid, inflammatory, giant cell, and angiomatoid variants. The present case was considered to be a combined storiformpleomorphic and angiomatoid type. The angiomatoid type is common in the extremities of young patients. At the light microscopic level, MFH may be confused with rhabdomyosarcoma, leiomyosarcoma, malignant shwannoma, and malignant hemangiopericytoma. Our case was also difficult to differentiate from these non-epithelial tumors. Immunological studies have shown that the tumor cells were positively stained with several antibodies such as vimentin, a 1-antitrypsin, and a 1-antichymotrypsin. On the other hand, desmin, muscle specific actin, and S-100 protein were negative. These results showed that the tumor was not of muscle or neural origin. Electronmicroscopically, spindle-shaped cells contained abundant intermediate filaments and many lysosomes. Most of the cytoplasmic constituents in the cell were replaced by a feltwork of lOnm filaments. Morphologically, these filaments were different from actin filaments, and lacked dense bodies. These filaments correspond to the presence of immunoreactive vimentin. These findings are consistent with previous reports on the ultrastructural features of MFH. 4 •5 The treatment of MFH is not standardized but usually consists of wide local excision. Radiation therapy results have been difficult to assess because of limited data. Radio- and chemotherapy has been reserved for patients with a positive surgical margin of the resected tumor or proven metastasis. 3•6 According to Weiss and Enzinger the 2 year survival rate is 60% in postoperated patient from all areas of the body, the local recurrence rate is 26% and the metastasis rate 42%. There is a high incidence of metastases in lung, lymph node, liver, and bone. In the head and neck area, the local recurrence rate is 20 to 42% and systemic metastases occur in 25 to 35% of cases. 1' 7' 8 In our case there was no local recurrence or distant metastasis for about 3 years. We are grateful to Miss Naoko Abo for her technical assistance.
REFERENCES I.
2. 3. 4.
Blitzer A, Lawson W, Zak FG, et al: Clinical-pathological determinants in prognosis of fibrous histiocytomas of head and neck. Laryngoscope 91:2053-2069, 1981. Daou RA, Attia EL, Viloria JB: Malignant fibrous histiocytomas of the head and neck. J Otolaryngol 12:383-388, 1983. Weiss SW, Enzinger FM: Malignant fibrous histiocytoma. An analysis of 200 cases. Cancer 41: 2250-2266, 1978. ChadD, Colvin RB, Digirolami U, et al: Spindle cell neoplasms. Dikersin GR (ed): Diagnostic Electron Microscopy. pp 158-260, Igaku-shoin, New York/Tokyo, 1944.
M. MORITA eta!
214
5.
6.
7. 8.
Henderson DW, Papadimitriou JM, Coleman M: Fibrohistiocytic Tumors, Ultrastructural Appearances of Tumors. pp 221-230, Churchill Livingstone, Edinburgh/London/Melbourne/New York, 1986. Kearney MM, Soule EH, Ivins JC: Malignant fibrous histiocytoma. A retrospective study of 167 cases. Cancer 45:167-178, 1980. Barns, L: Tumor and tumorlike lesion of the soft tissue. Barns L (ed): Surgical Pathology of the Head and Neck. pp 725-880, Marcel Dekker Inc., New York, 1985. Barns L, Kanbour A: Malignant fibrous histiocytoma of the head and neck. Arch Otolaryngol Head Neck Surg 114:1149-1156, 1988.
Request reprints to:
Dr. M. Morita, Department of Otolaryngology, Tokyo Women's Medical College, 8-1 Kawada-cho, Shinjuku-ku, Tokyo 161, Japan
