We describe a case of malignant melanoma, which appeared on the plaque of multiple congenital melanocytic nevuses (CMN) . A 33-year-old woman had a huge plaque of multiple CMN on her back at birth. The plaque was composed of numerous, brown to black, up to 2cm in size, papules or macules intermingling with normal skin. One of the black nodules had been gradually enlarged with occasional bleeding. The lesion was clinically and histologically diagnosed as malignant melanoma. Wide excision was performed in association with chemotherapy. After operation no reccur-ence has been noted.
The studies on cutaneous malignant lymphoma have made great progress during the last twenty years, especially the last ten years. In this paper, we reviewed the historical overview on the concept of cutaneous malignant lymphoma, especially (1) cutaneous T-cell lymphoma (CTCL) and cutaneous B-cell lymphoma (CBCL), (2) on the pathogenesis of CTCL and CBCL, (3) on the histopathological classification of non-Hdgkin's lymphoma, (4) on the clinical staging classification of primary CTCL and CBCL, (5) on the relationship between CTCL and adult T-cell leukemia/lymphoma (ATL), (6) on the molecular biology for diagnosis of CTCL and CBCL, (7) on the mechanism of the onset of primary CTCL and CBCL, (8) on the treatment and management of primary CTCL and CBCL and (9) on the future of the research for primary CTCL and CBCL.
A 82-year-old male patient with Merkel cell carcinoma (MCC) associated with Bowen's disease was reported. The patient developed a clearly demarcated tumor 14×17×3mm which has erythematous plaque 22×30mm at the flexor surface of the right thigh. Histologically, the epidermis showed Bowenoid changes. There were tumor nests consisting of round and oval cells in the dermis. Atypical nuclei and mitoses were markedly seen, with a trabecular pattern. Immunohistochemistry revealed positivity to NSE. Electron microscopically, dense core granules and intermediate filaments were observed. Based on these findings, the diagnosis was made.
We reported a case of epidermodysplasia verruciformis (EV) in which Merkel cell carcinoma developed. When he was 51 years old, he had received post-operative radiotherapy for squamous cell carcinoma on his occipital scalp. The patient was a 63-year-old Japanese man with a six-month history of eroded lesion on his temporal scalp. Physical examination revealed 20×20mm well-circumscribed, eroded lesion of the left temporal scalp. Multiple erythematous or brown scaling macules and plaques (1 to 3 cm) were scattered over his trunk. There was no superficial lymphadenopathy. Histopathologically, the specimen from the temporal lesion demonstrated proliferation of small, round and basophilic atypical cells from superficial to lower dermis. The overlying epidermis was necrotic. In the remaining epidermis around the tumor, atypical cells with hyperchromatic nuclei were observed. Immunohistochemically, the basophilic cells proliferating in the dermis expressed low-molecular-weight cytokeratin (CAM5.2), epithelial membrane antigen, neuron-specific enolase and chromoglanin A, but not leukocyte-common antigen, S-100 protein, vimentin or involucrin. Atypical cells in the neighbouring epidermis were positive for CAM5.2. Electron microscopically, the tumor cells in the dermis contained dense core granules in the cytoplasms. Although several types of skin tumor often developed on sun-exposed area of EV patients, development of Merkel cell carcinomas has been rarely reported. Preceding X-ray therapy might be related to the development of Merkel cell carcinoma in the present case.
DTIC was first introduced to Japan in 1977, and DAV combination chemotherapy using DTIC, ACNU and VCR became widely used to treat malignant melanoma. Used especially as a postoperative adjuvant chemotherapy, improvement in survival rates has been reported in multicenter research. Meanwhile, clinical trials commencing in 1978 of natural IFN-β produced from human fibroblasts demonstrated an efficacy rate of 50% for cutaneous metastasis of malignant melanoma, and Feron® was subsequently approved, in 1985. It has also been shown that Feron has an antineo-plastic effect: after subcutaneous administration at the site of melanoma in malignant melanoma patients. Feron levels taper gradually, but still remain in the local region 24hours later, while the drug is transported to regional lymph nodes in high concentrations. A concomitant effect for DAV+IFN-β was investigated in basic experiments, in which additive suppression of tumor proliferation was observed. With a view to further improving prognosis, therefore, we developed a protocol for Feron plus DAV combination therapy, in which DAV as postoperative adjuvant therapy would be provided in combination with the local administration of Feron, commencing a trial in centers across Japan in 1988. By 1995, 957 cases at 67 centers nationwide had been registered, the results tabulated and prognoses examined. Excellent results were obtained for 5-year suvival rate, classified by Stage, as follows: 98.0% for Stage I, 85.4% for Stage II, 65.8% for Stage III, and 35.0% for Stage IV. These results suggest that prognosis of malignant melanoma is better improved by DAV Feron therapy than by DAV therapy alone, and that combination therapy using Feron and DAV is therefore preferable.
In 1992, NIH Consensus Deveropment Conference, U.S.A, proposed that “early melanoma” is malignant melanoma in situ and invasive melanoma less than lmm in Breslow's tumor thickness. Patients with the early melanoma are cured by excision of the lesion with narrow margin. Thus, correct diagnosis and appropriate treatment of the neoplasm in this early stage are very important to improve the prognosis. In this paper, we described the clinical and histopathological characteristics of the early melanoma. Clinical guidelines for the early detection and appropriate treatment of the early lesion were also described.
Fundamental principles and surgical procedures how to treat genital skin cancers are discussed and clinical examples are presented. What should be taken into consideration are the age, general conditon, expected prognosis and quality of life of the patients. As for the disease it self, the biological behaviour -if it is lethal or not-, progression -staging-, and the anatomical characteristics of genital region should be kept on mind at the same time. Surgical procedure consists basically of wide resection and closure of the surgical defect with skin graft, local skin flap or musculucutaneous flap.
This article reviews the surgical management for cutaneous malignant neoplasms affecting acral portions, particulary malignant melanoma and squamous cell carcinoma of hand and foot. In most cases, surgical excision is the treatment of choice for primary lesions of cutaneous malignant melanoma and squamous cell carcinoma. In addition to an adequate excision, functional and cosmetic reconstruction are essential in the treatment of the neoplasm of hand and foot. Based on several randomized sutudies with long-term follow-up, decreased size of the resection margin for cutaneous melanoma has been recommended. The narrow surgical margin not only decreases functional loss but also makes treatment more simple. Correct diagnosis and accurate staging of the disease are important to decide size of the margin, and if necessary, excisional or incisional biopsy should be done even in malignant melanoma. Although there are many ways of reconstruction of defect after excision of malig-nant neoplasm of hand and foot, our first choice is simple way. Artificial dermis is recently available and useful to close skin defect exposing bone and/or tendon. Split thickness or full thickness skin graft is the most standard technique to close skin defect. Free flaps, such as wrap-aroud flap and pollicization, are good ways to reconstruct thum after its amputaion. Optimal surgical management with adequate margin associated with appropriate reconstruction is essential for the better quality of life of the patients.
A 29-year old man noticed a small bluish-brown spot on his right knee about 3 months ago. In the last 2 weeks it has suddenly enlarged, darkened and taken irregular shape. It was 3mm in diameter, black, having elevated macule with slight irregularity at the margin. Histopathological findings showed the lesion to be asymmetrical with unsharp lateral margins and mild inflammatory infiltrates in the upper dermis. Epidermis wasslightly acanthotic and there were tumor cells with atypical nuclei nested and scattered not only in the mid and lower epidermis but also up to the granular layer with casting off of melanocytes and melanin. The nests were of irregular sizes and shapes, not equidistant and tended to coalesce with a few mitoses. Therefore, we strongly suspected a malignant melanoma in situ in spite of it's very small size, although there was disagreement among the commentators.
A-16-year old girl developed a red nodule on her left thigh. The lesion had already been shaven by a surgeon and been diagnosed histopathologically an amelanotic melanoma when she first visited our hospital. She says the nodule was about 5mm in diameter, dome shaped, red color and rough surface. Histopathplpgically, main lesion is almost symmetrical and excists in inverted triangle shape. The tumor cells are arranged mostly in fairly well circumscribed nests and the nests exist in the epidermal-dermal junction through the lower dermis. There also are nests in middle epidermis, but it is not remarkable. The tumor cells are almost epitheloid and have large nuclei. Maturation of cells are recognized with increasing depth. Mitosis is rare. Cellular DNA content is 2.82C whitch is higher than that of Spitz nevus reported before and tetraploid cells are more than usual. Although analysis of cellular DNA content sugested that it was malignant, histopathology showed Spits nevus.
A 53-year-old man had noticed a pigmented lesion on his left palm from infancy. The pigmented lesion began to increase in size 10 years previously. The physical examination revealed a 17×12mm dark brown, irregularly shaped tumor. Histologically, there was a symmetrical growth of tumor cells from the epidermis into the lower dermis. The tumor cell showed slight atypia in the epidermis and papillary dermis, but cells in the lower dermis showed no cellular atypism. The patient was treated by the single excision and skin graft without elective lymphnode dissection. He died 7 years later due to the widespread metastases affecting left axillar lymphnode, colon and brain. We considered as a malignant melanoma showing histological similarity to nevocellular nevus.
A survival analysis of 58 patients with malignant melanoma was performed using the Kaplan-Meier life table method. To take quality of life (QOL) into consideration, we adjusted the survival time by the following equation : quality adjusted life year (QALY)=(survival time)-α × (hospitalization period). When α=0, 0.5, 1, the mean survival time (months) in stage I, II, III, IV was as follows: 60, 60, 40, 19 in α=0; 59, 59, 38, 17 in α=0.5; 59, 59, 35, 15 in α=1 respectively. In stage III, the patients were hospitalized in 12% of overall survival time. In stage IV, the hospitalization period formed 22% of survival time. In pT3, 4N0M0, the mean survival of the patients who received postoperative adjuvant chemotherapy was 60, 59, 58 months in α=0, 0.5, 1, respectively, and the mean survival of the patients who did not receive postoperative adjuvant chemotherapy was 59, 57, 56 months in α=0, 0.5, 1, respectively. The survival rate of the former was 100%, and the latter was 67% in α=0. Since the prognosis of the patients who did not receive adjuvant chemotherapy was poor, hospitalization required in the end stage results in diminished QOL assessed by our methods.
A 74-year-old female presented in January 1995 with indurated plaques and ulcerson her left thigh. These lesions had first appeared as nodules at the age of about 60 years and had slowly spread and ulcerated. The patient had never received anytreatment for the skin lesions because of her antipathy to doctors. Physical examination revealed numerous indurated erythematous plaques withsuperficial ulcers and nodules on the left thigh and edematous swelling of the entireleft leg. Several indurated erythematous plaques and nodules were also seen on theleft lower leg, perineum and right inguinal resion. A chest X-ray showed pulmonarymetastatic lesions and atelectasis of the left lung field. CT scan disclosed swelling ofthe celiac lymph nodes but no tumor originated from internal organs. Serum CA-125 level was elevated in all the course of the disease. We supposed that the neoplasm onthe patient's left thigh originated from the skin. She died of multiple organ failure in March 1995. Histologically, the many tumor cell nests were seen. in the dermis. Some of themshowed restiform-like pattern. Vacuoles were also seen in the tumor cell. Electronmicroscopical examination revealed tonofilaments in tumor cells, cell membraneswith complex interdigitating microvilli-like processes, desmosomes and intracellulartubules. These findings suggest that the neoplastic cells differenciated to eccrinesweat gland, and especially resemble eccrine porocarcinoma.
A case of giant keratoacanthoma on the nose of a 58-year-old woman was reported. The tumor reached a size of 8cm within 4 months. Proliferation of the tumor ceased after systemic administration of Peplomycin. Gradually the tumor flattened and took place with a scar later 10 months of onset.
We report two patients with Cole-Engman syndrome (dyskeratosis congenita) . Case 1: A 48-year-old Japanese man had noticed reticulated skin hyperpigmentation on his whole body from 11-year-old. He came to our dental hospital with acomplain of a tumor in the oral cavity. It was diagnosed as leukoplakia and squamous cell carcinoma arising from leukoplakia of the mucous menbranes on the left mandibular gingiva. He had also dental anomalies, nail dystrophy and lacrimal duct obstruction. He was diagnosed as Cole-Engman syndrome, comlete type. Case 2: A 49-year-old Japanese man visited our hospital with a complain of nummular eczema and prurigo-like eruption with itching. He also had reticulated skin hyperpigmentation, nail dyatrophy, hyperkeratosis of palms and soles, angular stomatitis and leukoplakia on right lateral side of tongue. We diagnosed him as Cole-Engman syndrome, comlete type. We followed up them very carefully.
Three patients with squamous cell carcinoma on the lower lip who were in mental hospital for a long time were reported. They had no history of taking a lot of sunshine because they had been in the hospital for 25 or 40 years. All of them were smokers but not so heavy, and had histories of the burn on the lower lip, because they usually smoke until the end of tabacco for the restriction to the number of it a day. They had cancer lesions at the site on which they always put the tabacco. For these reasons we suspect the inducer of these carcinoma is the burn scar not sunshine. And had much trouble in the treatment of these patients for their mental disease. We must plan operations without post-operation rest because of their disturbance after awakening of anethesia, and need much help of psychiatrists.
Three cases of giant cancer-in-scar, which histopathologically were diagnosed assquamous cell carcinoma with some complications and our therapeutic approach areintroduced. To our experience, two of the most important operative points for cancer-in-scarcould be extensive resection of tumor and related lymphatics, and reconstructiveprocedure using free skin graft in initial step. However in some compromised patients with high demand for quality of their life (cosmetically or functionally), more advanced operative method such as pedicled or free flaps could be performed initially. Even in such cases the size of tumor resection should not be compromised. In the course of treatment of cancer-in-scars collaboration of pathologists, dermatologists and experienced plastic surgeons in early stage could drastically present disastrous outcomes.
A 63-year-old, otherwise healthy woman consulted us because of ulcerated node on the parietal scalp. She got trauma on her scalp 48 years ago when she was a factory girl. Her parietal hairs were totally removed, leaving alopecia cicatrisata. She had hidden the lesion by a wig over 40 years, and her family did not notice the lesion. Anerosion developed on the alopecic plaque 10 years ago, and the lesion gradually elevated since 2 years. However, she never consulted us, despite she visited us because of eczematous lesion on the trunk. The physical examination revealed an well-demarcated, 11×10cm alopecic scar on the parietal and frontal scalp. A 4.4×7 ×1cm in size, granular-surfaced, cratered node with pus and bloody crust located on thecenter of the plaque. Radiologic examination revealed osteolytic change of the skull and thickening of the dura mater. Biopsy of the node was histologically diagnosed as SCC, and the node and alopecic plaque including affected skull were excised in Neurosurgery department. Totally 150mg of peplomycin sulfate was administrated for the treatment of remaining nodes on the dura mater, however the patient died of pneumonia 5 months after the operation. The present case suggests the importance of careful examination for other than chief complaint of the patient.
We reported a case of 24 year-old office girl patient with Bowen's disease occurring on her right fourth finger. Histological findings were mild atypical feature for the diagnosis of Bowen's disease. We found no evidence of human papilloma virus (HPV) in this case by methods of immunohistochemistry, southern blot hybridization, and electon microscopy. Of 6 patients with Bowen's disease developed on fingers who visited to our clinic in the period from July 1974 to March 1996, we recognized the HPV antigen in 2 cases by the PAP method. It is possible that the etiology of Bowen's disease on fingers is related to HPV infection.
We have encountered a case of multiple superficial basal cell epitheliomas of the dorsal thoracic region which appear to be histopathologically rare, and report the findings. A 66-year-old man visited our clinic with a presenting complaint of multiple pigmented spots in the dorsal thoracic region. He had a past history of arsenic ingestion, but no history of radiation. The skin eruptions were distinctly demarcated pigmented spots of sizes varying 1×1cm to 10×10cm, scattered in the region from the upper to the low back region, and two similar spots were also found in the anterior region of the chest. Biopsy was diagnostic of superficial basal cell epitheliomas, with all the spots made up of basophilic cells which were continuous with the epidermis, forming honeycomb-like foci of various sizes and proliferating bud-like form in the cutis, with the cells around the cell foci being of palisade arrangement.
We report a case of basal cell carcinoma (BCC) in the area of the genital Paget's disease. A 85-year-old woman noticed erythema on her vulvar region 3 years before, and the erythema gradually increased in size and followed erosion and ulcus on the erythema. Histologically, erythema revealed Paget's disease and the ulcus was the solid type of BCC. In the bordered area of Paget's disease and BCC, Paget cells infiltrated into the mass of BCC in the dermis, which were demonstrated with an immunopathological method using anti-CEA antibody. We speculated that the genital Paget's disease and BCC developed isolatedly in the different places nearly, after that, the BCC was surrounded by Paget's disese.
The human CD34 surface antigen is expressed on hematopoietic stem / progenitor cells. In the skin, vascular endothelial cells and mesenchymal cells including dermal dendritic cells and perifollicular cells are stained with the anti-CD34 monoclonal antibody. Interestingly, other than those mesenchymal cells in the dermis, the outer-root sheath enithelia of the lower hair follicle, which corresponded to the site of the stem cells of the hair follicle, are also positively immunostained. We examined the CD34 immunohistochemical localization in the various stage of the sebaceous nevus. In the sebaceous nevus with development of secondary tumors, ectopic CD34+ epithelial cells within the epidermis or at the base of infundibula-like structure were present with scant CD34+ mesenchymal microenvironment.
We reported a 75-year-old male patient, who had a large tumor mass, about 5×7×4cm, on the left lower eyelid. Histopatholgically, this was sebaceous carcinoma (SC) . Physical examination could not revealed regional lymph nodes swelling. He had advanced hepatocellular carcinoma (HCC) and liver cirrhosis. Wide excision and exenteration and Thiersch's skin graft was performed. He died of HCC. Autopsy was performed. There was no evidence of metastasis of SC to the regional lymph nodes and distant sites. Sebaceous carcinoma is a rare malignant tumor. It is subdivided into periocular and extraocular type. In periocular type, its size is usually at most thumb finger tip size, larger one is very rare. Our case may be the largest one.
A 77-year-old woman had noticed a red papule on her head about 2 years before. The eruption gradually increased in size. So she visited our clinic on June 19th, 1995. Clinically, it consisted of the firm, nodular, reddish lesion, the size of which was 25×20mm in diameter. Total resection was carried out 1cm apart from the margin of the lesion. Histologically, tumor cells showed a trabecular pattern or bands with a rather uniform appearance, which extended from the dermis into subcutis. The tumor cells had oval, vesicular nuclei and scanty cytoplasm. Numerous mitotic figures were also present. Ultrastructurally, there were observed paranuclear aggregation of intermediate filaments and dense core neurosecretory granules which were located in the cytolasmic processes or near the Golgi apparatus. Immunohistochemically, cytokeratin 8, 18, 19, 20 and epithelial membrane antigen were positively stained within tumor cells. Tumor cells showed partially positive labeling against chromogranin A, neurofilament and neuron specific elorase. S-100 and HMB-45 were negative. From the findings above, this case was diagnosed as Merkel cell carcinoma. It was suggested that perinuclear bandles were related to the partial positive staining of neurofilament.
A 66-year-old male with Merkel cell carcinoma is reported. A red nodule was noticed on his right temporal region about a year before visiting our clinic. The nodule was excised by a practicing doctor and was histopathologically diagnosed as basal cell carcinoma. When he visited to our clinic, the metastatic tumors were seen on his scalp and trunk, and were found in the mediastinal, axillary and abdominal lymph nodes, and in the brain by computed tomography. We histopathologically examined the tumor from the primary lesion, and the metastatic tumor on his back. Light microscopy of the primary lesion revealed that nests of tumor cells localized in the upper and middle dermis. Tumor cells had round nuclei and poor cytoplasm. Mitosis was seen in many tumor cells. Immunocytochemically, tumor cells were positively stained with anti-neuron-specific enolase antibody and anti-cytokeratin20 antibody. Electron microscopically, the intermediate filaments were observed around the nuclei. Desmosome-like dense materials were seen in the intercellular spaces of tumor cells. No dense core granules with a halo were found. Some tumor cells appeared apoptotic. The tumors responded to treatment with cisplatin/etoposide.
A 78-year-old man with malignant hemangioendothelioma on the scalp was successfully treated with a surgical operation and intra-arterial injection of recombinant interleukin-2 (rIL-2). A tumor grew on his forehead after he stabbed with his comb 5 months ago. He visited our hospital with the skin lesion, measuring 6×3cm, after local excision of a mass at another clinic. Histologically, the tumor showed irregular vascular channels and bleeding, and the tumor cells showed positive factor VIII-related antigen. Extensive resection and reconstruction using the lattisimus dorsi myocutaneous flap was performed in May, 1995. Intra-arterial injection of rIL-2 through a catheter at the right superficial temporal artery started just before the operation. At his 14-month postoperative evaluation, he had no evidence of any recurrences and metastases. It is suggested that extensive resection and intra-arterial injection of rIL-2 before and after the resection are effective to an early malignant hemangioendothelioma.
We reported a 69-year-old male case of multiple myeloma. His chief complain was a skin tumor, and it was localized on his forearm. After general examination of whole the body, atypical myeloma cells were found in the bone marrow. We selected radiation therapy, because of his age, past disease history, and so on. The tumor expressed good response, and reduced clinically. But, in a short time, it formed metastatic lesions in both thoracis vetebra and forearm arround the first lesion. Multiple myeloma is a disease, which is usually found and mainly complained chronic anemia, morbid bone fracture, and lumbago. This case that we reported was found as extramedullary plasmacytoma on the skin, and in that point, it was comparatively rare clinically.
The authers report a case of malignant skin tumor in the right leg followed by old burn scar. This case was reconstructed by distally based sural flap combined with venous switching method and favorable result was obtained. A 81-year-old male patient received a contact burn at his right leg 30 years ago by hot water tank for leg warming. Erosive lesion was observed from Aug., 1995 at the scarred area. He was referred to us by his family doctor. A biopsy was performed before the operation and the result was basosquamous cell carcinoma. Radical tumor excision was carried out and the skin defect was reconstructed with a distally based sural flap whose small saphenous vein was anastomsed to the great saphenous vein at the proximal side of the defect for obtaining the normal venous cirulation. Six months had elapsed and no recurrence was observed.
We report herein a case of mammary Paget's disease complicated by multiple Bowen's disease in a patient who had received successive chemotherapy during follow-up for surgical resection of breast cancer preserving the nipple 8 years earlier. Subject: The patient was a 52-year-old housewife. In 1988, she underwent left breast cancer resectomy preserving the nipple at another hospital. In 1991, erosion on the left nipple was noted. In 1993, erythema on the inner portion of the right breast was noticed. In 1995, since erosion of the left nipple expanded to the areola and was accompanied by gradual extension of erythema on the inner portion of the right breast as well as dispersion of erythema on other parts of the trunk, the patient consulted a local dermatologist. Because Bowen's disease was diagnosed by biopsy of the left areola with erythema due to suspected Paget's disease, the patient was reffered to our hospital. At our department, biopsy of the left areola as well as the erythematous area of the trunk was performed again. On histopathological examination, the patient was diagnosed with mammary Paget's disease and multiple Bowen's disease. The findings of the biopsy performed at the other hospital also indicated Paget's disease.
A case of extramammary Paget's disease with pedunculated tumor formation is reported. A 65-year-old woman had noticed a pruritic eruption on her left vulva 4 or 5 years ago. At her first visit, a pedunculated tumor about 3cm in diameter was found within an erythematous plaque intermingled with depigmented spots on her left vulva. Inguinal lymph nodes on both sides were palpable. Serum levels of CA19-9 as well as CEA were elevated. Histopathological examination revealed that the epidermis was permeated with Paget cells in groups, and the dermis was also infiltrated with a mass of Paget cells. Immunohistochemically, CEA and CA19-9 were expressed on tumor cells. After radical excision, serum levels of CEA and CA19-9 decreased rapidly. But 5 months after the operation, the tumor apparently recurred or metastasized because serum levels of CEA and CA19-9 became elevated again.
We describe two cases of metastatic skin cancer originating from pneumonic adenocarcinoma. In both cases, skin metastases were discovered prior to the detection of the primary tumor. Case 1: An 83-year-old man developed a reddish nodule on his back. A pathological study revealed solid proliferation of adenocarcinoma cells in the subcutaneous fat tissue. Examination of the entire body revealed pneumonic adenocarcinoma. Metastases to the liver, adrenal gland and mediastinal lymph nodes were detected, and the patient died 2 months after the first visit. Case 2: A 64-year-old man developed two reddish nodules on his scalp. A pathological study revealed solid proliferation of adenocarcinoma cells in the dermis and subcutaneus fat tissue. A whole-body examination revealed pneumonic adenocarcinoma. Metastases to the bones (acromion, rib, ilium) were detected, and the patient died 5 months after the first visit.
A case of extraskeletal osteosarcoma, which has been suspected to be malignant fibrous histiocytoma, is reported. The patient was a 62-year old man. He noticed an asymptomatic nodule on his forehead 1 month before the first visit to our clinic. There were 5 firm nodules on his forehead. Wide excision of the lesions and skin grafting was performed. Biopsy specimens revealed dermal and subcutaneous infiltrating tumors without discernible borders, which were composed of spindle-like, histiocyte-like and osteoblast-like cells with pronounced nuclear atypia. In some areas, immature osteoid structures were also found. Immunohistochemical examination revealed that tumor cells react positively with actin, myosin, α1-antitrypsin, S-100 protein, and lysozyme. On the other hand, vimentin, CD34 and factor VIII were negative. Multiple matastatic lesions in the scalp were revealed with a CT scan and MRI. The right-sided hemiplegia was appeared and gradually increased. Therefore, the patient was treated only with anti-edema therapy. He died 3 months after the first visit to our clinic. Compared with osteosarcoma of bone, extraskeletal osteosarcoma is rare. There are 68 cases of this tumor recorded in the Japanese literature.