We reported a case of cutaneous T cell lymphoma (CTCL) associated with multiple seborrheic keratosis and vitiligo. A reddish nodule appeared on the chest of a 63 years old Japanese male patient, and the noduler lesions continued to appear on his trunk, head and neck. Examinations including skin biopsy established the diagnosis of CTCL of non-mycosis fungoides type. Two years later, multiple seborrheic keratosis suddenly appeared on his trunk accompanied by severe itching. In addition, vitiligo-like depigmentation developed surrounding a lymphoma lesion in his left axilla. We concluded this case was unique CTCL showing Laser-Trélat sign and associated with vitiligo-like depigmentation.
A 48-year-old Japanese male patient visited us with a 3-year-history of scattered nodules on the right leg and foot. A nodule 2×1cm in size with a cratral scab and ulcer was observed on the swollen right leg and several small nodules ranged from 1 to 3mm in diameter were scattered on the foot. Biopsy specimen obtained from the nodule of the leg showed the histological features of epithelioid sarcoma. In addition to the positive staining for keratin and vimentin, tumor cells were also positive for CD34 and Factor VIII. Patient received an amputation at the right thigh followed by inguinal lymph node dissection. A metastatic lymph node at the distal portion of the saphenous vein was histologically detected. Patient was alive more than 2 years after the surgical procedure. During this period a small metastatic nodule appeared on the occipital region which was excised surgically.
Recently developed regimens for the treatment of patients with advanced malignant melanoma were reviewed. Combination chemotherapy of CDDP/BCNU/DTIC/tamoxifen (Dartmouth regimen) and sequential (concurrent) chemoimmunotherapy composed of CDDP based chemotherapy associated with IL-2 and IFN α have been reported to increase overall response rates and CR rates. However, they are still in the clinical trial setting. Temozolomide is an analogue of DTIC that has the advantage of being orally absorbed and crossing into the central nervous system (CNS). Fotemustine, a new nitrosourea, is also active in CNS metastasis. Results of the following two clinical trials of our study group were also described: DAC-Tam therapy using DTIC, ACNU, CDDP and tamoxifen, and sequential biochemotherapy using CDDP, IL-2 and IFN β.
The difficulty in diagnosing sweat gland tumors lies in the different classifications of the tumors used in textbooks. As for malignant tumors, the naming is more complicated for there are malignant tumors as the counterparts of the benign tumors as well as the undifferentiated carcinomas mimicking the benign tumors, and often a name used by an author means a different tumor in another textbook. So when diagnosing the sweat gland tumors, it is necessary to state the classificatin and to name the text you used to avoid the misunderstanding.
This report may be the first trial to introduce palliative care of the patient with advanced skin malignancy to dermatologists in Japan. It might be rare to take care of the patients in terminal stage. However, even dermatologist cannot keep away from the problem when treating malignancy. Recently it is also rare to treat the patient with extended skin involvement on malignant tumor. In many cases primary lesion was removed by surgery and local recurrence is rare. Then we take care of the patient with distant metastasis without skin involvement in terminal stage. There is no generalization of palliative care. Principle theory is to keep good QOL removing anypains of the patient.
A primary goal of palliative care is to improve the quality of patient's life. Pain management is one of the most important aspects of care for terminal cancer patient. Two patients who suffered from severe cancer pain were reported. Morphine relieved their pain. The pain relief helped the patients to live a comfort enf-of-life.
A 59-year-old Japanese male had a solitary tumor on the thigh for 3 years. Histopathology of the tumor showed nodular and reticulated patterns of proliferation of basaloid cells and cords of squamoid cells throughout the entire dermis. In the center of the tumor, dilatation of a hair follicle and numerous infundibular cysts were noted. Some hints of differentiation to dermal papilla were noted. These histopathologic features were considered to be compatible with the diagnosis of infundibulo-cystic basal cell carcinoma. Unusual site and rather deep invasion of the tumor suggest that infundibulo-cystic basal cell carcinoma may be one type of true malignant skin tumor but not a benign tumor.
Two cases with squamous cell carcinoma (SCC) occured on the hand induced by the occupational exposure to ionizing radiation were reported. The patients were a 57-years-old osteopathic physician and a 66-years-old dentist presented with ulcerated skin tumors on fingers. Both patients had primary tumors extending to the bone and later developed lung metastasis. Although the incidence of radiationindueced SCC are decreasing in Japan, the patients who recieved occupational radiation exposure should be followed-up carefully.
The case of a 71-year-old male with keratoacanthome pluri-kystique pseudo-sébacé (Degos) is reported. The patient noticed a plaque on his chin two months ago. The plaque gradually enlarged and on the first visit to our hospital, a 45×40mm sized, irregularly bordered and shaped reddish plaque that was composed of yellowish multilobular cysts was observed. On light microscopy, many cysts lined by stratified squamous epithelium with a granular layer and filled with layers of keratin were observed. Cysts in the dermis extended into the muscle layer. This is a most peculiar case consistent with keratoacanthome pluri-kystique pseudo-sébacé (Degos), which we regard as a type of squamous cell carcinoma.
Malignant fibrous histiocytoma (MFH) is a malignant tumor of the soft tissue which is mainly found extremities and breech. We showed five cases of MFH we had experienced, and we reported one case with MFH of the face, who is 92-year-old female. She visited us with the complaint of the nodule on her left cheek in 1996. Skin biopsy was performed and the pathological report showed MFH. According to the general examinations, there was no metastasis. Wide excision was performed with maximal 5cm margin and zygomatic bone was partially resected. The tissue defect was reconstructed with a cervico-facial flap, glabellar flap and skin grafting. If the first operation is inappropriate, MFH tends to recur and metastasize. So enough resection is necessary. A cervico-facial flap does no require microsurgery and gives us cosmetically excellent results. We conclude that a cervico-facial flap is suitable reconstructive method in this case.
A 75-year-old female was seen in June 1997 with a 4-year history of tumor on heel of her right-foot. The ulcerated part of the tumor was biopsied in another hospital and diagnosed as malignant melanoma. When we saw her, operation scar and brownblack macule were recognized. Three months after the wide resection of the primary lesion by us, she had multiple, in-transit metastases on her right skin. We performed a subtotal integumentectomy, initially from primary operation site to knee level, then subsequebtly from the knee to inguinal site with inguinal nodes dissection. However, three months after the subtotal integumentectomy, she developed multiple vinceral metastases and she deceased in April 1998. Although the subtotal integumentectomy is an option of treatment of in-transit metastasis of malignant melanoma, we think the validity should be evaluated by increasing cases treated with this procedure.
The authers found 4 subungual melanomas (SUM) among 17 cases of cutaneous melanoma (24%) at their clinic last 5 years. All cases occurred on the hands. Case 1: A 24-year-old female had SUM in situ on her left little finger. Total nail excision was done. The defect was covered with artificial dermis and delayed skin graft. Case 2: A 35-year-old female had SUM, Stage II (pT3apNOMO) on her left right finger. She was underwent amputation at I-P joint and wide local skin excision, followed by reversed forearm flap transfer. Then axilla dissection and DAV-interferon β therapy were performed. Case 3: A 89-year-old male had SUM, Stage II (pT3aNOMO) on his right middle finger. Ray amputation and interferon β therapy were performed. Case 4: A 68-year-old female had SUM, Stage III (pT4apNOMO) on her right index finger. Ray amputation, axilla dissection and DAV-interferon β therapy were performed.
Spontaneous regression of a primary lesion was observed in two patients with metastatic malignant melanoma. A 58-year-old man had had a greyish-brown nail and a greyish-brown pigmentation on the tip of the right index finger. The nail subsequently dropped off. The pigmentation gradually decreased in size and color followed by a swelling of right axillary lymph nodes. Another patient, 87-year-old man, had had a brown, grey, bluish, mottled pigmentation on his left sole for 30 years. He developed a tumor in his left inguinal region three years before which gradually enlarged. In both cases, the pigmented lesions considered to be primary lesions were slightly greyish-brown, mottled pigmentation within the boundaries of primary lesions, in which skin markings were obliterated. Histological findings of primary lesions in these patients were compatible with those reported in spontaneously regressing malignant melanoma. The patients were treated with repeated chemotherapy consisting of CDDP, DITC, ACNU, and Tamoxifen. They are still alive one and three years, respectively, after the recognition of the metastatic lesions.
The coincidence of malignant melanoma and acute myelocytic leukemia is very rare and our case is considered with the first case. A case of 52-year-old man who combined malignant melanoma in acute myelocytic leukemia simultaneously was reported. Melanotic freckle of irregular shape and black color of 3×4cm was locating with the right sole. The right groin lymph node didn't feel. Excision of tumor with 3cm of margin was performed on plantar fascia during remission induction treatment. Split thickness skin graft was done to skin defect region. Groin lymph node dissection wasn't done. Histopathologic findings was acral superficial spreading melanoma and stage I (pT1NOMO). Although metastasis wasn't noticed during course, Acute myelocytic leukemia had a relapse, and the patient died for organ invasion.
A case of atypical fibroxanthoma is reported. A 79-year-old man had noticed a small hemorrhagic nodule on the parietal area of the scalp for about 6 months. It was a pale red, elastic and firm nodule 12×12mm in diameter and 3mm in height. When he first visited our hospital. Histopathologically, the tumor was mainly composed of fibroblast-like cells and pleomorphic histiocyte-like cells in the dermis. Mitotic figures and many foam cells were also observed. No obvious striform pattern was observed in the fibroblast-like cells. On immunohistochemical examination, the tumor stained positive for vimentin, and negative for cytekeratin, desmin and s-100 protein. There is no evidence of recurrence 15 months after excision.
A 47-year-old woman, suffering from von Recklinghausen's disease, visited us because the tumor grew rapidly on her right neck. Except for numerous pigmented spots and cutaneous neurofibromas, there was a 18×10cm tumor on the area from right lateral neck to the right supraclavicular fossa, in addition to an indurated tumor measuring approximately 5cm in diameter on the right breast. According to the operative findings of an emergency operation for the lesion on the right neck, it was suspected of malignant tumor. Histopathologic diagnosis from department of clinical pathology was malignant peripheral nerve sheath tumor complicated with von Recklinghausen's disease. However, since the lesion had intralesional small nests and scattered large clear cells, it was suspected of undifferentiated cancer. Immunohistochemistry for myoglobin, desmin, NSE, S-100, EMA etc. could not show definite result. On the other hand, a biopsy specimen from her right breast was histopathologically diagnosed as breast cancer. Based upon all the above-mentioned histopathologic findings of both lesions of the right neck and the right breast, the lesion of the right neck was finally diagnosed as metastatic lesion of the breast cancer.
We have experienced 15 cases of extramammary Paget's disease since 1986. Male to female ratio was 11: 4. The average of age was 72 year old. Thirteen cases were affected genital area, one case was axiller, and so-called triple Paget's disease was one case. Extensive excision was performed for 12 cases. Histologically, Paget's cells were observed in papillary dermis of all cases.
We report a case of malignant chondroid syringoma affecting the preauricular portion of the left ear of a 67-year-old man. There were submental lymph node metastasis and multiple pulmonary metastases two months later. The local recurrence occurred at the primary site about 12 months after the initial insufficient surgical procedure. We suspected clinically squamous cell carcinoma was suspected. However, the findings of the histopathological, immunohistochemical and ultrastructual examinations for reccurent tumor led us finally to make a diagnosis of malignant chondroid syringoma. Patient received electron beam radiation followed by administration of chemotherapeutic agents including CDDP, 5-FU and irinotecan and taxotere. We discussed the usefulness of such combination therapy, particulary the chemotherapeutic agents including taxotere.
Regarding the 35 patients with skin metastases from internal cancer that we examined in a recent 10-year period: 1) the number of metastases increased more than 3 times compared to an earlier 10-year period; 2) patient age at the development of metastases was nearly 10 years older than in the earlier 10-year period; 3) metastases from breast cancer in women have particularly increased in recent years; 4) the lung was the most frequent primary site (55%) for men, and the breast was the most frequent (62%) for women; 5) skin metastases from the stomach occurred in only 2 cases, and no cases of metastases from the uterus were noted.
A 76-year-old man with angiosarcoma (malignant hemangioendotherioma) on his right frontal scalp was successfully treated with intra-aortic infusion chemotherapy. Its prognosis is extremely poor, even though treated with surgical excision, radiation and chemotherapy, so intra-aortic infusion chemotherapy through a catheter at the right and left superficial temporal artery has been done with IL-2, THP-ADM, DTIC, IF and ADM for 36 days and the tumor size was getting extremely small. He visited our hospital, the nodule resion was measuring 6×5cm, the macula resion was 16×12cm, but after treatment, the nodule resion was disappeared and the macula resion was 8×6cm. It is suggested that intra-aortic infusion chemotherapy are effective to an angiosarcoma.
Between 1990 and 1997, 10 patients with angiosarcoma on the face and scalp were seen at Yokohama City University School of Medicine. The patients were six men and four women with an average age of 80 years. Initial features were solitary or ulcerative nodules or bruise-like lesion. Distant metastasis had developed in seven patients. All patients had treated with IL-2 immunotherapy; four patients with surgical exision, six patients with radiotherapy. Three patients are survival and one of them is disease free for about 2 years. All of the three patients was treated with intra-arterial injection of IL-2.
We report a case of Stewart-Treves syndrome. A 71-year-old Japanese woman visited us with a reddish nodule on the right upper arm. She had radical mastectomy with lymph node dissection for the right breast cancer at the age of 53. On physical examination, a solitary darkreddish nodule measured 3.0×2.5×0.8cm with subcutaneous infiltration was found on the right upper arm showing postmastectomy lymph edema. Histology of the nodule revealed numerous round cells with marked cellular atypia forming slit-like space. The diagnosis of Stewart-Treves syndrome was confirmed. The surgical excision then was performed. After the surgical resection, she was treated with electron beam irradiation and intravenous recombinant human IL-2 therpapy. The patient had no local recurrence or metastasis for 12 months after the excision.
Langerhans cell histiocytosis (LCH) is a rare disease of Langerhans cell proliferation, has a broad spectrum of clinical symptoms, course and prognosis. We report the case of a 41-year-old man with LCH who exhibited dark red nodules on the trunk. The patient also exhibited swelling of the cervical lymph nodes and bilateral lung and skull lesions in X-ray examination. As there were no signs of severe physical symptoms or organ failure, we prescribed a course of oral treatment only (PSL 30mg/day for 7 weeks) and prohibited smoking. After treatment, the skin lesions disappeared. In this report, we present a case of the typical LCH case and in addition, examined the statistical relationship between clinical cutaneous symptoms and prognosis of 66 adult LCH cases reported from 1993 to 1998 in Japan. In result, cutaneous lesions in adult LCH are relatively common, mostly present as papules or nodules. Not infrequently, there are petechiae or purpuric lesions, which are considered as poor signs, and a rapid enlargement of cutaneous lesions is also. Lahey score is almost correlate with prognosis of adult LCH. These factors may be useful way to determine a prognosis of adult LCH.
We present a 65 year-old man with acute monocytic leukemia who demonstrated cutaneous leukemic infiltration over most of his body. He was admitted because of chest pain, bleeding tendency, high grade fever. The red blood cell count was 182×104/μl, Hb 5.8g/dl, The Ht was 18.7%, The platelet count was 33, 000/μl, The white blood cell count was 76, 000/μl with a defferential count of 54% blasts. The blasts were negative for peroxidase, but positeve for butyrate and chloroacetate esterase. Bone marrow aspirtation revealed necrosis. Acute monocytic leukemia was diagnosed. 6 hours later he was died of respiratory insufficiency and DIC. Autopsy revealed extensive leukemia cell infiltration in the lung, liver, spleen, kidney, stomach, bone marrow, adrenal glands, skin, and lymphnodes of porta hepatis.
We report a case of cutaneous B cell lymphoma in a 29-year-old male. About one and half years before consultation, he noticed firm and red colored nodules on his left knee. Histopathological examination revealed a dense infiltrate of large lymphoid cells with mitotic figures in the dermis. In an immunohistochemical study, most of these cells were found to be positive for CD20. Southern blot analysis demonstrated rearranged bands in the immunoglobulin heavy-chain gene. On physical examination lymph node swelling was not noted. CT and Ga scintigraphy showed no evidence of metastasis. The patient was diagnosed as having malignant lymphoma of the diffuse, large cell type by REAL classification associated with cutaneous B cell lymphoma, stage 1A, by Burg's staging. Excision of the lesion with a 2cm margin was performed followed by local irradiation with a total dose of 30Gy.
We reported a patient with actinic keratosis and keratoachantoma. Both tumors were on her dorsum pedis. Since those area were notably close, to investigate the cause of diseases, immunohistochemical staining and TdT-mediated dUTP-biotin nick end labeling (TUNEL) method were performed on theses skin samples. P53 immunoreactivity was not observed in the keratoacanthoma, and positive staining was seen in the actinic keratosis. In contrast, apoptosis was not detected on the actinic keratosis and was well detected on the central and peripheral portion of keratoacanthoma. This suggests P53 immunoreactivity and detection of apoptosis are very beneficial methods for differentiation of diagnosis of keratoacanthoma and squamous cell carcinoma.
A case of a 37-year-old man with papillary eccrine adenoma is reported. The lesion presented clinically as a slowly growing nodule on the malleolus lateralis of the right foot. Histopathologically, the tumor was composed of multiple dilated tubular structures lined by two or more layers of epithelial cells. In some of the tubules, the inner cell layer formed intraluminal papillary projections. Papillary eccrine adenoma is a rare benign sweat gland neoplasm. There is no consensus on the cellular origin of this tumor. But in this study, we performed immunohistochemical staining study by several monoclonal antibodies against cytokeratin antigens. We reveal that the portion of the two layers of this tumor may defferentiate towards the dermal eccrine duct, whereas, the portion of the papillary projections may defferetiate towards the eccrine secretory portion.