Rapid Growth of Dermatofibrosarcoma Protuberans Associated with Bilateral Adrenalectomy for Cushing's Syndrome

We describe a 50-year-old Japanese patient with dermatofibrosarcoma protuberans (DFSP) rapidly growing after bilateral adrenalectomy for Cushing's syndrome that reduced the serum level of cortisol from 17.1 to 0.8 mg/dl. It is known that glucocorticoids decrease the transcriptions of the COL1A1 gene and the PDGFB gene, which is under the direct control of the COL1A1 gene in most DFSP. Therefore, the hypersecretion of glucocorticoids in Cushing's syndrome might suppress the development of DFSP. To the best of our knowledge, this is the first case of rapid growth of DFSP that may be associated with bilateral adrenalectomy for Cushing's syndrome

Therapy-Resistant, Spontaneously Remitting Generalized Neutrophilic Eccrine Hidradenitis in a Healthy Patient Decreases the Expression of Dermcidin in Affected Eccrine Glands

We describe a healthy 69-year-old Japanese man with generalized neutrophilic eccrine hidradenitis (NEH). He visited our outpatient clinic with a 15-year history of disseminated pruritic papules on his trunk and extremities; the eruptions, however, were limited to the summer months. Histological findings reveal a dense accumulation of neutrophils around the sweat glands with degenerated secretary coils. Interestingly, immunohistochemical staining showed that the expression of dermcidin on the secretory portion of eccrine glands was significantly decreased in the affected lesion. To our knowledge, this is the first report in English of generalized NEH in a healthy adult that shows the downregulation of the expression of dermcidin in affected eccrine glands

Cutaneous Lymphadenoma: A Case Report and Immunohistochemical Study

Cutaneous lymphadenoma is a rare neoplasm, and characterized by a florid mononuclear cell infiltration that appears to be an integral component of the tumor. Several reports suggested that these infiltrated lymphocytes mainly consist of T cells and Langerhans cells, but no detailed phenotypical analysis of these cells has been demonstrated yet. In this report, we describe a 70-year-old Japanese patient with cutaneous lymphadenoma. In the present case, we investigated the immunohistochemical profiles of tumor-infiltrating cells in cutaneous lymphadenoma, especially focusing on the profiles of T cell subsets. In addition, there are abundant mucin deposits within the tumor stroma, and tumor lobules are composed of BerEP4-positive cells. To the best of our knowledge, this is the first report that employed immunohistochemical staining of cutaneous lymphadenoma to verify the types of cells composing such tumors with special focus on the profiles of the T cell subsets

Basal Cell Carcinoma with Spontaneous Regression: A Case Report and Immunohistochemical Study

Spontaneous regression of basal cell carcinoma (BCC) is rare, and characterized by various tumor-infiltrating lymphocytes (TILs) in the tumor. A previous report suggested that these infiltrated lymphocytes consist of type 1 helper T cells, but no detailed phenotypical analysis of other TILs has been demonstrated yet. In this report, we describe an 84-year-old Japanese patient with spontaneous regression of BCC. In the present case, we investigated the immunohistochemical profiles of TILs, not only focusing on the cytotoxic T cells, but the profiles of immunosuppressive cells, such as regulatory T cells and tumor-associated macrophages. Our present study sheds light on the immunological mechanisms of tumor rejection in human BCC

Myeloid Sarcoma in a One-Month-Old Infant

Myeloid sarcoma, which sometimes accompanies recurrent systemic leukemia, is a rare manifestation of congenital leukemia. We describe the case of a 1-month-old Japanese girl with myeloid sarcoma, who did not fulfill the criteria of systemic leukemia. The physical examination revealed a bruise-like plaque on the right side of her chest. Histopathologically, there were CD4+ CD56– CD68+ atypical large lymphocytes densely infiltrating the upper dermis and the subcutaneous tissue. Biopsy specimens from bone marrow contained only 3.4% of blast cells, showing an abnormality of the mixed-lineage leukemia gene. The skin lesions disappeared 10 days after chemotherapy, and there was no recurrence for one and a half years

Phaeohyphomycosis Caused by Phaeoacremonium rubrigenum in an Immunosuppressive Patient: A Case Report and Review of the Literature

Phaeohyphomycosis (PHM) is a rare, deep fungal infection of the skin and subcutaneous tissues caused by dematiaceous fungi. In this report, we describe a case of PHM caused by Phaeoacremonium rubrigenum, which is generally known to infect woody plants. We detected the gray-blackish villi by biopsy culture material, and slide culture revealed the conidia arising from slightly tapering phialides. Furthermore, we differentiated these fungi as P. rubrigenum by Basic Local Alignment Search Tool (BLAST) algorithm. We performed surgical debridement of disseminated nodules and administered oral itraconazole for a duration of 4 weeks. One year after stopping itraconazole, there was no sign of relapsing subcutaneous nodules. To our knowledge, this is the third case report of PHM developing from skin infection by P. rubrigenum in human

Tumor-Associated Macrophages: Therapeutic Targets for Skin Cancer

Tumor-associated macrophages (TAMs) and regulatory T cells (Tregs) are significant components of the microenvironment of solid tumors in the majority of cancers. TAMs sequentially develop from monocytes into functional macrophages. In each differentiation stage, TAMs obtain various immunosuppressive functions to maintain the tumor microenvironment (e.g., expression of immune checkpoint molecules, production of Treg-related chemokines and cytokines, production of arginase I). Although the main population of TAMs is immunosuppressive M2 macrophages, TAMs can be modulated into M1-type macrophages in each differential stage, leading to the suppression of tumor growth. Because the administration of certain drugs or stromal factors can stimulate TAMs to produce specific chemokines, leading to the recruitment of various tumor-infiltrating lymphocytes, TAMs can serve as targets for cancer immunotherapy. In this review, we discuss the differentiation, activation, and immunosuppressive function of TAMs, as well as their benefits in cancer immunotherapy

Malignant Fibrous Histiocytoma with In-Transit Metastasis

Malignant fibrous histiocytoma (MFH) is the most common fibroblastic tumor, but its cutaneous metastasis, especially in-transit metastasis, is extremely rare. We describe the case of a 30-year-old Japanese man with a recurrent MFH on the scalp accompanied by in-transit metastasis, which had been treated as a benign skin tumor 8 years before. The main bulk of the recurrent tumor was located in the dermis, but the metastatic tumor was mainly located in the subcutis. Generally, atypical fibroxanthoma, also known as cutaneous MFH, is rarely metastasized and presents a benign clinical course. Since there is a great difference between the prognosis of MFH and atypical fibroxanthoma, precise diagnosis of the primary tumor is essential

CD30-Positive Angioinvasive Lymphomatoid Papulosis (Type E) Developing from Parapsoriasis en Plaque

Angioinvasive lymphomatoid papulosis (LyP) type E is a rare variant characterized by angiocentric and angiodestructive features with CD30+ CD8+ lymphocyte infiltration. In rare cases, LyP type E is concomitant with mycosis fungoides, but there is no English report that describe LyP type E developing from parapsoriasis en plaque. In this report, we described a case of angioinvasive LyP (type E) developing from parapsoriasis en plaque, in which we employed immunohistochemical staining for the investigation of its pathomechanisms

Possible Delayed Foreign Body Reactions against Titanium Clips and Coating Materials after Unruptured Cerebral Aneurysm Surgery

Delayed foreign body reactions to either or both clipping and coating materials have been reported in several small series; however, studies in the titanium clip era are scarce. This study aims to survey the contemporary status of such reactions to titanium clips and coating materials. Among patients who received a total of 2327 unruptured cerebral aneurysmal surgeries, 12 developed delayed intraparenchymal reactions during outpatient magnetic resonance imaging (MRI) follow-up. A retrospective investigation was conducted. The patients\u27 average age was 58.6 (45-73) years, and 11 were women. The aneurysms were located in the middle cerebral artery (n = 7), internal carotid artery (n = 4), or anterior communicating artery (AComA, n = 1). In 10 patients, additional coating with tiny cotton fragments was applied to the residual neck after clipping with titanium clips; however, only the clipping with titanium clips was performed in the remaining two. The median time from surgery to diagnosis was 4.5 (0.3-60) months. Seven (58.3%) patients were asymptomatic, and three developed neurological deficits. MRI findings were characterized by a solid- or rim-enhancing lobulated mass adjacent to the clip with surrounding parenchymal edema. In 11 patients, the lesions reduced in size or disappeared; however, in one patient, an AComA aneurysm was exacerbated, necessitating its removal along with optic nerve decompression. In conclusion, cotton material is a strongly suspected cause of delayed foreign body reactions, and although extremely rare, titanium clips alone may also induce such a reaction. The prognosis is relatively good with steroid therapy; however, caution is required when the aneurysm is close to the optic nerve, as in AComA aneurysms