Microcystic adnexal carcinoma (MAC) was first described in 1982 by Goldstein. Considered a rare malignant skin appendageal tumor, it is often underdiagnosed due to its clinical and histopathological resemblance to other cutaneous neoplasms. MAC is locally aggressive with infiltration of perineural spaces, subcutaneous tissue, skeletal muscles, and so on. Aggressive treatment including wide local excision, Mohs micrographic surgery, or radiation therapy is necessary owing to the high recurrence rate. Herein, we report a case of a 47-year-old Korean woman with a skin-colored hardened plaque on the scalp with a clinical diagnosis of cicatricial alopecia and histopathological diagnosis of MAC. After treatment by Mohs micrographic surgery, the patient is being followed up regularly without any sign of recurrence. This case demonstrates an uncommon topography of MAC on the scalp with secondary cicatricial alopecia and highlights the need for awareness of the potential for MAC in the diagnosis of alopecia with a slow-growing tumor.
Alopecia* ; Diagnosis ; Female ; Humans ; Middle Aged ; Mohs Surgery ; Muscle, Skeletal ; Pathology ; Recurrence ; Scalp ; Skin ; Skin Neoplasms ; Subcutaneous Tissue

Child, Preschool ; Diagnosis, Differential ; Female ; Humans ; Inflammation ; pathology ; Neoplasms, Muscle Tissue ; diagnosis ; pathology ; surgery ; Prognosis

OBJECTIVETo study the clinicopathologic features, immunophenotype, molecular genetics and differential diagnosis of solid variant of angiomatoid fibrous histocytoma.

METHODSThe clinicopathologic features of 3 cases of solid variant of angiomatoid fibrous histocytoma were analyzed and the literature was reviewed.

RESULTSThere were a total of 2 males and 1 female. The age of patients ranged from 9 to 12 years. The patients presented with a painless mass located in left forearm, left knee or back. The lesions were treated by complete surgical resection. On gross examination, the tumors varied from 1.6 cm to 4.5 cm in greatest dimension. They were well-circumscribed and had pale yellow to grayish-red solid cut surface. Histologically, the tumor was composed of histocytoid cells arranged in sheet-like pattern. A fibrous pseudocapsule surrounded by lymphocytes and plasma cells was identified. Immunohistochemical study showed that the tumor cells in all cases were positive for vimentin and CD68. They were negative for S100 protein, cytokeratin, CD34, CD31, smooth muscle actin, CD35, CD21 and CD30. Two cases also expressed CD99 and one of them was positive for desmin and epithelial membrane antigen. Fluorescence in-situ hybridization was positive for EWSR1 gene.

CONCLUSIONSSolid type represents a variant of angiomatoid fibrous histocytoma and is considered as tumor of borderline malignant potential. Definitive diagnosis requires thorough histologic examination and clinical correlation. Immunohistochemistry and EWSR1 gene study are helpful in further delineation and differential diagnosis. Complete resection or wide local excision with post-operative follow up is the main modality of treatment.

Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Back ; Calmodulin-Binding Proteins ; genetics ; Child ; Dendritic Cell Sarcoma, Follicular ; metabolism ; pathology ; Diagnosis, Differential ; Female ; Forearm ; Histiocytoma, Malignant Fibrous ; genetics ; metabolism ; pathology ; surgery ; Humans ; Knee ; Male ; Neoplasms, Muscle Tissue ; pathology ; Neurilemmoma ; metabolism ; pathology ; RNA-Binding Protein EWS ; RNA-Binding Proteins ; genetics ; Soft Tissue Neoplasms ; genetics ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

Adolescent ; Adult ; Aged ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Child ; Cyclophosphamide ; therapeutic use ; Diagnosis, Differential ; Doxorubicin ; therapeutic use ; Female ; Follow-Up Studies ; Humans ; Ki-1 Antigen ; metabolism ; Leukocyte Common Antigens ; metabolism ; Lymphoma, Extranodal NK-T-Cell ; metabolism ; pathology ; Lymphoma, Large B-Cell, Diffuse ; metabolism ; pathology ; Lymphoma, Large-Cell, Anaplastic ; drug therapy ; metabolism ; pathology ; Male ; Melanoma ; pathology ; Middle Aged ; Mucin-1 ; metabolism ; Neoplasms, Muscle Tissue ; metabolism ; pathology ; Prednisone ; therapeutic use ; Receptor Protein-Tyrosine Kinases ; metabolism ; Retrospective Studies ; Vincristine ; therapeutic use ; Young Adult

Actins ; metabolism ; Adult ; Diagnosis, Differential ; Female ; Fibroma ; pathology ; Fibrosarcoma ; pathology ; Follow-Up Studies ; Humans ; Hysterectomy ; Inflammation ; Keratins ; metabolism ; Leiomyoma ; pathology ; Neoplasm Recurrence, Local ; Neoplasms, Muscle Tissue ; drug therapy ; metabolism ; pathology ; surgery ; Receptor Protein-Tyrosine Kinases ; metabolism ; Uterine Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Young Adult

OBJECTIVETo study the clinicopathologic features and differential diagnosis of extranodal Rosai-Dorfman disease (RDD) of the upper respiratory tract.

METHODSThe clinical, pathologic and immunohistochemical features of 10 cases of RDD were evaluated.

RESULTSAmong the 10 cases studied, there were 3 males and 7 females. The age of patients ranged from 20 to 61 years old (mean 38 years). The lesion arose in the nasal cavity (7 cases), nasopharynx (2 cases) or hard palate to trachea (1 case). Most of the patients presented with nasal obstruction, rhinorrhagia or tumor mass in the nasal/nasopharyngeal regions. CT scan often showed the presence of soft tissue lesion without bone destruction. Histologically, extranodal RDD was characterized by light-staining bands alternating with dark-staining bands. The light-staining bands were formed by aggregates of large round or polygonal histiocytes with emperipoiesis. The dark-staining bands were formed by abundant lymphoplasmacytic infiltrates. Immunohistochemical study showed that the histiocytes strongly expressed S-100 protein and partially expressed CD68. Six patients had no recurrence after surgical resection.

CONCLUSIONSExtranodal RDD of the upper respiratory tract is a rare disorder of histiocytic proliferation, which usually involves the nasal cavity and paranasal sinuses. RDD can easily mimic rhinoscleroma, mainly due to the overlapping morphologic appearance. Immunohistochemical study is helpful in the differential diagnosis.

Adult ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Histiocytic Sarcoma ; metabolism ; pathology ; Histiocytosis, Langerhans-Cell ; metabolism ; pathology ; Histiocytosis, Sinus ; diagnostic imaging ; metabolism ; pathology ; surgery ; Humans ; Male ; Middle Aged ; Nasal Cavity ; pathology ; Nasopharyngeal Diseases ; diagnostic imaging ; metabolism ; pathology ; surgery ; Neoplasms, Muscle Tissue ; metabolism ; pathology ; Nose Diseases ; diagnostic imaging ; metabolism ; pathology ; surgery ; S100 Proteins ; metabolism ; Tomography, X-Ray Computed ; Young Adult

Aged, 80 and over ; Antigens, CD20 ; metabolism ; CD79 Antigens ; metabolism ; Diagnosis, Differential ; Humans ; Interferon Regulatory Factors ; metabolism ; Ki-67 Antigen ; metabolism ; Leukosialin ; metabolism ; Lymphoma, Large B-Cell, Diffuse ; metabolism ; pathology ; surgery ; Male ; Neoplasms, Muscle Tissue ; metabolism ; pathology ; Prostatic Neoplasms ; metabolism ; pathology ; surgery ; Prostatitis ; metabolism ; pathology ; Proto-Oncogene Proteins c-bcl-2 ; metabolism ; Receptor Protein-Tyrosine Kinases ; metabolism

Actins ; metabolism ; Aged ; Breast Neoplasms ; metabolism ; pathology ; surgery ; Carcinoma ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Fasciitis ; metabolism ; pathology ; Female ; Fibroma ; metabolism ; pathology ; surgery ; Humans ; Keratin-5 ; metabolism ; Mastectomy, Modified Radical ; Neoplasms, Muscle Tissue ; metabolism ; pathology

Abdominal Cavity ; Adult ; Anemia, Aplastic ; therapy ; Antigens, CD34 ; metabolism ; Carcinoma ; metabolism ; pathology ; Dendritic Cell Sarcoma, Follicular ; metabolism ; pathology ; Diagnosis, Differential ; Hematopoietic Stem Cell Transplantation ; adverse effects ; Humans ; Ki-67 Antigen ; metabolism ; Lymph Nodes ; pathology ; Male ; Neoplasms, Muscle Tissue ; metabolism ; pathology ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Sarcoma, Kaposi ; etiology ; metabolism ; pathology ; Viral Proteins ; metabolism

Myolipoma is a rare benign tumor, composed of irregularly admixed adipose tissue and smooth muscle fibers. Few literature studies have described the radiologic appearance of myolipoma, especially in the soft tissue. No MRI findings in subcutaneous myolipoma of an extremity have been reported. Here, we report on the case of a 34-year-old woman with myolipoma in the subcutaneous tissue of the ankle and describe MRI features of the lesion.
Adult ; *Ankle ; Female ; Humans ; Lipoma/*diagnosis/pathology ; *Magnetic Resonance Imaging ; Muscle, Smooth/pathology ; Soft Tissue Neoplasms/*diagnosis/pathology ; Subcutaneous Fat/pathology