Inflammatory myofibroblastic tumor （IMT） is a rare mesenchymal soft tissue tumor characterized by borderline or low-grade malignancy. It is rare childhood tumor with an average age of onset of 10 years old. It is even rarer in infants and toddlers, and the etiology and pathogenesis of this tumor are still unclear. The clinical presentation of IMT is non-specific and are related to the location of the tumor. When the tumor compresses adjacent organs, it can cause pain and functional impairment. According to the current literature, IMT is most commonly found in the digestive and respiratory systems, but also occasionally occur in the genitourinary system, head and neck, and limbs. At present, there have been no reports of nasopharyngeal IMT involving nasal cavity of infants and toddlers at home and abroad.This article reports a case of a massive inflammatory myofibroblastic tumor involving the nasal cavity and nasopharynx in an infant. Plasma-assisted minimally invasive surgery was performed through multiple surgical approaches and achieved satisfactory therapeutic results. This case report may provide valuable reference for the treatment of similar diseases.
Humans ; Infant ; Granuloma, Plasma Cell/pathology* ; Nasopharynx/pathology* ; Neoplasms, Muscle Tissue ; Soft Tissue Neoplasms

Objective: To investigate the clinicopathological features, diagnosis and differential diagnosis of breast myofibroblastoma. Methods: The clinicopathological data and prognostic information of 15 patients with breast myofibroblastoma diagnosed at the Department of Pathology of the First Affiliated Hospital of Zhengzhou University, Zhengzhou, China from 2014 to 2022 were collected. Their clinical characteristics, histological subtypes, immunophenotypes and molecular characteristics were analyzed. Results: There were 12 female and 3 male patients, ranging in age from 18 to 78 years, with a median and average age of 52 years. There were 6 cases in the left breast and 9 cases in the right breast, including 12 cases in outer upper quadrant, 2 cases in inner upper quadrant and 1 case in outer lower quadrant. Most of the cases showed a well-defined nodule grossly, including pushing growth under the microscope in 13 cases, being completely separated from the surrounding breast tissue in 1 case, and infiltrating growth in 1 case. Among them, 12 cases were classic subtype and composed of occasional spindle cells with varying intervals of collagen fiber bundles; eight cases had a small amount of fat; one case had focal cartilage differentiation; one case was epithelioid subtype, in which epithelioid tumor cells were scattered in single filing or small clusters; one case was schwannoma-like subtype, and the tumor cells were arranged in a significant palisade shape, resembling schwannoma, and one case was invasive leiomyoma-like subtype, in which the tumor cells had eosinophilic cytoplasm and were arranged in bundles, and infiltrating into the surrounding mammary lobules like leiomyoma. Immunohistochemical studies showed that the tumor cells expressed desmin (14/15) and CD34 (14/15), as well as ER (15/15) and PR (15/15). Three cases with histologic subtypes of epithelioid subtype, schwannoma-like subtype and infiltrating leiomyoma-like subtype showed RB1 negative immunohistochemistry. Then FISH was performed to detect RB1/13q14 gene deletion, and identified RB1 gene deletion in all three cases. Fifteen cases were followed up for 2-100 months, and no recurrence was noted. Conclusions: Myofibroblastoma is a rare benign mesenchymal tumor of the breast. In addition to the classic type, there are many histological variants, among which the epithelioid subtype is easily confused with invasive lobular carcinoma. The schwannoma-like subtype is similar to schwannoma, while the invasive subtype is easily misdiagnosed as fibromatosis-like or spindle cell metaplastic carcinoma. Therefore, it is important to recognize the various histological subtypes and clinicopathological features of the tumor for making correct pathological diagnosis and rational clinical treatment.
Female ; Humans ; Male ; Antigens, CD34 ; Biomarkers, Tumor/analysis* ; Leiomyoma/pathology* ; Neoplasms, Muscle Tissue/pathology* ; Neurilemmoma ; Adolescent ; Young Adult ; Adult ; Middle Aged ; Aged

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

OBJECTIVE: To investigate the clinical features, therapeutic methods and therapeutic efficacy of imflammatory myofibroblastic tumor(IMT)of the nasal cavity and paranasal sinuses. METHOD: Clinical data of 14 cases diagnosed as IMT by pathology were retrospectively analyzed. There were 8 males and 6 females,age ranging from 18 to 77 years. 12 cases of them were treated by surgery while 2 cases received postoperative radiotherapy. RESULT: All cases were operated. All the patients were followed up for a period of 1 to 7 years after operation and two cases were proved low grade IMT pathologically. Eight cases survived with no recurrence until the last follow-up and 6 cases relapsed, of which 4 cases died and 2 were alive with tumor. CONCLUSION IMT of the nasal cavity and paranasal sinuses is very rare. The diagonosis of IMT is based on pathology and immunohistochemistry. Proper diagnosis is essential to avoid mutilating and disfiguring surgical procedures. Radical excision is still the first choice of treatment for IMT of the nasal cavity and paranasal sinuses. Chemotherapy and radiotherapy may not be helpful to prevent recurrence after operation. Due to high recurrence rate, long-term follow up is necessary after operation.
Adolescent ; Adult ; Aged ; Female ; Humans ; Immunohistochemistry ; Male ; Middle Aged ; Nasal Cavity ; pathology ; Neoplasm Recurrence, Local ; Neoplasms, Muscle Tissue ; pathology ; radiotherapy ; surgery ; Paranasal Sinus Neoplasms ; pathology ; radiotherapy ; surgery ; Paranasal Sinuses ; pathology ; Retrospective Studies ; Young Adult

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

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

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

Hoarseness, associated with a pharyngeal abnormal sensation, intermittent cough, without the symptoms of bloody sputum, dyspnea, fever, fatigue, weight loss and other symptoms. No lymphadenectasis or other abnormal symptoms. Routine tests, just like blood and urine routine, liver and kidney function, blood coagulation routine, ECG, chest radiograph, no abnormal results.
Humans ; Laryngeal Neoplasms ; pathology ; Male ; Middle Aged ; Neoplasms, Muscle Tissue ; pathology