Objective: To evaluate the efficacy of endoscopic transnasal surgery for sinonasal and skull base adenoid cystic carcinoma (ACC), and to analyze the prognostic factors. Methods: Data of 82 patients (43 females and 39 males, at a median age of 49 years old) with sinonasal and skull base ACC who were admitted to XuanWu Hospital, Capital Medical University between June 2007 and June 2021 were analyzed retrospectively. The patients were staged according to American Joint Committee on Cancer (AJCC) 8th edition. The disease overall survival(OS) and disease-free survival(DFS) rates were calculated by Kaplan-Meier analysis. Cox regression model was used for multivariate prognostic analysis. Results: There were 4 patients with stage Ⅱ, 14 patients with stage Ⅲ, and 64 patients with stage Ⅳ. The treatment strategies included purely endoscopic surgery (n=42), endoscopic surgery plus radiotherapy (n=32) and endoscopic surgery plus radiochemotherapy (n=8). Followed up for 8 to 177 months, the 5-year OS and DFS rates was 63.0% and 51.6%, respectively. The 10-year OS and DFS rates was 51.2% and 31.8%, respectively. The multivariate Cox regression analysis showed that late T stage and internal carotid artery (ICA) involvement were the independent prognostic factors for survival in sinonasal and skull base ACC (all P<0.05). The OS of patients who received surgery or surgery plus radiotherapy was significantly higher than that of patients who received surgery plus radiochemotherapy (all P<0.05). Conclusions: Endoscopic transonasal surgery or combing with radiotherapy is an effective procedure for the treatment of sinonasal and skull base ACC. Late T stage and ICA involvement indicate poor prognosis.
Male ; Female ; Humans ; Middle Aged ; Carcinoma, Adenoid Cystic/surgery* ; Retrospective Studies ; Skull Base/pathology* ; Disease-Free Survival ; Prognosis

Adenoid cystic carcinoma (ACC) is a malignant neoplasm of glands commonly occurs in salivary glands. Primary cutaneous adenoid cystic carcinoma (PCACC) is a rare form of ACC that primarily presents on the skin. Herein, we represent a rare case of PCACC occurred in the umbilicus in a 66-year-old Korean male patient. The patient visited our center with erythematous indurated patch on the umbilicus diagnosed as ACC by incisional biopsy at another center. The diagnosis of PCACC was confirmed by additional histopathologic examination and imaging study. We proceeded Mohs micrographic surgery and reconstructed umbilicus with tacked purse string suture. Local recurrence and distant metastasis were not observed during 30-month follow-up. We report this rare case of PCACC on the umbilicus so that dermatologist can aware of the rare disease. Furthermore, we recommend MMS and tacked purse string suture as effective methods for treatment of PCACC and immediate umbilical reconstruction.
Adenoids ; Aged ; Biopsy ; Carcinoma, Adenoid Cystic ; Diagnosis ; Follow-Up Studies ; Humans ; Male ; Mohs Surgery ; Neoplasm Metastasis ; Rare Diseases ; Recurrence ; Salivary Glands ; Skin ; Sutures ; Umbilicus

OBJECTIVE: To analyze the diagnosis, therapy and prognosis of the rare cases of malignant tumors in external auditory canal. METHOD: Eight cases of rare malignant tumors in external auditory canal were collected except squamous cell carcinoma and adenoid cystic carcinoma. The clinical manifestation, staging, surgical method, secondary treatment and prognosis were analysed. RESULT: There were 5 cases of basal cell carcinoma and 2 cases of mucoepidermoid carcinoma and 1 case of myoepithelial carcinoma. Three cases of the 5 basal cell carcinoma in external auditory canal have been misdiagnosed for a long time. After admission, 4 of the 5 basal cell carcinoma were T1 stage and cured only by a complete resection of tumor. One case of T2 stage basal cell carcinoma was found recurrence 2 years later after the first excision of tumor, and was treated with radiotherapy. Tumor was controlled. Two cases of mucoepidermoid carcinoma had been misdiagnosed as a benign tumor and received a resection. One case was found metastasis to the parapharyngeal space and nasopharynx and was treated with concurrent chemotherapy. Tumor was also controlled after 5-years follow-up. Another case was found metastasis to parotid gland and received an expanding tumor resection. No recurrence was detected after a 1-year followed up. One case of T4 stage myoepithelial carcinoma in external auditory canal was a metastasis of parotid and received a partial temporal bone resection. No-recurrence was found 1 year later. CONCLUSION Because the tumors above have a very low incidence in external ear canal and the location of tumors are hidden, they are often misdiagnosed and delayed in treatment. Therefore, otologists should pay more attention to avoid the misdiagnosis. To the treatment, complete surgical resection of early-stage tumor is important and for late-stage tumor, a supplemented by chemoradiotherapy may be needed.
Carcinoma, Adenoid Cystic ; diagnosis ; surgery ; Carcinoma, Basal Cell ; diagnosis ; surgery ; Carcinoma, Squamous Cell ; diagnosis ; surgery ; Ear Canal ; pathology ; Ear Neoplasms ; diagnosis ; surgery ; Humans ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Parotid Gland ; pathology ; Prognosis ; Retrospective Studies ; Temporal Bone ; surgery

OBJECTIVETo explore the reconstruction approaches and indications in repairing of postoperative defect after resection of oral floor carcinoma.

METHODSTo review the clinical data of 106 patients with oral floor carcinoma treated by radical resection with simultaneous reconstruction in the Department of Head and Neck Surgery at our hospital from July 2003 to March 2013, and to evaluate the advantages and disadvantages of various repair methods.

RESULTSThe patients were followed up for 2 months to 10 years. Their 3-year survival rate was 63.6% (42/66) and 5-year survival rate was 57.7% (30/52). One case had sternocleidomastoid myocutaneous flap necrosis, and was successfully repaired with elective pectoralis major myocutaneous flap. Another one case showed partial strap muscle flap necrosis, and the wound was well healed after debridement and dressing changes. Three cases of free flap crisis because of poor blood supply were successfully cured by flap emergency exploration. The rest were well healed. The eating and language function of the patients could meet the general needs of life.

CONCLUSIONSAt the time of radical resection of oral floor carcinoma, an appropriate repair method should be selected according to many factors such as disease conditions, defect size, patients' physical constitution, etc. Generally, adjacent pedicle flap is not recommended. Prosthodontics membrane, free forearm flap, free anterolateral thigh flap, pectoralis major myocutaneous flap and free fibula flap can basically meet the need of repair of the postoperative defect after resection of oral floor carcinoma, therefore, are recommended.

Adult ; Aged ; Carcinoma, Adenoid Cystic ; pathology ; surgery ; Carcinoma, Squamous Cell ; pathology ; surgery ; Female ; Follow-Up Studies ; Humans ; Lymphatic Metastasis ; Male ; Middle Aged ; Mouth Floor ; Mouth Neoplasms ; pathology ; surgery ; Neck Dissection ; Reconstructive Surgical Procedures ; methods ; Surgical Flaps ; Survival Rate

OBJECTIVETo explore the clinical features, management approach and treatment outcomes for adenoid cystic carcinoma (ACC) of the breast.

METHODSThe clinicopathological data of 25 patients with breasts ACC treated in our hospital from years 1990 to 2012 were retrospectively reviewed and their prognosis was analyzed.

RESULTSThe median age of these 25 patients was 53 years (ranged from 31 to 81 years). With the exception of one male case, all patients were female including 17 cases of postmenopausal women. The most frequent presenting symptom is breast lumps, most (48.0%) were in the upper outer quadrant and areola area of the breast. Core needle biopsy was performed in five patients. The specimen finding were adenoids in three and invasive carcinoma in two cases. Axillary lymph node dissection was performed in 23 patients. Only two patients had histologically positive lymph nodes (3 of 14 and 2 of 20). Expression of ER and PR in 14 cases was detected by immunohistochemistry, showing one PR-positive and three ER-positive cases. The median follow-up of the 25 cases was 118 months (ranged from 12 to 244 months). Two patients died of lung metastases at 3 and 10 years after the surgery, respectively.

CONCLUSIONSDue to the complexity of the histology of ACC, adequate sampling of specimens is essential for accurate diagnosis. ACC of the breast is a rare disease with a relatively good prognosis. The low incidence of axillary lymph node metastasis suggests that axillary node dissection is not recommended as a routine procedure. Breast ACC are often with negative ER and PR expression, and the value of adjuvant therapy needs to be further investigated.

Adult ; Aged ; Aged, 80 and over ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Axilla ; Breast Neoplasms ; drug therapy ; metabolism ; pathology ; surgery ; Breast Neoplasms, Male ; drug therapy ; metabolism ; pathology ; secondary ; surgery ; Carcinoma, Adenoid Cystic ; drug therapy ; metabolism ; pathology ; surgery ; Chemotherapy, Adjuvant ; Cyclophosphamide ; therapeutic use ; Female ; Fluorouracil ; therapeutic use ; Follow-Up Studies ; Humans ; Lung Neoplasms ; secondary ; Lymph Node Excision ; Lymph Nodes ; pathology ; Lymphatic Metastasis ; Male ; Mastectomy ; methods ; Methotrexate ; therapeutic use ; Middle Aged ; Postmenopause ; Receptors, Estrogen ; metabolism ; Receptors, Progesterone ; metabolism ; Retrospective Studies

Carcinoma, Adenoid Cystic ; diagnosis ; surgery ; Endoscopy ; Humans ; Neoplasm Recurrence, Local ; diagnosis ; Nose ; surgery ; Nose Neoplasms ; diagnosis

Adenoma, Pleomorphic ; diagnosis ; diagnostic imaging ; surgery ; Adolescent ; Adult ; Aged ; Angiography, Digital Subtraction ; Carcinoma, Adenoid Cystic ; diagnosis ; diagnostic imaging ; surgery ; Chemotherapy, Adjuvant ; Child ; Female ; Follow-Up Studies ; Head and Neck Neoplasms ; diagnosis ; diagnostic imaging ; surgery ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Neurilemmoma ; diagnosis ; diagnostic imaging ; surgery ; Oral Surgical Procedures ; methods ; Postoperative Complications ; Radiotherapy, Adjuvant ; Tomography, X-Ray Computed ; Young Adult

Adult ; Breast Neoplasms ; metabolism ; pathology ; secondary ; Carcinoid Tumor ; metabolism ; pathology ; surgery ; Carcinoma, Adenoid Cystic ; pathology ; Carcinoma, Lobular ; metabolism ; pathology ; secondary ; Cervical Intraepithelial Neoplasia ; metabolism ; pathology ; surgery ; Chromogranin A ; metabolism ; Diagnosis, Differential ; Female ; Humans ; Hysterectomy ; Keratins ; metabolism ; Neoplasms, Multiple Primary ; metabolism ; pathology ; surgery ; Ovarian Neoplasms ; metabolism ; pathology ; Sex Cord-Gonadal Stromal Tumors ; metabolism ; pathology ; Synaptophysin ; metabolism ; Uterine Cervical Neoplasms ; metabolism ; pathology ; surgery

Nasopharyngeal adenoid cystic carcinoma (NACC) is a rare malignancy with high local invasiveness. To date, there is no consensus on the imaging characteristics of NACC. To address this, we retrospectively reviewed 10 cases of NACC and summarized the magnetic resonance imaging (MRI) features. MR images of 10 patients with histologically validated NACC were reviewed by two experienced radiologists. The location, shape, margin, signal intensity, lesion texture, contrast enhancement patterns, local invasion, and cervical lymphadenopathy of all tumors were evaluated. Clinical and pathologic records were also reviewed. No patients were positive for antibodies against Epstein-Barr virus (EBV). The imaging patterns of primary tumors were classified into two types as determined by location, shape, and margin. Of all patients, 7 had tumors with a type 1 imaging pattern and 3 had tumors with a type 2 imaging pattern. The 4 tubular NACCs were all homogeneous tumors, whereas 3 (60%) of 5 cribriform NACCs and the sole solid NACC were heterogeneous tumors with separations or central necrosis on MR images. Five patients had perineural infiltration and intracranial involvement, and only 2 had cervical lymphadenopathy. Based on these results, we conclude that NACC is a local, aggressive neoplasm that is often negative for EBV infection and associated with a low incidence of cervical lymphadenopathy. Furthermore, MRI features of NACC vary in locations and histological subtypes.
Adult ; Aged ; Carcinoma, Adenoid Cystic ; diagnosis ; pathology ; surgery ; Female ; Humans ; Lymphatic Metastasis ; Magnetic Resonance Imaging ; methods ; Male ; Middle Aged ; Nasopharyngeal Neoplasms ; diagnosis ; pathology ; surgery ; Neoplasm Invasiveness ; Neoplasm Staging ; Retrospective Studies

OBJECTIVETo investigate the clinicopathologic and immunohistochemical features as well as the differential diagnoses of the solid variant of mammary adenoid cystic carcinoma with basaloid features.

METHODSClinical and pathological data were collected in four cases of the solid variant of mammary adenoid cystic carcinoma with basaloid features, and microscopic pathological examination and immunohistochemistry EnVision method were performed. The relevant literature was also reviewed.

RESULTSThe four patients were female, with age ranged from 46 - 65 years old (average 56 years) and the maximum tumor diameter ranged from 1.5 to 2.5 cm. Microscopically, the tumors exhibited a predominantly solid architecture with a myxoid or hyalinized stroma. The tumor cells showed moderate to marked nuclear atypia, and a basaloid appearance with scanty cytoplasm and inconspicuous nucleoli, and ≥ 5 mitotic figures per 10 high power fields. Glandular space embedded within tumor islands could be noticed. These spaces were genuine glandular structures and the cells lining these true glandular lumens had more abundant and eosinophilic cytoplasm. Pseudoglandular spaces of cribriform pattern or variable shape were also occasionally seen, and these cysts contained homogenous eosinophilic material. Focal necrosis was found. All cases were negative for ER, PR and HER2. Immunohistochemical staining for CK5/6, CK7 and CK14 was positive in the genuine glandular structures. All cases were positive for CD10, but also positive with varying intensity from weak to strong for vimentin and CD117. Staining for Ki-67 in three patients showed 10% - 50% positive.

CONCLUSIONSThe solid variant of mammary adenoid cystic carcinoma with basaloid features is a histologically distinctive and also a rare subset of the mammary adenoid cystic carcinoma. Awareness of its pathological features can help with the diagnosis as well as differential diagnosis. More cases are still needed for accurately assessing the prognosis of this particular tumor.

Aged ; Breast Neoplasms ; metabolism ; pathology ; surgery ; Carcinoma, Adenoid Cystic ; metabolism ; pathology ; surgery ; Carcinoma, Basal Cell ; metabolism ; pathology ; surgery ; Carcinoma, Ductal, Breast ; metabolism ; pathology ; Carcinoma, Small Cell ; metabolism ; pathology ; Diagnosis, Differential ; Female ; Humans ; Immunohistochemistry ; Keratin-14 ; metabolism ; Keratin-5 ; metabolism ; Keratin-7 ; metabolism ; Mastectomy ; methods ; Middle Aged ; Proto-Oncogene Proteins c-kit ; metabolism ; Vimentin ; metabolism