Adenoid cystic carcinoma mimicking an oroantral fistula: a case report.
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ABSTRACT:
SUBMITTER: Monteiro BV
PROVIDER: S-EPMC4297002 | biostudies-other | 2014 Apr
REPOSITORIES: biostudies-other
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Project description:Adenoid cystic carcinoma of the Bartholin Gland (BG-ACC) is a rare form of vulval cancer with only approximately 350 reported cases since 1864. A review of available literature and case reports suggests an aggressive nature with protracted clinical symptoms and a tendency for local recurrence despite adequate surgical excision with or without adjuvant radiotherapy. Survival rates of 71% and 59% are reported at five and ten years. This case report endeavours to add to our body of knowledge regarding this rare disease and thus help broaden and improve our understanding of management and treatment success.
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Project description:Adenoid cystic carcinoma (ACC) is a malignant neoplasm, frequently affecting the salivary glands, and rarely occurring in other locations. ACC is characterized by slow growth, perineural invasion, local and late recurrence after original treatment. However, renal metastasis of ACC is very rare. To our best knowledge, only 11 cases of ACC metastasis to the kidney have been reported in the English literature to date. Herein, we presented a rare case of a 70-year-old man with renal metastasis from ACC of the right lung after 3 years of primary presentation. Our patient underwent right radical nephrectomy and histologically confirmed as Metastatic Adenoid Cystic Carcinoma.
| S-EPMC6819778 | biostudies-literature
Project description:Adenoid cystic carcinoma (ACC) of the breast is a rare tumor comprising <0.1% of all breast malignancies. The present study reports the case of a 66-year-old woman who underwent multiple imaging techniques, including mammography, ultrasonography and magnetic resonance imaging (MRI). The patient reported gradual enlargement of a mass in the left breast. Mammography identified a high-density, irregular mass, with nipple retraction. Ultrasonography revealed an irregular, complex cystic and solid mass with an unclear margin in the subareolar region. The internal vascularity was seen on Doppler examination. The lesion on MRI was also mixed cystic and solid, with an irregular shape and irregular margins. The solid part of the lesion appeared heterogeneously isointense on T1-weighted imaging (WI) and a slightly high signal on T2WI, and exhibited internal septations with a low signal on T2WI. Following injection of contrast agent, the enhancement of the solid part was rapid and heterogeneous. The cystic parts of lesion appeared as high-signal on T1WI and T2WI. The tumor infiltrated the left nipple and adjacent skin. The patient underwent a left modified radical mastectomy with axillary lymph node dissection. The pathological examination confirmed the tumor as ACC of the left breast. The axillary lymph nodes were negative for tumor metastasis. Therefore, the differential diagnosis of mixed cystic and solid breast masses with unclear margins and internal septations on T2WI exhibiting delayed enhancement, must include ACC.
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Project description:Introductioncutaneous adenoid cystic carcinoma is a rare epithelial tumor with slow development, it is most often described in the scalp; palpebral involvement is very rare. And is accompanied by local aggressiveness and frequent recurrence. The aim of this work is to discuss the management and the interest of a close surveillance of adenoid cystic carcinoma.Case presentationThe authors report a case of palpebral cystic adenoid carcinoma in a 65-year-old patient who underwent surgical excision followed by reconstruction of the loss of substance.DiscussionCutaneous cystic adenoid carcinoma of the eyelids remains a very rare entity that must be evoked in front of a nodular cutaneous palpebral tumor that progresses slowly. An early diagnosis and a complete excision guarantee a better prognosis.ConclusionAdenoid cystic carcinoma, because of its local aggressiveness and its capacity to recur despite satisfactory surgical removal, requires long-term surveillance. Its surgery may be responsible for functional and aesthetic sequelae.
| S-EPMC8209184 | biostudies-literature
Project description:BackgroundPrimary adenoid cystic carcinoma (ACC) of the lung, which arises from the bronchial gland and is rare, accounting for only 0.04-0.2% of all primary lung tumors. The genetic profiling of bilateral ACC of unknown primary site and application in postoperative decision-making are less reported.Case presentationA 57-year-old male with a smoking history of over 30 years and multiple nodules in both lungs was present to our department. After assessing the bilateral solid nodules in his Positron Emission Tomography-Computed Tomography (PET/CT) scan, malignant lesions at the left lower lung, right lower lung, and right middle lung are suspected. Sequential selective video-assisted thoracoscopic surgeries (VATS) were performed. A genetic alteration test of 425 cancer-related genes and global gene expression profile of the specimens revealed intrapulmonary metastasis existed. The patient was followed up for three years without recurrence and tissue mutations in liquid biopsy.ConclusionWe present a way of omics-based multiple pulmonary lesions origin assessment, facilitating post-operative differential diagnosis and treatment decision for difficult cases.
| S-EPMC7921630 | biostudies-literature
Project description:RationaleAdenoid cystic carcinoma (ACC) is an infrequent malignant neoplasm of the salivary glands. The clinical and pathological characteristics include slow growth, perineural invasion, and local recurrence. ACC of the mobile tongue is rarely reported in the literature.Patient concernsWe describe a case of a 43-year-old man patient with numbness and growth in the tongue for 1 month duration. The patient reported a long-term smoking and drinking habit.DiagnosesA biopsy was carried out and histopathological analysis confirmed as diagnosis of adenoid cystic carcinoma. Computed tomography (CT) of the head and neck enhanced scanning revealed an ill-defined high density mass with altered enhanced signal entities involving the anterior 2/3rd of the tongue.InterventionsThe patient was treated with surgery and adjuvant radiotherapy. Incision was placed over the anteriorly till 2/3rd of tongue and then the femoral anterolateral free flap was used to repair and reconstruct the defect of tongue.OutcomeThe patient is currently under a postsurgical 29-month regular follow-up, showing good swallowing and speaking function without any recurrence and metastasis.LessonsThis case report suggests that surgery and adjuvant radiotherapy are suitable procedures for patients with resectable ACC of the mobile tongue.
| S-EPMC6494219 | biostudies-literature
Project description:RATIONALE:Tracheobronchial adenoid cystic carcinoma (TACC) is a rare malignancy. Surgical resection remains the standard treatment of choice. But it is frequently unresectable due to its local extension. The practicability and safety of hypofractionated radiotherapy (HRT) for TACC remains unknown since most of the TACCs are centrally located. PATIENT CONCERNS:A 57-year-old female presented with paroxysmal cough, expectoration, and hemoptysis. DIAGNOSES:Computed tomography (CT) scan of the chest revealed a nodule originating from the wall of right primary bronchus, with 1.9*1.2?cm in size. Bronchoscope confirmed the node on the medial wall of the right primary bronchus extending towards the carina, with a close distance of 0.5?cm. Biopsy from the node was considered as adenoid cystic carcinoma (ACC). The clinical stage of the patient was T3N0M0. INTERVENTIONS:The patient underwent HRT with a total dose of 60Gy in twelve fractions. OUTCOMES:The patient experienced complete remission after HRT. No symptomatic radiation-induced toxicity (?grade 2) was observed during the follow-up. LESSONS:HRT may be a safe and effective modality for inoperable TACC.
| S-EPMC6310550 | biostudies-other
Project description:IntroductionAdenoid cystic carcinoma (ACC) is an uncommon tumour of the breast. It is known for its rare lymph node involvement and distant metastasis. A triple-negative breast cancer that has a favorable prognosis compared to other triple negative ductal carcinomas, it accounts for approximately 0.1-1% of all breast cancers.Presentation of caseWe report a case of a 69-year-old female with a palpable left breast mass who underwent multiple imaging modalities with significant size variance between the studies. Breast conserving therapy (BCT) was performed with axillary sentinel lymph node biopsy (SLNB) followed by radiation therapy (RT). Pathological examination confirmed the tumour as ACC.DiscussionACC, known as an persistent if low-grade malignant tumour of salivary gland, is considered to have low-malignant potential in the breast. It is a very rare subtype and from this scant data, there is minimal mention about size discrepancy between imaging modalities such as ultrasound and MRI.No consistent MRI features have been demonstrated, with the exception of T2 hyperintensity in larger lesions and T2 iso-intensity in smaller lesions. Ultrasound demonstrates primarily a hypoechoic or heterogenous mass with minimum vascularity, consistent with our radiographic findings.ConclusionACC is a rare entity in breast cancer pathology. Its size can be highly variable as measured by various radiographic modalities, and final Pathology from the surgical specimen is, as always, required for an accurate tumoral diameter. With that caveat, careful utilization of pre-operative imaging modalities is critical in pre-surgical planning to choose the appropriate surgery.
| S-EPMC6529769 | biostudies-literature
Project description:Adenoid cystic carcinomas (ACCs) are among the most enigmatic of human malignancies. These aggressive salivary gland cancers frequently recur and metastasize despite definitive treatment, with no known effective chemotherapy regimen. Here we determined the ACC mutational landscape and report the exome or whole-genome sequences of 60 ACC tumor-normal pairs. These analyses identified a low exonic somatic mutation rate (0.31 non-silent events per megabase) and wide mutational diversity. Notably, we found mutations in genes encoding chromatin-state regulators, such as SMARCA2, CREBBP and KDM6A, suggesting that there is aberrant epigenetic regulation in ACC oncogenesis. Mutations in genes central to the DNA damage response and protein kinase A signaling also implicate these processes. We observed MYB-NFIB translocations and somatic mutations in MYB-associated genes, solidifying the role of these aberrations as critical events in ACC. Lastly, we identified recurrent mutations in the FGF-IGF-PI3K pathway (30% of tumors) that might represent new avenues for therapy. Collectively, our observations establish a molecular foundation for understanding and exploring new treatments for ACC.
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Project description:Adenoid cystic carcinoma (ACC) of the breast, a rare malignancy that makes up less than 0.1% of all breast malignancies, is much rarer in males than in females. Due to the rarity of this disease, an optimal treatment strategy for male breast ACC has not been established, and therapy for male patients is currently based on guidelines for female patients. According to previous reported cases, some authors believe that male breast ACC may have higher invasive potential than female breast ACC and the prognoses in male patients may be worse than those in female patients. Therefore, a more proactive diagnosis and treatment regimen may be required. However, the clinical feature of our case is inconsistent with this view. Herein we report the case of a 24-year-old male without any antecedent medical or family history who presented with a slow-growing lump on his left chest wall for 5 years. The patient initially underwent lumpectomy, and the mass was pathologically diagnosed as breast ACC. Systemic examination was performed, and no evidence of distant metastasis was found. Then, he received modified radical mastectomy and ipsilateral axillary lymph node dissection. The mastectomy pathological examination revealed that no cancerous tissue was detected around the primary tumor bed, and all 22 axillary lymph nodes were negative. The patient did not receive postoperative chemotherapy, radiotherapy or endocrine therapy and remained well after 28 months of follow-up. In this study, we review the literature and summarize the clinical manifestations, imaging and histopathological characteristics, treatments and outcomes of male breast ACC. We share our experience in the hopes that this evidence will aid in the development of better therapeutics.
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