Sphenopalatine artery pseudoaneurysm masquerading as a second primary maxillary carcinoma.

PURPOSE: Maxillary sinus carcinomas usually present as a locally advanced disease at the time of diagnosis and it is extremely unusual to have a second primary maxillary carcinoma on the contralateral side after many years of completion of treatment of the first malignancy. We present here a case report of a sphenopalatine artery (SPA) pseudoaneurysm mimicking the second primary maxillary carcinoma. METHODS: We reviewed the literature for SPA pseudoaneurysm. RESULTS/CASE REPORT: This report describes the case of a 90-year-old man with a background of adenoid cystic carcinoma of the right maxillary sinus, diagnosed and treated with surgery and radiotherapy 14 years ago, who presented with a history of multiple episodes of epistaxis. The radiological evaluation showed a heterogeneously enhancing mass with a central hemorrhagic component and surrounding bony erosions in the left maxillary sinus and the patient was planned for biopsy from the suspicious mass along with SPA ligation. However, on opening the maxillary antrum there was excessive bleeding and it was determined unsafe to proceed further. The patient was subsequently taken to interventional radiology for diagnostic angiography which revealed an SPA pseudoaneurysm that was subsequently embolized successfully. CONCLUSIONS: Sphenopalatine artery pseudoaneurysms should be considered as a differential for recurrent epistaxis in patients with a history of sinonasal malignancy. In such cases, endovascular embolization is a viable management option.

[Tracheal Tumours]. / Trachealtumoren.

Malignant primary tracheal tumours are rare. The most common histological subtypes are squamous cell carcinoma and adenoid cystic carcinoma. These two entities have different prognoses and growth patterns. Tracheobronchoscopy and thoracic sectional imaging are standard diagnostic tools for tumour staging and local evaluation. Complete surgical resection of the affected tracheal segment is the treatment of choice for limited disease without distant metastases. Incomplete gross tumour resection with additional irradiation is an acceptable therapeutic option for adenoid cystic carcinoma. Interventional endoscopy with tumour debulking or tracheal stenting and/or definitive mediastinal radiotherapy are treatment alternatives in either a locally advanced or palliative setting.

Primary and Recurrent Intraosseous Adenoid Cystic Carcinoma-Analysis of Two Cases and Literature Review.

Adenoid cystic carcinoma (ACC) is a rare malignant tumor that mostly occurs in minor glands, especially in the palate. Intraosseous adenoid cystic carcinoma (IACC) is rarer. There is no clear conclusion on the clinical, radiologic and pathological characteristics of IACC because of few reported IACC cases, leading to insufficient understanding of IACC. We reviewed 52 previous reports of primary IACC (PIACC) and analyzed the clinical features of those patients involved, attempting to provide a better understanding of PIACC. Moreover, we present a case of primary PIACC and a case of recurrent IACC (RIACC). The two patients showed similarities in clinical and pathological results, along with slight differences in radiological and immunohistochemical results. The patient of case 1 seemed to display a worse prognosis, which can only be proved after long term follow-up.

A case of metastatic adenoid cystic (basal cell) carcinoma of the prostate: Systemic therapy for a rare disease.

BACKGROUND: Metastatic adenoid cystic (basal cell) carcinoma of the prostate is an exceedingly rare disease entity. As a result, no current consensus exists for optimal systemic therapy. METHODS: We present a patient with metastatic adenoid cystic (basal cell) carcinoma of the prostate who subsequently received systemic treatment, including chemotherapy and immunotherapy. We comprehensively reviewed all published data on therapy outcomes in advanced disease. RESULTS: Our patient benefited from combination chemotherapy (carboplatin and paclitaxel), with objective radiographic response and reduction in cancer-related pain. However, chemotherapy was stopped due to cumulative neurotoxicity, and subsequent immunotherapy with atezolizumab did not produce any response. Our literature review revealed inconsistent outcomes with various treatments but showed most promise with chemotherapy. Targeted therapy and immunotherapy seem to benefit specific cases, and androgen deprivation therapy had minimal evidence of benefit. CONCLUSION: Based on the findings of our case report and literature review, we suggest platinum-based chemotherapy doublets as first-line treatment for metastatic cases of adenoid cystic (basal cell) carcinoma of the prostate, reserving targeted therapy or immunotherapy for select cases based upon molecular profiles.

Cylindroma of the breast with CYLD gene mutation: a case report and review of the literature.

BACKGROUND: Cylindroma of the breast is a rare benign neoplasm. Since its first description in 2001, 20 cases have been reported in the literature. METHODS AND RESULTS: We report another case of this rare tumor in a 60-year-old woman with demonstration of the underlying molecular alteration. Histologically, the tumor showed the typical "jigsaw" pattern of a dual population of cells with a triple-negative phenotype. The pathognomonic mutation of the CYLD gene mutation was detected by whole exome sequencing. Cylindromas show morphological overlap with the solid-basaloid variant of adenoid cystic carcinoma, which renders this differential diagnosis difficult. However, distinction of these two lesions is of outmost importance, since cylindromas, in contrast to solid-basaloid variant of adenoid cystic carcinoma, behave in an entirely benign fashion. CONCLUSIONS: Careful evaluation of morphological features such as mitotic figures and cellular atypia is crucial in the diagnostic work-up of triple-negative breast lesions. It is important to keep cylindroma in mind as a pitfall and possible differential diagnosis for the solid-basaloid variant of adenoid cystic carcinoma. Molecular detection of CYLD gene mutation is helpful in cases with ambiguous histology. With this case report, we aim to contribute to a better understanding of mammary cylindroma and facilitate the diagnosis of this rare entity.

Computed Tomography and Magnetic Resonance Imaging Findings Contribute to Differentiating Solid- and Nonsolid-Type Adenoid Cystic Carcinoma in Maxillary Sinus.

PURPOSE: This study aimed to evaluate the imaging features of maxillary sinus adenoid cystic carcinoma (ACC) on computed tomography (CT) and magnetic resonance imaging (MRI) and to investigate the imaging differences between solid and nonsolid maxillary sinus ACC. METHODS: We retrospectively reviewed 40 cases of histopathologically confirmed ACC of the maxillary sinus. All the patients underwent CT and MRI. Based on the histopathological characteristics, the patients were classified into 2 groups: ( a ) solid maxillary sinus ACC (n = 16) and ( b ) nonsolid maxillary sinus ACC (n = 24). Imaging features such as tumor size, morphology, internal structure, margin, type of bone destruction, signal intensity, enhancement changes, and perineural tumor spread on CT and MRI, were evaluated. The apparent diffusion coefficient (ADC) was measured. Comparisons of imaging features and ADC values were performed between the solid and nonsolid maxillary sinus ACC using χ 2 and nonparametric tests. RESULTS: The internal structure, margin, type of bone destruction, and degree of enhancement significantly differed between solid and nonsolid maxillary sinus ACC (all P < 0.05). The ADC of the solid maxillary sinus ACC was considerably lower than that of the nonsolid maxillary sinus ( P < 0.05). CONCLUSIONS: Computed tomography and MRI may aid in the differentiation of solid and nonsolid types of maxillary sinus ACC.

Polar Vessel: A New Ultrasound Sign for Complementary Diagnosis of Major Salivary Gland Adenoid Cystic Carcinoma.

OBJECTIVES: To investigate the characteristic ultrasonographic findings of adenoid cystic carcinoma (ACC) in major salivary glands and identify the value of polar vessel in color Doppler flow imaging (CDFI) for the diagnosis of ACC. METHODS: From January 2017 to December 2021, 76 patients with parotid and submandibular gland tumors, including 14 patients with ACC, as confirmed by surgery and histopathology, were enrolled. Their clinicopathologic information and ultrasound (US) features were recorded and analyzed. The performance of polar vessel in CDFI for differentiating ACC from non-ACC (benign tumors and mucoepidermoid carcinoma [MEC]) was analyzed. RESULTS: ACC in the major salivary gland was more likely to be associated with pain symptoms (P = .027) and unclear borders and rough edges in grayscale US (P = .002, .015, respectively) than benign tumors. Compared to MEC, ACC tended to feature a homogeneous internal echo (P = .008). ACC of the major salivary gland had a significantly higher incidence of polar vessel sign than that of non-ACC (benign tumors and MEC) (P < .0001, .0001, respectively). The polar vessel sign showed good performance in distinguishing between ACC and non-ACC, with an area under the receiver operating characteristic curve of 0.857, a sensitivity of 71.4%, a specificity of 100%, and an accuracy of 94.7%. Positive predictive value and negative predictive value were calculated at 100% and 93.9%, respectively. CONCLUSIONS: The US sign of polar vessel has high diagnostic efficiency, and it may have important potential for use as a new complementary sign for the diagnosis of ACC in major salivary glands.

Adenoid Cystic Carcinoma Mimicking Bronchial Asthma.

A 41-year-old male presented to the Department of Pulmonary Medicine, with shortness of breath for 1 year. Though on treatment for bronchial asthma, he was not responding. The chest radiograph was normal (Fig. 1). On spirometric examination, the flow-volume curve showed flattening of the expiratory limb, suggesting variable intrathoracic obstruction. Fiber-optic bronchoscopy was, hence, done and it revealed a growth in the trachea (Fig. 2). Biopsy was deferred due to the risk of bleeding. Computed tomography (CT) of the chest also showed tracheal growth (Fig. 3). The patient was planned for rigid bronchoscopy. Meanwhile, the patient presented with expectoration of a piece of that growth. Histopathological examination revealed an adenoid cystic carcinoma (Fig. 4).

[Comparison of four convolutional neural networks for histopathological diagnosis of salivary gland carcinomas]. / Ein Vergleich von 4 konvolutionalen neuronalen Netzen in der histopathologischen Diagnostik von Speicheldrüsenkarzinomen.

BACKGROUND: Malignant salivary gland tumors represent a particular diagnostic challenge due to the large number of histopathological entities, their rare occurrence, and the diverse clinical and histological presentations. The aim of this work is to investigate and compare convolutional neural networks (CNNs) as a diagnostic tool for histological diagnosis of salivary gland cancer. METHODS: From salivary gland cancer preparations of 68 patients, 118 histological slides were digitized at high resolution. These virtual sections were then divided into small image sections, and the resultant 83,819 images were sorted into four categories: background, connective tissue, non-neoplastic salivary gland tissue, and salivary gland cancer tissue. The latter category grouped the entities adenoid cystic carcinoma, adenocarcinoma (not otherwise specified), acinar cell carcinoma, basal cell carcinoma, mucoepidermoid carcinoma, and myoepithelial carcinoma. The categorized images were then processed in a training, validation, and test run by the ImageNet pretrained CNN frameworks (Inception ResNet v2, Inception v3, ResNet152, Xception) in different pixel sizes. RESULTS: Accuracy values ranged from 18.8% to 84.7% across all network architectures and pixel sizes, with the Inception v3 network achieving the highest value at 500â€¯× 500 pixels. The recall values/sensitivity reached up to 85% for different pixel sizes (Inception v3 network at 1000â€¯× 1000 pixels). The minimum F1 score achieved was 0.07 for the Inception ResNet v2 and the Inception v3 at 100â€¯× 100 pixels each, the maximum F1 score achieved was 0.72 for the Xception at 1000â€¯× 1000 pixels. Inception v3 was the network with the shortest training times, and was superior to all other networks at any pixel size. CONCLUSION: The current work was able to demonstrate the applicability of CNNs for histopathological analysis of salivary gland tumors for the first time and provide a comparison of the performance of different network architectures. The results indicate a clear potential benefit for future applications.

Unexpected recurrence and rapid progression of lacrimal gland adenoid cystic carcinoma during pregnancy: a case report.

Adenoid cystic carcinoma (ACC) is the most common malignant neoplasm involving the lacrimal glands, with high rates of recurrence and metastasis. During the pregnancy, reports of recurrence of ACC of the salivary glands and trachea have previously been published, but no lacrimal gland ACC recurrence has been reported. We present a 35-year-old woman with lacrimal gland ACC who was initially treated by surgical resection and adjunctive radiotherapy, but her cancer recurred during pregnancy, with rapid progression to cavernous sinuses and brain. Estrogen and progesterone receptors have been detected on lacrimal glands and ACCs of salivary glands. Thus, hormonal changes during pregnancy might contribute to the recurrence of ACC. However, the inherent invasive and recurrent nature of ACC could also account for the regrowth in this patient and further molecular studies can provide more accurate explanations.

Adenoid cystic carcinoma of the breast and intraoperative electron radiotherapy: single case report and review of literature.

Adenoid cystic carcinoma (ACC) of the breast is a very rare neoplasm. It presents a triple-negative phenotype in most cases, but its prognosis is generally considered to be better than other breast cancers with the same immunohistochemical pattern. Due to its controversial features, no data are available in the literature regarding a consensus approach for ACC treatment, especially for subtypes with worse prognosis like solid basaloid ACC. We present for the first time a rare case of ACC with multifocal presentation treated with breast-conservative surgery and intraoperative electron radiotherapy, thus supporting this treatment of ACC in selected patients like young women affected by the solid basaloid variant who commonly present a worse prognosis. In this case, no local or systemic recurrence was detected after 30 months of follow-up.

Plain language summary Breast cancer is a large group of tumors with different and specific features. Because of its variety, no univocal guidelines are available to medical doctors for the treatment of this disease, especially for the rarest presentations. This is the case for breast adenoid cystic carcinoma, a rare tumor which accounts for less than 0.1% of all breast cancers and about which few instructions for its therapeutic approach or prognosis are described in the literature. This case report describes our experience using partial breast resection in combination with a specific protocol of intraoperative radiation for the treatment of an aggressive variant of breast adenoid cystic carcinoma. Good cosmetic results and no recurrence of the disease were shown, suggesting that a conservative approach could avoid unnecessary total breast resection as supported by some previous authors.

Frequency and imaging features of the adjacent osseous changes of salivary gland carcinomas in the head and neck region.

PURPOSE: The association between salivary gland carcinomas and adjacent osseous changes in the head and neck region is not clear. We evaluated the frequency and imaging features of such changes and investigated the specific characteristics of salivary gland carcinomas associated with them. METHODS: A total of 118 patients with histologically proven salivary gland carcinomas were retrospectively reviewed. The imaging characteristics of osseous changes were sorted into three categories based on computed tomography images: sclerotic change, erosive change, and lytic change. The frequency of all these osseous changes and any one of them was compared between different pathologies using Fisher's exact test. Odds ratios were calculated to evaluate the association between these changes and perineural spread. RESULTS: Osseous changes were found in 21 (18%) of 118 cases. Among these, seven (6%) cases were with sclerotic, nine (8%) with erosive, and nine (8%) with lytic changes (four with mixed change). Adenoid cystic carcinoma showed a significantly higher frequency of sclerotic and erosive changes, and either osseous change, than the other salivary gland carcinomas (p < 0.001 for each). Sclerotic changes were only present in the adenoid cystic carcinomas. Perineural spread was a significant factor in showing higher osseous change frequencies (odds ratio = 3.98, p = 0.006). CONCLUSION: Among salivary gland carcinomas in the head and neck region, adenoid cystic carcinomas had a significantly higher frequency of adjacent osseous changes, especially sclerotic changes, than other salivary gland carcinomas.

Intraorbital and intracranial extension of adenoid cystic carcinoma without clinical or radiological lacrimal gland involvement.

Adenoid cystic carcinoma (AdCC) is a rare epithelial neoplasm of the head and neck, most commonly found in the salivary glands. Orbital AdCC is an uncommon clinical entity arising from the lacrimal glands, however primary orbital AdCC has been previously described in a small number of case reports. The exact origin of the neoplasm with uninvolved lacrimal gland in the orbit is unknown, however it may arise from ectopic lacrimal or salivary gland tissue, or extension from nearby epithelial structures. We describe the clinical characteristics, investigations and management of a 55-year-old man presenting with vertical diplopia, found to have left posterior orbital AdCC invading the skull base with intracranial extension involving the inferotemporal fossa, pterygopalatine fossa, left carotid artery, cavernous sinus and temporal lobe dura, without clinical or radiological lacrimal gland involvement or systemic metastases.

Adenoid cystic carcinoma of the palpebral lobe of the lacrimal gland - case report and literature review.

Epithelial tumors of the lacrimal gland are rare and usually develop in the orbital lobe. We report the exceedingly rare occurrence of a primary adenoid cystic carcinoma in the palpebral lobe of the lacrimal gland. A 26-year-old female was referred for evaluation of a gradually enlarging mass in the lateral upper eyelid, previously diagnosed as a chalazion. Computed tomography revealed a heterogeneous round lesion anterior to the orbital rim. Excisional biopsy was compatible with an adenoid cystic carcinoma. After excluding distant metastasis, and as the patient refused adjuvant radiotherapy, a second surgical procedure, with wide local excision, was indicated. Follow-up showed no recurrence. This case highlights the importance of performing a thorough clinical examination when diagnosing any lateral upper eyelid mass. A high index of suspicion for malignant tumors of the lacrimal gland should always be maintained, and a complete excision with histological analysis should be preferred whenever possible.

Primary adenoid cystic carcinoma arising from an ectopic lacrimal gland involving both nasal orbits: a rare clinical entity.

Adenoid cystic carcinoma (ACC) is a slow-growing, locally invasive tumor of epithelial origin. The common sites of origin are the minor and major salivary glands and also the lacrimal glands. ACC accounts for 4.8% of primary orbital neoplasms and commonly arises superolaterally from the main lacrimal gland. Primary ACC of the orbit from an extra lacrimal region is quite rare. We present a case of ACC that occurred in ectopic lacrimal gland tissue involving the medial region of both orbits. A 74-year-old woman was admitted with a 4-week history of progressive painful proptosis in her left eye. Examination revealed a medium-size bilateral nasal canthal mass. Computed tomography (CT) scan revealed a nasal mass in both orbits with bone erosion. The patient underwent bilateral anterior orbitotomy and incisional biopsy was performed. Histopathology exam revealed an ACC with perineural and bone invasion. She refused orbital exenteration and radiation therapy was initiated.

Adenoid cystic carcinoma of external auditory canal: A rare disease.

External auditory canal, like other epithelialized surfaces is predisposed to malignancies such as Squamous cell carcinomas (SCC), melanomas and adenocarcinomas. In this background, malignancies like adenoid cystic carcinoma (ACC) are a rare occurrence and need to be thoroughly evaluated both locally and for distant extension. The malignancy needs to be addressed with an aggressive approach surgically with adequate marginal clearance. The role of radiation is debatable considering the outcomes in the limited data. Here we present the case of a 35-year-old female who came in with otalgia and otorrhea associated with a mass in the external auditory canal. After biopsy and imaging, a diagnosis of adenoid cystic carcinoma was made and the patient was managed accordingly.

[A Case of Adenoid Cystic Carcinoma of the Breast].

Primary adenoid cystic carcinoma(ACC)of the breast is a rare type of breast cancer. A 53-year-old woman with a right breast mass was examined at our institute. Ultrasonography showed 12.5×10.3×8.4 mm sized an ill-defined hypoechoic mass at zone C of the right breast. Pathological examination of core needle biopsy revealed atypical cells with solid and cribriform growth pattern. Computed tomography did not reveal lymph node metastases or distant metastases. The preoperative diagnosis was Stage ⅠA(cT1cN0M0, ER/PgR/HER2=-/-/1+)invasive ductal carcinoma or ACC. Surgery consisted of breast-conserving surgery and sentinel node biopsy. Pathological examination of the excised specimen revealed a so- called adenoid cystic pattern, so the final diagnosis was Stage ⅠA(pT1cN0M0, ER/PgR/HER2=-/-/1+)ACC. After 1 year of observation without adjuvant treatment, there has been no recurrence.

Whole-Body MRI Surveillance of Cancer Predisposition Syndromes: Current Best Practice Guidelines for Use, Performance, and Interpretation.

OBJECTIVE. Whole-body MRI is a valuable tool in the surveillance of cancer predisposition syndromes (CPSs). Because it allows wide-FOV imaging without ionizing radiation, whole-body MRI is ideal in pediatric patients, enabling efficient assessment of different organ systems for multifocal disease. This article summarizes the use of whole-body MRI in pediatric patients with CPSs for earlier detection of malignancy, provides evidence where available, and offers guidance where lacking because of the rarity of CPSs. Protocol modifications and technique performance in specific CPSs are also considered. CONCLUSION. Whole-body MRI is the preferred imaging modality for surveillance of pediatric patients with CPSs, and the growing literature supports its importance in presymptomatic cancer detection.

Adenoid Cystic Carcinoma of Lacrimal Gland with Bone Remodeling.

Adenoid cystic carcinoma is the most common type of malignancy seen in the lacrimal glands and is generally characterized by invasive malignant appearance with irregular margins and associated bone erosion or destruction. The authors report an unusual patient with a large expansile, well-circumscribed lacrimal adenoid cystic carcinoma with adjacent bone remodeling, radiologically mimicking a benign lesion.