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Pathology, Research and Practice Apr 2024Solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms constituting less than 2% of all soft tissue tumors. They typically originate in the thoracic cavity,...
Solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms constituting less than 2% of all soft tissue tumors. They typically originate in the thoracic cavity, mainly in the pleura, but can also occur in other various sites such as lung parenchyma, pericardium, and bronchus. In this study, a 49-year-old non-smoking female with a history of allergies presented to our pulmonary clinic with a chronic cough. An explorative bronchoscopy revealed an intrabronchial mass in the left superior bronchi, and a 68 Ga-DOTATOC positron emission computed tomography suggested a carcinoid tumor. Subsequent pulmonary segmentectomy unveiled a well-circumscribed polypoid lesion diagnosed as a low-grade bronchus SFT through histopathological and immunohistochemical assessments. The patient was asymptomatic after surgical excision and showed no other lesion during the 6-month follow-up. The endobronchial location of SFT is uncommon, with only a few reported cases in the literature, underscoring the necessity of considering various differential diagnoses, including carcinoid, mucoepidermoid carcinoma, endobronchial pleomorphic adenoma, hamartoma, leiomyoma, and metastasis, depending on location and imaging features. This report underscores the importance of careful histological and immunohistochemical evaluation in understanding and appropriately stratifying the risk associated with polypoid lesions.
Topics: Humans; Female; Middle Aged; Solitary Fibrous Tumors; Diagnosis, Differential; Soft Tissue Neoplasms; Bronchi; Neoplasms, Connective and Soft Tissue
PubMed: 38492357
DOI: 10.1016/j.prp.2024.155240 -
International Journal of Clinical... Jun 2024Salivary gland-type cancers (SGTCs) are histologically heterogeneous and can affect organs other than the salivary glands. Some tumors outside the salivary glands are...
BACKGROUND
Salivary gland-type cancers (SGTCs) are histologically heterogeneous and can affect organs other than the salivary glands. Some tumors outside the salivary glands are diagnosed on their unique histological characteristics. Comprehensive cross-organ studies on SGTCs are limited.
METHODS
We retrospectively analyzed the data of patients with salivary duct carcinoma (SDC), adenoid cystic carcinoma (AdCC), mucoepidermoid carcinoma (MEC), epithelial-myoepithelial carcinoma (EMC), acinic cell carcinoma (AcCC), and polymorphous adenocarcinoma (PAC) who visited our institution between 2009 and 2019. The primary tumor sites were classified into four categories; major salivary glands, head/neck (H/N) excluding (exc) major salivary glands (MSG) regions, broncho-pulmonary regions, and "others". H/N exc MSG was further divided into three subcategories, nasal/paranasal sinus, oral and pharynx/larynx.
RESULTS
We identified 173 patients with SGTCs, with SDC, AdCC, MEC, EMC, AcCC, and PAC accounting for 20%, 42%, 27%, 3%, 8%, and 1% of the cases, respectively. The most frequent primary site was the major salivary glands (64%), followed by H/N exc MSG regions (27%), broncho-pulmonary regions, and "others", thus non-salivary gland origins accounted for 9% of all cases. Patients with SDC, MEC, AcCC, or SGTC of the major salivary glands and broncho-pulmonary regions were more frequently treated by surgery. The overall survival time of the patients with MEC was significantly better than that of patients with SDC or EMC.
CONCLUSIONS
This cross-organ study highlights the clinical significance of SGTCs, underscoring the need for developing novel therapies for this rare disease entity.
Topics: Humans; Salivary Gland Neoplasms; Female; Male; Middle Aged; Retrospective Studies; Aged; Adult; Carcinoma, Mucoepidermoid; Carcinoma, Adenoid Cystic; Aged, 80 and over; Carcinoma, Acinar Cell; Young Adult; Adolescent; Adenocarcinoma; Salivary Glands
PubMed: 38492066
DOI: 10.1007/s10147-024-02505-3 -
Journal of the West African College of... 2024Reconstruction of large facial defects is quite a challenging and difficult task. Various surgical options are available, each with its challenges and complications....
Reconstruction of large facial defects is quite a challenging and difficult task. Various surgical options are available, each with its challenges and complications. Galeo-pericranial flap has provided a suitable technique for reconstruction of radical parotidectomy defects with satisfactory outcomes. A 50-year-old farmer with a histologically diagnosed mucoepidermoid carcinoma of the right parotid gland of 15 years duration had radical parotidectomy and reconstruction of the defect with galeo-pericranial flap. The patient was followed up for 2 years, and the flap was completely taken with no donor site morbidity.
PubMed: 38486657
DOI: 10.4103/jwas.jwas_78_23 -
Orphanet Journal of Rare Diseases Mar 2024Locoregional recurrence is a critical factor in the prognosis of sinonasal malignancies. Due to the rarity of these tumours, as well as the heterogeneity of histologies...
BACKGROUND
Locoregional recurrence is a critical factor in the prognosis of sinonasal malignancies. Due to the rarity of these tumours, as well as the heterogeneity of histologies and anatomical subsites, there is little evidence regarding the rate and location of regional metastases in sinonasal malignancies. Elective regional lymph node dissection in the therapy of sinonasal malignancies has become controversial. On the one hand, elective regional lymph node dissection is considered to be an overtreatment in the cN0 cases. On the other hand, undetected occult lymphatic metastases are associated with a poor prognosis. In this study, we discuss the role of sentinel lymph node biopsy as a minimally invasive procedure in the treatment of sinonasal malignancies based on our two years of practical experience and the currently available data.
RESULTS
This is a descriptive, monocentric, retrospective study, including 20 cases of cN0 malignant sinonasal neoplasm, that underwent a surgical therapy between 2020 and 2022. The following aspects were investigated: tumour entity, localisation of the primary tumour, tumoral stage, localisation of the sentinel lymph nodes, and postoperative complications. Squamous cell carcinoma was the most frequently diagnosed tumour entity (50%), followed by adenocarcinoma (20%) and malignant melanoma (15%), adenoid cystic carcinoma and mucoepidermoid carcinoma. Sentinel lymph nodes were most frequently found in the ipsilateral neck region I (45%), followed by the ipsilateral neck region II (40%). In all cases, the removed lymph nodes were free of malignancy. There were no postoperative complications due to lymph node biopsy. There were no recurrences during the study period.
CONCLUSION
Sentinel node biopsy could add more safety to the management of cN0 sinonasal malignancies due to its low morbidity. Whether SNB could provide an alternative to elective neck dissection in the management of SNM should be investigated in further studies.
Topics: Humans; Sentinel Lymph Node Biopsy; Retrospective Studies; Neoplasm Recurrence, Local; Skin Neoplasms; Lymphatic Metastasis
PubMed: 38481240
DOI: 10.1186/s13023-024-03127-8 -
Ear, Nose, & Throat Journal Mar 2024Salivary gland tumors are a heterogenous group of lesions with variable pathology and clinical outcomes. Most published data are derived from studies conducted at...
Salivary gland tumors are a heterogenous group of lesions with variable pathology and clinical outcomes. Most published data are derived from studies conducted at tertiary care centers. Our study analyzed the experience from a community setting to determine significant differences, if any, in pathological distribution and clinical outcomes compared to the existing literature. We performed a retrospective analysis of all major salivary gland tumors that presented to a large community practice over a 20 year period. Retrospective chart analysis was performed for demographics, clinical presentation, imaging, cytology, histopathology, and clinical outcome data. Of 806 patients, the parotid gland was the most common site in 683 patients (84.7%), followed by submandibular in 78 (9.7%) and sublingual in 45 (5.6%). A total of 203 patients were managed conservatively with observation without definitive diagnosis or lost to follow-up. A total of 495 patients underwent surgical intervention within the community practice. Twenty-six patients underwent surgical excision at an outside hospital. Eighty-two patients were determined to have a benign diagnosis based on ultrasound-guided fine needle aspiration or excisional biopsy alone. Final histopathology was benign in 505 cases (83.7%), while 98 tumors (16.3%) received a diagnosis of primary or secondary malignancy. For the parotid gland, pleomorphic adenoma (155) and Warthin's tumor (155) were the most common benign diagnoses, while mucoepidermoid carcinoma (13), adenocarcinoma (8), and acinic cell carcinoma (8) were the most common primary malignancies. We found a higher rate of benign tumor pathology compared to the existing literature. While the outcome data on surgical treatment of benign tumors are comparable to the existing literature, the same conclusion cannot be drawn for malignant tumors, given relatively small numbers in our series and likely disparity in the complexity of the surgical cases in tertiary care centers.
PubMed: 38445603
DOI: 10.1177/01455613241233085 -
Cureus Jan 2024Mucoepidermoid carcinoma is a rare neoplasm of the salivary gland of which the intraosseous variety is commonly observed with a female predilection and the affected side...
Mucoepidermoid carcinoma is a rare neoplasm of the salivary gland of which the intraosseous variety is commonly observed with a female predilection and the affected side is more commonly in the mandible. It is usually perceived as an asymptomatic swelling that increases in volume over a few months to a year. They more frequently present as a cortical bulging and are mostly discovered as an accidental finding in a routine radiograph as a well-defined unilocular or multilocular radiolucency resembling an odontogenic cyst. The most widely accepted treatment is radical surgical resection due to its recurrence or metastatic nature. The current case is quite unusual developing in the posterior jaw as a result of an impacted third molar in a 22-year-old female patient.
PubMed: 38435903
DOI: 10.7759/cureus.53355 -
Diagnostic Pathology Mar 2024Primary mucoepidermoid carcinomas (MECs) of the sinonasal tract and nasopharynx are rare entities that represent a diagnostic challenge, especially in biopsy samples.... (Review)
Review
AIMS
Primary mucoepidermoid carcinomas (MECs) of the sinonasal tract and nasopharynx are rare entities that represent a diagnostic challenge, especially in biopsy samples. Herein, we present a case series of MECs of the sinonasal and skull base and its mimics to evaluate the clinicopathological and molecular characteristics in order to avoid misdiagnosis.
METHODS
We reviewed the pathology records of patients diagnosed from 2014 to 2022. Thirty MECs were consecutively diagnosed during that period.
RESULTS
Based on morphological and fluorescence in situ hybridization (FISH) analyses, 30 tumors originally diagnosed as MECs were separated into MAML2 fusion-positive (7 cases) and MAML2 fusion-negative groups (23 cases), in which 14 tumors were positive for the EWSR1::ATF1 fusion; these tumors were reclassified to have hyalinizing clear cell carcinoma (HCCC). The remaining nine MAML2 FISH negative cases were reconfirmed as squamous cell carcinoma (SCC, 3 cases) which showed keratinization and high Ki-67 expression; DEK::AFF2 carcinomas (2 cases), in which DEK gene rearrangement was detected by FISH; and MECs as previously described (4 cases) with typical morphological features. Including 7 MAML2 rearrangements tumors, 11 MEC cases had a male-to-female ratio of 4.5:1, and 6 tumors arose from the nasopharyngeal region, while 5 tumors arose from the sinonasal region. The prognosis of this series of salivary gland-type MECs was favorable.
CONCLUSIONS
Our study confirmed that HCCC runs the risk of being misdiagnosed as MEC in the sinonasal tract and nasopharynx, particularly with biopsy specimens. Careful histological evaluation with supporting molecular testing can facilitate pathological diagnoses.
Topics: Humans; Male; Female; Carcinoma, Mucoepidermoid; In Situ Hybridization, Fluorescence; Transcription Factors; Salivary Gland Neoplasms; Carcinoma, Squamous Cell
PubMed: 38429827
DOI: 10.1186/s13000-024-01466-5 -
Biomedicines Jan 2024Mucoepidermoid carcinomas (MECs) represent the most common malignant neoplasms of the salivary glands, but they have also been described in other unusual sites. Primary...
Mucoepidermoid carcinomas (MECs) represent the most common malignant neoplasms of the salivary glands, but they have also been described in other unusual sites. Primary MECs originating in the thyroid gland are exceedingly rare, accounting for less than 0.5% of thyroid tumors. Owing to their low to medium grade, they are usually associated with an indolent evolution and a good long-term prognosis, generally being managed surgically based on the extent of the disease. However, this does not always apply, as primary thyroid MECs may present as metastatic or locally advanced diseases. While several treatment options have been explored in such cases, no consensus currently exists on their optimal treatment plan, and they should be managed in a multidisciplinary fashion. We report the case of a 67-year-old patient with primary MEC of the thyroid, which behaved aggressively, with extensive pulmonary metastasis, ultimately leading to the rapid clinical deterioration and death of the patient.
PubMed: 38397887
DOI: 10.3390/biomedicines12020285 -
Medicine Feb 2024Mucoepidermoid carcinoma (MEC) of the breast is an extremely rare primary breast tumor. Between 1979 and June 2022, only 50 cases were reported. The pathological... (Review)
Review
INTRODUCTION
Mucoepidermoid carcinoma (MEC) of the breast is an extremely rare primary breast tumor. Between 1979 and June 2022, only 50 cases were reported. The pathological morphology and biological behavior of breast MEC remain poorly understood.
PATIENT CONCERNS
A 47-year-old female was presented with a 10-day-old left breast mass detected by physical examination.
DIAGNOSES
Ultrasonography could not distinguish whether the breast tumor was benign or malignant. After a biopsy of a breast tumor excision specimen, combined with immunohistochemical results, the patient was diagnosed with high-grade mucoepidermoid breast carcinoma.
INTERVENTIONS
The patient underwent a modified radical mastectomy for her left breast.
OUTCOMES
The patient was still free from local recurrence or metastases at 1-year follow-up.
CONCLUSION
A high-grade MEC case without MAML2 rearrangement shows good recovery without complications. The diagnosis was confirmed by histomorphology and immunohistochemical markers. It is sometimes necessary to distinguish it from adenosquamous, adenoid cystic, or mucinous carcinoma. The primary treatment is surgical resection, and the prognosis is closely related to the pathological grade.
Topics: Humans; Female; Middle Aged; DNA-Binding Proteins; Trans-Activators; Breast Neoplasms; Carcinoma, Mucoepidermoid; Mastectomy; Transcription Factors
PubMed: 38394503
DOI: 10.1097/MD.0000000000037163 -
Discover Oncology Feb 2024Salivary gland tumors are histologically diverse. Ionocytes and tuft cells, rare epithelial cells found in normal salivary glands, might be associated with salivary...
PURPOSE
Salivary gland tumors are histologically diverse. Ionocytes and tuft cells, rare epithelial cells found in normal salivary glands, might be associated with salivary tumors. Here, we explored the expression of FOXI1 and POU2F3, master regulators of ionocytes and tuft cells, respectively, for common salivary neoplasms using immunohistochemistry.
METHODS
We analyzed normal salivary tissues and nine salivary gland tumors; Warthin tumors (WT), pleomorphic adenomas (PA), basal cell adenomas, and oncocytomas were benign, whereas mucoepidermoid, adenoid cystic, acinic cell, salivary duct carcinomas, and polymorphous adenocarcinomas were malignant.
RESULTS
Normal salivary glands contained a few FOXI1- and POU2F3-positive cells in the ducts instead of the acini, consistent with ionocytes and tuft cells, respectively. Among the benign tumors, only WTs and PAs consistently expressed FOXI1 (10/10 and 9/10, respectively). The median H-score of WTs was significantly higher than that of PAs (17.5 vs. 4, P = 0.01). While WTs and PAs harbored POU2F3-positive cells (10/10 and 9/10, respectively), the median H-score was higher in WTs than in PAs (10.5 vs 4, respectively). Furthermore, WTs exhibited a unique staining pattern of FOXI1- and POU2F3-positive cells, which were present in luminal and abluminal locations, respectively. Whereas none of the malignant tumors expressed FOXI1, only adenoid cystic carcinoma consistently expressed POU2F3 (5/5), with a median H-score of 4.
CONCLUSION
The expression patterns of the characteristic transcription factors found in ionocytes and tuft cells vary among salivary gland tumor types and are higher in WT, which might be relevant for understanding and diagnosing salivary gland neoplasms.
PubMed: 38358561
DOI: 10.1007/s12672-024-00892-7