Pleomorphic adenoma (mixed tumor) of the upper lip: A case report.

BACKGROUND: Salivary gland tumors are relatively rare. Most minor salivary gland tumors are malignant with benign tumors accounting for 18% of the tumors. Pleomorphic adenoma (PA) is the most common salivary gland tumor. Lip PA is uncommon with 9.8% occurring in the upper lip. We are adding on the knowledge of the rare upper lip PA (benign mixed tumor). CASE SUMMARY: We report an upper lip PA (benign mixed tumor) in a 28-year-old man. His complaint was a painless swelling on the upper lip. A painless, non-tender, well-circumscribed, slightly mobile, sessile, nodular, and rubbery (in consistency) tumor measuring 5.0 cm x 2.0 cm was noted on the left side of his upper lip. The overlying skin was not fixed and of normal color. There was no ulceration, and palpation did not elicit pain or bleeding. There was no history of trauma. Blunt dissection was used to completely excise the nodular, whitish, and encapsulated tumor. Microscopy showed a well-circumscribed and partly encapsulated biphasic lesion, with large lobules of myxo-chondroid stroma and intervening cellular nodules of basaloid cells, well-formed tubules containing eosinic secretion, and nests of myoepithelial cells. A diagnosis of PA (benign mixed tumor) was confirmed. CONCLUSION: Blunt dissection is indicated to preserve the cosmesis and function of the upper lip.

Parotidectomy for deep lobe pleomorphic adenomas is associated with higher rates of complications and recurrence.

BACKGROUND: Pleomorphic adenoma (PA) is a common parotid tumor, yet due to the relative rarity of deep lobe PA (DLPA), there is a paucity of information about its clinical presentation and surgical outcomes. METHODS: We reviewed the charts of patients with previously untreated parotid PA between the years 1990 and 2015. Clinical parameters and surgical outcomes were compared between superficial lobe PA (SLPA) and DLPA. RESULTS: The cohort comprised 147 cases of DLPA and 222 cases of SLPA. DLPA were larger (median 2.6 cm vs. 2.0 cm, p < 0.001), more often discovered incidentally on imaging (33%, n = 48) and had unique presentations (pharyngeal mass, dysphagia, otalgia). Postsurgical complications were more frequently observed in DLPA (41% vs. 30% in SLPA, p = 0.025), mainly transient facial nerve weakness. DLPA also showed higher recurrence rates (n = 6, 4.1% vs. n = 1, 0.4%, p = 0.016). CONCLUSIONS: Parotidectomy for DLPA carries a higher risk of complications and recurrence compared to SLPA.

Benign mixed mammary tumor with sebaceous differentiation in a dog.

We describe here a case of canine mammary benign mixed tumor with sebaceous metaplasia in the right fifth mammary gland of an eight-year-old, intact female Poodle dog. Grossly, the mass was firm with off-white, poorly lobulated cut surfaces. Histologically, the luminal epithelial cells and myoepithelial cells proliferated with cartilage formation and focal squamous metaplasia. Moreover, a large number of nests of various sizes, which were filled with foamy cells in the center and associated with basaloid reserve-like cells in the periphery, showed sebaceous gland-like structures. Immunohistochemically, myoepithelial cells and reserve-like cells in the metaplastic sebaceous gland-like structures were CK14, α-smooth muscle actin (α-SMA) and p63 positive, suggesting a possibility that these two components may have a common cell of origin.

Diagnosis and treatment of mixed salivary gland tumor previously diagnosed as cutaneous chondroid syringoma proximal to the oral commissure: A case report.

Subcutaneous tumors of the head and neck resembling cutaneous mixed tumors may be misdiagnosed pleomorphic adenomas of salivary gland origin. Physicians should consider salivary mixed tumors in the differential diagnosis for suspected cutaneous tumors.

Chondroid syringoma of the nose: A rare case report and literature review.

INTRODUCTION AND IMPORTANCE: A chondroid syringoma is an exceptionally rare benign lesion of the sweat glands also known as mixed tumor of the skin (MTS). It can occur in different areas of the head and neck such as the lips, cheek, nose and scalp (Gotoh et al., 2022 [1]). It is usually painless and grows slowly. Based on pathological features it can be differentiated into apocrine or eccrine (Mixed cutaneous tumor: chondroid syringoma a case report, 2019 [2]). CASE PRESENTATION: Our patient presented with a nasal lesion in the left soft triangle, progressively increasing in size. He did not undergo any surgeries to the nose or any history of trauma. Due to the COVID-19 lockdown our patient did not seek early medical advice. In addition, the implementation of facemasks enabled for the concealment of the abnormality, which reduced the need of seeking treatment. CLINICAL DISCUSSION: Chondroid syringoma is a non-ulcerative tumor that grows slowly with an average diameter between 0.5 and 3 cm, however lesions reaching 9 cm have been also seen (Wan et al., 2018 [4]). The mainstay method of management is surgical excision while maintaining the aesthetic appearance of the patient. CONCLUSION: Owing to its rarity, clinical misdiagnosis is common, however absolute diagnosis is achieved by histopathology. This case delineates the rarity of this lesion and the mainstay method of management, which is surgical excision.

The comparison of pure uterine serous carcinoma and mixed tumor with serous component: a single-institution review of 91 cases.

BACKGROUND: Pure uterine serous carcinoma (p-USC) and mixed tumors with serous component (m-USC) are aggressive subtypes of endometrial cancer associated with high mortality rates. This retrospective study aimed to compare clinicopathologic features and outcomes of p-USC and m-USC in a single center. METHODS: This study retrospectively reviewed patients diagnosed with USC at Peking University People's Hospital between 2008 and 2022. T-tests and chi-square tests were used to compare clinicopathological characteristics between p-USC and m-USC. Kaplan-Meier survival curve and Cox regression analysis were used to analyze the impact of clinical and pathological variables on OS and PFS. RESULTS: Among the 91 patients who underwent surgery, 65.9% (n = 60) were p-USC, and 34.1% (n = 31) were m-USC. Patients with p-USC had earlier menopause (P = 0.0217), a lower rate of progesterone receptor(PR) expression (P < 0.001), and were more likely to have positive peritoneal cytology (P = 0.0464). After a median follow-up time of 40 months, 28 (46.7%) p-USC and 9 (29%) m-USC patients had progression disease, 18 (30%) and 8 (25.8%) patients died of their disease. 5-year PFSR were 51.2% and 75.3%, respectively, and 5-year OS rates were 66% and 67.4%. Kaplan-Meier survival analysis showed that p-USC was more likely to relapse than m-USC (P = 0.034), but there was no significant difference in OS. Cox regression analysis showed that lymph node metastasis and surgical approach were risk factors for OS, and myoinvasion depth ≥ 1/2 was an independent risk factor for PFS. CONCLUSIONS: p-USC was more likely to relapse than m-USC, but there was no significant difference in OS between the two subtypes.

SOX10-Internal Tandem Duplications and PLAG1 or HMGA2 Fusions Segregate Eccrine-Type and Apocrine-Type Cutaneous Mixed Tumors.

Cutaneous mixed tumors exhibit a wide morphologic diversity and are currently classified into apocrine and eccrine types based on their morphologic differentiation. Some cases of apocrine-type cutaneous mixed tumors (ACMT), namely, hyaline cell-rich apocrine cutaneous mixed tumors (HCR-ACMT) show a prominent or exclusive plasmacytoid myoepithelial component. Although recurrent fusions of PLAG1 have been observed in ACMT, the oncogenic driver of eccrine-type cutaneous mixed tumors (ECMT) is still unknown. The aim of the study was to provide a comprehensive morphologic, immunohistochemical, and molecular characterization of these tumors. Forty-one cases were included in this study: 28 cases of ACMT/HCR-ACMT and 13 cases of ECMT. After morphologic and immunohistochemical characterization, all specimens were analyzed by RNA sequencing. By immunohistochemistry, all cases showed expression of SOX10, but only ACMT/HCR-ACMT showed expression of PLAG1 and HMGA2. RNA sequencing confirmed the presence of recurrent fusion of PLAG1 or HMGA2 in all cases of ACMT/HCR-ACMT, with a perfect correlation with PLAG1/HMGA2 immunohistochemical status, and revealed internal tandem duplications of SOX10 (SOX10-ITD) in all cases of ECMT. Although TRPS1::PLAG1 was the most frequent fusion, HMGA2::WIF1 and HMGA2::NFIB were detected in ACMT cases. Clustering analysis based on gene expression profiling of 110 tumors, including numerous histotypes, showed that ECMT formed a distinct group compared with all other tumors. ACMT, HCR-ACMT, and salivary gland pleomorphic adenoma clustered together, whereas myoepithelioma with fusions of EWSR1, FUS, PBX1, PBX3, POU5F1, and KLF17 formed another cluster. Follow-up showed no evidence of disease in 23 cases across all 3 tumor types. In conclusion, our study demonstrated for the first time SOX10-ITD in ECMT and HMGA2 fusions in ACMT and further refined the prevalence of PLAG1 fusions in ACMT. Clustering analyses revealed the transcriptomic distance between these different tumors, especially in the heterogenous group of myoepitheliomas.

Cytohistological and Immunohistochemical Correlation of Cutaneous Mixed Tumors: A Series of Four Cases and Review of Recent Molecular Updates.

Fine needle aspiration cytology (FNAC) is an established diagnostic modality today, but its utilization in skin tumors is limited by the fact that most of these lesions are small and easily accessible for clinicians to perform an excision biopsy. As a result, our knowledge of the cytological features of some of the uncommonly encountered skin tumors is still lacking. The aim of this study was to correlate the cytological features of cutaneous mixed tumors (CMTs) with histological and immunohistochemical findings in four cases of CMT in commonly and uncommonly encountered locations. We also review the recent updates highlighting the various genetic rearrangements and newer more specific immunohistochemical markers described so far. This was a retrospective study, and all the slides were taken from our departmental archives. Case 1 was a 25-year-old male who presented with a gradually increasing painless swelling over the right angle of the mouth of 1.5 years duration. Case 2 was a 45-year-old male with swelling on the right forearm for the last three years. Case 3 was a 35-year-old female with a forehead swelling of one year duration. Case 4 was a 55-year-old female with left cheek swelling for two years. On clinical examination, all four nodular swellings were predominantly in the skin/subcutaneous tissue. On cytology, all cases showed abundant chondromyxoid material with clusters of benign epithelial cells and a fair number of predominantly singly scattered myoepithelial cells. The diagnosis of all four cases was further confirmed on histopathology and immunohistochemistry, and the findings correlated well with cytology. The cytological features of CMT closely correlate with their histopathological and immunohistochemical features. Newer immunohistochemistry (IHC) marker pleomorphic adenoma gene 1 (PLAG1) may be helpful in making a definitive diagnosis of CMT on cytology and cell block preparation along with a good clinical correlation. Complete surgical excision is the treatment of choice, and recurrence is rare.

Incidental combined hepatocellular-cholangiocarcinoma in liver transplant patients: Does it have a worse prognosis?

Background and Aim: Combined hepatocellular-cholangiocarcinoma (CHC) requires attention clinically and pathologically after liver transplantation (LT) because of its unique biology, difficulties in diagnosis, and being rare. We aimed to present our single-center experience for this incidental combined tumor. It is aimed to present our single-center experience for this incidental combined tumor. Materials and Methods: Seventeen patients with CHC were included in the study. There were 260 hepatocellular carcinoma (HCC) patients determined as the control group. Patients were evaluated for demographic, etiological, pathological features, and survival. Results: Macrovascular and microvascular invasion levels were significantly higher in the CHC group (p<0.05). P53, CK19, and CK7 levels were significantly higher in the CHC group (p<0.05). Hepatocyte-specific antigen level was significantly higher in the HCC group. The mean overall survival was significantly higher in the HCC group (p<0.05). Conclusion: Even though CHC is a rare liver tumor, it has features that need to be clarified regarding both survival and tumor biology. Investigating prognostic factors, especially in terms of survival and recurrence, will be very beneficial to identify candidates who will benefit from LT and be included in the indications for LT for CHC. This study evaluated the outcomes of patients showing combined HCC-intrahepatic cholangiocarcinoma in explant pathology.

Mixed Eccrine Cutaneous Tumor with Folliculo-Sebaceous Differentiation: Case Report and Literature Review.

Background/Introduction: Cutaneous mixed tumor is a rare benign neoplasm that exhibits a wide range of metaplastic changes and differentiation in the epithelial, myoepithelial, and stromal components, which is often confused with various other skin lesions. Case report: We present an unusual case of a 58-year-old woman with a mixed tumor of the upper lip, previously misdiagnosed as adnexal carcinoma on a preoperative biopsy. The excision biopsy shows a well-circumscribed lesion composed of various cells and structures featuring folliculo-sebaceous differentiation embedded in a prominent chondromyxoid stroma. The immunohistochemical study proves the various lineages of differentiation and classifies the neoplasm as the less common eccrine subtype of cutaneous mixed tumor. Discussion: The common embryologic origin of the folliculo-sebaceous apocrine complex leads to a great histological variety of cellular components of mixed tumors and the formation of structures that resemble established types of adnexal neoplasms, which could be a diagnostic pitfall, especially on a small incision biopsy.

A Review of the Current Literature on Pleomorphic Adenoma.

Pleomorphic adenomas (PA) are the most common benign salivary gland tumors. They arise from the major salivary glands, as well as the minor salivary glands. They may arise rarely from the palate, oral cavity, neck, and nasal cavity also. Yet, the fourth, fifth, and sixth decades of life are the most common for them to manifest. Forty percent of them occur in males and 60% in females. It is a benign mixed tumor, which has three components: an epithelial component, a myoepithelial cell component, and a mesenchymal component. A fibrous capsule separates these cells from the surrounding tissues. It generally presents as a slowly progressing painless swelling, which is well-delineated, nonsymptomatic, and not involving the facial nerve. Salivary gland tumors can be accurately diagnosed before surgery using fine-needle aspiration (FNA), ultrasonography (USG), and computed tomography (CT) scan. Calponin, cluster of differentiation 9 (CD9), glial fibrillary acidic protein (GFAP), Mcl-2, metastasis suppressor gene (NM23), p63, S-100, smooth muscle actin (SMA), and SRY-box transcription factor 10 (SOX10) exhibit the majority of the positive reactions in pleomorphic adenomas. The diagnostic marker pleomorphic adenoma gene 1 (PLAG1) is frequently employed since it is specific for pleomorphic adenoma. Although benign, these epithelial tumors have a propensity to recur and undergo malignant transformation if incompletely excised, leading to increased morbidity in these patients. A review of the consensus guidelines and literature was conducted, and the online literature on the subject from 2002 was included. This article is not a complete review of all the available literature; rather, it is a comprehensive review of the topic.

Management of a giant pleomorphic adenoma of the soft palate: A case report.

Key Clinical Message: Benign mixed salivary gland tumor is comprised of epithelial and myoepithelial cells and represents up to 80% of tumors of the parotid gland. It is relatively rare in the soft palate and in other minor salivary glands. Surgery is the standard care. Abstract: Salivary gland tumors are relatively rare and morphologically diverse group of lesions. Pleomorphic adenoma (PA) is the most common salivary gland tumor, accounting for approximately 80% of all major and minor salivary gland tumors. PA usually affects the parotid gland. Huge PA occurring in soft palate is extremely rare. Patients with these tumors are usually between at the age of 40 and 60 years. The tumors exhibit pleomorphic nature microscopically that may pose diagnostic challenges to pathologists as may confuse PA histopathologically with other salivary gland tumors. Surgery is the standard treatment. The purpose of writing this case study is to describe unusual case of a giant PA of the soft palate found in a 44-year-old male successfully managed at our facility.

The Rare Presentation of Multiple Chondroid Syringomas in One Patient: A Case Report.

Chondroid syringomas are rare, benign tumors originating from sweat glands occurring as singular lesions of the head and neck. This case report presents a patient in whom multiple chondroid syringoma lesions were found. Thus illuminating the possibility for such occurrences in the future of which physicians should be aware.

Common Tumor in an uncommon location: Pleomorphic Adenoma of nasal cavity - a case Report.

Pleomorphic adenoma (PA) is the most common benign salivary gland tumor of which parotid gland involvement is the most common. PA may arise from minor salivary glands as well, however, PA is very rare in the sinonasal and nasopharyngeal areas. It usually affects middle aged females. They are frequently misdiagnosed due to high cellularity and myxoid stroma, leading to delay in diagnosis & further appropriate management. Here we present a case report of a female who presented with gradually progressive nasal obstruction, on examination found to have a nasal mass in right nasal cavity. Imaging was done and the nasal mass was excised. Histopathological report revealed a PA. Common tumor in an uncommon location: Pleomorphic adenoma of the nasal cavity - a case report.

Thirty synchronous medullary and papillary thyroid carcinomas.

Synopsis for table of contents: An exceptional number of synchronous MTC/PTC in the same thyroid gland is presented. This may be the most numerous case series reported in the literature. Synchronous PTC/MTC in the same thyroid gland were classified into 4 subtypes and the clinical and pathological aspects as well as the results are presented. Background and objectives: The synchronous occurrence of multiple neoplastic processes in the thyroid gland is unusual. We investigated the clinicopathological features of 30 medullary thyroid carcinomas (MTC) in association with papillary (PTC). Method: Retrospective analysis of operated patients for thyroid tumors. Synchronous PTC/MTC in the same thyroid gland were classified into 4 subtypes: (type I) True mixed MTC/PTC, MTC and PTC closely intermingled. (Type II) Collision MTC/PTC, i.e. tumors that meet at the same site, invade each other and appear as a single mass in the thyroid gland, i.e. MTC and PTC merge. (Type III) Synchronous anatomically separate tumors in the same thyroid lobe, i.e. separated from each other by non-tumorous thyroid parenchyma. (Type IV) Synchronous tumors occurring in separate anatomical lobes or in the isthmus. Clinical and pathological data were reviewed. Location: Department of thyroid surgery, China-Japan Union Hospital, Jilin University. Time frame: 14 years (June 2008-November 2022). Results: Thirty patients were identified with an overall prevalence of 28621 (0.1%). 17 (56.7%) were male, 13 (43.3%) female, mean age 51.3 ± 11.0 years, mean BMI 23.6 ± 3.6kg/m2. Mean duration of symptoms was 11.2 ± 18.4 months. Mean calcitonin level was 133.7 ± 196.4 pg/ml. Fine needle aspiration (FNA) was offered in 21 cases: 9 (42.9%) were suspected carcinoma, 9 (42.9%) PTC, 1 (4.8%) MTC, 2 (9.4%) MTC/PTC. Pathology revealed type I 4 (13.3%), type II 2 (6.7%), type III 14 (46.7%), type IV 10 (33.3%). The mean diameter of MTC was 1.6 ± 2.0cm, 18 (60%) were micro-MTC. The mean diameter of PTC was 0.9 ± 1.9 cm, 26 (86.7%) were micro-PTC. In 16 (53.3%) micro-PTC/-MTC occurred in synchronous sequence. Four patients had a recurrence: 2 had to be re-operated due to MTC recurrence, 2 died due to distant metastases (bone, liver). Conclusion: We report an exceptional number of MTC/PTC in the same thyroid gland. This may be the most numerous case series reported in the literature. The clinical and pathological aspects as well as the results are presented.

A primary gastric Adenosquamous carcinoma: Case report.

INTRODUCTION: Adenosquamous carcinoma (ASC) is too rare accounting for <0.5 % of all gastric cancers and has a worse prognosis than adenocarcinoma. CASE PRESENTATION: We report a case of a man with digestive symptoms and epigastric discomfort came to a Gastrointestinal clinic. CT scan of the abdomen and pelvis showed a large mass localized in the gastric fundus and cardia. A PET-CT scan showed a localized lesion in the stomach. Gastroscopy revealed a mass in the gastric fundus. Biopsy from the gastric fundus reported a poorly-differentiated squamous cell carcinoma. A laparoscopic abdominal exploration found a mass and Infected Lymphatic Nodes on the abdomen wall. a second biopsy reported an Adenosquamous cell carcinoma grade II. The therapeutic was open surgery and then chemotherapy. CLINICAL DISCUSSION: Adenosquamous carcinoma usually presents at an advanced stage with metastasis (Chen et al., 2015). In our case, the patient presented with a stage IV tumor; including two lymph nodes metastasis (pN1, N = 2/15) and abdominal wall involvement (pM1). CONCLUSION: Clinicians should be aware of this potential site of adenosquamous carcinoma (ASC) because this carcinoma has a poor prognosis even if diagnosed at an early stage.

Carcinosarcoma ovárico asociado a carcinoma seroso intraepitelial tubárico

El carcinosarcoma primario ovárico es una neoplasia de baja incidencia, que suele ser diagnosticado en estadios avanzados y cursa con un mal pronóstico. Se comunica el caso de una paciente de 64 años con una tumoración abdominopélvica de 15 cm. El examen histológico evidenció una neoplasia maligna bifásica ovárica asociada a un carcinoma seroso intraepitelial tubárico, hallazgo que estaría en relación con la patogénesis de esta neoplasia.

Primary ovarian carcinosarcoma is a low incidence neoplasm that is usually diagnosed in advanced stages and has a poor prognosis. We report the case of a 64-yearold female patient with a 15 cm abdominopelvic tumor. Histological examination revealed a malignant ovarian biphasic malignancy associated with a serous tubal intraepithelial carcinoma, a finding that would be related to the pathogenesis of this neoplasm.

Chondroid syringoma of an upper eyelid tumor: Unusual case report.

We present the case report of a 58-year-old man with recurrent chondroid syringoma, which was histopathologically confirmed, who underwent exenteration surgery of the right eye. Furthermore, the patient was receiving postoperative radiation therapy, and presently there is no local and/or distant evidence of disease in the patient.

[Myoepithelial tumors of soft tissue: A case of mixed tumor]. / Tumeurs myoépithéliales des tissus mous : à propos d'un cas de tumeur mixte.

Myoepithelial neoplasms of soft tissue represent a rare entity which has been described only recently when compared to salivary gland tumors with whom they share histopathological and molecular features. The most common locations are the superficial soft tissues of the limbs and limb girdles. However, they can rarely occur in the mediastinum, abdomen, bone, skin and visceral organs. Benign forms (myoepithelioma and mixed tumor) are more frequent than myoepithelial carcinoma and the latter mostly affects children and young adults. Diagnosis is mainly based on histology, which shows a proliferation of myoepithelial cells of variable morphology with or without glandular structures in a myxoid background, and immunohistochemistry, which shows co-expression of epithelial and myoepithelial markers. Molecular tests are not mandatory, but in selected cases FISH analysis can prove useful as about 50% of myoepitheliomas show EWSR1 (or rarely FUS) rearrangements and mixed tumors show PLAG1 rearrangements. Here, we present a case of a mixed tumor of the soft tissue occuring in the hand with expression of PLAG1 in immunohistochemistry.

WT1 as a myoepithelial marker: a comparative study of breast, cutaneous, and salivary gland lesions.

WT1 immunostain is expressed in various benign and malignant neoplasms, as well as normal myoepithelial cells. WT1 shows differential expression in non-neoplastic, benign, and malignant neoplastic myoepithelial cells of the salivary gland. In this study, WT1 immunostain and other myoepithelial markers were compared to investigate the value of WT1 as a myoepithelial marker, and to delineate the expression profile of WT1 in nonsalivary gland myoepithelial cells. WT1, p63, and calponin immunostains were performed on normal and lesional tissues from the breast (adenosis, sclerosing adenosis, lactating adenoma, nipple adenoma, tubular adenoma, adenomyoepithelioma, and adenoid cystic carcinoma [ACC]), skin (cutaneous mixed tumor, hidradenoma, spiradenoma, and ACC), and salivary gland (pleomorphic adenoma and ACC). The stained slides were digitized and orientated with H&E images and assessed simultaneously using QuPath. A total of 129, 58, and 56 breast, cutaneous, and salivary gland lesions, respectively, were included. There was poor agreement between WT1-p63 and WT1-calponin (κ < 0.1) in all organs, with absence of WT1 expression in normal salivary gland myoepithelium and most ACCs. There were no significant differences in WT1 expression in myoepithelial cells in normal breast tissue and benign breast neoplasms. Compared to pleomorphic adenomas, cutaneous mixed tumors showed lower WT1 expression (P < .001). WT1 is a less sensitive myoepithelial marker than calponin and p63. However, its unique pattern of expression in salivary gland primary for pleomorphic adenomas/cutaneous mixed tumor can favor a diagnosis of benign salivary gland tumors, particularly in small biopsy specimens.