Endocrine mucin-producing sweat gland carcinoma - newly described skin appendageal tumours

@#Introduction: Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a recently described adnexal tumour with a predilection for the face particularly the eye-lids. Considered to be a precursor lesion of mucinous adenocarcinoma, it may represent part of a morphological spectrum. We described a case of this entity, which we believe is the first case to be reported in Malaysia. Case report: A 59-year-old Chinese male presented with a slow-growing cystic lesion over the left lower lateral canthal region. The lesion became progressively larger and nodular within the last 6 months. Histologically, the lesion is a well-circumscribed intradermal tumour with pushing borders extending into the subcutaneous tissue. The tumour cells were arranged in lobules of solid, papillary and cribriform architecture. The cells displayed uniform, medium-sized, round to oval nuclei with stippled chromatin pattern and ample eosinophilic granular cytoplasm. Intracellular mucin (as highlighted by mucicarmine stain) was observed in areas with focal extracellular mucin seen. Mitotic figures were not particularly impressive. By immunohistochemistry study, the tumour cells expressed ER, PR, CK7, GCDFP-15, mammaglobin and EMA diffusely. Chromogranin A and synaptophysin highlighted a significant number of tumour cells. Discussion: The morphology and immunohistochemical profile similarities between EMPSGC and solid papillary carcinoma of the breast (SPCOTB) makes the former considered as the cutaneous analogue of the latter. In fact, one should rule out the possibility of metastatic SPCOTB before considering the diagnosis of EMPSGC.

Clinicopathological features of stratified mucin-producing neoplastic lesions of the cervix / 中华病理学杂志

Objective@#To investigatethe clinicopathological features of stratified mucin-producing intraepithelial lesion (SMILE) and invasive stratified mucin-producing carcinoma (ISMC) of the cervix with review of the literature.@*Methods@#Sixteen patients with SMILE/ISMC components of the cervix were collected from files in the Department of Pathology at Xijing Hospital from January 2007 to March 2019. Clinicopathological data included age at diagnosis, clinical presentation, histological type, depth of invasion measurement, the status of lymphovascular space invasion (LVSI) and lymph node metastasis, FIGO staging and follow-up. Histochemistry AB and PAS-D staining and immunostaining for cytokeratin (CK) 7, p16, p63, p40, PAX8, MUC6, p53 were performed simultaneously. In addition, twelve cases with invasive endocervical adenocarcinoma were evaluated using three-tiered pattern-based system (also called Pattern Classification).@*Results@#The average age at diagnosis for patients was 49.7 years (range, 33 to 65 years), and vaginal bleeding occurred in the majority of cases (13/16). The characteristic morphology of SMILE and ISMC was present as non-invasive and invasive stratified epithelium that the full-thickness cells contained different amount of cytoplasmic mucin, respectively. Five cases of SMILE coexisted with adenocarcinoma in situ (1 case), high-grade squamous intraepithelial lesion (1 case), and invasive adenocarcinoma (3 cases). Thirteen cases with ISMC components included pure ISMC (3 cases), mixed with usual-type endocervical adenocarcinoma (8 cases) or squamous cell carcinoma (2 cases). All pure ISMC had lymph vascular space invasion and depth of invasion exceeded 10 mm. All eleven cases of invasive adenocarcinoma with ISMC components belonged to Pattern C tumors, which typically showed diffusely destructive stromal invasion, solid or poorly differentiated components. The results of histochemical staining confirmed that SMILE/ISMC cells were rich in acidic and neutral mucin. The immunohistochemical staining for CK7 and p16 was diffusely strong positive in SMILE (4/4) and ISMC (8/8) components. The positive expressions of p63 and p40 was located in peripheral cells of stratified epithelial nests or merely in a few cells of SMILE (1/3) and ISMC (2/8) components. In ISMC tissues, there were partial expression of MUC6 (5/7), focal expression of PAX8 (2/8), and wild-type expression pattern (4/10) or completely negative expression (6/10) of p53 protein. All thirteen patients with follow-up data were alive (mean 50.5 months, range 4 to 140).@*Conclusions@#As new tumor entities, SMILE is a rare and unique endocervical intraepithelial lesion, while ISMC belongs to an invasive leison. Given that the tumors with ISMC components may have aggressive behavior, it is important for clinicians and pathologists to fully understand the clinicopathological features of SMILE and ISMC.

Endocrine mucin-producing sweat gland carcinoma of the eyelid: A clinical and histological conundrum

A 60-year-old male presented with a 6-month-old history of a left upper lid mass. The mass was excised, and histopathological evaluation showed a well-circumscribed, multinodular, intradermal tumor consisting of round-to-oval cells with round nuclei and mucin filled cysts. On immunohistochemical analysis, the tumor cells stained positively for cytokeratin (CK)-7, CK-8, estrogen receptor (ER), progesterone receptor (PR), mucicarmine, synaptophysin, gross cystic disease fluid protein-15 (GCDFP-15), and neuron-specific enolase (NSE). A diagnosis of endocrine mucin-producing sweat gland carcinoma (EMPSGC) of the eyelid was made and at 6-month follow-up, no recurrence was noted. In this communication, we discuss the pathology and treatment options of EMPSGC of the eyelid. Although an uncommon entity, EMPSGC may be considered as a differential when encountered with a suspicious, potentially malignant eyelid mass.

Mucin-producing bile duct tumors / 中华普通外科杂志

Objective To summarize experience of diagnosis and treatment of the mucin-producing bile duct tumors(MPBTs). Methods Clinicopathological features of 7 patients with MPBT undergoing surgery from Nov 2002 to May 2005,were retrospectively reviewed.The clinical radiography characteristics and the resection type were summarized respectively. Results Fluctuant iaundice was the most common manifestation of MPBTs,with different characteristics of magnetic resonance cholangiopancreatography when compared with gallbladder carcinoma,hilar cholangiocarcinoma and distal bile duct cancer.All the 7 patients with MPBT underwent successful surgical resection and were cured. Conclusion Appropriate diagnosis and treatment of MPBTs made it possible to achieve long-term survival of these patients.

Mucin-Producing Intrahepatic Cholangiocelluar Carcinoma Presenting as a Focal Dilatation of the Intrahepatic Bile Duct

A case of a mucin-producing intrahepatic cholangiocellular carcinoma (MPCC) is reported. A 58-year old female presented with epigastric discomfort of several years duration. The physical examination and laboratory findings were normal. Abdominal ultrasonography (US) and computed tomography (CT) showed a focal dilatation of the right posterior intrahepatic bile duct. There was no abnormal mass in the liver parenchyma. Endoscopic retrograde cholangiopancreaticography (ERCP) showed a filling defect in the right posterior hepatic duct. There was no anatomical abnormality and abnormal staining on the heaptic angiography. At the operation, the right posterior hepatic duct was filled with mucin. The patient had a right posterior segmentectomy. Histologically, a 2.5 X 0.6 X 0.6 cm sized mucin-producing intrahepatic cholangiocellular carcinoma was found in segment 6 of the liver. The postoperative recovery was good, and the patient has had a good social life for the last 3 years, with no evidence of tumor recurrence. In patients with a focal dilatation of the intrahepatic bile duct on CT or US with no underlying cause, an intrahepatic malignancy has to be suspected.

Mucin-Producing Bile Duct Tumor

BACKGROUNDS: A mucin-producing bile duct tumor is a rare disease causing diffuse bile duct dilatation and intermittent obstructive jaundice due to mucin accumulation in the bile duct. A massive amount of mucin in the dilated bile duct is confirmed during surgery, endoscopic drainage, or percutaneous drainage. These tumors are usually slow-growing, well-differentiated papillary adenocarcinomas that shows intraductal spreading or an intraductal papillary growth pattern. PURPOSE: The purpose of this study was to evaluate the clinical, radiological, and histopathological characteristics of mucin-producing bile duct tumors. METHODS: We treated 10 patients with mucin-producing bile duct tumors during the recent 5 years. Clinical features were reviewed, including symptoms and signs, radiologic characteristics, operative findings, and pathological characteristics. RESULTS: The main clinical symptoms were recurrent abdominal pain, fever, chill, and intermittent jaundice. The characteristic radiologic findings were marked dilatation of the bile ducts distal to the tumors on computed tomography (CT) and ultrasonography and multiple, large, amorphous filling defects on cholangiography. In three cases, percutaneous transhepatic cholangioscopy (PTCS) was useful not only in making a diagnosis but also in delineating the extents of the tumors. In seven of ten cases involved (70%), the tumors were located mainly in the left intrahepatic duct. Pathologically, six (60%) cases were differentiated papillary adenocarcinoma. CONCLUSIONS: A mucin-producing bile duct tumor should be suspected if the patient has diffuse bile duct dilatation without definite evidence of stones, and it can be confirmed by a large amount of mucin secretion. If this type of tumor is suspected, careful preoperative evaluation, including PTCS, should be considered for making an accurate preoperative diagnosis and for determining the extent of the tumor.

A Case of Conjunctival Mucoepidermoid Carcinoma with invioving Cornea

The mucoepidermoid carcinoma on the cornea and conjunctival is so rare that only a few cases have been reported worldwide and no case in Korea. We experienced that a patient whose complaint was a protruding mass on the conjunctiva, after tumor removal it was histologically defined as mucoepidermoid carcinoma. So we report a case of conjunctival mucoepidermoid carcinoma involving the cornea, with reviewing reference.