What is the optimal (neo)adjuvant strategy of extremity high-risk soft tissue sarcomas (ESTS)?

Currently, the standard treatment for extremity high-risk soft tissue sarcomas (ESTS) combines surgery and pre- or post-op radiation therapy (RT). In some selected cases, chemotherapy (CT) is incorporated into the therapeutic algorithm as a neoadjuvant approach to enable conservative management. Given the risk of local or metastatic relapse, this paper discusses the potential benefits of CT and RT in high-grade ESTs. The role of adjuvant chemotherapy in addition to neoadjuvant CT, the prognostic value of the pathological response to neoadjuvant treatment, and the role for an adjuvant "boost" following resection after pre-operative radiotherapy will be discussed.

[HER2 epidermal growth factor receptor, tests used to look for its amplification in breast cancer: Principles and limitations]. / Récepteur du facteur de croissance épidermique HER2, tests utilisés pour rechercher son amplification dans le cancer du sein : principes et limites.

HER2-positive breast cancer accounts for 20 % of all invasive breast cancers. It is associated with increased recurrence, a higher risk of brain metastases and a shorter overall survival than luminal A and B cancers. The presence of ERBB2 gene amplification is predictive of a good response to anti-HER2 therapies. Determining HER2 status is a hot topic, given recent studies on HER2-low subtypes. Although several techniques are available, the latest 2018 update from the American Society of Clinical Oncology and College of American Pathologists (ASCO/CAP) recommends a first immunohistochemical study followed by a fluorescence in situ hybridisation study indicated only for equivocal cases. This article summarises the different techniques used to determine HER2 status and provides a detailed review of the literature on pre-analytical factors and intrinsic tumour-specific factors that can distort or complicate the interpretation of results.

Exploring real-world practices and challenges of sarcoma diagnosis in Morocco: A survey-based study among 144 pathologists.

BACKGROUND AND AIM: Sarcoma diagnosis is a challenge for laboratories of cytopathology and anatomic pathology. We conducted this survey-based study to better elucidate real-world practices and challenges of sarcoma diagnosis.  Methods: This was a cross-sectional study using an anonymous questionnaire. The survey was distributed among pathologists using snowball sampling. Descriptive statistics were used to report the finding of this survey. RESULTS: A total of 144 Moroccan pathologists were surveyed. Most respondents were from academic institutions (41.7%) and those with a specialization in sarcoma diagnosis represented 57%, through training using post-graduate fellowships followed by internships abroad and inter-university diplomas. Remarkably, 36.8% of participants reported no training on sarcoma pathology during their career. Regarding frequency of sarcoma diagnosis, 64.6% of pathologists reported having received less than one case of sarcoma per week and more than 70% did not receive sufficient information from referring clinicians. The majority of pathologists reported their nonattendance in multidisciplinary meetings. Morphology and immunohistochemistry were the most frequently used diagnostic tools, while fluorescence in situ hybridization and other molecular biology techniques were accessible for only 27% and 20.1% of pathologists, respectively. Response time during pathological diagnosis of sarcoma was 14 days according to 52.8% of surveyed participants. In addition, a second opinion was needed for 66% of pathologists. CONCLUSIONS: Specialized training on sarcoma pathology in Morocco is lacking. A national working group on sarcoma pathology is needed to provide second opinions, and therefore, improve the management of this malignancy of poor prognosis. (www.actabiomedica.it).

Trial of a Rare Diagnosis in a Core Needle Biopsy of Breast.

OBJECTIVE: Mucinous carcinoma is a rare, special subtype of breast carcinoma associated with a good prognosis. It often presents a confusing appearance with a benign process. For all these reasons, differential diagnosis could be challenging. CASE REPORT: Eighty years-old woman with a soft, lobulated, and well circumscribed mass at the left breast mimicking a benign process. Pathological examination of a core needle biopsy showed pure mucinous carcinoma. DISCUSSION: It is a disease of elderly patient, over 60 years, and usually occurs in postmenopausal women. It accounts for only 2% of total breast carcinomas. On pathologic analysis, to be defined as pure mucinous carcinoma, a carcinoma must be made up of at least 90% intracellular or extracellular mucin. CONCLUSION: Examination of core biopsy material should be done carefully respecting this criteria to confirm the diagnosis of this rare entity and eliminate differential diagnosis.

Adenoid Cystic Carcinoma of the Uterine Cervix: A Report of 2 Cases.

Adenoid cystic carcinoma is malignant tumor that exceptionally occurs in the uterine cervix. It is mostly seen in postmenopausal women and has an aggressive clinical course. We report two cases of an adenoid cystic carcinoma associated with a high grade squamous intraepithelial lesion and invasive squamous cell carcinoma of the uterine cervix and discuss briefly its clinical and pathological characteristics.

Primary atypical teratoid/rhabdoid tumor of the optic nerve: a rare entity in an exceptional location.

Atypical teratoid/rhabdoid tumors are rare and highly malignant central nervous system tumors. They have no specific radiological features and often present several histological components that make a problem in differential diagnosis with medulloblastoma and primitive neuroectodermal tumors. We present the case of a newborn girl complained of a gradual proptosis of the left eye secondary to an expansive lesional process of the optic nerve. The location at the optic nerve, reported only twice in the literature, and an exclusive rhabdoid appearance on biopsy added additional differential diagnosis problems. The proptosis worsened and the infant died few days after two cycles of chemotherapy. VIRTUAL SLIDES: The virtual slides for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2037718783145212 .

Conjonctival melanoma metastatic to the breast: a case report.

BACKGROUND: Breast metastasis is fairly uncommon and prognosis is dismal. Breast metastasis might be the first symptom or may occur during the course of other malignancies dominantly arising from the contralateral breast. Leukemia, lung cancer and conjunctival melanoma may spread to the breast. CASE PRESENTATION: A 43-year-old female patient was operated on for conjunctival melanoma. After two years the disease progressed quickly and cutaneous nodes appeared on the back and paraumbilical region. Physical and radiological examination showed a breast mass. No palpable lymph's nodes were noted. She underwent an open biopsy. Histopathologic examination and immunohistochemistry confirmed breast metastases from melanoma. During post-operative staging multiple nasopharyngeal and oropharyngeal lesions were also objective. The patient was given palliative dacarbazine (250 mg/m2 per day for 4 days) for 4 cycles. She died 4 months after the diagnosis of breast metastases. CONCLUSION: Histopathological evaluation should be mandatory in patients with medical history of malignancies in order to differentiate new primary tumors, metastases, and benign tumors.

Successful management of metastatic eccrine porocarcinoma.

Eccrine porocarcinoma (EPC) is a rare tumor. It develops from the intraepidermal ductal portion of the eccrine sweat gland. Metastatic disease is rare. We report a new case of metastatic eccrine porocarcinoma with a successful management and a good response to docetaxel. A 54-year-old man was admitted with a mass in the breast. Biopsy specimen found carcinomatous tumor proliferation with large anastomosing ducts. Cellular atypia were noted, with eosinophilic cytoplasm and round to oval nuclei. The tumor showed positive immunoreactivity for ACE and negative to anti-PS-100. Resection was performed. One year later, he presented with local and metastatic recurrences. The patient had received 3 cycles of cisplatin and 5-fluorouracil; he progressed with increase in mass size and number of lung lesions. He has been undergoing three cycles of docetaxel with complete response in the lung and regression of the breast mass. The mass was excised. Porocarcinoma is a very rare entity and poorly understood. In the metastatic phase, it has modest or no sensitivity to anticancer treatment. Docetaxel should be considered in the metastatic eccrine porocarcinoma.

Primary immature teratoma of the uterus relapsing as malignant neuroepithelioma: case report and review of the literature.

Background. Although some mature cases of teratomas have recently been described in the cervix, they are not commonly found in the uterus, especially in immature forms. An immature uterine teratoma relapsing after surgery as malignant neuroepithelioma has never been reported in the literature. Case Presentation. We describe a case of immature teratoma which occurred primarily in the uterus in a 56-year-old female. Treatment consisted of total simple hysterectomy. Three months after surgery, the patient relapsed with voluminous pelvic mass and was treated with bleomycin, etoposide, and cisplatin-containing chemotherapy regimen. Conclusion. In this report and according to the pertinent literature, clinical and pathological features and management of uterine immature teratomas are discussed. The mainstay of treatment is surgery. The prognosis of this unusual disease remains uncertain.