Malignant eccrine spiradenoma is an extremely rare skin tumor of sweat gland origin. In most cases, it arises in pre-existing benign eccrine spiradenoma. We report an additional case of malignant eccrine spiradenoma. The present case is of a 75-year-old man with malignant eccrine spiradenoma developed in the right shoulder. He had a 6-year history of a recurrent mass that was removed 2 years ago. The patient then underwent a complete excision. The gross pathologic specimen showed a large cutaneous and subcutaneous multinodular tumor, measuring 6 cm in maximal dimension. Microscopically, there were two distinct morphological components: a benign eccrine spiradenoma and a malignant eccrine spiradenoma of low grade with extensive necrosis. Different histologic patterns were observed such as cylindromatous features. Morphological differentiation in malignant eccrine spiradenoma is variable, sometimes with almost complete loss of eccrine differentiation. Extensive sampling to look for a probable previously benign component is necessary. In the other hand, malignant changes can be easily missed without adequate sampling.
BackgroundSince the advent of immunohistochemistry for the diagnosis of stromal tumours, the incidence of leiomyosarcomas has significantly decreased. Nowadays, gastric leiomyosarcoma is an exceptionally rare tumour. We report the second case in the English literature of gastric leiomyosarcoma revealed with massive bleeding and hemodynamic instability and diagnostic pitfalls that we encountered.Case presentationA 63-year-old woman, with 2 years’ history of dizziness and weakness probably related to an anaemic syndrome, presented to the emergency room with hematemesis, melena and hemodynamic instability.On examination, she had conjunctival pallor with reduced general condition, blood pressure of 90/45 mmHg and a pulse between 110 and 120 beats per minute. On digital rectal examination, she had melena. Laboratory blood tests revealed a haemoglobin level at 38 g/L.The patient was admitted to the intensive care department. After initial resuscitation, transfusion and intravenous Omeprazole continuous infusion, her condition was stabilized. She underwent upper gastrointestinal endoscopy showing a tumour of the cardia, protruding in the lumen with mucosal ulceration and clots in the stomach. Biopsies were taken. Histological examination showed interlacing bundles of spindle cells, ill-defined cell borders, elongated hyperchromatic nuclei with marked pleomorphism and paranuclear vacuolization. Immunohistochemistry showed positivity for Vimentine, a strong and diffuse immunoreactivity for smooth muscle actin (SMA). Immunoreactivities for KIT and DOG1 were doubtful.Computed tomography scan revealed a seven-cm tumour of the cardia, without adenopathy or liver metastasis.The patient underwent laparotomy. A total gastrectomy was performed without lymphadenectomy. Post-operative course was uneventful.Histological examination of the tumour specimen found the same features as preoperative biopsies with negative margins. We solicited a second opinion of an expert in a reference centre for sarcomas in France, who confirmed the diagnosis of a high grade gastric leiomyosarcoma.ConclusionGastric leiomyosarcoma is a rare tumour. Diagnosis is based on histological examination with immunohistochemistry, which could be sometimes confusing like in our case. The validation of a pathological expert is recommended.
Several hundred cases of placental hemangiomas have been reported in the literature. However, the umbilical cord is extremely uncommon as a site of occurrence. We present a case of postnatal discovery of giant hemangioma of the umbilical cord (HUM) in a Coronavirus Disease 2019 (COVID 19) positive mother. To our knowledge, this is the first reported case of HUM synchronous to a maternal infection with COVID 19. We aim, through this case and a review of the literature, to study the clinicopathological characteristics of this singular entity. Our patient, a 37‐year‐old woman, presented to the Department Of Obstetrics And Gynecology for respiratory distress and loss of fetal movements. Ultrasound examination concluded to intrauterine fetal desmise. After stabilization of the patient, a cesarean section was performed. A macerated fetus was extracted. Placenta showed a giant mass attached to the cord. It was submitted for pathological examination. Gross examination showed that the umbilical cord was inserted eccentrically with a fusiform dilation. Near its placental end, three cohesive solid angiomatous nodules were noted. Microscopic examination revealed lobules of dilated blood‐filled capillaries set in a myxoid stroma. The diagnosis of HUM have been established. HUM arise from endothelial cells of the umbilical vessels. Their etiology, physio‐pathology and pathways of tumorigenesis are not yet well defined. Further studies are needed to explore the pathways of tumorigenesis and to determin the implication of COVID‐19 in HUM.
Ectopic molar pregnancy is a rare occurrence and consequently not often considered as a diagnostic possibility. We report two cases of molar hydatidiform tubal pregnancy. Diagnosis of ectopic pregnancy was confirmed on clinical biological and sonographic investigations. Diagnosis of molar pregnancy was done on histopathology. The clinical course was favorable for both patients. Although rare, molar changes can occur at any site of an ectopic pregnancy. Clinical diagnosis of a molar pregnancy is difficult but histopathology is the gold standard for diagnosis.
SMARCA4-deficient thoracic sarcoma (SMARCA4-DTS) is a recently identified aggressive subtype of sarcoma. We present the case of a 44-year-old man who underwent a surgery for a perforated small intestine. Compued tomography scan revealed a tissular mediastino–pulmonary mass.Histopathological examination of the intestinal mass shown a malignant tumour with a typical epithelioid and rhabdoid cells, numerous mitoses and large necrosis. A large panel of immunohistochemistry revealed loss of SMARCA4 and SMARCA2 and allowed the diagnosis of SMARCA4-DTS. It is important to consider SMARCA4-deficient thoracic sarcoma in the differential diagnosis of tumours showing suggestive morphologic features in patients of all ages, especially in the case of metastasis associated with thoracic mass.
Neuroblastoma‐like schwannoma (NLS) is a rare variant of a common tumor. The aim of this study is to discuss, through a literature review, the differential diagnoses of NLS while emphasizing the importance of ancillary studies. So far, 24 cases have been reported. We describe an additional case in a 64‐year‐old woman who had a 3‐cm subcutaneous tumor on her flank. The histological examination showed an encapsulated neoplasm. Most of the tumor was made of giant rosettes. These rosettes had several sizes reaching 2.6 mm. They showed a palisade of rounded cells. Nuclei were hyperchromatic but bland. The center was made of eosinophilic cores of collagenous radiating fibrils. Neoplastic cells stained strongly for S‐100 protein. In the capsule, perineural cells stained for epithelial membrane antigen (EMA). No expression of Mucin 4 (MUC4) was present. This was consistent with NLS. The same peculiar appearance of giant rosettes has been described in hyalinizing spindle cell tumor with giant rosettes (HSCT). We propose the term “neuroblastoma‐like schwannoma” when there are small rosettes, “HSCT‐like schwannoma” for tumors with giant rosettes and “collagen‐rich schwannoma” when there are “ill‐defined” structures reminiscent of rosettes. Immunohistochemical panel containing S100, EMA, and MUC4, as well as molecular testing when needed should be performed.
Intranodal palisaded myofibroblastoma (IPM), also known as “intranodal hemorrhagic spindle cell tumor with amianthoid fibers,” is a rare benign mesenchymal tumor originating from smooth muscle cells and myofibroblasts, often with the presence of amianthoid fibers. Usually IPM affects inguinal lymph nodes, but three cases have been described in the submandibular and cervical lymph nodes. We report a new case of a 44-year-old women with submandibular mass. Cervical ultrasound showed a suspect right submandibular adenomegaly. The patient underwent an excision of the submandibular mass. Histological features of the tumor include an encapsulated fusocellular proliferation, with nuclear palisading, amianthoid fibers, hemosiderin pigment, and extravasated erythrocytes. In the light of these results, we made the diagnosis of IPM. No recurrence was found 5 years after surgery.
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