Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors originating from perivascular epithelioid cells. In gynecological system, the uterus is one of the most common sites affected by PEComas. Most PEComas are benign, and patients usually have a good prognosis. However, malignant uterus PEComa is rare, and better comprehensive epidemiological investigations are needed. To date, there are a few reported cases of uterus PEComa. We herein report a rare case of malignant PEComa occurred in the uterine corpus and cervix, possibly accompanied by pulmonary lymphangioleiomyomatosis (PLAM). In addition, 55 cases of malignant uterus PEComa were picked out and collected in the data base of PubMed and Medline. On the one hand, the age of onset, population distribution, clinical manifestations, metastatic sites and routes of metastasis were analysed. On the other hand, a summary of the epidemiology, pathogenesis, diagnosis, and treatments of uterus PEComa was given.

UNASSIGNED: Ipsilateral proximal, shaft, and distal femur fractures are extremely uncommon. It might be challenging and contentious to treat ipsilateral multi-level femur fractures. There are still unanswered questions regarding the order of fracture types that should be repaired first and the type of implant that should be used.
METHODS: A twenty-nine-year-old male patient was assessed at the emergency department after a motorcycle accident. The patient had a clearly deformed left lower extremity and was complaining of pain in the left thigh. Preoperative radiographs revealed ipsilateral multi-level femur fracture on the left thigh involved basicervical fracture of femur (AO/OTA 31-B3) with transverse shaft fracture of femur (AO/OTA 32-A3) and extra articular supracondylar femur fracture (AO/OTA 33-A2).
UNASSIGNED: First, we performed proximal femur nail antirotation in order to stabilize the fracture of the femur neck and reduce the incidence of nonunion and avascular necrosis of the femoral head in young adults. The next step to fix the shaft and distal femur fracture was to perform the distal femur locking plate. The EQ5D and Harris Hip Score questionnaires showed improvement after implementing these procedures.
CONCLUSIONS: Ipsilateral multi-level femur fractures have challenges and controversies in their management. In this situation, proximal femur nail antirotation and distal femur locking plates are viable options due to the condition of the injury and the higher risk of negative effects. After all fractures have been fixed, it is important to closely monitor the hip and knee joints to avoid stiffness or contracture.

BACKGROUND: Ossifying fibroma (OF) is a benign lesion could occur in different areas of the body and contain fibers, cells and non-organic materials in non specific amounts. It is growing could be slow or rapid and according to this an array of treatment options must be considered to avoid future complications.
METHODS: In this article, we present a case report of a 40-year-old female patient who visited the dentist with the intention of having a normal check up. Bilateral lesion was observed in the mandible, the patient had no medical history of trauma. The lesion was removed surgically and analyzed histologically and the result was ossifying fibroma in both sides.
CONCLUSIONS: Ossifying fibroma is a rare tumor to be found in the oral cavity, and the family of fibro-osseous lesions (FOLs) has in general the same pathologic features and differ from each other in clinical view so the definitive diagnosis is based on mixture of all these elements. The treatment is complete surgical excision.
UNASSIGNED: 11 cases were discovered and archived since 1968 until now, the distribution of cases is nearly equal in the oral cavity, and the infection of females is higher than males.

UNASSIGNED: Odontogenic keratocyst (OKC) is a developmental odontogenic cyst that usually can be seen within the jaw bones. The cyst arises from the remnants of odontogenic epithelial cells in the jaw bones. In rare cases the cyst can arise in the extraosseous tissues like the gingiva which is the most common site. However, other uncommon sites like oral mucosa and orofacial muscles have been reported.
METHODS: In this article we present a case report of 17-years-old male patient who visited the dentist complaining of a swelling in the right cheek for almost 2 years. He had no medical history with medications or genetic diseases. The mass was removed by the oral surgeon then subjected to histological examination; it was found to be an intramuscular odontogenic keratocyst.
UNASSIGNED: Intramuscular odontogenic keratocyst is a rare cyst that can be seen in the orofacial muscle, and it can be difficult to diagnose when only clinical and radiographic features are based, and the definitive diagnosis is based on histological examination. The treatment is complete surgical excision.
CONCLUSIONS: 39 cases were reported and achieved since 1971 until now, most of them presented in the gingiva and buccal mucosa and extremely rare within the muscles.

暂无摘要。

Becker\'s nevus, also known as Becker\'s melanosis or Becker\'s pigmentary hamartoma, is a concurrent melanosis first described by S. William Becker. It is a type of acquired hyperpigmentation characterized by well-defined, unilateral lesions with regular borders. It is associated with hypertrichosis and hyperpigmented brownish patches with a mean diameter of 15 cm. The shoulder, scapular area, and upper arms are the most commonly affected areas, but it can occur on any area of the body, including the forehead, face, neck, lower trunk, extremities, and buttocks. The lesion usually appears around puberty, and males are more likely to be affected than females. A 27-year-old male of Arabic descent who was medically free presented to the dermatology clinic complaining of bilateral, symmetrical, hyperpigmented patches on the upper back. The lesions started almost at birth, gradually growing in size and darkening in color over time. On local skin examination, the lesions were bilateral, symmetrical, hyperpigmented patches on the upper back. They were both homogeneous and brown in color with irregular borders and blotchy hyperpigmented macules on both sides of the upper back associated with sparse hair development. Histopathological examination revealed epidermal hyperkeratosis, acanthosis, and focal regular elongation of rete ridges with clubbing. Increased basal layer pigmentation was noticed. The dermis showed focal areas with pigment incontinence. Based on the above clinicopathological findings, the patient was diagnosed with Becker\'s melanosis. He was referred to the laser clinic for further treatment.

Turner Syndrome (TS) is a genetic disorder caused by total or partial loss of an X chromosome. The isochromosome X (i(X)) is a known variant of TS, however, double i(X) is a very rare variant, reported very few times in the literature. We report on a rare case of TS with double i(X). This is an 11-year-old female patient , addressed to the medical genetics consultation for short stature and facial features suggestive of TS. We performed a constitutional postnatal karyotype from a peripheral blood sample, with lymphocyte culture, and an R band analysis, performed on 70 metaphases. Metaphases analysis in our patient identified the presence of three cell populations: 45,X[22]/46,X,i(X)(q10)[30]/47,X,i(X)(q10),i(X)(q10) [18]. The first has total chromosome X monosomy, the second with a normal X chromosome and one isochromosome of the long arm of the other X chromosome and the third with a normal X chromosome and two isochromosomes of the long arm of the X chromosome. A control cell culture was performed from a second blood sample of the patient and confirmed the abnormality. This paper will discuss this case in comparison with other rare cases described, as well as the formation of the double isochromosome, based on the literature.

Löfgren syndrome is an acute presentation of sarcoidosis that comprises fever, bilateral and symmetric hilar lymphadenopathies, polyarthritis, and erythema. We present the case of a 34-year-old male patient who presented with ankle monoarthritis without a history of a traumatic event. Contralateral ankle arthritis associated with erythema nodosum and fever developed one week later. Laboratory tests showed anemia, thrombocytosis, and elevated inflammatory parameters. A chest CT revealed symmetrical mediastinal and hilar adenopathies. A transbronchial biopsy was compatible with granulomatous lymphadenitis, and the diagnosis of Löfgren syndrome was confirmed. Our case report and literature review emphasize the wide web of mimicry of acute sarcoidosis. Secondary forms of acute sarcoidosis are likely to benefit from additional and more complex immunomodulatory therapies. Close monitoring and follow-up should be conducted because it is possible that these patients experience higher rates of recurrence or relapse.

BACKGROUND: Lipoma is a benign tumor that arises at the expense of mature adipose tissue, it can occur anywhere in the body that contains adipose tissue. However, the intraosseous lipoma is considered a rare bone tumor, that affects the long and flat bones, but it is uncommon in the jaws. Usually, diagnosis based on clinical and radiographic features is insufficient and histopathology analysis is of utmost importance for the final diagnosis.
METHODS: In this article, we present a case report of a 28-year-old female patient who visited the dentist with the intention of extracting an impacted tooth. She had no medical history with medications or genetic diseases. The tooth was extracted with a small soft mass that was subjected to histological analysis and it was found to be an intraosseous lipoma.
CONCLUSIONS: Intraosseous lipoma is a rare tumor of the oral cavity, and it can be difficult to diagnose when only clinical and radiographic features are based, and the definitive diagnosis is based on histological examination. The treatment is complete surgical excision.
UNASSIGNED: 30 cases were discovered and archived since 1948 until now, most of them were in the posterior region of mandibular, and the infection of females is higher than males with different final sub-diagnosis of types of intraosseous lipoma.

UNASSIGNED: Pterygoid hamulus syndrome, a painful oral and facial syndrome, has been described in literature to be correlated with morphological changes in the length of the pterygoid hamulus of the sphenoid bone.
UNASSIGNED: The current case report describes the treatment for severe, continuous pain in the posterior right palate. Despite numerous conservative treatments given to the patient, no improvement was seen. Cone beam computed tomography (CBCT) measurements revealed an elongation as well as a significant medial deviation of the lower extremity of the medial pterygoid plate. The surgical resection was performed under local anesthesia. The pain subsided two days after the surgery, and there were no relapses in the weeks that followed.
UNASSIGNED: The medial deviation of the hamulus appeared to be important in the etiology of this painful syndrome. Additional research based on CBCT measurements will be required.