Massive localized lymphedema (MLL) is a rare disease caused by the obstruction of lymphatic vessels with specific clinical morphological and radiological characteristics. People with morbid obesity are mainly affected by MLL. Lymphedema is easily confused with soft tissue sarcoma and requires differential diagnosis, both the possibility of an MLL and also carcinoma manifestations in the soft tissues. The possible causes of massive lymphedema include trauma, surgery, and hypothyroidism. This report is the first case of MLL treated surgically in the Russian Federation. Detailed computed tomography (CT) characteristics and an electron microscope picture of MLL are discussed. A 50-year-old woman (body mass index of 43 kg/m 2 ) with MLL arising from the anterior abdominal wall was admitted to the hospital for surgical treatment. Its mass was 22.16 kg. A morphological study of the resected mass confirmed the diagnosis of MLL. We review etiology, clinical presentation, diagnosis, and treatment of MLL. We also performed an electron-microscopic study that revealed interstitial Cajal-like cells telocytes not previously described in MLL cases. We did not find similar findings in the literature. It is possible that the conduction of an ultrastructural examination of MLL tissue samples will further contribute to the understanding of MLL pathogenesis.

In dermatology, \"pseudo\" is often used as a prefix for entities resembling another standard condition, either morphologically or histopathologically. Correspondingly, \"pseudotumor\" is a term encompassing dermatological conditions which are not true proliferations, but either have a clinical resemblance to a known tumor (e.g., Pseudokaposi\'s sarcoma is actually a non-neoplastic condition) or a histopathological resemblance to one (e.g., pseudo-myogenic hemangioendothelioma named due to a histopathological resemblance between myocytes and tumor cells). Often such a nomenclature can create confusion and unnecessary alarm for both the physicians and the patients. Through this article we attempt to summarise \"pseudotumors\" in dermatology and classify them into clinical and histopathological \"pseudotumors\", so as to produce a ready reckoner for this confusing nomenclature.

Nodular fasciitis (NF) is a benign proliferation of fibroblasts and myofibroblasts occurring most commonly in the upper extremities that can mimic a variety of mesenchymal tumors including sarcoma. Although reported in almost all anatomic locations, only 7 cases of intraneural nodular fasciitis have been reported in English literature. The CTNNB1::USP6 gene fusion has not been previously reported in intraneural nodular fasciitis, although it has been reported in three entities including aneurysmal bone cyst, nodular fasciitis, and intravascular fasciitis. We report a case of a 29-year-old female with a 6-month history of left leg weakness, myalgia, and paresthesia of the left foot prompting a clinical diagnosis of a peripheral nerve sheath tumor. Surgical resection was performed, and histologic sections revealed a circumscribed lesion composed of banal spindle cells with variable interstitial collagen and occasional mitotic figures. By immunohistochemistry, the lesional cells were positive for smooth muscle actin, smooth muscle heavy chain myosin, p16, and H-caldesmon and negative for desmin, S-100, SOX10, HMB45, CD34, and beta-catenin. Fluorescence in Situ Hybridization for USP6 gene rearrangement was positive and consistent with the diagnosis of nodular fasciitis. Next-generation sequencing uncovered the presence of a CTNNB1::USP6 gene fusion involving CTNNB1 gene in exon 1 at the genomic position chr3:41241161 and the USP6 gene in exon 1 at the genomic position chr17:5033231. This gene fusion was confirmed by Sanger sequencing. Herein, we report a case that underscores the rare incidence of intraneural nodular fasciitis and highlights the pitfalls associated with the clinical differential diagnoses of intraneural tumors.

BACKGROUND: Ischemic fasciitis is a rare pseudosarcomatous fibroblastic lesion. It is considered benign and generally occurs in the elderly. Long-term follow-up is rare therefore; the natural history is largely unknown. It is excised without specific attention to margins.
METHODS: We present a case of a 94-year-old female with ischemic fasciitis on the shoulder for which we have three years of follow-up. During this time, multiple re- excisions for recurrence were required. This lesion eventually progressed to myxofibrosarcoma.
UNASSIGNED: We suggest ischemic fasciitis may be a precursor to malignancy. Ischemic fasciitis itself is noted to have features similar to sarcoma on histopathology. Simple excision of ischemic fasciitis without margins may be inadequate therapy.
CONCLUSIONS: Ischemic fasciitis or pseudosarcomatous fibroblastic lesion and myxofibrosarcoma have many histological similarities. A pseudosarcomatous fibroblastic lesion may be a precursor to myxofibrosarcoma and we recommend complete excision with negative margins at the initial treatment to prevent a recurrence.

Proliferative fasciitis (PF) is a rare pseudosarcomatous lesion arising from the subcutaneous fascia and the fibrous septa. Only few hundred cases have been reported in the literature. In the largest series of 53 patients, only two patients had PF lesion arising from the flank. The most common site of origin is extremities followed by abdomen and head and neck. Its origin from the abdominal wall layer and presentation as the fever has been rarely reported in the literature. A PF lesion larger than 5 cm dimension has been sparsely noted. We report the presence of this rare entity in a 68-year-old gentleman who presented to us with low-grade fever and the presence of large lump arising from the abdominal wall. In our patient, the lesion was arising from transervsalis fascia and was excised in toto laparoscopically without damaging the abdominal muscles. It is imperative to differentiate both these lesions from sarcoma on histopathological examination as the follow-up treatment protocols for both vary.

Nodular fasciitis is a benign proliferation of myofibroblastic cells affecting subcutaneous tissue, muscles, and fascia. This rare disorder is most commonly observed on the upper extremity of adults. We present a case of nodular fasciitis of the cheek of a 12-year-old girl.

Massive localized lymphedema (MLL) is a rare disease observed in people with morbid obesity. MLL is easily confused with soft tissue sarcoma. Therefore, MLL is sometimes called as pseudosarcoma in the literature. MLL was initially described by G. Farshid and S. Weiss in 1998. However, etiology of MLL is still unknown despite certain relationships with injuries, operations and hypothyroidism. Since the term «MLL» was introduced only 20 years ago, there are no reliable statistical data on the prevalence of MLL. According to the World Health Organization data (2016), 13% of the adult world population are obese. Therefore, the risk of MLL is increased in these people. Thigh is the most common site of lesion. Abdominal wall lesion is rare and can cause diagnostic difficulties due to large dimensions and appearance. We report a 50-year-old obese woman with MLL of anterior abdominal wall. She experienced discomfort while walking and sleeping due to giant MLL of anterior abdominal wall that significantly reduced quality of life. Removal of MLL (weight 22160 g) was followed by favorable cosmetic and functional outcome. Analysis of differences between MLL and soft tissue sarcoma in the era of «obesity epidemic» is valuable for correct diagnosis and treatment of this rare complication. This report is the first case of MLL, registered in Russia. In our opinion, this is associated with insufficient awareness of physicians about this complication of obesity. We hope our observation will help clinicians to identify and treat this complication.
Массивная локализованная лимфедема (МЛЛ) — редкое заболевание, наблюдается у людей с патологическим ожирением, ее легко спутать с саркомой мягких тканей, в связи с этим в литературе она упоминается под названием «псевдосаркома». С первоначального описания G. Farshid и S.W. Weiss в 1998 г. этиология МЛЛ остается неизвестной, хотя сообщается о связях с травмой, операциями и гипотиреозом. Поскольку термин «массивная локализованная лимфедема» ввели лишь 20 лет назад, достоверных статистических данных о распространенности МЛЛ нет. По данным ВОЗ 2016 г., 13% взрослого населения Земли страдают ожирением, соответственно растет и риск развития МЛЛ у этой группы людей. Бедро — наиболее частый участок поражения. Локализация на брюшной стенке встречается редко и может вызвать диагностические трудности из-за больших размеров и внешнего вида. Приводим клиническое наблюдение МЛЛ брюшной стенки у женщины, 50 лет, с ожирением. Из-за гигантских размеров образования передней брюшной стенки она испытывала дискомфорт во время ходьбы и сна, что значительно снижало качество жизни. После удаления этого образования массой 22 160 г достигнут хороший косметический и функциональный результат. Описание различий между МЛЛ и саркомой мягких тканей в эпоху «эпидемии ожирения» позволяет правильно диагностировать и лечить это редкое осложнение. Данное сообщение является первым случаем МЛЛ, зарегистрированным в России, что, на наш взгляд, связано с недостаточной осведомленностью врачей об этом осложнении ожирения. Надеемся, что наше наблюдение поможет клиницистам выявлять и лечить это осложнение.

Benign myofibroblastic lesions can clinically and histologically be mistaken for sarcoma. Excessive and potentially disfiguring surgical treatment can be avoided by ruling out malignancy. We present the case of a low-grade, myofibroblastic lesion of the lip, which shows how detailed clinical examination rather than reliance on histopathological information alone helped to achieve this. Differential diagnoses of myofibroblastic lesions are also discussed.

BACKGROUND: Carcinosarcoma of the esophagus or esophagogastric junction (EGJ) is a rare malignancy with both carcinomatous and sarcomatous components. There is no report of carcinosarcoma arising from the EGJ wherein the carcinomatous element was adenocarcinoma. We describe a patient with carcinosarcoma of the EGJ in which the carcinomatous element was adenocarcinoma.
METHODS: A 52-year-old man was diagnosed with carcinoma on his EGJ after complaining of appetite loss. All tumor markers (carcinoembryonic antigen, squamous cell carcinoma antigen, alpha-fetoprotein, and carbohydrate antigen 19-9) were within the respective normal ranges. Esophagogastroduodenoscopy showed a 150-mm (100 mm esophageal side and 50 mm gastric side) type 1 tumor on his EGJ. A histopathological examination of a biopsy specimen revealed well-differentiated tubular adenocarcinoma at the gastric side; however, only necrotic tissue was noted on the esophageal side. Contrast-enhanced computed tomography did not reveal any invasion of the adjacent structures; however, it did show five swollen regional lymph nodes. 18F-Fluorodeoxyglucose positron emission tomography with computed tomography did not reveal distant metastases. We performed thoracic subtotal esophagectomy, total gastrectomy, and two-field plus left cervical paraesophageal lymphadenectomy. Macroscopically, the lesion consisted of two components: a 7.5-cm type 2 tumor and a 9-cm type 1 tumor at the proximal end of the type 2 tumor. Microscopically, the type 2 tumor showed predominantly solid or cribriform proliferation of tumor cells with clear cytoplasm, which was moderately differentiated adenocarcinoma with enteroblastic-like differentiation. The tumor cells of the adenocarcinoma component had periodic acid-Schiff (PAS)-positive globules and were positive for sal-like protein 4 (SALL 4) and negative for α-fetoprotein (AFP) or human epidermal growth factor receptor type 2 (HER2). The type 1 tumors consisted of the adenocarcinoma-like type 2 tumor and spindle cells (sarcomatous component). Part of the sarcomatous component showed cartilage differentiation. The type 2 and type 1 lesions were continuous lesions. The epicenter of the tumor was located at the EGJ. The adenocarcinoma component was present in 10 of 27 resected lymph nodes. The tumor was diagnosed as carcinosarcoma of the EGJ.
CONCLUSIONS: We report a rare patient with carcinosarcoma of the EGJ wherein the carcinomatous element was adenocarcinoma.

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