Clinical Case Reports for the Evidence-Based Era

Spontaneous, nontraumatic retroperitoneal hemorrhage or Wunderlich syndrome (WS) is a rare but potential life-threatening condition. It can prove fatal if not recognized and treated aggressively at the appropriate time. A shock caused by massive retroperitoneal or intraperitoneal haemorrhage may develop in isolated cases [1]. The clinical findings are not specific, which is why the differential diagnosis must be made with a multitude of benign and malignant renal masses until the correct diagnosis can be made by the pathologist. The CT and US images are not characteristic. The most common cause of bleeding is angiomiolipoma. Less often are drug-induced vasculitis, kidney cancer, pheochromocytoma or renal cyst. We present the case of 53-year old man with symptoms of renal haemorrhage in the retroperitoneal cavity as the first manifestation of renal cell carcinoma.

Neurofibromas Plexiform neurofibromas are benign tumors of the peripheral nerve sheath. They can be found as single or multiple nodules, which are part of the of the clinical picture in any type of neurofibromatosis, and can become malignant. Neurofibromas arise from Schwann cells which are part of the nerves of the skin throughout the body, nerves innervating internal organs and cranial nerves. The most characteristic symptoms include the presence of scattered, elongated, resilient subcutaneous nodules. These are fibromas, histologically resembling individual neurofibromas, appearing along the the course of peripheral nerves or cranial nerves. They most often occur on the face and neck. Sometimes they lead to massive enlargement of a limb or other part of the body (neurofibromatous elephantiasis) [1,2]. Another equally common common symptom is skin discoloration, looking resembling coffee spots ("giant café-au-lait spot"), as well as freckle-like lesions localized around the axillae and axillary pits [3]. Neurological symptoms are associated with the compression of neurofibromas on adjacent structures. The patient then complains of neuralgia of the peripheral nerves. In the literature we can find isolated cases of neurofibroma plexiformis without any association with neurofibromatosis type 1 [4-6]. Treatment of patients with plexiform neurofibromas is not well defined defined and is mainly aimed at controlling symptoms. Surgical excision is the only available therapy, as there there are no drugs that can prevent their formation or treat them conservatively conservatively. At present, the results of surgical excision are not fully satisfactory, and these procedures can be complicated due to the size, location, condition of the vessels, involvement of the neural involvement, microscopic staging and high rates of tumor re-growth [7]. It should also take into account the fact that there are known there are many causes causing tumor-like lesions to neurofibromas . These include traumatic soft tissue injuries, arthritis, tendon diseases (e.g. tendinosis, tendon sheath inflammation, tendon rupture), non-neoplastic soft tissue lesions (e.g. ganglion, bursitis, granuloma) , and, less commonly, tumors and their metastases to soft tissues [8]. Focal lesions of soft tissues soft tissues are often the cause of pain and palpable tumor mass [9]. W these cases, especially in the early stages, accurate diagnosis is crucial, to establish a diagnosis and treatment strategy. Therefore, w when confronted with a focal tumor lesion the logical seems is to follow established algorithm of procedure. First, it should provide an accurate description of the lesion in correlation with the patient's age, location, type of involved tissue, the condition of the adjacent environment. For this purpose, in addition to detailed clinical examination, ultrasound should be performed of the occupied area, and if a bony lesion is suspected then an x-ray examination in two projections with adjacent joints. The advancement of bone lesions, it is necessary to deepening diagnosis through CT examination. However, lesion limited only to soft tissues on ultrasound will require MRI diagnosis, which will more accurately visualize the infiltration by its neighborhood. If imaging studies show a high probability of proliferative disease, an important diagnostic element is to obtain material for pathomorphologic examination, taken during open biopsy or oligobiopsy and establish the histological diagnosis [10]. If the neoplastic process is confirmed, it is always necessary to X-ray/CT scan of the chest, ultrasound/CT scan of the abdomen and even even PET to exclude metastases [11]. The second stage of the implemented algorithm dictates that treatment should be planned based on the answers to the following questions: - Is the lesion likely to be a normal variant, i.e., "leave me alone, don't touch me." lesion of a benign type, requiring no further imaging or supplies, but only observation? [12] - Does the lesion have features suggestive of an aggressive nature, and then how far advanced should be treatment? [13]. The heterogeneity of the clinical picture of unidentified focal nodular lesions in the metacarpal region, the variety of available methods of diagnosis do not make the task any easier the task, both at the stage of making the initial diagnosis and during communication between members of the interdisciplinary team, who, on more than one occasion, should handle the treatment. Therefore, it seems important to cooperate on the line of orthopedist-oncologist-pathomorphologist. Two cases of isolated neurofibromatosis of the palm of the hand are described below because of their uniqueness. The second of the described cases indicated an additional possibility of postoperative treatment of the patient in the form of prolotherapy injections to improve the function of the treated limb.

Oncological treatment is associated with a high risk of malnutrition in children. Complications of treatment often prevent oral intake of food, which requires proper dietary modification and consideration of parenteral nutrition.

Breast sarcomas are rare, accounting for 1% of malignant gland tumors and less than 5% of all tumors mesenchymal neoplasms.1 The low incidence makes it difficult to conduct trials randomized clinical trials, and available data include descriptions of retrospective series and single cases. The number of known factors that increase risk of developing the disease is small. A genetic predisposition in the form of syndrome Li-Fraumeni (TP53 gene mutation) increases the risk of primary sarcomas also in the thoracic location.2 Chronic lymphedema occurring in some patients after axillary lymphadenectomy and axillary irradiation predisposes to lymphangiosarcoma in the affected arm (Stewart syndrome and Treves syndrome).3 Secondary sarcomas occasionally occur in breasts previously irradiated after breast conserving treatment (BCT), performed for breast cancer, the most common malignancy in women, and after radiation therapy administered for malignant granuloma.4 The cumulative risk of of radiotherapy-induced sarcoma (RIS) is 3.2 per 1,000 over 15 years vs. compared to 2.3 per 1,000 for primary sarcoma in a population without radiation therapy.5 Compared to women who were not irradiated, patients treated with radiation therapy for breast cancer, the risk of developing radiotherapy-induced hemangiosarcoma (radiotherapy-induced angiosarcoma, RIA) is five times higher 6 , in general, however, the possibility of of developing angiosarcoma after radiation therapy is low and, according to the literature literature, it is 0.03 - 0.2% in more than 10 years of follow-up.7,8 It should be clearly emphasized the benefits enjoyed by women with breast cancer due to radiation, an obligatory part of breast-saving treatment.9 There has been no correlation of the incidence of RIS with the use of chemotherapy.10 For the The diagnosis of radiotherapy-induced sarcoma can be made using criteria proposed by Cahan in 1948,11 as interpreted by Kirova15 (At that time, Cahan described more than a dozen cases of bone sarcomas in patients irradiated according to the treatment standards of the time for benign lesions and bone tuberculosis, the first point in the original description was the non-neoplastic nature of the lesion treated with radiation therapy)11: 1. History of treatment with irradiation 2. asymptomatic latency period between two cancers lasting several years 3. occurrence of sarcoma in a previously irradiated area, 4. Histopathologic confirmation of sarcoma as a second neoplasm.10,11,15 However Cahan described the latency period as "relatively long," amounting to more than 5 years in his paper, the literature a case has been described that appeared before a year, also described as a RIS (RIA).12 Among the histopathological types diagnosed among secondary sarcomas include angiosarcoma, hemangiosarcoma, leiomyosarcoma, malignant fibrous histiocytoma, liposarcoma, and fibrosarcoma. Also described were chondrosarcoma and osteosarcoma. RIS develop most often in the chest wall, less often in the parenchyma of the of the irradiated breast.10 The method of choice for patients with for breast sarcomas is excision of the tumor with a margin of healthy tissue. While in the case of primary sarcomas, follow-up treatment after sparing surgery often consists of irradiation of the operated area, when treating patients with for sarcoma occurring in a previously irradiated area, repeat radiotherapy of the of the same area is not recommended. The optimal type of treatment in such a case is mastectomy in this case.