In resection margins, we have: intralesional → marginal (option 3) → Extended → Radical (options 1 and 4). This is a high-grade tumor, in which extended resection including tumor-free margins is indicated. Radical amputation surgery has not been shown to improve survival being reserved for selected cases (invasion of neurovascular structures, pathologic fracture...). Therefore, we discard both option 1 and 4; option 3 is also discarded, because it is a smaller margin than the extended one; by elimination we are left with option 2.

In resection margins, we have: intralesional → marginal (option 3) → Extended → Radical (options 1 and 4). This is a high-grade tumor, in which extended resection including tumor-free margins is indicated. Radical amputation surgery has not been shown to improve survival being reserved for selected cases (invasion of neurovascular structures, pathologic fracture...). Therefore, we discard both option 1 and 4; [HIDDEN], because it is a smaller margin than the extended one; by elimination we are left with [HIDDEN].

44-year-old patient with a rapidly growing lesion (16x8x12 cm) involving the soft tissues of the proximal third of the right thigh. He reports that he has started to notice the mass, which has a hard consistency and is adhered to deep planes, in the last 6 months. Previously, she had been a regular athlete and had even finished a marathon. On examination, collateral circulation was observed, but no right inguinal lymph nodes were palpable. After biopsy it was confirmed to be a high-grade undifferentiated pleomorphic sarcoma, which in the imaging studies was found proximal to the lesser trochanter. The extension CT scan is negative. What would be the therapeutic approach?

Treatment with ifosfamide and tamoxifen for 6 cycles prior to resection surgery to reduce the size of the lesion.

Marginal surgery removing the pseudocapsule that these tumors usually form in response to their rapid growth.

[Adamantinoma of the proximal end of the tibia. A case].

Adamantinoma is a rare primary tumour of long bones containing mesenchymatous and epithelial cells. There has been some controversy over the pathogenesis. We observed a localization in the right tibia. A 22-year-old patient from Algeria was seen for spontaneous progressively increasing pain in the upper part of the right tibia. The patient's general health had deteriorated somewhat, with fever. On examination there was ulceration of the skin, costal and pelvic pain and inflammatory right inguinal lymph nodes. Radiography revealed lateral metaphyseal lytic image with interruption of the cortex also seen on magnetic resonance imaging which revealed invasion of the soft tissue and multiple bilateral pulmonary nodules. Bone scintigraphy showed several zones of hyperfixation. The diagnosis of adamantinoma was confirmed by pathology examination of the biopsy specimen. On surgical exeresis, the capsule of the knee joint was found to be involved without invasion of the knee joint. Node dissection showed inguinal and popliteal invasion. Macroscopically, the surgical specimen was a red-whitish osteolytic tumour. Microscopically, the tumour was composed of hyperchromatic epithelial cells in an abondant fibrous stroma. Immunohistochemical studies were negative for vimentine, cytokeratin and factor VIII. Adjuvant chemotherapy was based on a sarcoma protocol. Unfortunately, after two cycles, white cell counts fell sharply and multiple skin nodules appeared together with progression of the bone metastases. The chemotherapy was modified without any therapeutic effect and the patient died in February 1993. An epithelial origin would appear most probable, but at least two groups of adamantinoma can be described: one with typical epithelial differenciation and one overlapping to the differential diagnosis of osteofibrous dysplasia. Although considered as a low grade malignant tumour, we emphasize the aggressive forms with local relapse or metastatic resistance to chemotherapy. Treatment relies on wide surgery and prognosis is generally good. Neither chemotherapy nor radiotherapy has stood the test of time in cases with metastasis.

Femoral nerve compression caused by a hibernoma in the right thigh: a case report and literature review.

A hibernoma, also known as a brown fat tumor, is a rare benign soft tissue tumor, which originates from brown adipose tissue remaining in the fetus after the gestational period. It is often detected in adult men, presenting as a painless slow-growing mass. Hibernomas of the thigh have been reported; however, motor and sensory disorders caused by the tumors compressing the femoral nerve have not been reported. We report a case of a histopathologically proven hibernoma that induced femoral mononeuropathy. A 26-year-old man was admitted to the hospital due to a mass, approximately 11.0 × 9.0 × 4.0 cm in size, that had developed 5 years ago in the anterolateral aspect of the proximal thigh. Furthermore, he had a history of hypoesthesia 1 month prior to his admission. He had signs and symptoms of both a motor and sensory disorder, involving the anterior aspect of the right thigh and the medial aspect of the calf, along the distribution of the femoral nerve. During surgery, the femoral nerve was found to be compressed by the giant tumor. The resultant symptoms probably caused the patient to seek medical care. Marginal resection of the mass was performed by careful dissection, and the branches of the femoral nerve were spared. Histopathology examination showed findings suggestive of a hibernoma. At the 4-month follow-up, no femoral nerve compression was evident, and local tumor recurrence or metastasis was not found. Asymptomatic hibernomas do not require treatment; however, in cases of hibernomas with apparent symptoms, complete marginal surgical excision at an early stage is a treatment option because it is associated with a low risk of postoperative tumor recurrence.

[Synovial sarcoma. Case report].

In 2013 there were 94,770 new cancer patients reported in Hungary. Synovial sarcoma accounts for 0.05-0.1% of all cancers and, therefore its incidence is predicted to be 47-94 patients/year in Hungary. The authors report the history of a 18-year-old man who was operated on a right upper abdominal wall tumor with R1 resection. During the next 5 months the tumor grew up to 8 cm in largest diameter. Histology revealed monophasic synovial sarcoma. Immunohistochemistry showed bcl2, focal CD99 and high molecular weight cytokeratin positivity, while smooth muscle actin, S100 and CD34 immunostainings were negative. Becose of this reoperation was not possible, curative six cycles of doxorubicine and ifosfamide with granulocyte colony stimulating factor support and 60 Gy radiotherapy was given to the tumor bed. After these treatments computed tomography scan was negative and the patient attended regular imaging every 3 months. At the age of 20 years the patient developed two neoplastic lesions in the surgical scar measuring 10 mm and 45 × 10 mm in size. R0 resection, partial rib resection and abdominal wall reconstruction were performed. Histology confirmed residual monophasic synovial sarcoma. Radiotherapy was not given because of a risk of intestinal wall perforation. Staging positron emission tomography-computed tomography proved to be negative. At the age of 22 years magnetic resonance imaging scans indicated no tumor recurrence, but after one month a rapidly growing tumorous lesion was found on ultrasound in the surgical scar measuring 20 × 20 × 12 mm in size. Cytology confirmed local recurrence and fluorescence in situ hibridization indicated t(x;18). R0 exstirpation and partial mesh resection were performed and histology showed the same monophasic synovial sarcoma. Because of the presence of vascular invasion and a close resection margin (1 mm) the patient underwent 3 cycles of adjuvant chemotherapy (doxorubicine and ifosfamide) with granulocyte colony stimulating factor support and 3 cycles of ifosfamide. After 2 years follow up at the age of 24 years, imaging studies did not reveal any local or distant recurrence.

Five-year evolution of a telangiectatic osteosarcoma initially managed as an aneurysmal bone cyst.

We present the clinical, radiographic, and pathologic features of a telangiectatic osteosarcoma (TOS) of the right femoral neck in a 20-year-old man which was initially diagnosed and managed as an aneurysmal bone cyst (ABC). The lesion recurred twice. At the second recurrence TOS was diagnosed. The first local recurrence was recognized 4 years 8 months after the first operation. The clinical diagnosis for the recurrent lesion was recurrent ABC, and curettage and bone graft with internal fixation were performed. The second local recurrence was observed 8 months after the second surgery. The right lesser trochanter appeared destroyed on the radiograph, and a large medial soft tissue mass was demonstrated by computed tomography. The patient underwent wide resection of the tumor with prosthetic replacement of the right proximal femur. The histologic section for this lesion showed a blood-filled cystic lesion, and its wall contained sarcomatous cells with atypical mitoses and tumor osteoid. The histologic diagnosis for the second recurrent lesion was high-grade TOS. The retrospective review of the histologic section for the primary lesion showed similar features to ABC except for a few bizarre cells without mitosis in the tissue of cystic wall. The patient demonstrates no evidence of disease 13 months after the last surgery without adjuvant therapy (he declined chemotherapy).

InternalMed_Harrison

AJCC stage I patients are adequately treated with surgery alone. Stage II patients are considered for adjuvant radiation therapy. Stage III patients may benefit from adjuvant chemotherapy. Stage IV patients are managed primarily with chemotherapy, with or without other modalities. Soft tissue sarcomas tend to grow along fascial planes, with the surrounding soft tissues compressed to form a pseudocapsule that gives the sarcoma the appearance of a well-encapsulated lesion. This is invariably deceptive because “shelling out,” or marginal excision, of such lesions results in a 50–90% probability of local recurrence. Wide excision with a negative margin, incorporating the biopsy site, is the standard surgical procedure for local disease. The adjuvant use of radiation therapy and/or chemotherapy vascular structures are involved so that resection will result in serious functional consequences to the limb.

[A malignant lymphoma (non-hodgkin lymphoma, follicular type) patient who achieved complete remission by radiochemotherapy].

A 54-year-old female patient visited our hospital as an outpatient in August 2008 for her growing mass thigh, without pain, redness or fever. She had suffered discomfort from that swelling since she had been diagnosed with erysipelas by a dermatologist she had visited 6 months before and received some medication. Suspicious of a subcutaneous soft tissue tumor, we performed a biopsy, and histological examination of the lesion indicated malignant lymphoma (follicular lymphoma grade 2). PET-CT (as of September, 2008) showed abnormal migration at the right inguinocrural and right external iliac lymph nodes. In view of her age and the that rapid exacerbation, we considered chemotherapy (rituximab, cyclophosphamide, adriamycin, vincristine and prednisolone: R-CHOP) and involved field radiotherapy (IFRT) better to perform than 'watchful wait'. PET-CT (as of November, 2008) after 3 courses of R-CHOP therapy showed abnormal migration had been significantly improved (complete remission on PET-CT) and completely disappeared in the right external iliac lymph nodes. For long-term prognosis and prophylaxis of recurrence, we then added 2 courses of chemotherapy (rituximab alone) and IFRT (30 Gy/20 Fr) as radical therapy. She is now doing well and visits our hospital once a month for follow-up after achieving CR with RECIST guideline.

From Morphea to Dermatofibrosarcoma Protuberans.

Dear Editor, Morphea profunda (MP) is a chronic autoimmune disease, a subtype of localized scleroderma that presents clinically as local discomfort due to the impairment of skin motility (1). Dermatofibrosarcoma protuberans (DFSP) is a rare soft tissue neoplasm that not only infiltrates the dermis and subcutaneous tissue, but can also affect the muscles and bones with finger-like extensions, usually present on the trunk and the proximal extremities (2). DFSP is known for its indolent clinical course, locally aggressive behavior, and high local recurrence rates, but relatively low risk of metastatic spread (2). DFSP frequently arises in middle-aged adults, affecting both sexes equally with an incidence of 4 per 1,000,000 people (3). We report the case of a 39-year-old female patient who first presented to our clinic at the age of 20 years due to a brownish atrophic coin-sized lesion appearing on the left side of the abdomen. Medical reports indicated that biopsies had been performed previously on 3 occasions, and histopathologic findings confirmed the diagnosis of MP. The aforementioned lesion on the abdomen had been growing slowly over the years, and the patient finally visited our clinic 15 years later after noticing two palpable nodules developing within the affected skin (Figure 1, A, B). Clinical examination revealed an indurated ill-defined plaque measuring 10 cm with partially atrophic surface and 2 centrally located palpable nodules measuring between 3 and 5 mm. A deep biopsy of the lesion was performed, and histopathology and immunohistochemical analysis of CD34 expression confirmed the diagnosis of dermatofibrosarcoma protuberans (Figure 1, C, D). Computed tomography scans of the thorax, abdomen, and pelvic region were subsequently performed, revealing no further disease progression. Complete excision of the tumor was performed and followed by wide scar re-excision due to narrow surgical margins of only 1 mm. No further disease progression or recurrences have been noted during the follow-up, and the patient has been disease-free for one year postoperatively. Although the etiology of DFSP is unknown, trauma has been hypothesized as a predisposing factor. It usually presents on the trunk and the proximal extremities (4). Patients usually report disease progression over a long period of time, ranging from several months to years. The tumor is associated with variable color changes, even proximal skin discoloration, and often presents with a slowly growing indurated dermal plaque or firm nodule attached to the skin (4). Clinically, it can be difficult to distinguish DFSP from a wide number of diagnoses, including morphea, idiopathic atrophoderma, atrophic scar, anetoderma, lipoatrophy, cellular dermatofibroma, fibrosarcoma, malignant fibrous histiocytoma, atypical fibroxanthoma, desmoplastic melanoma, Kaposi sarcoma, and solitary fibrous tumors (5). Immunohistochemistry staining for CD34 cells can be helpful in differentiation, since spindle cells stain positively in DFSP (6). Due to alteration of dermal collagen, histopathological differential diagnoses of DFSP includes lichen sclerosus, atrophic scars and keloids, as well as morphea (7), atrophic dermatofibroma, and undifferentiated pleomorphic sarcoma (6). The mainstay of DFSP treatment is tumor excision performed either by wide local excision or Mohs surgery and having surgical margins between 1 and 5 cm. Several studies have confirmed that patients treated with the Mohs technique have significantly lower recurrence rates (8). Due to the high number of unsatisfactory primary excisions, wide free surgical margins are important for disease control (3). Radiotherapy might be considered as a therapeutic option for inoperable tumors or relapses, as well as an adjuvant therapy after primary excision or re-excision with positive margins (8). Furthermore, recent findings indicate positive therapeutic efficacy after administration of imatinib mesilat - a tyrosine kinase inhibitor due to over expression of PDGFβ (9). Clinical follow-up of patients with DFSP after tumor excision should be performed every six months for the first five years, followed by yearly intervals thereafter for up to 10 years (3). Previous case reports have claimed that the diagnosis of DSFP is commonly delayed as a result of slow tumor growth and nonspecific initial clinical findings (10). To the best of our knowledge, our case is the first description in the literature of DFSP developed within a MP plaque. We speculate that trauma from repeated punch biopsies taken from the sclerotic morpheaform plaque may represent the trigger for the development of the DFSP. Another notable clinical challenge was the surgical excision itself, since the majority of cases presented in literature mentioned unsatisfactory resection margins and a high risk of local disease recurrence. Although complete excision of the neoplasm was performed, re-excision was performed in order to provide wider resection margins. Surgical resection remains the main treatment for dermatofibrosarcoma protuberans, with the main challenge being the achievement of clean excision margins. Proper management of the disease and continuous follow-up are important in order to prevent local recurrence of dermatofibrosarcoma protuberans or its potential metastases.

[Treatment of a patient with giant solitary fibrous tumor of the left hip].

The authors report surgical treatment of a 59-year-old female with a giant solitary-fibrous tumor of the left hip. Previous excision of hip hemangioma was performed in 2016. Recurrent tumor with fast growth has occurred since 2018. The neoplasm consisted of 2 tumors connected by vascular structures. CT revealed soft tissue neoplasms in the upper third of the left thigh (anterior, medial and dorsal regions). Large tumors with an isthmus along the inner surface had tuberous contours and internal septa. The last ones and walls accumulated contrast agent. No structural damage to femoral muscles was observed. The patient underwent resection of tumor and vascular ligation (great saphenous vein and its tributaries along the anterior surface, arteries from the deep femoral artery system along the posterior surface of tumor). There were no early postoperative complications. There are no complaints within 6 month after surgery. There is no lower limb dysfunction.

Hemipelvectomy to treat sarcoma of the proximal portion of the femur in a rabbit.

A 7-year-old sexually intact female rabbit was admitted to the hospital because of a 6-month history of chronic right pelvic limb lameness. Clinical examination revealed a prominent right pelvic limb lameness and signs of pain on manipulation of the right hip joint, with a focal, well-defined soft tissue mass palpable in the right pelvic area. Pelvic radiography revealed a lytic hip joint lesion and CT detailed an expansile lesion within the proximal portion of the femur with an appearance consistent with a soft tissue mass. Histologic evaluation of incisional biopsy samples of the soft tissue mass revealed a poorly differentiated sarcoma. A hemipelvectomy was performed, and histologic evaluation of the soft tissue mass confirmed the diagnosis, with tumor-free margins achieved. The patient recovered well from surgery and had good mobility. The patient survived 21 months after surgery and died of a non-cancer-related disease. Anatomic dissection was described in a cadaver rabbit to aid future surgeries. To the authors' knowledge, this was the first report of a hemipelvectomy performed in a rabbit. Hemipelvectomy is more routinely performed in canine and feline patients, but with the right candidate and owner commitment to aftercare, it may be safely and successfully performed in rabbits.

[Methods of treatment of tumors of the soft parts and bones of the limbs and extremities. Analysis of case records].

218 patients examined for localized cancer of the extremities (50% benign, 50% malignant) are reported. The incidence is considered by sex and age. Demolition surgery was carried out in 35 patients and 27 locoregional treatment with antiblastics and radioactive isotopes was employed. Conservative surgery was used in 158. Operative mortality was less than 1% (2 cases out of 218) and did not reach 6% if amputations and disarticulations alone are considered. There were no deaths in cases submitted to radiochemotherapy and inguinocrural hymphadenectomy. The series includes a 15-years survival for giant-cell tumour of the femur treated with radioactive microsphores, an 11-years survival for a popliteal melanoma submitted to radiocolloid infiltration, a 9-year survival for femoral osteosarcoma for which hip disarticulation was carried out and a 4-year survival for a recurrent rhabdomyosarcoma of the hip operated by hemipelvectomy.

Current and Emerging Therapies for HER2-Positive Women With Metastatic Breast Cancer.

<bCASE STUDY</b DE, a 31-year-old premenopausal woman with a nonsignificant medical history, noticed a right breast mass after playing basketball in September 2011. She initially attributed the mass to slight trauma, but after 2 weeks, she realized the mass was increasing in size. Her primary care physician ordered a bilateral screening mammogram and ultrasound. Mammography revealed no evidence of malignancy in the left breast. In the right breast, at the 7 o'clock position, a loose cluster of faint calcifications spanned a 2.2-cm area. Ultrasound confirmed an irregular hypoechoic mass in the right breast measuring 3.5 × 2.7 × 2.8 cm. Ultrasound of the right axilla identified two enlarged right axillary lymph nodes. Ultrasound core-needle biopsy of the suspicious right breast mass confirmed invasive ductal carcinoma, nuclear grade 2, Ki67 index of 55%, estrogen receptor-positive (H score of 180), progesterone receptor-positive (H score of 135), HER2-positive (3+ on immunohistochemistry). Utilizing the TNM (tumor, node, metastasis) staging system, she was clinically staged with a stage IIB (cT2, cN1, M0) invasive breast tumor. The computerized axial tomography (CT) scan of the chest, abdomen, and pelvis demonstrated the known right breast mass and two enlarged right axillary lymph nodes; however, no metastatic disease was noted. Nuclear bone scan revealed no bone metastases. Her medical oncologist recommended she receive neoadjuvant chemotherapy. The patient was treated with 6 cycles of neoadjuvant docetaxel at 75 mg/m², carboplatin at an AUC (area under the curve) of 6, and trastuzumab (Herceptin) at 6 mg/kg (TCH), which she tolerated well. She then underwent a right segmental mastectomy with axillary lymph node dissection and was found to have a residual 1.0-cm invasive ductal carcinoma, representing a 60% tumor volume reduction. None of 13 axillary lymph nodes were positive for disease. Pathologic staging confirmed a stage IA (ypT1, ypN0, M0) tumor. DE completed 33 fractions of radiation therapy to the right breast. She initiated endocrine therapy with tamoxifen at 20 mg daily and received 1 year of maintenance trastuzumab (6 mg/kg). Due to vaginal discharge and weight gain, endocrine therapy was switched from tamoxifen to toremifene (Fareston), which she tolerated relatively well. She continued routine follow-up, with no evidence of disease. In September 2014, DE presented to her primary care physician complaining of left hip pain. Magnetic resonance imaging (MRI) of the left hip revealed T2 hyperintense masses within the right anterior superior iliac crest, right sacrum, and left iliac body consistent with skeletal metastases. She was referred back to her medical oncologist, and per National Comprehensive Cancer Network (NCCN) guidelines, a biopsy of the suspicious lesion was obtained. The bone biopsy of the lytic lesion was consistent with metastatic breast cancer, which was estrogen receptor-positive, progesterone receptor-positive, and HER2-positive (3+ on immunohistochemistry). Restaging CT scan of the chest, abdomen, and pelvis revealed new 4- to 6-mm pulmonary nodules, hilar and mediastinal lymphadenopathy, new liver lesions, and bone lesions. Nuclear bone scan confirmed multiple bone metastases of the right and left iliac bones and sternum. Complete blood cell count with differential and complete metabolic panel were within normal ranges. The CA 27-29 tumor marker for breast cancer was elevated at 495 U/mL (normal range, < 37 U/mL). DE was understandably devastated by the new diagnosis. She questioned how the treatment plan was to be established. Her medical oncologist struck a somewhat optimistic tone. He explained that metastatic breast cancer was not yet considered to be curable, but periods of disease stability and chronicity were possible. He explained that the HER2 positivity was perhaps the most important factor in delineating her treatment options. He told her that current treatment options were numerous and increasing in number.

[The analysis of the treatment of giant cell tumor of the pelvis and sacrum].

To discuss the surgical management, local recurrence rate and complications of giant cell tumor (GCTs) of the pelvic and sacrum. From December 1997 to December 2005 the data of 46 patients of GCTs of pelvic and sacrum treated were reviewed. There were 25 men and 21 women with the average age of 32 years old ranging from 17 to 64. Out of 46 patients,there were 24 tumors involved the sacrum and the others at the pelvis. According to the site of the tumors on the bone, they was classified into three regions: 8 patients involved region I (ilium), 10 region II (acetabulum) and 4 region III (ischiopubic). Two patients had lesions located at S(1-5), 4 at S(1-4), 12 at S(1-3), 5 at S(1-2) and 1 at S(3-5). Surgical management: 2 patients received 3 times of operations and 7 underwent 2 operations. There were 19 patients managed with intralesional marginal excision and 2 patients with intralesional marginal excision and adjuvant radiotherapy, another 3 patients with widely marginal excision as the treatment of sacral lesions. Nineteen patients underwent the enbloc excision except 2 involved ischium and 1 involved ilium for the managements of pelvic lesions. One recurrent patient with the large, ragged tumor died of serious infection in 2 weeks after the second surgery. The remain 45 patients had followed-up durations ranging from 12 months to 8 years. One patient of malignant giant cell tumor of sacrum died at 15 months after surgery. One patient with postoperation sarcoma underwent reoperation and radiotherapy but died at the 13th month. One patient with sacral lesion occurred pulmonary metastases in two years after surgery, and received chemotherapy with ADM, DDP and IFO. One year later there was no much change in metastatic tumor. One patient with acetabular lesion underwent curettage before local recurrence and pulmonary metastases were found in 2 years. The patient was treated with pulmonary radiation, widely marginal excision and hemi-pelvic prostheses reconstruction. There was no progression in metastatic lesion during the following up. The local recurrence rate of GCT at sacrum was 9/24 (37.5%), and at pelvis was 2/22 (9.1%). However, 2 patients with ischium lesions both recurred after curettage. Nineteen lesions with enbloc excision showed no recurrence at all. The treatment for GCT of the pelvic and sacrum should be more aggressive because of high incidence of local recurrence after intralesional excision. Although it might induce sacral nerve deficit, widely marginal excision is the best surgical procedure because of its low recurrence rate.

Duration of Anti-Programmed Death-1 Therapy in Advanced Melanoma: How Much of a Good Thing Is Enough?

The Oncology Grand Rounds series is designed to place original reports published in the Journal into clinical context. A case presentation is followed by a description of diagnostic and management challenges, a review of the relevant literature, and a summary of the authors' suggested management approaches. The goal of this series is to help readers better understand how to apply the results of key studies, including those published in Journal of Clinical Oncology, to patients seen in their own clinical practice. A 53-year-old healthy man presented with recurrent in-transit melanoma of the right lower extremity. Eight years prior he had undergone wide local excision and sentinel lymph node biopsy for invasive melanoma of the anteromedial aspect of the distal right thigh. Pathology revealed an ulcerated melanoma, Breslow depth 3.5 mm, and with one involved micrometastatic inguinal lymph node. Staging studies did not demonstrate distant metastases. Superficial inguinal node dissection was performed and did not identify any additional metastatic nodes of 14 retrieved for a final pathologic staging of T3bN1aM0 (stage IIIB) cutaneous melanoma. He received 12 months of adjuvant high-dose interferon alfa-2b. Two years later, he developed a 1.2-cm subcutaneous focus of in-transit recurrence approximately 4 cm proximal to the original melanoma site in the right thigh, which was treated with surgical resection followed by adjuvant radiotherapy. Over the next 4 years, he underwent six additional surgeries for isolated in-transit recurrences affecting the same limb. He was referred for therapeutic options at the time of his latest in-transit recurrence. Examination revealed three palpable subcutaneous nodules in the right thigh in the setting of lymphedema. A core biopsy confirmed recurrent melanoma (Fig 1). Whole-body fluorodeoxyglucose positron emission tomography imaging revealed at least 17 hypermetabolic cutaneous and subcutaneous nodules in the right thigh, four fluorodeoxyglucose-avid nodules below the right knee, but no distant metastases (Fig 2A). Brain magnetic resonance imaging was normal. His serum chemistry profile, including lactate dehydrogenase, was normal. Molecular analysis demonstrated presence of BRAF V600E mutation in the tumor. After multidisciplinary evaluation, an isolated limb infusion procedure of the right lower extremity was not believed to be feasible, secondary to the proximal extent of the recurrence. Therapy was initiated with pembrolizumab at 2 mg/kg intravenously every 3 weeks.

Outcome after disarticulation of the hip for sarcomas.

To review the oncological and functional outcome in 80 patients who underwent disarticulation of the hip as part of their treatment. Eighty patients had disarticulation, of whom 46 had a bone sarcoma and 34 a soft tissue sarcoma. In 42 patients the operation was done as the first definitive surgical procedure for that patient. In 38 patients the disarticulation followed local recurrence after unsuccessful limb salvage, three of these patients had palliative amputations already having metastatic disease. All patients had adjuvant therapy when appropriate. The overall survival of the patients following the amputation was 56% at 1 year, 39% at 2 years, 27% at 5 years and 21% at 10 years. The 5-year survival of patients having the amputation as a primary procedure was 32%, for those with local recurrence it was 25% whilst for those with a palliative amputation it was nil. Local recurrence developed in 10 patients following the amputation, and was related to close margins of excision; all of these patients subsequently died. Function was on the whole poor, with only one surviving patient regularly using an artificial limb. Disarticulation of the hip remains a disabling procedure usually carried out for high grade sarcomas with extensive involvement of bone and soft tissues in the thigh. Long term survival is possible if wide margins of excision can be achieved.

Pleomorphic Liposarcoma of Femur: A Rare Soft Tissue Sarcoma Metastasized to the Bone-Case Report and Review of Literature.

Pleomorphic liposarcoma (PLPS) accounts for less than 5% of liposarcoma, and its metastasis to bone is rare. As a high-grade tumor, PLPS is reported to be more invasive with high local recurrence and distant metastasis. Here, we report a case of PLPS of the femur and undertake a review of the literature. <iCase Presentation</i. A 58-year-old man presented with a big mass at posterior aspect of his left thigh. The computed tomography of the chest for staging revealed two nodules at the left upper lung field. Wide resection of the soft tissue mass at the left thigh was performed by a general surgeon. Thoracotomy and wedge resection of the lung nodules was carried out by a cardiothoracic surgeon. Pathologic diagnosis suggested PLPS. Three years later, he was presented with sudden right hip pain after he slipped. The plain radiograph revealed an osteolytic lesion at the right proximal femur with minimally displaced pathological fracture. The MRI showed the presence of a tumor at the proximal part of the right femur and its soft tissue invasion. The patient underwent en bloc proximal femur wide resection followed by cemented long-stem bipolar hemiarthroplasty. The final histopathology report from definite surgery specimen revealed pleomorphic spindle, round, and polygonal cells arranged in sheets, short fascicles, and storiform arrays. There were no complications, adverse outcomes, or recurrence reported at six months after surgery. The patient could walk without gait aid and had good functional outcomes according to the TESS questionnaire. PLPS is a highly aggressive tumor with a high distant metastatic rate. The definite diagnosis of PLPS is made on the basis of histopathology. Surgical treatment involving wide resection that aims to achieve a negative margin is the best option currently available, and we recommend treating bone metastasis from PLPS as the primary site of the tumor. The effect of chemotherapy and radiotherapy in preventing postoperative recurrence is still unclear and requires further studies.

[Partial pelvic resection (internal hemipelvectomy) and endoprosthetic replacement in periacetabular tumors].

Treatment of tumors of the pelvic girdle by resection of part or all of the innominate bone with preservation of the extremity. Implantation and stable fixation using a custom-made megaprosthesis to restore painless joint function and loading capacity. The surgical goal is to obtain a wide surgical margin and local tumor control. Primary bone and soft tissue sarcomas, benign or semi-malignant aggressive lesions, metastatic disease (radiation resistance and/or good prognosis). Limited life expectancy and poor physical status, extensive metastatic disease, persistent deep infection or recalcitrant osteomyelitis, poor therapeutic compliance, local recurrence following a previous limb-sparing resection, extensive infiltration of the neurovascular structures and the intra- and extrapelvic soft tissues. Levels of osteotomy are defined preoperatively by a CT-controlled manufactured three-dimensional 1:1 model of the pelvis. Using these data, the custom-made prosthesis and osteotomy templates are then constructed by the manufacturer. The anterior (internal, retroperitoneal) and posterior (extrapelvic, retrogluteal) aspects of the pelvis are exposed using the utilitarian incision surgical approach. The external iliac and femoral vessels are mobilized as they cross the superior pubic ramus. The adductor muscles, the rectus femoris and sartorius muscle are released from their insertions on the pelvis and the obturator vessels and nerve are transected. If the tumor extends to the hip joint, the femur is transected at a level distal to the intertrochanteric line to ensure hip joint integrity and to prevent tumor contamination. A large myocutaneous flap with the gluteus maximus muscle is retracted posteriorly. The pelvitrochanteric and small gluteal muscles are divided near their insertion in the upper border of the femur. To release the hamstrings and the attachment of the sacrotuberous ligament, the ischial tuberosity is exposed. After osteotomy using the prefabricated templates, the pelvis is released and the specimen is removed en bloc. The custom made prosthesis can either be fixed to the remaining iliac bone or to the massa lateralis of the sacrum. The released muscles are refixated on the remaining bone or the implant. Time of mobilization and degree of weight-bearing depends on the extent of muscle resection. Usually partial loading of the operated limb with 10 kg for a period of 6-12 weeks, then increased loading with 10 kg per week. Thrombosis prophylaxis until full weight bearing. Physiotherapy and gait training. At follow-up, patients are monitored for local recurrence and metastases using history, physical examination, and radiographic studies. Between 1994 and 2008, 38 consecutive patients with periacetabular tumors were treated by resection and reconstruction with a custom-made pelvic megaprosthesis. The overall survival of the patients was 58% at 5 years and 30% at 10 years. One or more operative revisions were performed in 52.6% of the patients. The rate of local recurrence was 15.8%. Deep infection (21%) was the most common reason for revision. In two of these cases (5.3%), a secondary external hemipelvectomy had to be performed. There were four cases of aseptic loosening (10.5%) in which the prosthesis had to be revised. Six patients had recurrent hip dislocation (15.8%). In four of them a modification of the inserted inlay and an implantation of a trevira tube had to be performed respectively. Peroneal palsy occurred in 6 patients (15.8%) with recovery in only two. There were 4 operative interventions because of postoperative bleeding (10.5%). The mean MSTS score for 12 of the 18 living patients was 43.7%. In particular, gait was classified as poor and almost all patients were reliant on walking aids. However, most patients showed good emotional acceptance.

A case of quadruple primary malignancies including breast, tongue, and thyroid cancers and osteosarcoma in a young female without karyotype abnormality.

The patient was a 41-year-old, premenopausal woman with a chief complaint of well-circumscribed palpable, right breast mass without nipple discharge. Although she noticed the lump 3 months previously, the size of the tumor (1.1 × 0.9 cm(2)) had been stable. The patient's mother suffered from gastric cancer. Her previous history of the triple different malignancies was as follows: (1) left osteosarcoma [amputation of left lower leg at 15 years old (y/o)]. After the operation, she was treated with various kinds of anticancer drugs including a total of 45 g ifosphamide and 342 g methotrexate; (2) tongue cancer (right radical neck resection; 23 y/o); and (3) thyroid cancer (right lobectomy; 40 y/o). There was no evidence of recurrence of these malignancies at the present consultation. At the time of tongue cancer operation, chromosome abnormality was investigated, but the results were normal. Physical examination showed a well-delimited, elastic-firm, mobile tumor in the central outer right breast. Regional lymph nodes were not palpable. Mammography showed a focal asymmetry in the right upper breast on the mediolateral oblique view. Ultrasonography revealed a hypoechoic mass with irregular margins. Distant metastases could not be detected by whole-body computed tomography scan. The histology of the Mammotome(®) (vacuum-assisted core needle biopsy) specimen revealed that this tumor was low-grade ductal carcinoma in situ (DCIS). She underwent breast-conserving surgery with sentinel lymph node biopsy. On permanent histopathological examination, the diagnosis of the tumor was intracystic papilloma with low-grade DCIS. Surgical margin was negative, and sentinel lymph node metastases could not be observed. Estrogen and progesterone receptor (ER/PR) were strongly positive, but human epidermal growth factor receptor-2 (HER-2) overexpression was not tested because the lesion was DCIS. She has received no adjuvant therapy and is currently disease free 3 months after surgery.

Primary reexcision for patients with 'microscopic residual' tumor following initial excision of sarcomas of trunk and extremity sites.

Among 404 patients with primary tumors of extremity-trunk sites entered in the Intergroup Rhabdomyosarcoma Study (IRS) (1972 to 1984), 154 were placed in clinical group IIa, ie, with negative nodes but with "microscopic residual" (MR) disease, following the initial excisional (not biopsy) procedure. An elective reexcision of the area of the primary tumor (PRE) was performed in 41 of these patients within 35 days (mean interval, 14 days; SE, 0.9) with no intervening therapy. These procedures consisted of wider excision of the tumor "bed," resulting in a technical transfer of these patients from group IIa to group I, ie, complete excision. This reduced intensity of nonsurgical therapy (irradiation and chemotherapy). Among the 41 patients who underwent PRE, the 3-year survival estimate (Kaplan-Meier) was 91% (SE, 4%). This may be compared with the results in 113 patients who remained in group IIa, in which the 3-year survival estimate was 74% (SE, 4%). A second group for comparison consisted of the 73 patients with trunk/extremity tumors who were placed in group I after a single excisional procedure, ie, no PRE, in whom the 3-year survival estimate was 74% (SE, 5%). Recognized prognostic factors influencing survival in these groups were comparable, with the exception of tumor size, ie, the largest tumors (greater than or equal to 10 cm in diameter) were concentrated in groups I and IIa. When patients with tumors greater than or equal to 10 cm in diameter (9.7% of the total) were removed from all three study groups, patients undergoing PRE had longer survival duration estimates than patients in the control groups.

Fibromyxoid sarcoma in a four-year-old child: case report and review of the literature.

We present a child with a rare and chemotherapy-resistant form of soft-tissue cancer, low-grade fibromyxoid sarcoma, first noted when he was 4 years old. He is the youngest patient reported to date. An 11-year-old white male presented to. The University of Texas M.D. Anderson Cancer Center's Department of Pediatrics with a 7-year history of right thigh mass and pulmonary nodules, confirmed on examination. He had undergone extensive prior chemotherapy and surgery. He received chemotherapy with high-dose cyclophosphamide (7 g/m2) and later etoposide (150 mg/m2/day x 5), with only slight shrinkage of the thigh mass and none in the lungs. Subsequently the tumor in his proximal thigh and his lung metastases were resected, and radiation therapy was administered to the thigh. His disease remained stable for 12 months, but he then developed a pleural-based metastasis on the left side and new bilateral lung metastases also. The tumors on the left side were removed; residual disease is stable after treatment for 6 months with subcutaneous alpha-interferon-2b. Low-grade fibromyxoid sarcoma is very uncommon in children. It grows slowly and metastasizes to distant organs, chiefly to the lungs. It is resistant to conventional chemotherapy, and thus far only surgery seems to have a life-prolonging effect. Newer chemotherapeutic and possibly biologic agents should be tried in future patients, in order to find an effective way to control the disease.

[Tuberculosis of the greater trochanter. Apropos of 2 cases].

The authors report two cases of tuberculosis of the greater trochanter as an uncommon localization in osseous tuberculosis. 1st case: A 77 year old male presented with hip pain and swelling. Laboratory studies revealed an increased erythrocyte sedimentation rate. The patient had a positive skin test reaction for tuberculosis. Radiographs showed greater trochanter remodeling. M.R.I. demonstrated a bilobed abscess. There were enough arguments to initiate medical treatment, before surgery: excision of the abscess and partial trochanteric resection. 2nd case: In a 51 year old male, a large lytic lesion of the whole greater trochanter suggesting benign bone tumors was found an radiographs after a traumatism. Curettage and grafting were undertaken. The final diagnosis was made with the results of the intra-operative samples culture of and led to appropriate medical treatment. The outcome was good in both cases with four years follow-up for the first case, and two years for the second one. Trochanteritis Tuberculosis occurs in 1.8 per cent of bony tuberculosis cases. Local symptoms are discrete and the diagnosis is belatedly made when the tuberculous abscess appears. Radiographs and CT scan show the bony lesions: unevenness of the contours of the trochanter and several gaps in it. MRI shows the abscess and its spread. The microbiological test (when antibiotic therapy has not been initiated) and anatomo-pathological tests confirm the diagnosis. General treatment is based on a six month long specific antibiotic therapy. Local treatment concerns the abscess and the bony lesions. As the case may be; careful curettage is undertaken, or wide excision is performed.

Giant malignant peripheral nerve sheath tumor with cauda equina syndrome and subarachnoid hemorrhage: Complications in a case of type 1 neurofibromatosis.

Type 1 neurofibromatosis (NF1), which mainly involves ectodermal tissue arising from the neural crest, can increase the risk of developing malignant peripheral nerve sheath tumors (MPNSTs), soft tissue sarcomas and subarachnoid hemorrhage. We describe a patient with neurofibromatosis type 1 who developed soft tissue sarcoma, MPNST, and subarachnoid hemorrhage. A 22-year-old male reported right focal seizures consequence to severe headache. He had a weakness in both legs, could walk only with the support of a stick for the last 3 months and suffered from constipation and intermittent urinary retention for the past 1 week. The patient had a history of swelling in the back of left thigh for which surgical resection was done 6 months back. Cutaneous examination revealed multiple nodules of varying sizes all over the body, along with many café-au-lait spots and Lisch nodule in iris. Patient had weakness in bilateral hip abduction, extension, knee flexion, extension and ankle dorsiflexion and plantiflexion. Bilateral ankle reflexes were absent while other deep tendon reflexes were sub-optimal. A noncontrast computed tomography brain indicated subarachnoid hemorrhage in left perisylvian region. Ultrasound of left thigh showed a hypoechoic solid lesion in the posterior aspect of left thigh in muscle plane. Histopathology of the lesion following resection showed features suggestive of a low-grade pleomorphic rhabdomyosarcoma. Histology of cutaneous nodules was consistent with neurofibroma. Magnetic resonance imaging of the lumbosacral spine demonstrated a tumor arising from cauda equina. Histopathological examination of the tumor suggested high-grade MPNST. Unfortunately, the patient's MPNST was inoperable, and he received palliative radiotherapy for local control of the disease. The care of a patient with neurofibromatosis requires a comprehensive multisystem evaluation. MPNST occurs in 8-13% patients with neurofibromatosis. Early diagnosis and surgical resection are key to prolong survival. Though rare, rhabdomyosarcoma can occur with a higher frequency in NF1, necessitating through clinical investigation. Subarachnoid hemorrhage can occur due to aneurismal rupture or vascular friability in NF1 patients.

A novel surgical approach for the treatment of tumors in the lesser trochanter.

The proximal femur is a common location for the development of primary benign bone tumors. However, there is currently no surgical technique designed specifically for treating tumors located in the lesser trochanter. In the present study, a novel procedure was developed for directly exposing the lesser trochanter for surgical intervention. This technique may be particularly suited to treating tumors that extend toward the lateral and anterior forward of the lesser trochanter. The new approach involved passing through the femoral triangle, separating the femoral nerve and femoral vessels (artery and vein) and resecting the tumor between the iliopsoas and pectineal muscles. The procedure was performed on six patients with various types of tumor, including one case with osteoid osteoma, one case with non-osteogenic fibroma, one case with osteoma, one case with liposarcoma and two cases of osteochondroma. The preliminary results indicated that the surgical durations were short (60-100 min), blood loss was minimal (30-200 ml) and that pain relief was achieved following surgery. Only one patient continued to experience mild pain, scoring 18 mm on a visual analog scale. The other patients were fully relieved of pain. Sensory dysfunction was experienced by one patient following surgery, with persistent numbness and paresthesias in the distribution of the femoral nerve. No cases of deep vein thrombosis, femur head necrosis, hip joint degeneration disease or local recurrence were identified in any patients during the follow-up period. In order to clarify the virtual tissue, such vessels, nerves and the available space in our approach area, we collected 20 cadaveric specimens and performed anatomical examinations in and around the formal triangle. The spaces between the femoral artery and femoral nerve were measured and analyzed, with the results demonstrating that a definite space existed. Therefore, the novel approach presented in the study may be useful in the resection of benign tumors and the preoperative palliative resection of malignant tumors. The technique may be particularly suited to tumors extending toward the lateral and anterior of the lesser trochanter.

Radiation recall pneumonitis induced by ifosfamide for malignant soft tissue tumor arising from the chest wall: A case report.

The present study reports a rare case of radiation recall pneumonitis attributed to ifosfamide that was used as a single antitumor agent for a recurrent malignant soft tissue tumor, arising from the chest wall. A 74-year-old man, who had a mass in his right upper chest wall, underwent surgical excision under the diagnosis of chronic expanding hematoma. He was referred to the present hospital with a recurrent tumor as the excised tumor was diagnosed as undifferentiated high-grade sarcoma. The extension of the tumor following previous surgery was aggressive and the tumor margins were unclear on magnetic resonance imaging. Wide excision was done with adequate margins for the new recurrent tumor as it was difficult to set sufficient tumor margins following the previous surgery. Radiotherapy (60 Gy) on his right subclavicular area and chemotherapy with ifosfamide (3.5 g × 4 days/cycle) was started 1 month following surgery to control the growth of residual tumors and to prevent metastases. The third cycle of chemotherapy with ifosfamide (3 g × 4 days) was started 80 days following the final surgery and 15 days following termination of radiotherapy. Following administration, he felt short of breath and dyspnea gradually increased. This condition was diagnosed as interstitial pneumonitis induced by ifosfamide following radiotherapy. Although corticosteroid pulse therapy was performed, he died due to respiratory failure. To the best of the author's knowledge, this is the first case report of radiation recall pneumonitis induced by ifosfamide used as a single anti-tumor agent. Caution is needed when ifosfamide is planned for patients who have undergone previous radiotherapy to the chest.

[Clinical treatment of 37 cases of soft tissue sarcomas in the past 6 years].

Since 1981, 37 cases of soft tissue sarcomas have been treated in our clinic. There were 20 males and 17 females with an average age of 43.5 years old. Histopathological classifications were liposarcomas 9 cases, MFH 8 cases, synovial sarcoma 5 cases, rhabdomyosarcoma 3 cases etc. Twenty-four cases (65%) were located in lower extremity, 11 cases (30%) in upper extremity, and 2 cases (5%) in trunk. All cases were treated with surgical intervention. Postoperative radiation was undertaken in 4 cases and adjuvant chemotherapy in 12 cases, respectively. There were 28 cases (76%) of excisional surgery; i.e., so-called limb salvage operation, and 9 cases (24%) of amputation or disarticulation. In August 1987, 10 cases had died of disease (DOD) and 27 cases were alive, but 4 cases were alive with disease (AWD). Each of the DOD and AWD cases were in stage III (by Enneking's surgical staging system) at first arrival to our clinic, or multioperated cases or cases with rapid distant metastases soon after operation. In conclusion, it was emphasized that in order to improve the clinical results, the soft tissue sarcomas must be treated earlier and that more effective chemotherapeutical protocols should be developed in the future.

Carcinosarcoma of the Tunica Vaginalis Following Radiation Therapy for Localized Prostate Cancer.

Spermatic cord tumors (SCTs) are rare neoplasms with 80% exhibiting benign pathology. Of the malignant SCTs, 90% are sarcomas. To date there has only been one documented case of primary CS of the spermatic cord which occurred in a 40 year old with no reported medical history. A 76-year-old male with a history of biopsy proven Gleason score 7 (3 + 4) prostatic adenocarcinoma underwent external beam radiation therapy (7920 cGy) in 44 fractions in 2004. He presented with a 3 year history of an asymptomatic right hydrocele. Several scrotal ultrasounds had been performed confirming a hydrocele, with the most recent revealing a hydrocele measuring 10 × 14 cm and several epididymal cysts. During the hydrocelectomy a firm suspicious mass was noted inside the tunica vaginalis and involving the spermatic cord. Given the intraoperative findings, decision was made to proceed with radical orchiectomy. Final pathologic examination revealed the tumor to have a biphasic pattern composed of spindled cells and also cells with an epithelioid morphology. Five months following orchiectomy he patient presented with a painful, enlarging right upper scrotal mass. A CT scan revealed new soft tissue lesions suspicious for necrotic lymph nodes within the right inguinal canal. Additional metastatic evaluation revealed multiple new pleural and parenchymal lung nodules. CT guided needle biopsy of a left lung nodule demonstrated spindle cells with high cellularity and areas of necrosis which were histomorphologically similar to the previous scrotal CS. Gemcitabine therapy was subsequently started. Four months following the diagnosis of metastatic CS, the patient expired. Dedifferentiation of prostatic adenocarcinoma to prostatic CS should be considered as a treatment outcome after localized radiation therapy to the prostate due to the highly aggressive nature of metastatic CS.

[Extra-abdominal desmoid tumors: 7-year outcome after surgical treatment in 12 patients].

The purpose of this study was to report the clinical course and outcome in 12 patients with extra-abdominal desmoid tumors. This retrospective series included 12 patients with extra-abdominal desmoid tumors treated between 1987 and 1996. Female gender predominated (8/12) and age ranged from 4 months to 60 years. The tumors were identified in the thoracic wall (n=2), the upper limb (n=4), and the lower limb (n=6) with a bony localization in the upper femoral metaphysis. Magnetic resonance imaging was highly contributive to the search for extension. Surgical treatment was given in all cases. All patients were living at last follow-up. Complete resection was achieved in three patients and incomplete resection in nine. Mean follow-up was seven years. There was one case of sarcomatous transformation and nine cases of recurrence. Mean time to recurrence was one year (range 6 months-4 years). Two tumors, in the forearm and the leg, did not recur. Secondary surgery was not attempted in four patients whose clinical situation has remained stable after recurrence. A second resection was performed for recurrence in four other patients who then remained recurrence-free to last follow-up. One tumor recurred three times after surgical resection. Functional outcome was good in nine patients. The poorly limited nature of extra-abdominal desmoid tumors makes resection a difficult task, particularly when located in the girdles or the most proximal part of the limbs. Wide first-intention surgical resection is recommended but mutilation must be avoided due to the benign nature of the tumor, its slow progression, and the possibility of regression or stability after recurrence. Nevertheless, surgical treatment of recurrent tumors is difficult and often insufficient, compromising functional outcome. Complementary treatment (radiotherapy) has been advocated for better control tumor growth.

Primary great saphenous vein leiomyosarcoma: report of a case.

Leiomyosarcomas rarely arise in primary veins, especially the great saphenous vein. We have found only 20 case reports of leiomyosarcoma arising in the great saphenous vein, most of which manifested as nonspecific symptoms of advanced disease, such as a palpable mass, swelling, and back or abdominal pain. We report the case of greater saphenous vein leiomyosarcoma diagnosed in a 48-year-old man with a 4-month history of an inguinal mass. Ultrasonography and computed tomography showed a 6-cm mass attached to the right superficial femoral vein. Fine-needle aspiration biopsy confirmed that it was a vascular sarcoma. At the time of surgery there was no evidence of distant metastasis; therefore, we removed the tumor en bloc along with the sartorius muscle, inguinal lymph nodes, and 10 cm of the common femoral vein, and replaced the femoral vein with a polytetrafluoroethylene graft. A pathological examination revealed poorly differentiated leiomyosarcoma of the great saphenous vein, involving the deep femoral vein, without lymph node involvement. During follow-up, a thrombosis of the prosthesis developed, followed by proximal stenosis, which was treated successfully with percutaneous transluminal angioplasty. The patient was found to have lung metastases 25 months after surgery and he died about 5 months later.

Surgery of a Rare Case of Multiple Synovial Osteochondromatosis of the Hip Joint.

Primary synovial osteochondromatosis of the hip is a benign disorder, which is not frequently seen clinically. The characteristic of this disease is proliferation of synovium and formation of loose bodies inside the joint. It is known that only the loose bodies derived from synovium were called synovial osteochondromatosis. Although it can take place in any joint, the knee is most commonly affected, involvement of the hip joint is relatively rare. We report a young man who has multiple synovial osteochondromatosis in his left hip. A 21-year-old young man, who had progressive pain and functional impairment of his left hip, came to our hospital. He complained that 2 years ago, he began to feel painful of his left hip joint when walking and squatting. Physical examination found that the motion range of the hip was obviously limited, with internal rotation, abduction in particular. The Harris hip score was just 38 points. X-ray shew multiple osteochondromatosis with mild degenerative osteoarthritis existing inside the hip joint. Magnetic resonance imaging shew that the synovial osteochondromatosis intersperse inside the hip joint. During the operation, posterolateral incision of the hip was chosen with dislocation of the femoral head for total exposure of the hip joint to debride extensive loose bodies and proliferative synovial tissue. Pulse irrigation was applied to flush out the residuals. More than 872 of osteochondromatosis were removed out of the hip joint (some small ones were sucked during the operation). The size of the loose bodies ranged from 3 cm × 3 cm × 2.5 cm to 0.2 cm × 0.2 cm × 0.15 cm. Post-operation radiography shew most pathological tissues were taken out. After the operation, the patient rehabilitated fast. The pain relieved and he could bear weight and walked 3 days postoperatively. 1 and 3 months postoperatively, the patient came to us for visit, and he said that he just had a relatively satisfactory function of his affected hip. He could walk, slowly run, and squat freely. But sometimes he could feel a bit painful on his left hip joint after long walking and fatigue. And sometimes he could feel and hear mild crepitus on the left hip joint. 12 months later, the patient came again and complained that the pain of his operated hip became a bit more serious than before since about 20 days ago. The range (internal rotation, abduction in particular) of this hip joint could not live up to normal, compared with the uninvolved hip in physical examination. X-ray shew osteoarthritis had become a bit severer. The patient accepted the suggestion of prospective total hip replacement. Multiple synovial osteochondromatosis is not a very uncommon condition. However, there may be unusual presentations. It can take place in any joint, the knee is most commonly affected, however, involvement of the hip joint is relatively rare as was seen in our patient.

Retroperitoneal dendritic cell sarcoma: A case report.

Interdigitating dendritic cell sarcoma (IDCS) is a rare sarcoma that originates from interdigitating dendritic cells in lymphoid tissue, the imaging characteristics of which are poorly defined. Pathological examination can identify the tumor, but reports on the imaging characteristics of IDCS are limited. Here, we report a case of IDCS in a 48-year-old female involving the retroperitoneal area. The patient had a lumbar mass on her right lower back for 4 years, and which started increasing in size 1 year before. An irregular soft tissue mass (10.1cm × 8.5 cm in size) in the right lower back of retroperitoneum was detected by CT examination with unclear borders, uneven density, and necrosis. The solid components of the mass were significantly enhanced on postcontrast imaging. The soft tissue was irregular and uneven. Cystic solid masses were observed on MRI examination in the right retroperitoneum, lateral abdominal wall, waist, and back. Necrosis, hemorrhage, and cystic transformation were observed inside the lesion. The cyst wall, separation, and wall nodules were significantly enhanced on the postcontrast image. No distant metastasis was observed. Postoperative pathology confirmed the diagnosis of IDCS. The patient underwent surgical resection. The resected margin was positive, and the patient received adjuvant radiotherapy 2 months after the surgery. Twelve months after radiotherapy, the patient's chest CT showed multiple metastases in both lungs. The patient was started on combination chemotherapy of doxorubicin and ifosfamide, and the follow-up is still ongoing. Imaging provides a unique advantage to determine the extent of the IDCS, the invasion of adjacent tissues, and the presence or absence of distant metastases.

Epithelioid sarcoma in the upper extremity.

Eighteen patients who had epithelioid sarcoma in the upper extremity were divided into two groups on the basis of treatment: marginal resection of the tumor or wide or radical resection of the tumor. Historical, demographic, histological, and staging data were recorded and were correlated with the type of treatment and the outcome. Marginal resection (excision through the reactive zone or pseudocapsule surrounding the tumor, with confirmation of a negative margin by a pathologist), with or without adjuvant therapy, led to a dismal outcome in patients who had been treated in this manner initially or secondarily (fifteen failures after seventeen procedures). Disease-free survival at ten years was significantly improved when wide resection (intracompartmental en bloc excision with a cuff of normal tissue of more than three centimeters) or radical resection was either the initial or the secondary treatment; success then was noted in nine of thirteen operations. Of twelve patients in whom a marginal resection had been done initially, three had had a recurrence, three had had a metastasis, and five had died, at a mean follow-up of seven years (range, twenty-seven months to sixteen years). At a mean follow-up of six years (range, two to fifteen years), only one of five patients in whom a secondary lesion had been treated with marginal resection was free of disease. Of the six patients who had been treated with wide or radical resection initially, none had died, one had had a recurrence, and one had had a metastasis, at a mean follow-up of seven years (range, twenty-two months to fourteen years).(ABSTRACT TRUNCATED AT 250 WORDS)

Radiation-Associated Sarcoma of the Breast in a Patient With a Germline Tumor Protein p53 Mutation.

Radiation-induced sarcoma of the breast is a rare complication that is primarily treated with surgical resection but in patients with advanced disease, a multimodality treatment approach is often required. This case report discusses a 37-year-old female with a history of a pT3N3M0, estrogen receptor (ER)+, progesterone receptor (PR)+, human epidermal growth factor receptor 2 (HER2)+, right breast cancer, and a germline tumor protein (TP) p53 mutation who underwent right modified radical mastectomy, adjuvant systemic therapy, and radiation therapy, and subsequently developed a radiation-induced sarcoma. The patient is a 37-year-old female who has a history of pT3N3M0, ER/PR+, HER2+, and right breast cancer diagnosed in 2014. At the time of diagnosis, she had locally advanced disease and underwent right modified radical mastectomy followed by adjuvant chemotherapy, radiation, delayed right breast implant-based reconstruction, and left breast augmentation with mastopexy. Upon completion of adjuvant chemotherapy, she was started on hormonal therapy. In February 2020, she underwent genetic testing given her early onset of breast cancer and was found to have a germline TP53 mutation. Routine MRI for breast implant evaluation showed two irregular enhancing masses with an additional satellite lesion in the right breast. Right breast ultrasound (US)-guided biopsy revealed two separate foci of high-grade pleomorphic fibroblastic/myofibroblastic sarcoma. Further staging workup with a whole-body MRI was negative for evidence of metastatic disease. Her case was discussed in multidisciplinary sarcoma tumor board and consensus was for surgical resection. She underwent radical resection of the right chest wall masses and subcutaneous tissue, removal of right breast implant and capsulectomy, and left breast mastectomy with left breast implant removal and capsulectomy. The final pathology revealed two separate foci of high-grade pleomorphic fibroblastic/myofibroblastic sarcoma, 1.2 cm and 1.1 cm in their greatest dimensions with negative margins. Her case was re-discussed in multidisciplinary sarcoma tumor board and due to T1 size of the tumors and the negative resection margins, close surveillance with annual whole-body MRI and quarterly chest MRI imaging was recommended. In patients with a germline TP53 mutation and breast cancer, the utilization of adjuvant radiotherapy should be considered cautiously given the increased risk of radiation-associated sarcoma.

[Surgical treatment for malignant soft tissue tumors of the hip].

Surgical treatment for malignant soft tissue tumors has resulted in a marked improvement in the grading of lesions, utilizing the surgical staging system introduced by Enneking. We attempted to divide malignant soft tissue tumors of the hip into four compartments; the anterior compartment, medial compartment including the neovascular bundle, posterior compartment including the sciatic nerve, and intra-bony lesions with tumors. A lesion localized in the anterior compartment would be resected with a wide margin. For resection of a lesion localized in the medial compartment, vascular reconstruction must be performed with replacement using artificial vessels for the femoral vein and the saphena magna femoral artery. As for the posterior compartment, for the lesions are easily resected with or without the sciatic nerve. In the last compartment of intra-bony lesions, disarticulation or endoprosthesis replacement after wide-resection surgery must be performed. In such cases, musculocutaneous reconstruction may be needed.