Lichen planus related to transforming growth factor beta inhibitor in a patient with metastatic chondrosarcoma: a case report.

Transforming growth factor-beta1 (TGF-ß1) is expressed in normal epidermis. TGF-ß1 potently inhibits keratinocyte proliferation and immunomodulatory properties, mainly by suppressing immune responses to self-antigens. Lichen planus (LP) is a form of dermatitis caused by cell-mediated immune dysfunction, but the exact pathogenic pathways are unknown, which poses therapeutic challenges. We report on a 68-year-old man who developed multiple pruritic, discrete, and well-demarcated, flat-topped red-purple papules and macules on the back and upper arms following 4 cycles of treatment with TGF-ß receptor I (TGFBR-I) inhibitor, ly3200882, for metastatic chondrosarcoma. The biopsy showed hyperkeratosis, wedge-shaped hypergranulosis, elongation of the rete ridges, and a dense band-like lymphohistiocytic infiltrate admixed with colloid bodies and pigment incontinence, consistent with LP. Temporal correlation suggested that the TGFBR-I inhibitor might be a trigger. Treatment with topical clobetasol and oral metronidazole led to partial resolution of the lesions with postinflammatory hyperpigmentation. We believe this is the first reported case of LP related to TGFBR-I inhibitor therapy. This report expands the list of cutaneous adverse events associated with this novel class of targeted therapy. More importantly, this report supports emerging evidence that failure of TGF-ß1 activation/signal transduction is an important mechanism in the pathogenesis of LP and suggests the TGF-ß1 pathway as a potential therapeutic target in this disease.

Reconstruction of forearm support with ulnar translocation after resection of chondrosarcoma in the proximal radius.

This is a report of the reconstructive surgery of a patient with chondrosarcoma in the proximal radius. After extensive resection of the proximal radius that contained the tumor, the skeleton of the forearm was reconstructed by ulnar translocation. This patient was followed for 2 years, no recurrence of the tumor was found and the function of the forearm was nearly normal. This case is reported and discussed and a literature review is presented.

Chondrosarcoma in the body of the C2 vertebral axis.

Chondrosarcoma, a malignant bone tumor, is rarely encountered in the cervical spine. This article describes a patient whose neck pain and dysphagia were caused by an expansive, destructive lesion with calcification that was located in the body of the axis (C2 vertebra), the first time a chondrosarcoma has been reported in this location.

Extra-intradural extracavernous subtemporal approach for chondrosarcomas: technical note and case report.

BACKGROUND: Skull base chondrosarcomas are rare tumors often invading the petrous apex and cavernous sinus, and many surgical approaches have been described. For most of them, these tumors grow slowly and their partial removal can be a first option before complementary radiotherapy. We described herein a minimally invasive approach that could be useful for soft non-calcified chondrosarcomas. METHOD AND RESULTS: We report a case of right parasellar chondrosarcoma, for which an extra-intradural extracavernous subtemporal approach allowed a safe effective partial removal. CONCLUSION: This surgical approach is indicated in selected cases to obtain good decompression or partial removal of lesions involving the parasellar space and the petrous apex.

Intralesional vs. extralesional procedures for low-grade central chondrosarcoma: a systematic review of the literature.

INTRODUCTION: Chondroid lesions are very common bone tumors. In most cases, they are benign enchondromas (EC) and, in a minor percentage, chondrosarcomas (CSs), the malignant counterpart. In the latter cases, surgery is the mainstay treatment, because they are chemo- and radio-resistant unless dedifferentiation occurs. If resection is recognized as the gold standard for intermediate-, high-grade tumors, and for low-grade chondrosarcoma (LG-CS) located in the spine and pelvis to reduce the risk of local recurrence, there is still no consensus in literature on the treatment of central low-grade chondrosarcoma (cLG-CS) located in the limbs. Our aim is to perform a review of literature on evidence supporting this approach or not. MATERIALS AND METHODS: An electronic research of the medical archives was carried out in March 2017 seeking papers evaluating the results of curettage and resection in cLG-CS. RESULTS: We selected 13 studies corresponding to our criteria. Unfortunately, they were descriptive, retrospective, non-randomized studies. We identified a population of 471 patients for a total of 473 low-grade chondrosarcomas. Two hundred and ninety-nine lesions were treated with curettage and 174 with wide surgery. The two groups were not homogeneous for diagnosis, size and staging, so no comparison between resection and curettage was possible. The global weighted average percentage of local recurrence was 6.7% (20 cases) and 10.9% (19 cases) after curettage and resection, respectively. No cases of metastasis were reported in the group treated with intralesional surgery, compared to five cases reported in the group treated with resection. Indications for surgery were given in most cases based on symptoms and imaging. CONCLUSIONS: The absence of a preoperative histological diagnosis and the lack of a scientific method to conduct the studies do not sufficiently support curettage for low-grade chondrosarcomas. In the absence of this, resection must be considered a general rule for every malignancy. In our opinion, based on the low biological growth rate of low-grade chondrosarcoma, every chondromatous lesion can be followed-up. Biopsies must be performed based on clinical and radiological suspicions such as pain, scalloping or increase in size, rather than on performing a PET scan to evidence more informative high metabolic areas.

[Comparative functional outcome of reverse shoulder prostheses implanted on degenerative and oncological indications]. / Degeneratív és onkológiai indikációk alapján beültetett reverz vállprotézisek funkcionális kimenetelének összehasonlítása.

INTRODUCTION: Several methods (bone graft, endoprosthesis) are used to reconstruct proximal humerus resections due to primary and metastatic tumours. One of the new procedures is the replacement of the joint surfaces and the removed proximal humerus end with reverse shoulder prosthesis, optionally supplemented with bone implantation (composite procedure) in the hope of achieving better shoulder function. AIM: The aim of this study was to compare the shoulder function of the patient group operated by the above procedure with reverse prosthesis to a patient group operated by indication of degenerative shoulder disease (rotator cuff arthropathy). METHOD: The study involved 23 patients (13 men, 10 women) who were operated between 2012 and 2016 in our institution. Among them, 15 had rotator cuff arthropathy indication of reverse prosthesis, while in 8 cases, resection of the humerus and prosthesis implantation were performed due to tumour lesions. The average age of our patients was 62.5 years (17-82) and the average follow-up was 23.5 months (5-57). We performed physical examination, X-ray pictures on their operated arm, and data were recorded and analysed based on functional score systems. RESULTS: The level of postoperative pain was almost the same in the two groups, but only the rotator cuff arthropathy group needed pain medications. In line with our expectations, we received slightly lower values by each of the three scoring systems in case of tumorous patients. The tumorous patients were satisfied with the results achieved. CONCLUSIONS: Following proximal humerus resections, reversed prosthesis implantation with occasional bone allografts is a reliable method of reconstruction, which approaches available functions and reliability as compared to non-tumour-indicating patients. Orv Hetil. 2018; 159(45): 1838-1843.

Reconstruction of Anterior Chest Wall with Polypropylene Mesh: Two Primary Sternal Chondrosarcoma Cases.

　Primary sternal chondrosarcoma is a rare malignant tumor that is refractory to chemotherapy and radiation. Effective therapy is radical resection of the tumor. We present two patients with primary sternal chondrosarcoma who underwent a radical resection of the lower half of the sternum and bilateral ribs, followed by reconstruction with 2 sheets of polypropylene mesh layered orthogonally. The patients have maintained almost the same pulmonary function as preoperative values, with stability of the chest wall. Although there are various ways to reconstruct the anterior chest wall, reconstruction with polypropylene mesh layered orthogonally is an easy-to-use and sufficient method.

Functional results after total cricoidectomy with medial femoral condyle free flap reconstruction.

Our goal was to describe a laryngeal-preserving single-stage procedure for the treatment of low-grade chondrosarcomas of the larynx: a total cricoidectomy with medial femoral condyle flap reconstruction. The study was designed as a case series of three consecutive patients with low-grade chondrosarcomas of the cricoid cartilage lamina, diameter 4.4, 5.2, 3.8 cm, respectively, who underwent total cricoidectomy in 2012. Single-staged reconstruction of the defect with medial femoral condyle flap including the periosteum and thin underlying cortical layer of the bone was conducted. Oncologic and functional results were observed during 3 years of follow-up. No evidence of tumor recurrence was detected during follow-up. One month after surgery, all patients were able to tolerate a soft diet and to speak satisfactorily. None of the patients reported aspiration after surgery nor experienced aspiration-related problems, which was confirmed by means of the Leipzig-Pearson scale. The speech ability was good, maximum phonation time was 14, 18, 21 s, respectively, and the voice handicap index scores ranged from 24 to 36 and had improved noticeably at 1, 3 and 6 months follow-up. Two patients were ultimately decannulated, and one female still has a tracheostomy; however, she is able to keep the tracheostoma closed for most of the time, maintaining good phonatory and swallowing functions. Total cricoidectomy with reconstruction by means of medial femoral condyle flap may replace the total laryngectomy in large low-grade chondrosarcomas of the cricoid cartilage.

[Chest Wall Reconstruction Using Titanium Plates Sandwiched Between Sheets after Resection of Chest Wall Chondrosarcoma].

Extensive chest wall resection carries the risk of difficult reconstruction and surgical complications. We report our experience on chest wall reconstruction using titanium plates for a wide thoracic defect after tumor resection. A 74-year-old man was diagnosed with chondrosarcoma of the 6th rib on the right. He needed extensive chest wall resection because of skip lesions on 4th rib noted on operative inspection, leaving a defect measuring 33 × 20 cm. Reconstruction using 5 transverse titanium plates sandwiched between an expanded polytetrafluoroethylene patch and a polypropylene mesh sheet stabilized the chest wall. This reconstruction allowed successful separation from ventilatory support after operation. The postoperative course was uneventful, and he was discharged on postoperative day 20. The advantages of this form of reconstruction over conventional prostheses are rigidity, and stability and usability.

[Combined Anterior and Posterior Surgical Approaches for Resection of a 2nd-rib Chondrosarcoma Occupying the Superior Sulcus].

A 77-year-old man with right chest wall chondrosarcoma invading vertebral bodies underwent resection. Computed tomography (CT) showed that the tumor occupied the right superior sulcus, and was close to mediastinal organs including the trachea and esophagus. We adopted a combined anterior and posterior approaches that made safe and curative resection possible. In the anterior approach, we dissected and mobilized the neurovascular structures and neighboring organs from the tumor. A-4 cm gutter on the ventral side of the 1st, 2nd, and 3rd thoracic vertebral bodies was created for safe resection. By the subsequent posterior approach, successful resection was achieved without violating tumor margins.

Bilateral chondrosarcoma of the jugular foramen: literature review and personal experience.

Chondrosarcomas (CS) are slow-growing malignant cartilaginous tumors with locally invasive behavior. They account for only 0.15% head and neck neoplasia. There have been no reports in the management of bilateral skull base CS in the literature to date. The synchronous presentation of bilateral CS of the jugular foramen (JF) was diagnosed in a 22-year-old woman with right abducens nerve palsy. Once evaluated the collateral intracranial venous discharge, the lesions were removed in two surgical stages through a bilateral petro-occipital trans-sigmoid (POTS) approach performing a bilateral closure of sigmoid sinus. The patient is disease free 15 years after surgery. No complications occurred. Diplopia improved after excision of the tumor on the right side. A review of relevant English literature was performed. The POTS approach to the JF proved to be safe and effective. Staged radical surgery alone, assessing intracranial venous flow at all stages of surgery, was a valid strategy for bilateral CS, achieving long-term disease control, avoiding early adjuvant radiotherapy, and carrying no complications.

Active raster scanning with carbon ions: reirradiation in patients with recurrent skull base chordomas and chondrosarcomas.

PURPOSE: To evaluate the safety and efficacy of reirradiation with carbon ions in patients with relapse of skull base chordoma and chondrosarcoma. PATIENTS AND METHODS: Reirradiation with carbon ions was performed on 25 patients with locally recurrent skull base chordoma (n = 20) or chondrosarcoma (n = 5). The median time between the last radiation exposure and the reirradiation with carbon ions was 7 years. In the past, 23 patients had been irradiated once, two patients twice. Reirradiation was delivered using the active raster scanning method. The total median dose was 51.0 GyE carbon ions in a weekly regimen of five to six fractions of 3 GyE. Local progression-free survival (LPFS) was evaluated using the Kaplan-Meier method; toxicity was evaluated using the NCI Common Terminology Criteria for Adverse Events (CTCAE v.4.03). RESULTS: The treatment could be finished in all patients without interruption. In 80% of patients, symptom control was achieved after therapy. The 2-year-LPFS probability was 79.3%. A PTV volume of < 100 ml or a total dose of > 51 GyE was associated with a superior local control rate. The therapy was associated with low acute toxicity. One patient developed grade 2 mucositis during therapy. Furthermore, 12% of patients had tympanic effusion with mild hypacusis (grade 2), while 20% developed an asymptomatic temporal lobe reaction after treatment (grade 1). Only one patient showed a grade 3 osteoradionecrosis. CONCLUSION: Reirradiation with carbon ions is a safe and effective method in patients with relapsed chordoma and chondrosarcoma of the skull base.

Chondrosarcoma of bone.

Chondrosarcoma is a cartilage forming neoplasm, which is the second most common primary malignancy of bone. Clinicians who treat chondrosarcoma patients must determine the grade of the tumor, and must ascertain the likelihood of metastasis. Acral lesions are unlikely to metastasize, regardless of grade, whereas axial, or more proximal lesions are much more likely to metastasize than tumors found in the distal extremities with equivalent histology. Chondrosarcoma is resistant to both chemotherapy and radiation, making wide local excision the only treatment. Local recurrence is frequently seen after intralesional excision, thus wide local excision is sometimes employed despite significant morbidity, even in low-grade lesions. Chondrosarcoma is difficult to treat. The surgeon must balance the risk of significant morbidity with the ability to minimize the chance of local recurrence and maximize the likelihood of long-term survival.

Malawer limb salvage surgery for the treatment of scapular chondrosarcoma.

Chondrosarcoma is a common malignant bone tumor, which accounts for 20% of all malignant bone tumors. It often occurs in the long bones, but the incidence of scapular chondrosarcoma is rare. Here, we describe a case of a large chondrosarcoma occurring in the scapula which was treated with Malawer limb salvage surgery. The patient retained considerable limb function after complete removal of the tumor tissue as assessed at the follow-up visit two years and ten months following surgery.

Extraskeletal myxoid chondrosarcoma arising in the femoral vein: a case report.

Soft tissue tumors arising in deep veins of the extremities are uncommon, although a few cases of synovial sarcoma or leiomyosarcoma arising in the femoral vein have been documented. However, to the best of our knowledge, an extraskeletal myxoid chondrosarcoma (EMC) arising in the femoral vein has not been reported in the English literature. We report a case of EMC arising in the femoral vein of a 70-year-old man who presented with right leg edema and was diagnosed with a deep venous thrombosis (DVT) by computed tomography (CT). Magnetic resonance imaging (MRI) revealed a mass in the right proximal thigh that was diagnosed as myxomatous sarcoma by aspiration cytology, and anticoagulant therapy was initiated. The mass was surgically resected en bloc, including the femoral vein and surrounding soft tissue, and the femoral artery was preserved. The femoral vein was not reconstructed. The histologic diagnosis was an extraskeletal myxoid chondrosarcoma. The patient received postoperative local radiation treatment, with a total dose of 60 Gy, and is currently doing well with no evidence of local recurrence or metastasis at 8 months after surgery. In summary, this case report shows that EMC can arise in the femoral vein, and that reconstruction of the femoral vein is not always necessary during surgery for soft tissue tumors.

Chondrosarcoma of the petrous apex with nerve abducens involvement in a woman with pre-existing contralateral amblyopia.

We report about a case of a woman in her 50s with a chondrosarcoma of the petrous apex. She has a strabism due to a right-sided amblyopia since childhood and noticed a slowly progressive squint angle in the last months. Her general practitioner (GP) referred her to an ophthalmologist who diagnosed a left abducens paresis and initiated an MRI demonstrating a mass in the left petrous apex. A transnasal endoscopic transsphenoidal biopsy revealed a grade I chondrosarcoma. The patient underwent an endoscopic transsphenoidal anterior petrosectomy approach with complete removal of the tumour. After surgery the squint angle gradually improved to previous levels, so that the patient was able to resume her work. This report demonstrates that even slight changes of a squint angle without diplopia, in this case because of pre-existing amblyopia, could be a hallmark of an intracranial process.

Surgical management of recurrent thoracolumbar spinal sarcoma with 4-level total en bloc spondylectomy: description of technique and report of two cases.

INTRODUCTION: The descriptions of total spondylectomy and further development of the technique for the treatment of vertebral sarcomas offered for the first time the opportunity to achieve oncologically sufficient resection margins, thereby improving local tumor control and overall survival. Today, single level en bloc spondylectomies are routinely performed and discussed in the literature while only few data are available for multi-level resections. However, due to the topographic vicinity of the spinal cord and large vessels, the multisegmental resections are technically demanding, represent major surgery and only few case reports are available. Surgical options are even more limited in cases of revision surgery and local recurrences when en bloc spondylectomy was considered to be not feasible due to high risk of vital complications in expanding resection margins. Deranged anatomy, implants in situ and extensive intra-/paraspinal scar tissue formation resulting from previously performed approaches and/or radiation are considered the principal complicating factors that usually hold back spine surgeons to perform revision for resection leaving the patient to palliative treatment. METHODS: We present two patient cases with previously performed piecemeal vertebrectomy in the thoracic spine due to a solitary high-grade spinal sarcoma. After extensive re-staging, both patients underwent a multi (4)-level en bloc spondylectomy in our department (one patient with combined en bloc lung resection). Except a local wound disturbance, there was no severe intra- or postoperative complication. RESULTS: After multilevel en bloc spondylectomy both patients showed a good functional outcome without neurological deficits, except those resulting from oncologically scheduled resection of thoracic nerve roots. After a median follow-up of 13 months, there was no local recurrence or distant metastasis. The reconstruction using a posterior screw rod system that is interconnected to an anterior vertebral body replacement with a carbon composite cage showed no implant failure or loosening. In summary, the approach of a multilevel en bloc surgery for revision and oncologically sufficient resection in cases of spinal sarcoma recurrences seems possible. However, interdisciplinary decision making in a tumor board, realistic evaluation of surgical resectability to attain tumor free margins, advanced experiences in spinal reconstructions and involvement of vascular, visceral and thoracic surgical expertise are essential preconditions for acceptable oncological and functional outcome.

Intra-articular extraskeletal myxoid chondrosarcoma of the ankle.

We describe an extremely rare case of an intra-articular extraskeletal myxoid chondrosarcoma causing devastating destruction to the ankle joint in a 71-year-old woman, which had been originally excised as a ganglion cyst. Histological analysis of the lesion revealed an extraskeletal myxoid chondrosarcoma. The patient proceeded to curative surgery with a below-the-knee amputation.

[A unique reconstructive technique in a young patient after manubrial recestion in grade II chondrosarcoma]. / Egyedi mutéti megoldás fiatal férfi betegen manubriumresectio után Grade II chondrosarcomában.

OBJECTIVE: Approximately 30% of malignant, primary bone tumors are chondrosarcomas, which occur on the anterior chest wall most frequently. Patients who are treated with adequate surgical intervention recover well, 10 years survival rate is 97%. Besides the aesthetic outcome, preservation of breathing and loading are crucial. METHODS: Authors present a case of a 44-year-old male patient who worked as a sport masseur operated on Grade II chondrosarcoma of the manubrium. Concerning the age of the patient, an immediate reconstruction was carried out. The involved part of the sternum was resected with wide margins. The reconstruction was performed with Dual Mesh covered by a pedicled left sided pectoral major muscle. As a unique technique, authors used the tendons of the semitendinous and gracilis muscles to fix both claviculas together to give the proper stability and function for the shoulder girdle. RESULT: After an uneventful postoperative period the patient had a fast recovery. CONCLUSION: There are no data in the literature for such a method of fixation of the anterior chest wall. The method is suggested by the authors.

Anterior thoracic wall giant tumor--special surgical procedure.

We describe the case of a 39 years old male known with an anterior thoracic traumatism in 1993 who observed ananterior thoracic wall tumor since 2000. After one and half year of empiric treatment the tumor starts growing very fast, reaching 30/40/35 cm by the date of his hospital admission (march 2003). Due to this accelerate growth and the size of the tumor the patient is proposed for a particular surgical procedure removing the tumor along with the anterior thoracic wall and the reconstruction using "spider web" technique, Thoratex reinforced mesh and muscle flaps. Thisprocedure had agood result, the patient returned to his anterior lifestyle few months after. The patient did not come for follow-up, although he was recommended to come every tree months. Four years after surgery (2007) the patient came back with local recurrence of the tumor. In 2007 the patient underwent another surgical intervention, removing the tumor recurrence. Chest wall tumors are complex surgical conditions requiring complex treatment. The empiric treatment and the fact that the patient did not come as planned for follow-up, prolonged his sufferance, leading to a second surgical intervention.