Cytopathology of Chondromyxoid Fibroma: Report of 2 Cases with Immunocytochemical Expression of GRM1.

INTRODUCTION: Chondromyxoid fibroma (CMF) is a rare, benign bone tumor that occurs predominantly in the second and third decades of life, more frequently in males. Overexpression of GRM1 as a consequence of tumor-specific gene rearrangement of GRM1 has recently been reported as a useful immunohistochemical marker for histopathological diagnosis of CMF. However, the usefulness of GRM1 staining of cytology specimens has not yet been evaluated. In this report, the cytological findings and GRM1 immunocytochemistry of two cases of CMF are described. CASE PRESENTATIONS: Case 1 was a 15-year-old girl with a rib tumor. Imaging findings suggested a benign neurogenic tumor such as schwannoma. The tumor had increased in size over a 2-year period and was resected. Case 2 was a 14-year-old boy with a metatarsal tumor involving his left first toe. Imaging findings were suspicious of a benign neoplastic lesion. Biopsy findings suggested a benign tumor, and the patient underwent tumor resection. Cytologically, in both cases the tumor cells were predominantly spindle-shaped or stellate, with a myxoid to chondromyxoid background matrix and multinucleated giant cells, and these matrices were metachromatic with Giemsa staining. Cellular atypia was more accentuated in case 2 than in case 1. Immunocytochemical staining for GRM1 was positive in both cases. CONCLUSION: Due to the overlap in cytological findings, it is often difficult to differentiate CMF from chondroblastoma and chondrosarcoma grade 2. Immunocytochemical staining for GRM1 may support the diagnosis of CMF, and the reuse of Papanicolaou-stained specimens is applicable. The present cases further demonstrated the difficulty of differentiating CMF from other mimicking tumors such as chondroblastoma and chondrosarcoma grade 2. In such instances, immunocytochemistry for GRM1 is applicable to the diagnostic process, the value of which is strengthened by reusing Papanicolaou-stained specimens.

Cancer may accelerate locomotive syndrome and deteriorate quality of life: a single-centre cross-sectional study of locomotive syndrome in cancer patients.

BACKGROUND: Thanks to recent advancement in cancer treatment, an increasing number of cancer patients are expected to live longer with cancer. The ambulatory ability is essential for cancer patients to spend their own independent lives, but locomotive syndrome (LS), a condition of reduced mobility due to impairment of locomotive organs, in cancer patients has been seldom examined. METHODS: This was a single-institutional cross-sectional study. Cancer patients receiving cancer therapy between April 2020 and March 2021 were asked to participate. LS was classified as stage 0-3, and compared with their performance status (PS). Physical component summary (PCS) and mental component summary (MCS) were calculated from the results of Short Form-8. Logistic regression analysis was performed to identify risk factors for LS stage 3. RESULTS: One hundred and seventy-six cancer patients were included. The rate of LS was 96.0%. That of LS stage 3 was 40.9% and as high as 29.7% even if limited to those with PS 0. The mean PCS and MCS were both inferior to the national averages. PCS decreased as the LS stage advanced. Old age and underweight were revealed as independent risk factors for LS stage 3. CONCLUSIONS: The ratio of LS in cancer patients was extremely high, and the LS stage correlated with physical QOL. Even those with PS 0 can have severe LS; thus, LS can be a sensitive detector of physical disability of cancer patients than PS. The improvement of LS can be a key to the preservation of their ADL and QOL.

Importance and Difficulty of Differentiating BMA-induced AFF Prodromal Symptoms from Hormonal Therapy-related Femoral Pain.

We herein report a case of breast cancer in a 74-year-old woman treated with exemestane as fourth-line hormonal therapy and bone-modifying agents for long time. She suddenly developed a right femoral shaft fracture during treatment. Her femoral fracture had a beaking sign on radiogram. Given this finding, her fracture was ultimately diagnosed as atypical femoral fracture (AFF). In this case, it was difficult to recognize the difference between groin pain as a prodromal symptom of AFF and that due to an adverse reaction to hormonal therapy. Therefore, clinicians should recognize the difficulty of this differentiation and consider the situation with caution.

Seipin-linked congenital generalized lipodystrophy type 2: a rare case with multiple lytic and pseudo-osteopoikilosis lesions.

Congenital generalized lipodystrophy (CGL), or Berardinelli-Seip syndrome (BSCL), is a part of lipodystrophic syndromes that constitute a heterogeneous group of genetic or acquired generalized or partial body fat loss disorders. It is a rare autosomal recessive disease characterized by a near-absence of adipose tissue from birth or early infancy and severe insulin resistance. CGL is classified as type 1-4, depending on the gene involved, and bone lytic lesion is found frequently in type 1 especially in long bones, but reported to be rare in type 2. Here we report an active lifestyle 25-year-old woman with type 2 CGL showing multiple bone lytic and pseudo-osteopoikilosis lesions in hands and feet. Radiograph bone survey showed no apparent abnormality in pelvic bone or axial skeletons. Bone marrow was completely absent and extra-skeletal general fat loss was also evident in whole-body magnetic resonance imaging sparing the orbital, axial, sole, and palmar regions. Radiographic bone survey is important even for type 2 CGL to find the change of bones to provide direction of preventing excessive overload or activity.

A trapdoor procedure for chondroblastoma of the femoral head: a case report.

Chondroblastoma located in the femoral head is one of the locations accounting for frequent recurrence. One of the reasons for this is the difficulty in obtaining appropriate surgical access to it for adequate removal of tumors. The authors present and illustrate a trapdoor procedure for the surgical treatment of chodroblastoma in the epiphysis of the femoral head. The surgical approach was made over the great trochanter and a trochanteric osteotomy was performed. The capsulotomy was made anteriorly and posteriorly, and the hip was dislocated anteriorly. Using a scalpel and an osteotome, the edges of a trapdoor segment were sharply dissected and the rectangular segment was lifted back to reveal an underlying subchondral tumor. The tumor tissue was thoroughly curetted and autologous cancellous bone was grafted. The trapdoor was replaced without any additional fixation, and the femoral head was reduced. The patient recovered good hip function without pain, and showed no recurrence of chondroblastoma at 5 years after surgery. The trapdoor procedure enabled sufficient access to complete curettage and autologous cancellous bone grafting for the chondroblastoma of the femoral head. This procedure proved to be a useful surgical approach for the treatment of chodroblastoma in the epiphysis of the femoral head in this case.

Hyperthermic isolated regional perfusion with CDDP for bone and soft-tissue sarcoma of the lower limb: pharmacokinetics, thermal dose, toxicity, and feasibility.

The objectives of this study were to investigate the pharmacokinetics of cisplatin (CDDP) and the thermal dose, toxicity, and feasibility of hyperthermic isolated regional perfusion (HIRP) with CDDP for bone and soft-tissue sarcomas of the lower limb. A total of 43 patients were treated with HIRP using CDDP. The dose of CDDP administered was 62.9+/-11.8 mg/limb (20 mg/m(2) +20 approximately 30 mg). The mean highest CDDP concentration was 56.9 microg/ml as total platinum (tPt) and 49.0 microg/ml as free platinum (fPt). The tPt concentration remained higher than 10 microg/ml. The highest temperature within tumor was 42.3+/-1.4 degrees C on average. The complications of HIRP were grade II toxicity in 30 patients, grade III in 9, and grade IV in 4. The mean necrotic ratio in the resected specimen was 84.5%, and the effect was grade IV (no viable tumor cells) in 13 patients, grade III(>90% necrosis) in 12, grade II (50 to <90%) in 9, and grade I (<50%) in 4. We obtained favorable levels of platinum concentration of the perfusate using a lower CDDP dosage compared with previous studies of HIRP. Considering our results of the pharmacokinetics of CDDP and clinical efficacy, we propose a lower dosage of CDDP for HIRP in the treatment of osteosarcoma. Multimodality treatment of HIRP with preoperative chemotherapy and surgery is a relatively safe and reliable therapeutic option for patients with limb sarcomas, and our method offers excellent local control.

Distraction osteogenesis for knee arthrodesis in infected tumor prostheses.

Three patients had distraction osteogenesis as a salvage method for infected endoprostheses. At the first operation, the infected prosthesis was removed and stabilization was achieved with an external fixator to preserve limb length. An additional external fixator was applied later for distraction osteogenesis after ensuring that there was no infection. Osteotomy was done at two sites on the femur, or tibia and femur, respectively, as a second operation. A third surgery was done at the docking site at the edge of the transported bone fragments. Curettage, refreshing, and soft tissue release were done to enhance bone union. The healing index was 18.3 days/cm in Patient 1, 17.7 days/cm in Patient 2, and 33.0 days/cm in Patient 3. All patients walk without a cane. It has been shown that patients can obtain a long-lasting and weight-bearable leg with our method, because their viable bone establishes biomechanical stability. Loss of knee function, a longer treatment period, and pin site treatment are the weaknesses of our method. Our method is indicated for patients in whom systemic disease can be controlled well and who have longer life expectancy.

Parachordoma with multiple metastases.

We present a very rare case of parachordoma with local aggressiveness and widespread metastases. A 68-year-old male presented with a mass in his left calf. The lesion was depicted as a poorly marginated mass with inhomogeneous signal intensity on magnetic resonance imaging. The tumor invaded surrounding muscles, neurovascular bundles, and bones. Widespread metastasis to lung, bone, and skin developed after amputation surgery. The histologic features of primary and metastatic lesions were the same and consistent with parachordoma.