Acral myxoinflammatory fibroblastic sarcoma: A case report and literature review

Acral myxoinflammatory fibroplastic sarcoma is an extremely rare soft-tissue sarcoma. It typically presents as an inflammatory mass in the distal extremities of adult patients. The authors present a review of the available literature as well as a discussion on the surgical management of a patient with acral myxoinflammatory fibroplastic sarcoma who originally requested conservative management but ultimately required a two-digit ray amputation after local recurrence.     

A case of lung metastasis in myxoinflammatory fibroblastic sarcoma: analytical review of one hundred and thirty eight cases

Purpose

Myxoinflammatory fibroblastic sarcoma (MIFS) is a rare soft tissue tumour first identified at the end of the 1990s. This study presents our experience and literature reviews focusing on risk of recurrence.

Methods

Rizzoli Orthopaedic Institute database and literature were searched for patients with MIFS observed from 1997 to 2012. Data were analysed in a new database.

Results

Five patients underwent surgery at our institute, and 133 cases were retrieved from the literature. Not all clinicopathological data were available: 76/138 were men (55 %), median age was 45 [interquartile range (IQR) 34–56] years, median tumour size was three (IQR two to five) centimetres. Common sites of occurrence were hand (24 %), fingers (23 %) and foot (20 %). Pain was present at diagnosis in 14/82 patients (17 %), with a median duration of seven (IQR three to 12) months. Surgery was performed for a suspected benign tumour in 88 patients (74 %). Resection was incomplete in 45/71 cases (63 %); re-excision was performed in 32/45 (71 %). At a median follow-up of 26 months, 26/118 patients (22 %) developed recurrent disease; median time to recurrence was 15 months (IQR seven to 26). Actuarial relapse-free survival (RFS) at one, three and five years was 93 %, 72 % and 67 %, respectively. At univariate analysis, only symptom duration of six months or less was significantly associated with a worse RFS (p = 0.046). Metastatic disease to lymph nodes and/or lungs was observed in four patients (3 %).

Conclusions

Clinicopathological findings confirm the low-grade nature of MIFS. However, local recurrence occurs, and patients may be affected by aggressive forms with a potential for distant metastases. Follow-up is strongly advised.     

A rare case of acral amelanotic melanoma,nodular type

Amelanotic melanoma is a rare melanoma subtype, and it is even more rare when it occurs at an acral site. We here present a case of a nodular amelanotic acral melanoma (NAAM) occurring on the heel of an 83‐year old female. It presented as an ulcerated pink nodular growth on the heel, which clinically mimicked other nodular non‐pigmented lesions, causing a delay in diagnosis until it was biopsied. This case is a demonstration of the critical importance to include NAAM in the differential diagnosis of nodular non‐pigmented skin lesions as to avoid delay in diagnosis and disease progression, in which early detection can provide the most modifiable prognostic factor.     

A rare case of synovial sarcoma of the prostate

Prostatic synovial sarcomas are exceedingly rare. To our knowledge, only six primary cases have been reported so far. We herein describe a primary synovial sarcoma of the prostate seen in a 25-year-old male patient, the youngest patient seen with this disease to date. He was referred to our department with the diagnosis of high-grade sarcoma of the prostate revealed by TRUS-guided biopsy. On admission he had a transurethral catheter for acute retention of urine. MRI revealed a solid prostatic tumor of 9.5 × 8 cm involving the rectum without any evidence of lymphatic or distant metastases. The patient underwent total pelvic exenteration and sigmoid end colostomy with ileal conduit. Histopathology revealed a synovial sarcoma of the prostate, immunoreactive to vimentin, Bcl-2 and cytokeratin. The patient is doing well at 18 months follow-up.     

Primary mediastinal synovial sarcoma: A rare case report

IntroductionSynovial sarcomas commonly occur in the extremities of young adults. A primary occurrence in the mediastinum is very rare with only a few reported cases in the world literature.We report a case of mediastinal synovial sarcoma. This paper is about a 47-year-old male who presented with retrosternal chest pain and shortness of breath on exertion. Imaging showed an anterior mediastinal mass. Pathological examination of the resected mass showed a biphasic neoplasm with a spindle cell component admixed with gland-like elements. The tumor showed positive staining with cytokeratin, epithelial membrane antigen and vimentin confirming the diagnosis of a biphasic synovial sarcoma.DiscussionA wide range of neoplasms, both primary and metastatic, occur in the mediastinum, which pose considerable diagnostic difficulties. A synovial sarcoma should always be considered in the differential diagnosis, and immunohistochemistry is an important adjuvant tool in this situation.ConclusionThis paper highlights the importance of recognizing an unusual presentation of this aggressive neoplasm to aid appropriate clinical management.     

Ewing's sarcoma of the hand: a case report

Ewing's sarcoma of the hand is rare. This tumor was first described in 1921, since then, only a few cases with hand involvement have been reported. We report a case of Ewing's sarcoma of the left hand, presenting as a swelling of the hand gradually enlarging over six months. Plain radiographs showed marked osteolysis of the second, third and fourth metacarpals. Histological confirmation was made following biopsy. Despite amputation and post-operative chemotherapy, death occurred two months later due marrow aplasia.     

A rare case of myeloid sarcoma presenting as anal fissure

Myeloid sarcoma is a tumor composed of myeloblasts occurring at an extramedullary site. It may develop in patients with acute myeloid leukemia, myeloproliferative or myelodysplastic syndrome, sometimes preceding onset of the systemic disease. Frequent sites of myeloid sarcoma are bones or various soft tissues. Gastrointestinal involvement is very rare. We report a unique case of myeloid sarcoma presenting as a painful anal fissure, in a patient with a history of acute myeloid leukemia. The diagnosis was achieved by a surgical excisional biopsy and immunoistochemical staining.     

A rare hand injury

Simultaneous dislocations of the carpometacarpal joints are relatively rare hand injuries. This is a case report of dislocations of the carpometacarpal joints II-V, combined with Bennett's fracture. The case is discussed in this article with particular reference to the therapeutical aspects.     

手背部滑膜肉瘤1例

患者,男,22岁,因发现左手背肿物4年、复发1 年于2019 年11 月3 日入院.患者约4年前无意中发现左手背一肿物,约玉米粒大小,无疼痛、瘙痒,表面皮肤无红肿,随着肿物逐渐增大,患者在淄博市中心医院高青院区行左手背肿物切除术,术后病理结果显示非典型纤维瘤,术后恢复好.     

Low-grade fibromyxoid sarcoma of the hand: a case report

We report a case of low-grade fibromyxoid sarcoma with giant collagen rosettes in the hand of a 21-year-old female. This is a clinically and radiological benign appearing tumor that has a high rate of recurrence and metastasis.     

A rare case of a solitary peripheral nerve sheath sarcoma of the scalp

Summary Malignant peripheral nerve sheath tumors are rare and most often appear in the clinical setting of von Recklinghausen's neruofibromatosis. On rare occasions they are seen in patients lacking any of the overt stigmata of this systemic disease. We report on a case of a peripheral nerve sheath sarcoma (PNSS) on the scalp and the back of the neck of a 13-year-old female arising in a solitary benign neurofibroma with no evidence of systemic neurofibromatosis. The tumor was examined by ultrastructural and immunohistochemical techniques as well as by routine histopathology. It was defined as a peripheral nerve sheath sarcoma, fibrosarcomatous type, of poorly differentiated perineurial cell origin. The possible significance of histogenetic subclassification in terms of surgical management and the oncological approach as well as the prognosis is discussed.     

A child with Ewing's sarcoma in scapula: A rare case report

IntroductionEwing's sarcoma is considered to be the second most frequent primary sarcoma in children. It affects children and young adults with a male predominance. Ewing's sarcoma is usually found in the femur, humerus, ilium and tibia; and in extremely rare cases Ewing's sarcoma might affect the scapula. There are only 15 papers in PubMed database regarding scapular Ewing's sarcoma.Case presentationA 14-year-old male, with no significant medical or surgical history, presented with a swelling in the right scapular area for two months. The patient also experienced mild pain and fever. On physical examination, there was a tender mass and restriction in the right shoulder joint movements. MRI showed a large scapular mass with the characteristics of a sarcoma. The final diagnosis was made based on pathologic findings. Eventually, the patient was subjected to neoadjuvant chemotherapy.DiscussionThe most common tumors affecting scapula are chondrosarcoma and osteosarcoma. In a cohort study about patients diagnosed with ES between 1988 and 2018, only 29 cases were involved in the scapula. There are 12 cases of congenital ES have been reported in the medical literature, 3 of them were in the shoulder girdle. Surgery with neoadjuvant chemotherapy is considered better for total survival in ES of scapula in comparison with chemotherapy and/or radiotherapy alone.ConclusionEwing sarcoma is extremely rare in the scapula and should be considered as a differential diagnosis for any patient with scapular tumor.     

Case report: A rare case of Ewing's sarcoma and osteosarcoma at different sites 10 years apart

Ewing's sarcoma of bone and osteosarcoma are rare tumors. A combination of high-grade osteosarcoma and Ewing's sarcoma of bone in anatomically unrelated sites is unique, especially in the absence of previous radiation or retinoblastoma. We present a patient with a rare case of Ewing's sarcoma of the scapula that showed no evidence of recurrence (after 10 years of continued followup) and who subsequently presented with a primary osteosarcoma of the femur.