Primary lymphoma of bone of the little finger: a case report and review of the literature.

Primary lymphoma of bone (PLB) is a rare, malignant lymphoid proliferation within bone accounting for less than 3% of all malignant bone tumors. In this case report, a 61-year-old female with past medical history of gout presented with pain and swelling in her right little finger. Initial radiographs demonstrated periostitis and soft tissue swelling about the right little finger. She returned three months later with progressive pain. Subsequent MRI and repeat radiographs demonstrated near complete destruction of the right little finger middle phalanx and periostitis with marrow infiltration at the right long finger. Given the rapid progression of disease, the differential diagnosis consisted primarily of aggressive neoplastic processes. The little finger ray was amputated through the level of the metacarpophalangeal joint and histopathology demonstrated large neoplastic cells that stained positive with CD45, CD20, and PAX5, compatible with diffuse large B-cell lymphoma. A subsequent normal bone marrow aspiration and PET-CT demonstrated no additional sites of disease, thus excluding secondary lymphoma to bone. To the best of our knowledge, this is the first case report of polyostotic PLB involving the hand. PLB of the hands may be initially misdiagnosed due to its rarity and clinical presentation mimicking rheumatological disease. Clinical vigilance in concert with close imaging follow-up is required to make the diagnosis in a timely fashion. We also review the existing PLB hand literature which consists of five cases.

Giant Cell Tumor of the Proximal Phalanx: Report of two Cases Treated by Two Different Methods and Review of the Literature.

The hand is an extremely rare site for giant cell tumor (GCT). There are only a few reported cases of GCT including the hand, and even fewer reporting involvement of phalanges. GCTs in small bones are typically more aggressive and have higher local recurrence and rate of metastasis in younger patients compared to long bone involvement, so the treatment is more clinically challenging in the hand. In this study, we present the management of giant cell tumors of the proximal phalanxin two patients treated with two different method; ray resection and arthrodesis using an iliac crest graft. Key words: giant cell tumor, phalanx, hand, recurrence.

Bilateral Lung Metastases From a Phalangeal Giant Cell Tumor of Bone.

We describe a rare pediatric case of a phalangeal giant cell tumor of bone with extensive bilateral lung metastases following curettage, wide resection, and amputation. Concurrent peripheral blood eosinophilia and pleural effusion with marked eosinophilia (47%) were present. To discover genetic changes driving tumor metastasis, genomic and transcriptome profiling of the metastatic lung mass as well as germline analysis were performed. Whole exome sequencing detected a histone H3F3A p.G35V missense mutation in tumor cells. RNA sequencing revealed overexpression of receptor activator of nuclear factor kappa-B ligand (RANKL). The patient is alive with no residual disease and uncompromised respiratory function 29 months after amputation of primary tumor and 19 months after surgical resection of his metastatic lung disease.

Huge aneurysmal bone cyst secondary to giant cell tumor of the hand phalanx: a case report and related literature.

BACKGROUND: Aneurysmal bone cyst (ABC) secondary to Giant Cell Tumor of bone (GCT) is a rare lesion, of which the incidence is about 0.011 to 0.053 per 100,000 every year. There are only a few previous case reports, and most of them occur in the spine, long bones or flat bones. CASE PRESENTATION: We report one case of a patient who complained of "progressive enlargement of the mass on right-hand fifth finger for 5 years with ulceration for 6 months". After the imaging examination in our hospital, it was diagnosed as a "huge bone tumor on the proximal phalanx of the right-hand fifth finger", then wide excision and amputation of the fifth finger were made. The pathological examination diagnosed the mass as aneurysmal bone cyst secondary to giant cell tumor, 13 × 8 × 6 cm3, with no local infiltration observed. No recurrence and metastasis occurred 18 months after the operation, and the patient recovered well. CONCLUSION: In this report, we discuss the etiology, diagnosis, differentiation, and management of Aneurysmal bone Cyst secondary to Giant Cell Tumor of bone, and review previous case studies.

Brachydactyly type A3 is caused by a novel 13 bp HOXD13 frameshift deletion in a Chinese family.

Brachydactyly type A (BDA) is defined as short middle phalanges of the affected digits and is subdivided into four types (BDA1-4). To date, the molecular cause is unknown. However, there is some evidence that pathogenic variants of HOXD13 could be associated with BDA3 and BDA4. Here, we report a Chinese autosomal dominant BDA3 pedigree with a novel HOXD13 mutation. The affected individuals presented with an obviously shorter fifth middle phalanx. The radial side of the middle phalanx was shorter than the ulnar side, and the terminal phalanx of the fifth finger inclined radially and formed classical clinodactyly. Interestingly, the index finger was normal. The initial diagnosis was BDA3. However, the distal third and fourth middle phalanges were also slightly affected, resulting in mild radial clinodactyly. Both feet showed shortening of the middle phalanges, which were fused to the distal phalanges of the second to the fifth toes, as reported in BDA4. Therefore, this pedigree had combined BDA3 and atypical BDA4. By direct sequencing, a 13 bp deletion within exon 1 of HOXD13 (NM_000523.4: c.708_720del13; NP_000514.2: p.Gly237fs) was identified. The 13 bp deletion resulted in a frameshift and premature termination of HOXD13. This study provides further evidences that variants in HOXD13 cause BDA3-BDA4 phenotypes.

Unusual localization and presentation of osteoid osteoma mimicking juvenile spondyloarthritis: a case report.

BACKGROUND: Osteoid osteoma is a painful benign skeletal tumour of unknown aetiology. Most often it occurs in the long bones of extremities and responds well to nonsteroidal anti-inflammatory medications. However, unusual localization and atypical presentation of this tumour might present a diagnostic challenge, especially if symptoms mimic that indicative of juvenile spondyloarthritis. CASE PRESENTATION: A misdiagnosed ten-and-a-half-year-old girl with osteoid osteoma involving the distal phalanx of a little finger is presented. Her initial symptoms were pain and swelling of the little finger resembling dactylitis, while various imaging modalities showed signs of tenosynovitis, indicating a possible development of juvenile spondyloarthritis. Several trials of different non-steroid anti-inflammatory drugs gave no satisfactory results and ultrasound guided triamcinolone-hexacetonide injection provided only a short relief. Finally, almost three years after initial presentation, persistent clinical symptoms warranted repeated imaging that raised suspicion of an osteoid osteoma. Directed treatment with surgical intervention led to almost immediate and complete resolution of her symptoms. CONCLUSIONS: Osteoid osteoma should be suspected in case of a tender swelling of a digit in children and adolescents, regardless of initial imaging findings and clinical presentation. Early diagnosis and treatment of this benign condition can have a substantial impact on quality of life of patients and their families and protect them from many unnecessary diagnostic procedures and treatment.

Osteoid Osteoma in the Thumb of an Adolescent Patient.

Osteoid osteoma is a relatively common benign tumor of bone, typically presenting in the diaphysis of long bones during the second or third decades of life. This tumor is rarely reported in the hand and wrist, making up only approximately 10% of cases. When reported in the hand, osteoid osteoma tends to occur more frequently in the proximal phalanx of the index and middle fingers. We present the case of an osteoid osteoma in an adolescent male in the distal phalanx of the thumb. The presentation of this osteoid osteoma was atypical owing to its location and lack of characteristic clinical features, making the initial work-up and final diagnosis challenging and pointing to the importance of considering this diagnosis on a differential for painful bony tumors in the hand.

Phalangeal Osteoid Osteoma of Thumb.

Osteoid osteomas are typically benign osteoblastic bone tumors. Patients often present in their twenties with progressively increasing pain that is worse at night and relieved with nonsteroidal anti-inflammatory drugs. These lesions rarely occur in the hand or wrist; however, when those are involved, the proximal phalanx is the most common location and the index finger is the most commonly affected digit. Lesions affecting the distal phalanges are least likely to occur and the thumb is least likely to be affected. Osteoid osteomas of the distal phalanges can cause great diagnostic challenges. They often present with atypical radiographic and physical examination findings. Although rare, osteoid osteomas of the distal phalanx can be a major cause of digit enlargement and pain. In a patient with a painful and/or swollen digit, the diagnosis should be considered.

Phalangeal microgeodic syndrome in childhood.

BACKGROUND: Phalangeal microgeodic syndrome is an uncommon benign self-limiting condition that often occurs during cold weather. The etiology and the pathogenesis of the disease remain unclear. OBJECTIVE: To report a series of children with phalangeal microgeodic syndrome. MATERIALS AND METHODS: Twenty children with phalangeal microgeodic syndrome were retrospectively identified at our hospital after 2007. The clinical data, radiologic manifestation and pathologic appearance were analyzed. RESULTS: The average age was 10.3 years (range: 6.5-14.6 years). Twelve patients were boys. Twenty-five phalanges were affected radiographically (23 middle phalanges [92%] and 2 proximal phalanges [8%]). On radiographs, there were multiple small phalangeal lacunae in all cases. Metaphyseal rarefaction was seen in 15 phalanges, and metaphyseal transverse lucent bands were found in 7 phalanges. Epiphyseal rarefaction was seen in three phalanges. On magnetic resonance imaging (MRI), diffuse signal abnormalities of affected phalanges were observed in all cases. Multiple other phalanges and metacarpals also showed marrow edema in three cases. CONCLUSION: Phalangeal microgeodes may represent bone absorption and destruction in response to exaggerated peripheral circulatory impairment following chilblain, and mainly occur in bone growth spurts.

A Rare Manifestation of Primary Bone Lymphoma: Solitary Diffuse Large B-Cell Lymphoma of the Little Finger.

Solitary primary non-Hodgkin bone lymphoma of the hand is a rare entity with only 3 cases reported in the literature. We report the case of a 77-year-old patient with isolated large B-cell bone lymphoma of the proximal phalanx of the little finger without rheumatoid arthritis or methotrexate treatment. The patient was treated with digital amputation and at 6 months' follow-up showed no relapse or dissemination of the disease.

Panoramic radiographs and quantitative ultrasound of the radius and phalanx III to assess bone mineral status in postmenopausal women.

BACKGROUND: Various mandibular indices have been developed to detect osteoporosis on panoramic radiographs. Quantitative ultrasound (QUS) is a low-cost, radiation-free method to assess bone status. The aim of this study was to compare mandibular morphometric analysis and QUS at the radius and proximal phalanx III finger. METHODS: The study involved 97 postmenopausal women, aged 48.5-71.5y (mean: 55.4). Mandibular morphometric analysis comprised: distance between upper and lower mandibular borders just behind the mental foramen (H), distance: mental foramen - inferior mandibular cortex (IM) and mandibular cortical width at the mental region (MCW). Then, ratios were calculated: MCW/IM = PMI (panoramic mandibular index), H/IM = MR (mandibular ratio). Mandibular cortical index (MCI) was used to classify the morphology of the mandibular cortex. Bone mineral status assessed using QUS at the radius and proximal phalanx III finger was compared to population mean apical bone mass (T-score). Linear regression analysis was used for correlations between continuous variables, Pearson's correlation coefficient r - for variables of normal distribution. Student's t-test was used to compare variables of normal distribution and for the latter - Mann-Whitney U-test. The level of significance was p < 0.05. RESULTS: Mandibular height was 13.42-34.42 mm. The mean mandibular cortical width was 3.31 mm. Mean values of PMI and MR were 0.33 and 2.57, respectively. Higher mean value of Ad-SoS was found in the radius than in the III finger. Phalanx T-score values ​​were lower than those of the radius. T-score of the radius was < - 1.0 in 22 patients, indicating osteopenia. Basing on phalanx T-score, osteopenia was found in 39 patients. Category C1 of Mandibular Cortical Index was found in 48 women, C2 - in 37 women and C3 - in 12 women. Higher scores of Mandibular Cortical Index were recorded in older women. MCI significantly correlated with the skeletal status (p = 0.01) as well as with H, MCW and MR. Phalanx T-score was not correlated to PMI, MR or MCW. CONCLUSIONS: 1. Mandibular Cortical Index can be used as a screening tool for detecting osteoporosis. 2. Quantitative ultrasound at the phalanx III constitutes a reliable way of assessing bone status.

[MALLET FINGER - DIAGNOSIS, CLASSIFICATION AND TREATMENT].

INTRODUCTION: Mallet finger is a common injury involving the extensor mechanism of the finger. It presents as an inability to extend the distal phalanx of the digit. In the right clinical setting, a thorough examination should be performed and supplemented by adequate radiographs. The mechanism of injury is usually a direct blow to an extended finger causing hyperflexion or hyperextension of the distal phalanx. Common occurrences are in young adults during sporting activities or in minor falls or trauma in osteoporotic individuals. Treatment includes use of a splint which maintains the distal phalanx in extension allowing for tendon healing or surgical intervention when indicated. In the vast majority of cases, prompt diagnosis and avid conservative treatment will result in a good outcome. Neglecting or missing this seemingly minor injury may result in a formidable functional handicap.

Preservation of Devitalized Bone in Necrotic Digits Following Crush Injury With Free Tissue Transfer.

Traditional management of necrotic digits has been to allow demarcation between viable and nonviable tissues to occur, and thus to indicate the level of amputation required. In this case report, the phalangeal length of the necrotic thumb was maintained in 2 patients who had sustained crush injury. After nonviable soft tissues were debrided, the devitalized bones were covered with soft tissue flaps. All wounds healed without infection. Both cosmetic and functional results of the reconstructed thumbs were satisfactory. In one patient, radiographs of the thumb demonstrated resorption of the distal phalanx, but this did not impede daily use. This experience supports an earlier observation suggesting that devitalized bone can be covered for salvage in some cases.

Distal Bone Metastasis From Primary Rectal Cancer: A Case Report.

Distal bone metastases from rectal cancer are uncommon. Our case report is from a patient with rectal carcinoma who presented with symptomatic middle finger metastases, and we describe the clinical characteristics of this presentation and the treatment provided. Metastases in bone tissues are a sign of a grave prognostic outcome due to the association of this with advanced terminal disease. Palliative treatment for symptom relief is the only option in this situation.

Primary intra-osseous myoepithelioma of phalanx mimicking an enchondroma.

Myoepitheliomas (MEs) are uncommon tumours of the soft tissue with an intermediate biological behaviour and uncertain differentiation. Primary intra-osseous MEs are rare and occur predominantly in the axial and proximal appendicular skeleton in middle-aged patients. The morphological variation of the tumour cells and stromal metaplasia may cause considerable diagnostic confusion, especially when it occurs in an unusual location. A wide panel of immunohistochemical markers is required to exclude other histological mimics. A 37-year-old male presented with a recurrent swelling in the right middle finger for 1-month duration. Radiographic images showed an expansile, lytic, intra-osseous lesion with high signal intensity on T2W fat-suppressed MR images in the proximal phalanx of the right middle finger without cortical breach, highly suggestive of an enchondroma. Histopathology revealed a lobulated tumour comprising of polygonal to spindle cells in groups and cords in a chondromyxoid stroma. No cellular atypia was noted. The tumour cells were immunopositive for epithelial membrane antigen (EMA), p63, S100 and smooth muscle actin (SMA), compatible with the diagnosis of an intraosseous ME. The proximal phalanx of the right middle finger was excised, revealing a similar tumour, and the patient has been on regular follow-up for the last 18 months without any recurrence. Primary intra-osseous MEs are extremely rare, and this is the second reported occurrence in small bones. A differential diagnosis of ME should be kept for enchondroma-like lesions of the bone for proper histopathological assessment and accurate diagnosis. Documentation of such cases and follow-up will enhance our understanding of their clinical course and prognosis.

[Tranverse acro-osteolysis: A rare cause of nail dystrophy]. / Acro-ostéolyse transversale : une cause rare d'onychopathie.

BACKGROUND: Acro-osteolysis (AO) involves partial or total destruction of the distal phalanges of the fingers or toes. The range of forms of AO is extremely wide. A distinction is generally made between the acquired forms and the genetic and idiopathic forms. Herein we report a case of idiopathic transverse single-finger AO associated with onychopathy. PATIENT AND METHODS: A 48-year-old woman consulted for a nail lesion involving brachyonychia in the right index finger alone (pseudo-clubbing appearance of the finger). The remainder of the clinical examination was normal, particularly in terms of neurological and dermatological investigations. Standard x-rays revealed transverse osteolysis of the middle third of the distal phalanx, occurring solely in the right index finger. DISCUSSION: While certainty regarding diagnosis of AO depends upon radiological findings, the radiological appearance is not specific. Two subgroups suggesting aetiological diagnosis may be distinguished: transverse AO with banding and longitudinal AO. With transverse AO, the diaphysis of the distal phalanx presents osteolysis with banding, but with sparing of the base of the band (pseudo-fracture appearance); this appearance is suggestive of toxic causes, congenital familial conditions or repetitive micro-trauma. Upon x-ray examination, the longitudinal forms exhibit concentric resorption of the band and these forms are more often seen in a setting of neurological, vascular or metabolic disorders. The associated nail involvement frequently present helps clarify the diagnosis, with nail plate shape being dependent on the integrity of the underlying bone. CONCLUSION: Herein we report the first case of transverse AO in a single finger associated with brachyonychia, and with no discernible cause.