Digital Gangrene as a Paraneoplastic Manifestation of Peripheral T-cell Lymphoma Not Otherwise Specified Type.

BACKGROUND: Peripheral T cell lymphoma (PTCL), not otherwise specified (NOS) is a heterogenous group of predominantly nodal T cell lymphomas that generally presents with lymphadenopathy with or without extra nodal involvement. Acral vascular syndrome clinically presents as digital ischemia with Raynaud's phenomenon and acral cyanosis. Although, this condition is commonly associated with connective tissue disorder, smoking and vasculitis, its association with lymphoid malignancy is very rare. Here, we present a case report of a patient with digital gangrene of all toes and fingers as a presenting symptom of PTCL-NOS. CASE DESCRIPTION: A 62 year old male presented with digital ischemia associated with pain, low grade fever, loss of appetite and significant weight loss of 6 kilograms over a period of 3 months. On examination, he was found to have bilateral inguinal and axillary lymph nodes with gangrenous changes over toes and fingers but peripheral pulses were palpable. On evaluation he had anemia, elevated ESR and CRP. CT angiogram revealed thinned out digital arteries with multifocal areas of narrowing. Patient was screened for other causes of digital gangrene and was tested negative for ANCA, ANA, cryoglobulins and viral markers. Lymph node biopsy with IHC was suggestive of peripheral T-cell lymphoma-NOS and was started on CHOP regimen. Lymph nodes size decreased and gangrenous changes resolved. CONCLUSION: Though digital ischemia is a rare paraneoplastic presentation of lymphoma, it should be considered if there is a rapid progression of gangrene. Early initiation of chemotherapy may result in the reduction of further progression of digital gangrene and thus prevent permanent disability. In our patient, progression of gangrene was prevented even though it was an aggressive variant of T cell lymphoma.

Isolated Distal Interphalangeal Joint Flexion Contracture of the Ring Finger due to Dupuytren Disease: A Case Report.

Dupuytren disease is a common benign fibromatosis of the palmar and finger fascia caused by pathological cord formation. As both the retrovascular and lateral cords are jointly responsible for the flexion contracture of the distal interphalangeal (DIP) joint, isolated DIP joint contracture caused by Dupuytren disease is rare. We present a 34-year-old right-hand-dominant male patient with a 6-month history of an isolated DIP joint flexion contracture in the right ring finger due to Dupuytren disease. Surgical fasciotomy of the retrovascular cord improved the contracture without adverse events. It is important to pay attention to the anatomical relationships between the pathological cord and neurovascular bundle to avoid neurovascular injury during fasciotomy in patients with Dupuytren disease, especially in isolated DIP joint contracture cases. Level of Evidence: Level V (Therapeutic).

Angioleiomyoma - A Case Report.

Angioleiomyoma is a rare benign soft tissue tumour arising from smooth muscle, representing <1% of upper limb soft tissue tumours. We report a 54-year-old male presenting with a progressively enlarging atraumatic lesion along the palmar side of the base of the ring and little finger. A biopsy was done to determine the diagnosis. Intraoperatively, the lump was found to be intimately related to the radial digital artery, it could not be excised en-bloc without transecting the radial digital artery of the little finger. Following excision, the ends of the digital artery were anastomosed. At 10-months follow-up, the hand was fully functional without any evidence of cold-intolerance or neurological deficit along the distribution of the digital nerve. We review the literature on angioleiomyoma and report careful resection of the tumour with digital artery transection and repair as a treatment option for angioleiomyoma of the digital artery. Level of Evidence: Level V (Therapeutic).

Plexiform Schwannoma of Digital Nerve.

Plexiform schwannoma is an uncommon benign tumour that grows in a plexiform pattern. We report a 47-year-old man with a mass on the palmar aspect of the metacarpophalangeal joint of the right index finger that had been growing gradually for more than 10 years. The mass was palpated from the distal carpal tunnel to the ulnar aspect of the proximal interphalangeal joint of the index finger, with tingling and numbness sensation. The tumour was a multinodular tumour involving the first common palmar digital nerve to the ulnar proper palmar digital nerve. It was resected and reconstructed with a sural nerve graft. Plexiform schwannoma is rare in the digital nerve, with only six cases reported. Generally, classic schwannomas can be enucleated without causing neurologic deficits; however, plexiform schwannoma may require nerve resection. There have been reports of recurrence of plexiform schwannoma; definitive resection and long-term follow-up are necessary. Level of Evidence: Level V (Therapeutic).

Recurrent osteoid osteoma of the index proximal phalanx: A case report with a new resection technique.

RATIONALE: Osteoid osteoma is a relatively common benign bone-forming lesion that often occurs in young adults with a predilection for the lower limbs and spine. It is characterized by the formation of osteoid tissue, a precursor to bone, and can cause pain and restricted mobility in affected areas. Osteoid osteoma of the hand and fingers is rare, thus representing a diagnostic challenge. A written informed consent was provided from the patient's father for the publication and sharing all images and clinical data concerning the patient. PATIENT CONCERNS: We are reporting a case of an 11-year-old girl who presented with persistent pain and swelling in her left index finger, as well as limited mobility, over the course of 1 year. Nonsteroidal anti-inflammatory drugs eased the pain, but it resurfaced once the medication was discontinued. DIAGNOSES: A series of investigations, showed a lytic lesion at the second proximal phalangeal neck, with features indicative of osteoid osteoma. INTERVENTIONS: Excision with bone grafting was performed as definitive therapy with pathological confirmation of the osteoid osteoma diagnosis. Nevertheless, the patient returned 2 years later with a recurrence of her previous symptoms, and further tests suggested a recurrence of osteoid osteoma. To address the recurrence, a modified open thermoablation technique was used. Thermoablation is a minimally invasive procedure that uses heat to destroy the tumor cells, and it has been shown to be effective in treating osteoid osteoma. The modified open approach involves making a small incision to access the tumor and delivering heat directly through a previously CT-guided inserted Kirschner wire to the affected area. OUTCOMES: The patient reported no pain at 1 month and 1 year after the surgery, with no radiological signs of recurrence, indicating complete excision of the lesion. LESSONS: Overall, this case highlights the challenges of diagnosing and treating osteoid osteoma in the hands and fingers. Further research is needed to better understand the underlying causes, potential risk factors, and optimal treatment for osteoid osteoma recurrence.

A Case of Circumscribed Palmar or Plantar Hypokeratosis and a Mini-review of the Literature.

ABSTRACT: Circumscribed palmar or plantar hypokeratosis (CPPH) is a new dermatologic disorder that firstly defined Pérez A et al in 2002. Since that time, further cases of CPPH have been reported by different authors in different countries. We report a 69-year-old Turkish woman who presented with asymptomatic, erythematous patches on the thenar region of the left hand and on the second left finger. Skin biopsy showed histological features of CPPH. In this article, it was emphasized that CPPH may be seen more frequently than expected and the clinical and pathological features of this disease with suspected malignant transformation should be known.

Bizarre parosteal osteochondromatous proliferation (Nora's lesion) of the hand: management of a rare clinical entity.

Bizarre parosteal osteochondromatous proliferation, or Nora's lesion, is a rare benign tumour of the bone, most commonly described in the hands and feet. We present the case of a female patient in her 20s attending the hand clinic with a sudden onset, atraumatic swelling on the proximal phalanx of her right ring finger. The patient retains good hand function and remains asymptomatic 3 months after surgical excision. Our case highlights the importance of being aware of this diagnosis and differentiating it from other malignant, as well as benign tumours of the hand.

Microreticular perineurioma: A new morphological subtype of a rare entity.

Perineurioma is a benign peripheral nerve sheath tumor. Several subtypes have been described, including plexiform, reticular, and sclerosing. The reticular variant has been previously described as having a net or lace-like growth pattern consisting of large anastomosing cords of spindle-shaped cells. We report a case of an 11-year-old male who presented with a 2-year history of a slowly enlarging, tan-white lesion on the finger. Microscopically, the lesion consisted of cells with ovoid nuclei and delicate, elongate cytoplasmic processes, arranged in a microreticular pattern. The lesional cells were markedly positive for epithelial membrane antigen and claudin-1. Based on these features and the unusual morphology, the case was called a microreticular perineuroma. The patient underwent complete excision of the lesion with no recurrence 9 months after follow-up. To our knowledge, this is the first reported case of this morphologic variant. Awareness of this entity is important to avoid inappropriate management.

Possible association of trichorhinophalangeal syndrome I and intracranial subependymoma.

Trichorhinophalangeal syndromes (TRPSs) are rare hereditary syndromes with autosomal dominant inheritance. Patients exhibit abnormalities including bulbous pear-shaped nose, broad columella, and long and flat philtrum, fine, sparse, brittle, slow-growing scalp hair, skeletal abnormalities, and short stature. Three families; age at subependymoma surgery, pathogenic TRPS1(NM_014112.5) variant, and subependymoma number are described.

Ungual Keratoacanthoma With Features of Regression.

ABSTRACT: Ungual keratoacanthoma (UKA) is an infrequent tumor. Different from keratoacanthoma in other parts of the skin, UKA rarely regresses, and grows aggressively with common destruction of the subjacent phalanx. Reported cases of UKA with features of regression are exceptional, and even dermatopathologists with reputed experience in nail pathology admit to having seen very few cases. We herein report a case of a 77-year-old man who presented a painful subungual lesion on the second finger of the right hand. An x-ray showed evidence of erosion of the subjacent distal phalanx. The patient was highly concerned about the lesion and rejected conservative treatment preferring amputation of the distal phalanx. The histopathologic examination revealed a UKA with features of regression.

Trichoepithelioma on the Finger: A Rare Location and Dermoscopic-Histopathologic Correlation.

ABSTRACT: Trichoepithelioma is a benign adnexal neoplasm of follicular germinative cells, with bulbs, papillae, and sheaths of perifollicular connective tissue as signs of follicular differentiation. Accordingly, trichoepithelioma may arise in any hair-bearing location, mostly on the face. That is why trichoepithelioma cannot appear in glabrous skin, and, although the dorsum of the hands and feet are a hair-bearing area, acral location is exceptional. We report the first case of trichoepithelioma localized in the finger of a 79-year-old man. It was a brown-pink, firm, 7-mm diameter, solid papule on the dorsal aspect of his left index finger. The lesion was completely asymptomatic, and he remembered to have it for many years. We describe this case highlighting its rare anatomical location and correlate its dermoscopic features with the histopathological appearance.

Intravascular papillary hemangioendothelioma disguised as a peripheral sheath tumor of median nerve at the wrist: a case report and literature review.

BACKGROUND: Intravascular papillary hemangioendothelioma (IVPH) is a benign lesion previously reported in the nasal cavity, neck, upper extremities, and breast. Diagnosis with cross-sectional imaging can prove difficult, with histopathological examination necessary for diagnosis. IVPH resulting in carpal tunnel symptoms is quite rare. CASE PRESENTATION: We report the case of a 37-year-old woman who presented with a radial, volar right wrist mass enlarging over the span of 5 years. She noted numbness and tingling in her wrist and thumb, exacerbated by minor accidental collisions and wrist hyperextension. There was no antecedent trauma. On examination, a mildly tender, mobile mass was evident at the volar aspect of the right wrist. Magnetic resonance imaging (MRI) with contrast demonstrated a lobulated, predominantly T2 hyperintense, heterogeneously enhancing mass thought to be a peripheral nerve sheath tumor. The patient elected for surgical excision of the mass, and the histopathological examination showed organizing thrombi with prominent papillary endothelial hyperplasia. At the 2-month follow-up, the patient had full range of motion of her fingers and wrist, with subjectively normal sensation in the distribution of the median nerve. CONCLUSION: Carpal tunnel syndrome, in exceedingly rare occasions, can result from an IVPH. MRI findings may be confused with more common entities. Histopathological confirmation remains necessary for conclusive diagnosis.

Glomus tumours of the knee: a case series.

AIM: To review clinical and magnetic resonance imaging (MRI) features of patients with glomus tumour of the knee, and compare the present findings with cases from the English literature. MATERIALS AND METHODS: The orthopaedic oncology and pathology databases at The University of Chicago were reviewed for cases of glomus tumour and small subcutaneous anterior knee masses. For all glomus tumours, the site of the tumour and age and sex of the patient were recorded. For those tumours arising in the knee, the symptom profile and MRI characteristics were recorded. The English literature was reviewed for cases describing glomus tumours in the knee. RESULTS: Thirty-four patients with glomus tumour were recorded. Five glomus tumours occurred in the anterior knee, all in men aged ≥30 years, four of whom had a several-year history of pain prior to diagnosis. MRI showed small (<2 cm) nodular enhancing masses. Review of the English literature supports the anterior knee as a common location for lower-extremity glomus tumours, with a male predominance and several years of pain preceding diagnosis. CONCLUSION: Although rare, glomus tumours occur in the knee. Unlike the classic description of glomus tumours occurring in the fingers most typically in women <30 years of age, those that occur in the knee tend to do so in men >30 years, and many years of pain can precede diagnosis. The astute radiologist may be the first to suggest this diagnosis if a small, painful, enhancing, nodular subcutaneous mass is noted on knee MRI examinations.

Metastases to the nail unit and distal phalanx: a systematic review.

Metastases to the nail unit/distal phalanx (NU/DP), although rare, carry a poor prognosis and are frequently misdiagnosed due to variable clinical presentation. Metastases to the NU/DP may be the initial presenting sign of a new or recurrent malignancy. Since the most recent systematic review of case reports (133 patients total) was conducted in 2001, we conducted a systematic review from 1900 to 2021 (244 patients total) to assess any changes in trends in demographics, clinical presentation, and morphology and to report on more updated differential diagnoses. We also examined cases for age, sex, race, ethnicity, Fitzpatrick skin type, laterality, distribution, and diagnostic methods. The PubMed database (1900-2021) was used to detect case-level data per Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. We found that the most common primary tumors were lung, kidney, and esophagus. A NU/DP metastasis was the presenting sign of malignancy in 31.00% of patients without a former cancer diagnosis. Male to female ratio was 2:1, with average age at diagnosis 58 years. Metastases most often affected a single digit (79.91%), particularly the thumb, followed by the fourth digit. This systematic review corroborates that metastases to the NU/DP may be the initial presenting sign of a new or recurrent malignancy and provides updated diagnostic guidelines. NU/DP metastasis should be considered in both healthy patients and patients with a former malignancy diagnosis presenting with nail changes involving a single digit. Prompt diagnosis and treatment may improve prognosis.

Pleomorphic acquired digital fibrokeratoma: A novel clinicopathologic entity.

A 26-year-old male presented with a 2-year history of a hyperkeratotic growth from the left index finger. Histopathology was consistent with an acquired digital fibrokeratoma with changes of a pleomorphic fibroma. Lesional cells were negative for CD34, Rb, and p53, and were positive for FXIIIa. We introduce the pleomorphic acquired digital fibrokeratoma as a novel clinicopathologic entity.

Mast cell presence in tendon sheaths of trigger fingers: implications on pathogenesis and clinical presentation.

Trigger finger is a common hand disorder; however, its pathogenesis remains unknown. In this study, we aimed to investigate mast cells, fibroblast activators that synthesize collagen, in the tendon sheaths of trigger fingers. We investigated the presence of mast cells and their association with changes in the collagen content of the tendon sheath and clinical data. We performed a multicenter prospective study of 77 adult patients with trigger finger who had undergone resection of the first annular pulley between August 2012 and January 2020. The tendon sheath was immunostained with an anti-tryptase antibody to confirm mast cell presence. The percentage of collagen in the tendon sheath was determined by picrosirius red staining observed through a polarization microscope. The clinical data, including the duration from symptom onset to surgery, severity, pain numerical rating scale, and Hand20 scores, were evaluated. Tryptase-positive mast cells were recognized in 83.5% of all specimens. The mast cell presence group (Group P) had a significantly higher percentage of type-3 collagen in the tendon sheath than the non-mast cell presence group (Group N) (Group P, 15.6%; Group N, 12.7%; p = 0.03). Moreover, Group P had significantly higher pain numerical rating scale (Group P; 5, Group N; 3, p = 0.04) and Hand20 (Group P; 35.5, Group N; 13.0, p = 0.01) scores than Group N. These findings suggest that mast cell presence in the tendon sheath of the trigger finger is related to the pathology and clinical symptoms of trigger finger.

Solitary Chondrosarcoma of the Right Ring Finger: A Case Report.

We present a case of solitary chondrosarcoma arising from the proximal phalanx of the ring finger in an elderly man. The chondrosarcoma developed over a period of 14 years, during which the phalanx became progressively more deformed. Several radiographic investigations were carried out, but the patient declined further suggested diagnostic examinations (computed tomography, magnetic resonance imaging, biopsy). Eventually, the lesion became significantly enlarged, and radiographs showed osteolytic lesions in the phalangeal bone. Ray amputation of the finger was required to establish a wide resection of the chondrosarcoma. Most osteochondral tumors arising from the phalanges are benign tumors such as enchondromas, but primary chondrogenic malignant bone tumors (chondrosarcomas) occasionally occur. Chondrosarcoma of the phalanx is difficult to distinguish from enchondroma of the phalanx, because histological investigations of the two neoplasms often produce similar findings. Even with a combination of clinical, biopsy, and imaging findings, differentiating these neoplasms is still challenging, because the characteristic clinical and radiological features of chondrosarcoma do not appear until it becomes aggressive and starts to cause destructive changes. Once that happens, radical expanded resection of the tumor is essential. Therefore, longstanding enchondroma-like lesions should be actively treated in elderly patients, even if a definite diagnosis of chondrosarcoma cannot be made.

Onychomatricoma on the fourth toenail: A rare tumor in a rare localization.

Onychomatricoma is a rare benign tumor of the nail matrix, characterized by finger-like projections that invade the nail plate. The fingernails of Caucasian women are most commonly affected. Because this tumor can easily mimic other more prevalent ungual diseases, it is crucial to be acquainted with its characteristic clinical and histopathologic features. The authors present a case of a 40-year-old man with an onychomatrichoma in the fourth left toenail, which was initially misdiagnosed and treated as onychomycosis.