Diffuse Large B-Cell Lymphoma of The Upper Limb and Face: Two Ends of a Rare Spectrum


Sirkeci C. B., Yalçın C. E., Aydın S. Y., Ercan A., Demiröz A.

12. Balkan Association of Plastic Reconstructive and Aesthetic Surgery Congress, Konya, Türkiye, 13 - 16 Eylül 2023, ss.270-272, (Özet Bildiri)

  • Yayın Türü: Bildiri / Özet Bildiri
  • Basıldığı Şehir: Konya
  • Basıldığı Ülke: Türkiye
  • Sayfa Sayıları: ss.270-272
  • İstanbul Üniversitesi-Cerrahpaşa Adresli: Evet

Özet

Diffuse large B-cell lymphoma is the most common lymphoma subtype in adults. There is still conflicting literature on the identification of primary extranodal disease when nodal and extranodal lesions are detected together. Although the incidence of primary extranodal lymphoma varies geographically, diffuse large B-cell lymphoma is the most common non-Hodgkin's lymphoma subtype with extranodal disease. Studies have reported that skin and skeletal muscle invasion is rare and skeletal muscle invasion is mostly observed in the lower extremities.

In this study, we aimed to present two patients, 61 and 56 years old, who presented to us with an open wound on the left arm and a nodular mass on the left cheek, who were diagnosed with diffuse large B-cell lymphoma. Thus, we wanted to show that extranodal diffuse large B-cell lymphoma may be an important option in the differential diagnosis of chronic open wounds in addition to cutaneous malignancies.

A 61-year-old woman was consulted to our outpatient clinic with a complaint of an open wound on the left elbow. In October 2020, the patient, who did not have a special anamnesis for trauma except for an only one bump 1 year ago, noticed an open wound about 2 cm in diameter on her elbow. Afterwards, she applied to an external center and topical treatment was recommended. After the patient did not benefit and the wound gradually grew, the patient applied to us 6 months later. In her anamnesis, she did not describe any comorbidities and therefore was not taking any chronic medication. Her surgical history was unremarkable except for hysterectomy, cholecystectomy and appendectomy. On initial examination, a wound with a diameter of approximately 15 cm with dry necrotic areas at the base, granulation tissue around it and hyperpigmentation and fragile vascular networks in the surrounding healthy skin tissue was found on the left elbow.  After debridement, Klebsiella spp. was found in the wound culture and outpatient follow-up with oral antibiotherapy was considered appropriate. However, there was no significant healing in the wound afterwards, and the patient was interned to our service.(Fig.1) After internalization, the patient's laboratory results were unremarkable except for acute phase increase (CRP: 36), mild normocytic anemia and HBsAg positivity. Gram-positive rods and coagulase-negative staphylococci grew in cultures sent from the open wound. Antibiotherapy was initiated in cooperation with infectious diseases department. Subsequently, For possible reconstruction needs and exclusion of mass lesions, magnetic resonance imaging (MRI) was requested.(Fig.2) Imaging showed a mass lesion in the distal half of the left arm extending subcutaneously through the muscle planes. An incisional biopsy was performed because the existing wound had not healed since the first follow-up and irregularities occurred in the tissues around the open wound. The incisional biopsy revealed atypical cells filling the dermis. After immunohistochemistrical evaluation these cells were found to be positive for Bcl-2 and C-myc. The patient was pathologically diagnosed with diffuse large B-cell lymphoma. Positron emission tomography scan (PET/CT) imaging  detected lesions related with metastasis in the left axilla, supraclavicular region, lung and spleen. According to this result, the patient was followed up by medical oncology and hematology departments for treatment.

A 56-year-old male patient without any comorbidity,  was evaluated at an external center because of a nodular mass in the left cheek area that had been gradually growing for 5 months. A punch biopsy was taken from the lesion. Because the biopsy result was suspicious for Merkel cell carcinoma, he was referred to our department.(Fig.3) We planned sentinel lymphadenectomy and excision operation with an initial diagnosis of Merkel cell carcinoma. Preoperative PET/CT imaging revealed no additional lesions suspicious for malignancy except the main lesion.  In the pathological evaluation of the excision material of the patient, CD 20 (+), CD 10 (+), Bcl-2 and Bcl-6 (+) were reported, and the synaptophysin and chromogranin required for Merkel cell carcinoma were negative. The patient was diagnosed with diffuse large B-cell lymphoma. Finally, the patient was followed up for treatment by medical oncology and hematology departments.

Primary cutaneous diffuse large B-cell lymphoma represents 4% of all cutaneous lymphomas. The median age at diagnosis has been reported to be approximately 78 years. It is more common in women. Extranodal diffuse large B-cell lymphoma originating from the skin is usually aggressive and presents as rapidly growing masses in the lower extremities. Lower extremities are affected in 80% of patients. Leg type is clinically aggressive, with high rates of relapse and metastasis. Average of 5-year survival has been reported to be 50-60%. On the other hand, non-leg-type lymphomas are presented with a 5-year survival rate up to 90%. They require effective treatment due to their aggressive character. Although palliative care, radiotherapy and non-aggressive chemotherapies have been reported, combined chemotherapeutic agents and rituximab treatments are common in the current literature. While it is obvious that cutaneous malignancy is one of the first options in the differential diagnosis of chronic open wounds, it is important to keep in mind that after exclusion of cutaneous malignancy, extranodal lymphoma is also a possible cause.