The Anatomic Pathology laboratory adjustments in the era of COVID-19 pandemic: The experience of a laboratory in a Portuguese central hospital.

The coronavirus disease 2019 (COVID-19) pandemic has dramatically changed the world over the past weeks, with already 8,25 million infections and 445,000 deaths worldwide, leading to an unprecedented international global effort to contain the virus and prevent its spread. The emergence of novel respiratory viruses such as the SARS-CoV-2 creates dramatic challenges to the healthcare services, including surgical pathology laboratories, despite their extensive daily experience in dealing with biological and chemical hazards. Here, we cover important aspects on the knowledge on COVID-19 gathered during the first six months of the pandemic and address relevant issues on human biological sample handling in the Anatomic Pathology laboratory in the context of COVID-19 global threat. In addition, we detail our strategy to minimize the risk of contamination upon exposure to the different biological products received in the laboratory, which can be of general interest to other laboratories worldwide. Our approach has enabled a safe work environment for laboratory staff, while ensuring the maintenance of high quality standards of the work performed. In times of uncertainty and given the lack of specific guidelines directed at Anatomic Pathology services to better deal with the global COVID-19 public-health emergency, it is essential to share with the community rigorous methodologies that will enable us to better cope with probable novel waves of COVID-19 infection and other viruses that will possibly arise in the near future.

Bullous lupus erythematosus with an erythema gyratum repens-like pattern.

Bullous lupus erythematosus is a rare clinical form of lupus. The diagnosis is challenging and involves the exclusion of other subepidermal bullous dermatoses. We present a 21-year-old woman with erythematosus, polycyclic plaques with vesiculobullae along the periphery, creating an erythema gyratum repens-like pattern on acral regions. The cutaneous biopsy, analytical, and autoimmune studies support the diagnosis of systemic lupus erythematosus. Dapsone and glucocorticosteroids were given with prompt resolution of the lesions within two weeks. To our knowledge this is the first case of bullous lupus erythematosus with this atypical acral presentation.

Ungual Tuberculosis: A Unique Clinical Case.

Cutaneous manifestations of tuberculosis (TB) are rare, particularly from an exogenous source. Involvement of the nail apparatus is extremely rare and has only previously been reported as a secondary involvement. We report the case of a 76-year-old female patient referred to our department with onychodystrophy with purulent drainage of the first left finger, which had developed during the preceding year. She had no previous traumatic history and had received treatment with multiple cycles of oral antibiotics and antimycotics, with no clinical improvement. Physical examination showed paronychia and onychodystrophy of the entire nail plate. Biopsy evaluation revealed epithelioid granulomas with central foci of necrosis, and laboratory cultures were positive for Mycobacterium tuberculosiscomplex. Chest computed tomography excluded primary pulmonary TB. X-ray of the left hand revealed the presence of dactylitis on the distal phalanx. Based on these findings, the patient was treated with rifampicin, isoniazid, pyrazinamide, and ethambutol for 2 months and with rifampicin and isoniazid for 7 months, resulting in complete resolution of the lesions. Cutaneous TB is a diagnostic challenge, particularly in rare cases such as involvement of the nail apparatus. It should be considered as a diagnostic hypothesis in cases of painless paronychia with refractory purulent drainage and associated onychodystrophy.

Cutaneous diffuse large B-cell lymphoma.

Cutaneous diffuse large B-cell lymphoma accounts for ~6% of all cutaneous lymphomas. It is associated with poor prognosis, and solitary lesions are relatively rare. It often requires an aggressive approach with multi-agent chemotherapy and radiotherapy. It is important to recognize these cases in order to offer rapid and appropriate management.

Subcutaneous metastasis of a pulmonary carcinoid tumor: A case report.

RATIONALE: Carcinoid tumors are derived from neuroendocrine cells and are most frequently found in the gastrointestinal tract and bronchopulmonary system. They are generally characterized by an indolent clinical course but may in some instances spread to regional lymph nodes or to distant sites. Subcutaneous metastases of carcinoid tumors are extremely rare; there are only few cases reported in the literature and the site of the primary tumor was mainly the gastrointestinal tract. Also, the diagnosis of this type of lesions many years after the surgical resection of the pulmonary carcinoid (PC) could be a challenge for clinicians. PATIENT CONCERNS: A nonsmoker woman diagnosed with a atypical carcinoid stage IA2 maintained follow-up at our institution. Seven years later she incidentally detected a subcutaneous nodular lesion in the lumbar region. DIAGNOSES: A positron emission tomography-computed tomography (PET/CT) was performed and showed pathological uptake of the refered lesion. An excisional biopsy was performed and with the support of immunohistochemistry the diagnosis of a subcutaneous metastasis from a pulmonary atypical carcinoid was made. INTERVENTIONS: The patient initiated chemotherapy with carboplatin plus etoposide and complied 4 cycles of treatment. OUTCOMES: She maintained tight follow-up at our center and for 12 months there were no signs of relapse. LESSONS: This extremely rare case highlights the difficulties in the differential diagnosis and the importance of diagnostic tests as PET/CT and immunohistochemistry in the establishment of a diagnosis. Physicians should be aware of signs of skin metastasis from lung malignancies even if the prognosis is good or many years have passed since the surgical resection.

Synchronous mucinous and non-mucinous lung adenocarcinomas with different epidermal growth mutational status.

In recent years, the spread of more-sensitive diagnostic methods has resulted in an increase of synchronous multiple primary lung cancer diagnosis. Nevertheless, its occurrence is still rare. Distinction between synchronous lesions from second independent primary tumors is a problem when dealing with multiple lung tumors, particularly if the histological type is the same. We present a case report of a 78-year-old female patient referred to our institution due to pneumonia. A subsequent thoracic computed tomography (CT) was performed showing two suspicious lesions, one in the right upper lobe and the other in the right inferior lobe. The CT-guided transthoracic needle biopsy of both pulmonary lesions revealed two adenocarcinomas, but with a rare combination of distinct morphologic variants, as well as different immunophenotypes and epidermal growth factor receptor (EGFR) gene status. The patient refused surgery and was submitted to stereotactic body radiation therapy (SBRT). She maintained tight follow-up and until now, she has not shown any signs of relapse or metastasis. A multidisciplinary approach with clinical, morphologic and molecular evaluation in multiple lung cancer is important to diagnosis and treatment guidance.

Role of epidermal growth factor mutational status for distinction between recurrent lung cancer and second primary lung cancer: case report.

INTRODUCTION: In a patient with previous radically treated lung adenocarcinoma, the detection of a new lung cancer raises the question whether recurrence or a second primary lung cancer is involved. Current criteria for differentiating multiple lung tumors lack a biologic and molecular basis and may lead to misclassification with impact on survival. OBJECTIVES: We report the case of a female patient with a recent diagnosis of lung adenocarcinoma and a previous lung adenocarcinoma submitted to curative surgical therapy 4 years before. As both lesions were resected, were of the same histologic subtype and presented the same immunohistochemistry profile; we decided to perform mutational analysis of the epidermal growth factor (EGFR) gene to differentiate between recurrence and second primary lung cancer. METHODS: The EGFR gene was screened for mutations in exons 18, 19, 20 and 21 using direct sequencing of polymerase chain reaction products in DNA obtained from paraffin preserved cells from both tumors. RESULTS: Mutational analysis of the EGFR gene, revealed different mutations in each tumor (both on exon 19) allowing the confirmation of the diagnosis of two metachronous primary lung cancers. CONCLUSIONS: In this patient, mutational analysis of the EGFR gene was superior to histologic and immunohistochemistry characterization in differentiating between recurrent lung cancer and second primary lung cancer; allowing confirmation of the diagnosis of two metachronous primary lung cancers.

Single symptomatic colonic metastasis as first presentation of a pulmonary mucoepidermoid carcinoma.

Metastatic lesions in the large bowel are rare. A case of single symptomatic colonic metastasis as first presentation of pulmonary mucoepidermoid carcinoma (MEC) is described. A 62-year-old male was admitted with constipation and rectal bleeding. Colonoscopy revealed extrinsic compression at sigmoid colon, and microscopy suggested a secondary origin. Subsequent chest computed tomography confirmed a right perihilar mass invading posterior mediastinum, carina and esophagus, and also a second consolidation on right lower lobe and ipsilateral mediastinal and subcarinal lymph nodes. After pathologic evaluation of a transthoracic biopsy specimen, the diagnosis of pulmonary high-grade MEC, stage IV (T4, N3, M1b - 7th ed. TNM), was established. Chemotherapy with carboplatin and paclitaxel was initiated, and disease progression occurred after two cycles with increasing colonic mass. Palliative surgery was rejected because of advanced stage, and the patient was submitted to palliative pelvic radiotherapy. Second-line chemotherapy with gemcitabine was initiated, but clinical status worsened and the patient died because of severe lung failure. Clinical, pathological and therapeutic aspects of this uncommon site of extrathoracic metastatic disease are discussed, emphasizing the important contribution of special stains and immunohistochemistry to the diagnosis.

Extracorporeal membrane oxygenation as bridge-to-decision in acute heart failure due to systemic light-chain amyloidosis.

PATIENT: Female, 58. FINAL DIAGNOSIS: Acute hear failure. SYMPTOMS: Dispnoea • edema • fatigue. MEDICATION: ­ CLINICAL PROCEDURE: Bone marrow biopsy • endomyocardial biopsy • abdominal subcutaneous fat biopsy under ECMO support. SPECIALTY: Cardiology. OBJECTIVE: Rare disease. BACKGROUND: Cardiac amyloidosis results from the amyloid deposition in heart tissue, either in the context of a systemic disease or as a localized form. Several pro-amyloid proteins can produce amyloid deposits in the heart. Each of these amyloidoses has characteristic clinical (cardiac and extracardiac) features, and a specific diagnosis and treatment. CASE REPORT: A 58-year-old woman who presented with acute heart failure and echocardiographic findings strongly suggestive of infiltrative cardiomyopathy needed percutaneous veno-arterial extracorporeal membrane oxygenation (ECMO) as bridge-to-decision. Amyloid deposition was found on endomyocardial and bone marrow biopsies. Bone marrow plasma cell infiltrate with acute renal lesion and hypercalcemia confirmed the diagnosis of multiple myeloma-associated systemic light-chain amyloidosis (AL). Refractory shock with multi-organic failure syndrome persisted and no improvements in left ventricular function and structure were seen. After extensive discussion by a multidisciplinary team, and with the patients' family, she was not considered eligible for high-dose chemotherapy and/or autologous stem cell transplantation, heart transplantation, or sequential heart with autologous stem cell transplantation. The patient died a few hours after ECMO withdrawal. During the 14 days of ECMO support no major bleeding or thrombotic complications occurred. CONCLUSIONS: The clinician must consider a diagnosis of cardiac amyloidosis in patients with heart failure, a restrictive type of cardiomyopathy with ventricular hypertrophy in the absence of valve abnormalities, or uncontrolled arterial hypertension. Although developments in chemotherapy have greatly improved the outcomes in AL amyloidosis, the prognosis of patients with severe cardiac involvement remains very poor. ECMO is potentially a reliable bridge-to-diagnosis and bridge-to-decision in these patients. An experienced ECMO team, careful patient selection, and rigorous management protocols with objective criteria to wean or stop ECMO are needed.

Sporadic exophytic hepatic angiomyolipoma.

Angiomyolipomas are rare mesenchymal tumours consisting of smooth muscle, blood vessels and adipocytes in variable proportions. These tumours are more usually seen in kidneys (incidence 0.3-3%), and the extrarenal location is rare. Since its initial description by Ishak in 1976, only about 200 cases of hepatic angiomyolipomas have been reported. We describe the case of a 59-year-old woman, without tuberous sclerosis, with a 6 cm tumour in hepatic segments II and III, gallbladder with parietal calcification and lithiasis. She was proposed to bisegmentectomy (II and III) and cholecystectomy, which were performed without complications. The pathological examination confirmed the diagnosis of hepatic angiomyolipoma (positive for HMB45 and focally positive for HHF35) and chronic cholecystitis. Although typically benign, the emergence of cases with malignant behaviour--recurrence or distant metastasis--led to a change in attitude towards these lesions, from clinical surveillance to surgical excision, with few exceptions.

Papillary adenocarcinoma of the thymus: case report and review of the literature.

A 44-year-old male with a mediastinal mass measuring 3.5 × 3.5 × 3 cm was diagnosed with papillary adenocarcinoma of the thymus. Other origins of papillary adenocarcinoma were excluded by clinical, imaging, and immunocytochemical methods before assuming this diagnosis. Residual thymus was seen under the microscope. Focal CD5 immunoreactivity was present. There was no associated thymoma. The patient underwent surgery, radiotherapy, and chemotherapy. He disclosed systemic recurrence at 18 months (subcutaneous nodule). He is alive after 24 months of follow-up with active disease. There had been only 7 cases of this rare entity published before.

[Kaposi's sarcoma arising in a patient with chronic myelomonocytic leukaemia]. / Sarcoma de Kaposi em doente com leucemia mielomonocítica crónica.

We present the case of a 84 year-old man from north Portugal with chronic myelomonocytic leukaemia (diagnosed 2 years ago), treated with erythropoietin and hydroxyurea. The patient was referred to our department due to brown macules, papules and patches with a smooth surface and whitish scaling, located in the lower limbs that had appeared two months ago. The diagnosis of Kaposi's sarcoma was histologically confirmed. He was HIV-1/HIV-2 negative and had no analytic detectable immussupression. The patient died a short time after the diagnosis before we could start treatment.

[Testicular tuberculosis: case report]. / Tuberculose testicular: Caso clínico.

Testicular tuberculosis is rare, occurring in approximately 3% of patients with genital tuberculosis. It is often clinically indistinguishable from lesions such as testicular tumour and infarction and may even mimic testicular torsion. Men aged 20-50 years old are most commonly affected and often present with painful or painless enlargement of the scrotum. Ultrasound (US) is currently the best technique for imaging the scrotum and its contents and for diagnosing testicular lesions. We present the case of a 58 year-old male, followed in pneumology for pulmonary tuberculosis, who complained of pain and right scrotum enlargement in the second month of anti-tuberculosis chemotherapy. First assessment, seconded by US, suggested a neoplasic lesion, and the final diagnosis revealed testicular tuberculosis.