Selected case from the Arkadi M. Rywlin international pathology slide series: diffuse dendriform pulmonary ossification: report of 2 cases with review of the literature.

Two cases of diffuse dendriform pulmonary ossification (DPO) are presented, one of the secondary type and the other of the idiopathic type. Case 1 was an adult female patient who underwent thoracic surgery to remove a posterior mediastinal bronchogenic cyst, which was discovered on a computed tomography scan performed after an episode of pneumonia when traction bronchiectasis with interstitial lung disease/fibrosis was also suspected in the lungs. Histologic examination performed on the resected lung tissue revealed numerous scattered small osseous spicules on a background of intense interstitial inflammation and fibrosis, leading to further clinical and laboratory investigations and the final diagnosis of DPO secondary to lung involvement by scleroderma. Case 2 was an adult male patient who underwent thoracoscopic exploration after a computed tomography scan, which revealed traction bronchiectasis with linear thickening of the interstitial lung tissue. Histologic examination of the lung tissue specimen revealed numerous osseous spicules in the absence of interstitial septal inflammation. Noteworthy in this case were also some nodules of collagenized tissue similar to those seen in the lungs of patients affected by Ehlers-Danlos syndrome. The absence of any clinical sign or symptoms related to Ehlers-Danlos syndrome attest to the nonspecificity of these pulmonary fibrous nodules. No case of DPO secondary to scleroderma has been reported in the literature so far, although around half of the patients with scleroderma manifest pulmonary diseases. Idiopathic DPO is even rarer, usually discovered postmortem, with only 20 cases diagnosed in life with lung biopsies taken by open surgery or through a thoracoscopic approach. DPO is often misdiagnosed radiologically as bronchiectasis and/or interstitial lung disease/fibrosis.

Selected case from the Arkadi M. Rywlin international pathology slide series: lymphangiomatosis of the spleen associated with ipsilateral abdominopelvic and lower extremity venolymphatic malformations: a case report and review of the literature.

Herein, we report a 26-year-old patient with lymphangiomatosis of the spleen associated with multiple lymphatic and venous malformations. This patient underwent excision of a large lymphatic malformation of the left abdominal wall during childhood. A venous malformation of her left lower limb was excised during adolescence. Additional lymphatic malformations were found in the soft tissue of her left thigh at the age of 20. During hospitalization for a huge vulvar hemangioma at the age of 26, she was incidentally found to have asymptomatic splenomegaly, for which she underwent splenectomy. Examination of the spleen revealed diffuse involvement by a lymphatic anomaly predominantly forming small cystic spaces. Lymphangiomatosis of the spleen is rare and is classically separated into an isolated or pure form and a generalized form when it is associated with involvement of other viscera and/or multiple soft-tissue planes. This patient was affected by a borderline form of splenic lymphangiomatosis with limited somatic involvement of the superficial soft tissues and blood vessels. Notably, all the additional vascular malformations in this patient were left sided, and at this time there was no additional involvement of internal organ. No hereditary or known syndrome was identified.