Life threatening rare lymphomas presenting as longitudinally extensive transverse myelitis: a diagnostic challenge.

Background and aims - Description of two cases of rare intravascular large B-cell lymphoma and secondary T-cell lymphoma diagnosed postmortem, that manifested clinically as longitudinally extensive transverse myelitis (LETM). We discuss causes of diagnostic difficulties, deceptive radiological and histological investigations, and outline diagnostic procedures based on our and previously reported cases. Case reports - Our first case, a 48-year-old female was admitted to the neurological department due to paraparesis. MRI suggested LETM, but the treatments were ineffective. She died after four weeks because of pneumonia and untreatable polyserositis. Pathological examination revealed intravascular large B-cell lymphoma (IVL). Our second case, a 61-year-old man presented with headache and paraparesis. MRI showed small bitemporal lesions and lesions suggesting LETM. Diagnostic investigations were unsuccessful, including tests for possible lymphoma (CSF flow cytometry and muscle biopsy for suspected IVL). Chest CT showed focal inflammation in a small area of the lung, and adrenal adenoma. Brain biopsy sample from the affected temporal area suggested T-cell mediated lymphocytic (paraneoplastic or viral) meningoencephalitis and excluded diffuse large B-cell lymphoma. The symptoms worsened, and the patient died in the sixth week of disease. The pathological examination of the presumed adenoma in the adrenal gland, the pancreatic tail and the lung lesions revealed peripheral T-cell lymphoma, as did the brain and spinal cord lesions. Even at histological examination, the T-cell lymphoma had the misleading appearance of inflammatory condition as did the MRI. Conclusion - Lymphoma can manifest as LETM. In cases of etiologically unclear atypical LETM in patients older than 40 years, a random skin biopsy (with subcutaneous adipose tissue) from the thigh and from the abdomen is strongly recommended as soon as possible. This may detect IVL and provide the possibility of prompt chemotherapy. In case of suspicion of lymphoma, parallel examination of the CSF by flow cytometry is also recommended. If skin biopsy is negative but lymphoma suspicion remains high, biopsy from other sites (bone marrow, lymph nodes or adrenal gland lesion) or from a simultaneously existing cerebral lesion is suggested, to exclude or prove diffuse large B-cell lymphoma, IVL, or a rare T-cell lymphoma.

Egyre gyakoribbá váló megbetegedés: pulmonalis dirofilariasis.

Due to the climate change zoonoses, dirofilariasis in particular is spreading. Hence, it is likely that we will encounter with this problem more frequently in the future Our intention with this case presentation is to draw attention to the increasing number of nematodes that cause differential diagnostic problems. The patient underwent surgery with suspicion of pulmonary malignancy suggested by a peripheral rounded opacity in imaging, but histological examination revealed dirofilariasis.

[Changing times - changing diseases. Review of the neuropathological autopsy documentations at the Markusovszky University Teaching Hospital (1964-2014)]. / Változó idok - változó betegségek. A Markusovszky Egyetemi Oktatókórház neuropatológiai autopsziás anyagának áttekintése (1964-2014).

INTRODUCTION: Nearly 6000 autoptic studies were carried out during the last 50 years at the Laboratory of Neuropathology, Markusovszky University Teaching Hospital, Hungary. AIM: The aim of the authors was to present those previously frequent and often fatal conditions that can be prevented or treated today. METHOD: Retrospective analyses of the neuropathological documentations. RESULTS: Measles-related subacute sclerosing panencephalitis caused death in 13 cases, the last occurred in 1991. The mandatory vaccination against the causative virus has eliminated this severe neurological complication. Fourteen lives were lost due to herpes simplex encephalitis, including the last case seen in 1999. Feasibility of early diagnosis and the availability of acyclovir therapy resulted in better outcome without fatality. Tuberculous meningitis still occurred in most recent years, although only sporadically. Recognition of this condition is not straightforward due to its rarity, and considerations for this disease are often omitted from the routine differential diagnosis. The generally low mortality rates in tick borne encephalitis further dropped after the introduction of vaccination. Altogether only 8 such cases were documented. The last fatal cases of neurolues were seen in the 1990s. However, syphilis itself has not disappeared, and the number of cases with newly acquired infection continues to rise. The introduction of intrathecal methotrexate and radiotherapy made possible the prevention or effective treatment of meningeal leukosis. A careful coordination of these treatment modalities, however, is important as nervous system complications may develop in the form of disseminated necrotizing leukoencephalopathy that is also reflected in the records. CONCLUSIONS: The 50-year neuropathology documentation reflects changes in the occurrence of diseases, and it calls attention to those disorders which can be prevented or treated today, but may represent diagnostic challenges.

[Littoral cell angioma]. / Dongasejtes angioma.

Splenic rupture secondary to blunt trauma is a common condition. Non-traumatic, also known as spontaneous or pathological splenic rupture is an uncommon, but potentially life-threatening condition. Spontaneous splenic rupture caused by a primary splenic tumor is rare. In this case study, we present a special, benign tumor causing splenic rupture. Our 78-year-old female patient was hospitalized due to left shoulder pain and chest discomfort. Her blood pressure was low, the laboratory tests showed anemia, and the chest CT scan involving also the upper abdomen raised the suspicion of a splenic rupture. During the emergency splenectomy, there was a large amount of blood in the abdominal cavity. Macroscopic pathological examination of the removed spleen showed multifocal cystic lesions that led to splenic rupture. Immunhistochemical analyses revealed a littoral cell angioma. Littoral cell angioma is a rare, benign vascular tumor of the spleen, which is thought to originate from the red pulp sinuses lined with littoral cells. The aim of our report is to describe an unusual cause of sudden splenic rupture without traumatic history, the histologically benign littoral cell angioma that has not been published in Hungary. Orv Hetil. 2023; 164(10): 393-397.

[Left pneumonectomy for intrapulmonary unicentric Castleman disease]. / Bal oldali teljes tüdoeltávolítás intrapulmonalisan elhelyezkedo Castleman betegség miatt.

Castleman disease is an uncommon lymphoproliferative disorder, located most commonly in the mediastinal lymph nodes. The intrapulmonary presentation is extremely rare, with 14 published cases in the English literature. The clinical presentation of the disorder is not specific and the diagnosis is often challenging. The main goal is to achieve an R0 surgical resection which gives the potential chance for a recurrence-free survival. We present the case of a symptomless, 15-year-old female patient with left-sided tumor mass. Transthoracal invasive tissue biopsy confirmed Castleman disease. The central mass involved the main structures in the left hilus and therefore left pulmonectomy was necessary to reach the complete, tumor-free resection margins. The patient had no local or distant relapse during the 7-year follow-up. Although Castleman disease treated by complete surgical resection provides excellent results, radical and extended lung resection is sometimes inevitable to reach tumor-free margins. Orv Hetil. 2023; 164(37): 1476-1483.

Our Doubts regarding the Identification of the Corpse of Louis II King of Bohemia and Hungary. / KÉTSÉGEINK II. LAJOS CSEH- ÉS MAGYAR KIRÁLY (1506-1526) HOLTTESTÉNEK AZONOSÍTÁSÁVAL KAPCSOLATBAN.

The coalitioned man-at-arms batallion of Louis II (1516-1526) King of Bohemia and Hungary had crushing defeated by overwhelmingly Turk army of Suleiman (1520-1566) the Magnificent at close to Mohács on 29 August 1526. Louis II and his trusty servant, István Aczél escaped from the losing battle, but both of them drowned into a brook (perhaps a local stream, named Csele) or a swampy fishpond. The one of the Bohemian noble, Ulrich Czettritz von Neuhaus royal court chamberlain was the single eyewitness of this luckless misadventure, furthermore - according to his first verbal report - he failed to help to the King from the sludge. The same Czettritz identified the King's dead body on the spot on 18 October of 1526, but the King's corpse turned up from a newly dug sepulchral mound instead of water, swamp or any kind of original surface. Ferenc Sárffy (royal castle head of Gyor) joined to Czettritz's scene investigation and Sárffy reported István Brodarics Chancellor about the case on 19 October 1526. The details of royal corpse identification is well-known from the copy of Sárffy's letter, which one has been preserve at Poland. According to the Sárffy's report the corpse of Louis II was taintless, in spite of the royal dead body spent underground circa 50 days and unknown persons previously removed it. In our opinion it would have been decompose because of late autodigestion or adipoceration. So if Sárffy's report is right about the undamaged condition of corpse, it must not Louis' dead body. It could not excluded that Ferenc Sárffy deliberately embellished the truth. Nevertheless Louis was not buried by Ulrich Czettritz or Maria Habsburg ­ Louis' widow, who sent Czettritz to the corpse identification ­, or Maria's brother Ferdinand Archduke of Austria, who aspirated to the throne. Point of interest that finally the King's funeral was made by John Szapolya vaivoda of Transylvania, who occupiedwith his army the crowning city (named Székesfehérvár), because he aspirated to the throne also and he was a politician on the other side of the fence. The ceremonial funeral became on 9 November 1526, when the royal body was dead for 72 days. This is the reason why we have been sceptical about Louis' corpse was in suitable condition for the procession of burial, where some important Hungarian people surely inspected him and nobody failed to notice that the King's body had had signs of advanced decompose. In our opinion - considering that date of the scene investigation, then circumstances of the medical examination, finally Czettritz was not a skilled doctor - could be 66 percent chances that Czettritz and Sárffy should not found the corpse of Louis II. An English document proves our statement. Thomas Wolsey Chancellor, Archbishop of York wrote a letter to Henry VIII King of England from Westminster on 23 October 1526. The Chancellor informed Henry VIII in this document about the founding and burying process of Louis II. Out of consideration for the distance between of England and Hungary, the news could not arrive from Gyor (Sárffy reported Brodarics on 19 October) to London (Wolsey reported Henry VIII on 23 October) within four days, because other letters, news and reports were coming 3 or 4 weeks delay. Our conclusions that Louis' corpse was finding at least 3 or 4 weeks before the scene investigation of Czettritz and Sárffy. Accordingly the corpse which was found on 18 October it should neither the King's body nor Louis' corpse was buried at Székesfehérvár on 9 November 1526.